Textbook of

Oral Pathology

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 Textbook of
Oral Pathology

Anil Ghom Shubhangi Mhaske

MDS (Oral Medicine & Radiology) MDS (Oral Pathology)
Professor and Head Associate Professor
Department of Oral Medicine and Radiology Department Incharge, Department of
Chhattisgarh Dental College and Research Center Oral Pathology, People’s Dental Academy
Sundra Rajandagao, Chhattisgarh Bhopal (MP)
India India

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Textbook of Oral Pathology

© 2008, Jaypee Brothers Medical Publishers
All rights reserved. No part of this publication and should be reproduced, stored in a retrieval system, or transmitted in any
form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of
the authors and the publisher.
This book has been published in good faith that the materials provided by authors is original. Every effort is made to
ensure accuracy of material, but the publisher, printer and authors will not be held responsible for any inadvertent
error(s). In case of any dispute, all legal matters are to be settled under Delhi jurisdiction only.

First Edition: 2009

ISBN 978-81-8448-402-1
Typeset at JPBMP typesetting unit
Printed at Replica

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 Dedicated
to
The students of dentistry

“Prayer is not to lighten your burden, but to strengthen your back, have FAITH in yourself
and your Almighty, as FAITH doesn’t move mountains but gives power to climb them’’

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 Contributors

Imtiyaz Nadaf Sangamesh Halawar

MDS (Oral Pathology) MDS (Oral Pathology)
Senior Lecturer Reader
Department of Oral Pathology Department of Oral Pathology
People’s Dental Academy CDCRI, Rajnandgaon, Chhattisgarh, India
Bhopal
Madhya Pradesh Anand Tegginamani
India MDS (Oral Pathology)
Senior Lecturer
Mandale Mandakini Coorg Institute of Dental Science, Virajpeth
MDS (Oral Pathology) Coorg, Karnataka, India
Senior Faculty
Govt. Dental College and Hospital Meghanand Nayak
Aurangabad MDS (Oral Pathology)
Maharashtra Senior Lecturer
India KGMC College, Luknow, India

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 Foreword

It gives me immense pleasure to write few words about the Textbook of Oral Pathology.
This book is the outcome of combined efforts of Dr Anil Ghom, Professor of Oral Medicine and
Radiology and Dr (Mrs) Shubhangi Mhaske (Jedhe), Associate Professor of Oral Pathology and
Microbiology. I know Dr Shubhangi as undergraduate as well as postgraduate student. I feel her
sincerity coupled with hard work and humanly approach toward the patients during her
postgraduate studies contributed in making this textbook.
My heartiest congratulations to Professor Anil Ghom and Dr (Mrs) Shubhangi Mhaske (Jedhe)
for their great endeavor in bringing out this book. This book contains six sections which are divided
into 37 chapters related to oral lesions/diseases inclusive of basic topics of oral pathology. The
topics like microscopy, stains and routine as well as special investigations are noteworthy. The lots
of updated information in this book will be helpful to undergraduates, postgraduates and also for
practising dental fraternity.
The total of over 600 clinical photographs, microphotographs and line diagrams incorporated in
the book are definitely useful for in-depth understanding of the subject. The textbooks available on
these subjects are many, but only a few ones cover both the subjects: oral medicine and radiology as
well as oral pathology. Contribution of Indian authors toward the books in dentistry is less as
compared to the foreign authors. Therefore, Mrs Mhaske (Jedhe) deserves a word of appreciation
for her sincere and painstaking efforts.
This book is an excellent contribution to a scientific literature in Indian scenario and thereby
facilitating our students to understand various diseases.
With regards and best wishes.

Dr Jagdish V Tupkari
Professor and Head
Dept of Oral Pathology and Microbiology
Govt Dental College and Hospital
Mumbai, Maharashtra
India

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 Preface

In the study of oral and dental sciences, oral pathology is the subject that concentrates on the
morphologic changes in oral tissues which causing diseases and the mechanisms of the disease
process. Most recently published works on the subject are the product of eminent authorities with
multiple authorship with current research advances. ‘Why another book in oral pathology’? This
question can probably be answered by the phrase,
“Nothing can be changed by changing the face
But many things can be changed by facing the change.”
The purpose of this book is mainly to provide the undergraduate and postgraduate students an
easy way of reference for covering a broad spectrum of oral pathology in lucid and simple language.
In support of all the composite works it can be stated that the progress of oral and maxillofacial
pathology in its many varied specialties coming up has made the subject too vast to be covered
adequately by a single author. Also a balance between the advances and the basic essentials is need
of the hour. With this balanced perspective and from the viewpoint of the graduate and postgraduate
students under training, the authors have endeavored to compile the oral and maxillofacial pathology
with respect to the related essential clinical oral medicine and radiology. The book gives an extensive
coverage and emphasises on detailed description, adequate well labeled illustration, flow charts,
recent developments and molecular aspects. Aside from oral pathology in general, the initial phase
of the book includes the basics of the embryology, anatomy and pathology. The study of microscope,
tissue processing, diagnostic tests and advanced techniques are also included. The photomicrographs
and the clinical photographs in the book signify the adage: “A picture is worth a thousand words”;
as the reader is encouraged to study the details and to clarify the confusion of the topics.
Inspite of sincere efforts, elements of human error or shortcomings are likely; the readers are
welcome to point out all such mistakes and render valuable suggestions for further improvements
and shall be greatly acknowledged.

Anil Ghom Shubhangi Mhaske

[email protected] [email protected]
[email protected] [email protected]
 Acknowledgements

Those who are lifting the word upward and onward are those who encourage more than criticize.
It is my esteemed pleasure to acknowledge the indebtedness to the many friends and colleagues
whose influence over the years enabled to enjoy and grow in my discipline. While they are too
numerous to mention, they continued to occupy a very special space in my heart and soul. Dr
Rupali Sharma, Dr Vaishali Gawande, Dr Anjusha Ganar, Dr Deepika Phatnani, etc. served an
indispensable organizational role throughout the preparation of this text which demands a very
special mention.
Every book has its share of contributor and influence and this book is certainly no exception. I
express my deepest gratitude to Dr Sangamesh Halawar, Dr Anand Tegginamani, and
Dr Meghanand Nayak. I am also thankful to management of CDCRI for their encouragement.
“Was anything real ever gain, without sacrifice of some kind?”
I am extremely thankful to my family specially my better half, Dr Savita Ghom for the appreciation
and unconditional support.
Our special thanks goes to Shri Jitendar P Vij, Chairman and Managing Director of Jaypee Brothers
Medical Publishers, and his staff for their excellent work in professionalism.
Last but not the least, we are extremely thankful to Almighty for His continuous blessing.

Anil Ghom

I dedicate my work to my child Sumedh who kept tremendous patience during the entire completion of mission
and to my mentor and inspirator, my father Shri Shrikant Jedhe.
I adore and express my deep sense of heartfelt indebtedness towards my caring, empathetic
husband Dr Ashok Mhaske (Prof and Head, Dept of Surgery), who has been a guiding light
throughout the intricate voyage of life and for bestowing the untiring, invaluable emotional support
that was required for completion this arduous task. No one can accomplish much without the help,
support and understanding of family. I acknowledge with affection the support extended to me by
my mother Sulochana, brothers, sisters and all the family members especially caring sister-in-law
Dr. Mandakini who has been supportive throughout. I am extremely thankful to Dr Imtiyaz Nadaf
for his tremendous efforts in providing the professional and technical support for exploring all the
possible accesses and avenues required for the latest references to be added in the text. Continuous
strong support of Dr Pooja Hazari, Dr Mahesh Masaram, Dr Bharti Sherke, Dr Swapnali is priceless.
I am grateful to Dr Raju Ragavendra for helping in proofreading and judicious suggestions. I extend
my gratitude towards my respected teacher Dr JV Tupkari, an educationalist with outstanding talent
and modesty, whose teaching are unforgettable.
XIV Textbook of Oral Pathology

Special thanks to:

Management of peoples group, Shri Suresh Vijayvargiya, Chairman
Dr Suresh Barpande (Dean, GDC, Aurangabad)
Dr MK Gupta (Dean, PDA, Bhopal)
Dr JJ Doshi (Prof, PDA, Bhopal, Ex-Dean, RDC, Loni)
Dr Vijay Deshmukh (HOD of OMFS, CSMSS, Aurangabad)
Dr Jyoti Bhavthankar, Dr Jayanti Humbe (Dept. of Oral Pathology, GDC, Aurangabad)
Dr Alka Kale, Dr Seema Hallikerimath, Dr Avninder Sethi, Dr Kunal Sah, Dr Surekha, Dr Deepak
Patel (Dept. of Oral Pathology, KIDS, Belgaum)
Dr Roshna, Dr Seema Rajnikanth, Dr Parimala Tyagi, Dr Rucha (PDA, Bhopal)
Dr Bharti Sherke, Dr Vrushali Deshmukh
Olympus India Ltd
Students of PDA, especially Ankit Gupta, Parvez, Amisha Jain, Vivek Parihar
Dept staff Ujwal Soni, Mamta, Rajesh Chourasiya
Librarian Mrs Krishna Verma and staff
Shubhangi Mhaske
 Contents

Section 1: Basics

1. Microscope ............................................................................................................................................ 3
2. Tissue Processing Methods ............................................................................................................. 19
3. Stains and Staining Methods .......................................................................................................... 28
4. Diagnostic Pathology and Caries Activity Test ........................................................................... 36
5. Advanced Diagnostic Techniques ................................................................................................. 47
6. Embryology and Anatomy of Oral Structures ............................................................................. 55
7. Hyperplasia, Hamartoma and Neoplasm ..................................................................................... 70
8. Healing of Oral Wounds .................................................................................................................. 84

Section 2: Pathological Aspects of Oral Diseases

9. Teeth Anomalies ............................................................................................................................... 91

10. Craniofacial Anomalies ................................................................................................................. 123
11. Benign Tumors ................................................................................................................................ 134
12. Premalignant Lesions and Conditions ........................................................................................ 170
13. Malignant Tumors .......................................................................................................................... 204
14. Salivary Gland Pathology .............................................................................................................. 242
15. Odontogenic Tumors ...................................................................................................................... 280
16. Cysts of the Orofacial Region ....................................................................................................... 318
17. Chemical and Physical Injuries .................................................................................................... 369
18. Allergic and Immunological Diseases of Oral Cavity ............................................................. 386
19. Granulomatous Diseases and Collagen Disorders ................................................................... 393

Section 3: Infections of Oral Cavity

20. Dental Caries .................................................................................................................................... 401

21. Sequel of Pulpal Infection ............................................................................................................. 420
22. Consequences of Oral Infection ................................................................................................... 444
23. Bacterial Infections ......................................................................................................................... 461
24. Viral Infections ................................................................................................................................ 484
25. Fungal and Parasitic Infections .................................................................................................... 507
XVI Textbook of Oral Pathology

Section 4: Diseases of Specific Organ

26. Periodontal Pathology .................................................................................................................... 525

27. Temporomandibular Joint Pathology ......................................................................................... 541
28. Diseases of Tongue ......................................................................................................................... 549
29. Diseases of Lip ................................................................................................................................. 571

Section 5: Systemic Diseases Manifested in the Jaws

30. Blood Dyscrasias ............................................................................................................................. 585

31. Bone Pathology ................................................................................................................................ 605
32. Skin Disorders ................................................................................................................................. 623
33. Nutrition and Oral Cavity ............................................................................................................. 643
34. Endocrine Disorders ....................................................................................................................... 669
35. Diseases Affecting Nerves and Muscles .................................................................................... 683

Section 6: Miscellaneous

36. Syndromes of the Orofacial Region ............................................................................................ 697

37. Forensic Dentistry ........................................................................................................................... 730

Appendices

Appendix I. Classifications ................................................................................................................ 741

Appendix II. Differential Diagnosis of Lesion ................................................................................ 754
Appendix III. Histology and Histopathological Diagrams of Oral Tissues ................................ 795
• Glossary ................................................................................................................................................ 821
• Bibliography ......................................................................................................................................... 839
• Index ....................................................................................................................................................... 843
 CHAPTER

1 Microscope

The light microscope, now 400 years old, is the Simple Microscope
standard instrument for the examination of
• A simple microscope consists of a single lens
histological preparations. The word microscope
or a magnifying glass.
is derived from two Greek words ‘micro’ meaning
‘small’ and ‘scope’ meaning ‘to view’. Thus, it is Principle: A lens of short focal length is used to
an instrument which enables us to view small produce an enlarged image of an illuminated
objects. It magnifies (enlarges) the image of the object at a short distance; the lens fixed in a frame
small object and thus enable it possible to be seen is adjustable to view the object. Shorter the focal
by the viewer. length, larger is the magnified image.
There are two main types of microscopy:
Compound Microscope
• Light microscopy (0.2 μ resolution)
– Visible light — Routine microscopy • A compound microscope consists of two or
– Ultraviolet light — Fluorescent microscopy more lenses.
• Electron microscopy (0.2 nm resolution) — Principle: If a lens of short focal length is used to
Beam of electrons in an electromagnetic field produce an enlarged image of an illuminated
are used to visualize the specimen. object at a short distance, then another lens can
be so fixed that it would produce a further
HISTORY OF MICROSCOPE enlargement of that image.

The early pioneers in the history of the CONSTRUCTION OF MICROSCOPE

microscope are Digges of England and Hans and
Light Source
Zachcharias Janssen of Holland. But it was Antonie
van Leeuwenhoek who first made and used a The light microscope uses natural daylight or
real microscope. Early microscopes were simple artificial visible light. The resolution of light
microscopes, but with advancement of science microscope is limited by wavelength of its light
compound microscopes were built. source. A progression of light source have been
4 Textbook of Oral Pathology

developed from oil lamps to the low voltage • Kohler illumination: This is used for specialized
electric lights of today. type of microscopy where an image of the
In histopathology laboratories microscopes light source is focused by the lamp collector
with three different types of light sources are or field lens in the focal plane of the
found; the conventional light microscope using condenser. The image of the field or lamp
natural or artificial visible light, the fluorescent diaphragm is focused in the object plane and
microscope using ultraviolet light, and electron the aperture diaphragm is in turn focused at
microscope using a beam of electrons. the back focal plane of the objective and can
be examined with the eyepiece removed
Light microscope: Uses a visible light of 400-800
(Fig. 1.1).
nm wavelength to illuminate an object upto 0.2
μm, made visible with perfect optics given in a Condensers
microscope. In light microscope two different
Light from the lamp is directed into the first
types of illuminations are used.
major optical component – the sub stage
Fluorescent microscope: Uses ultraviolet light with condenser-either directly or from a mirror or
a shorter wavelength below 400 μm which a light prism.
microscope cannot. It can demonstrate, with the The main purpose of the condenser is to focus
help of fluorchrome dyes (Thioflavin T for or concentrate the available light into the plane
amyloid, acridine orange for malignant cells in of the object i.e. the condenser collects the
exfoliative cytology), the tissue components that maximum possible light reflected by the mirror
are present in very low concentration. High or the inbuilt light source and condenses or
pressure mercury lamp, halogen lamps are used converges it to a very small area at the position
to generate ultraviolet light. Light source used of the specimen (Fig. 1.2).
in fluorescent microscope is different, i.e. in Condensers used for routine microscopy
modern microscope high intensity illumination should have the same numerical aperture as
systems are used. They should to be used with objective. The ideal condenser should form a true
specialized filters for protection of eyes. image of the light source. It is practically useful
to have a condenser with a top lens that can be
Electron microscope: This technique of microscopy swung out of the path of light, thus filling the
is different from light microscopy as it uses a whole field with light when very low power
stream of electrons in a magnetic field. This stream objectives are used.
of electrons has a very short wavelength (a 50 kv Three types of condensers are used (Fig. 1.3):
electron beam produces light of 0.0055 nm). This • Abbe Condenser: Named after Ernst Abbe. It
is one hundred thousandth that of the visible light. is simplest and least expensive type. Because
In light microscope two different types of of its simplicity and good light gathering
illuminations are used. capacity. It is used with most microscopes
• Critical illumination: When the object and light unless specified otherwise; it has a NA
source from the substage condenser is in (Numerical aperture) of 0.25. It consists of
the same plane it is called as the critical two lens elements. Abbe condenser is not
illumination, as commonly used in simple corrected for spherical and chromatic
equipments, but this produces uneven aberration but serves well for general
illumination of the object though modern observation. Some types of Abbe condensers
filament lamps are used. are “variable focus condensers” in which the
 Microscope 5

Figure 1.1: Types of illumination used in microscope

upper lens element is fixed and lower lens is

focusable. When the lower element is raised
upto its top position it is similar to the above
condenser. But when its position is lowered,
light is focused in between the elements, thus
the light can emerge as a large diameter
parallel bundle. For 10× the field area is
larger. To illuminate this large area the top
lens element is removed to achieve the
illumination of entire field. For medium and
high magnification the top lens element of the
variable focus condenser remains in place.
• Aplanatic Condensers: These types of
condensers are optically corrected for
spherical aberration. These are not available
from all microscope manufacturers, but are
Figure 1.2: Parts of condenser of better quality than Abbe condensers.
6 Textbook of Oral Pathology

Figure 1.3: Types of condensers used in modern microscopes

• The Achromatic Condensers: These are Every objective has a fixed working distance,
corrected for both spherical and chromatic focal length, magnification and numerical
aberrations. It has NA of 1.40. Because of its aperture (NA) (Fig. 1.5).
high degree of correction, it is recommended The working distance is the distance between
for research microscopy and for color an object in focus and the front of the lens system
photomicrography where the highest degree of the objective.
of perfection in the image is desired. The focal length is the distance from the center
of a simple lens to the point at which parallel
Object Stage rays of light are brought to a sharp focus; in the
A rigid platform above the condenser which compound lens it is the distance between an
supports the glass slide is object stage. This object object in focus and a point approximately
stage has an aperture in the center through which halfway between the component lenses of the
the light can pass to illuminate the specimen on objective.
the glass slide (Fig. 1.4). Total Magnification is product of magni-
The stage holds the slide firmly and allows fication values of eyepiece and objective in a
the slide movements with a mechanical vertical standard microscope.
and horizontal adjustment screws. The
mechanical stage is graduated with Vernier
scales and the x and y movements assist the
operator to return to an exact desired location in
the specimen. Traveling range in most of the
microscopes is 76 mm(X) 30 mm (Y).

Objectives
Performance of a microscope is dependent
wholly on the quality of the optics especially the
objectives. The main task of objective is to collect
maximum light possible from the object, unite it
and form a high quality magnified image some
distance above. Figure 1.4: Parts of mechanical stage
 Microscope 7

Numerical Aperture
The ability of the lens to distinguish fine structural
details in a specimen is known as the ‘resolving
power’. This ability is expressed in terms of
numerical aperture, as NA, as it is usually called.
Numerical aperture depends primarily on
the extreme range of the divergent rays that can
be made to enter the lens (angular aperture) and
secondarily on the refractive index of the
medium between the object and the objective.
The relation between numerical aperture,
angular aperture and refractive index is:
Figure 1.5: Specifications mentioned on objective NA = Refractive index × Sin (angular aperture)
The numerical aperture for any objective is
Magnification in a standard microscope with
always imprinted on its mount. A 10×
tube length of 160 mm is calculated using the
achromatic objective usually has a numerical
formula aperture of 0.25 and a 20× achromat will usually
Magnification =
have a numerical aperture of 0.50; Apochromatic
Tube length/Focal length of objective
objectives have higher numerical apertures than
For microscopes with tube length other than achromats.
160 mm
Magnification = Resolution
Tube length × Eye piece magnification It is the smallest distance between two dots or
Focal length of the objective lines that can be seen as separate entities. It
Magnification for low power objective with depends on the wavelength of light (λ) and the
focal length 16 mm and standard tube length of NA of the lens.
160 mm is The resolving power is the ability to resolve
Magnification = 160/16=10 the detail and can be measured. As the NA of the
objective increases the resolving power increases.
Color Codes It is calculated as:
Microscope manufacturers label their objectives Resolving = 0.61 λ/NA
with color codes to help in rapid identification
Types of Objectives
of the magnification (Table 1.1). In addition to
color coding other information is also embossed In most modern microscopes objectives are
on the objective. usually made up more than one lens. This series
of lenses is used to overcome certain limitations
Table 1.1: Color codes used for objectives in the lenses.
Magnification Color Code
Limitations of Lens (Optical aberrations)
4× Red
10× Yellow Aberration is the failure of a lens to produce exact
40× Light blue point to point correspondence between an object
100× White and its image. Every lens system has an
8 Textbook of Oral Pathology

aberration to a greater or lesser extent. To The objective can be both ‘apochromat’ and
improve the image quality, the lenses are ‘achromat’ types to correct these optical
designed by combining different lens shapes and aberrations.
glass materials.
Spherical Aberration
Chromatic Aberration
It is caused when light rays entering a curved
White light is composed of different colors which lens at its periphery are refracted more than those
on passing through a simple lens, will be rays entering the center of the lens and are not
refracted to a different extent, with blue being brought to a common focus (Fig. 1.7).
brought to a shorter focus than red. This defect Different types of objectives are:
of lens is ‘chromatic aberration’ and results in • Achromatic: Corrected for two colors red and
an unsharp image with colored fringes (Fig. 1.6). blue. It is the most widely used for routine
It is possible to construct compound lenses purposes.
of different glass elements to correct this fault. – Fluorite: Corrected for yellow green color.
An achromat lens is corrected for two colors, blue Green light is brought to a shorter focus
and red, producing a secondary spectrum of and violet light to a longer focus.
yellow/green. This secondary spectrum can be • Apochromat: All colors are brought to same
reduced by adding fluorite to objective. Such a focus. It is fully corrected for three colors. By
lens is called as fluorite lens. Fluorite lenses need the design of the lens and use of fluorite, the
to be corrected for yellow green, which is done formation of a secondary spectrum is almost
by adding more lens components. Such type of completely eliminated and all colors are
lens is ‘apochromat’ lens which is most expensive. brought to the same focus. These lenses are
used especially for photomicrography and
for screening cytological smears (Fig. 1.8).
– Plan-achromat: Although histological
sections are flat the image produced by the
microscope is not flat. It is saucer shaped;
it is not possible to focus the whole of the
field sharply at any one time. This
aberration is corrected using flat-field
objective also called as plan-achromat
lenses.

Figure 1.6: Chromatic aberrations and its correction Figure 1.7: Spherical aberrations
 Microscope 9

Figure 1.8: Design and arrangement of lens system in objectives

Nosepiece introduced in the image formed. To overcome

In most modern microscopes up to six objectives this limitation, most of the modern microscopes
are mounted on resolving nosepiece. For rapid use infinity corrected optics where image is
change of all objectives they should be at focus projected to the infinity. In this system, tube
and they should focus the same central area of length can be altered without affecting the
the section when brought into the position. Such quality of the image. Infinity-corrected systems
nosepieces are known as ‘par-focal’ and ‘par- have the advantage of being easier to design and
central’. also make possible the insertion of less costly
accessories in the "parallel" light path. This
Mechanical tube advanced new optical system allows
Light from the objective is received into the microscopes to support complex optical
bottom of the microscope bodytube. From there component clusters in the optical pathway
it travels to the eyepiece in a tube called between the objective and the lens tube. This is
mechanical tube. The distance from objective to especially useful for techniques such as confocal,
eyepiece is called as “mechanical tube length” and polarized, DIC, and fluorescence microscopy
it is defined as “the distance from the nosepiece where specialized lens systems must be
opening, where the objective is mounted, to the employed for optimum results.
top edge of the observation tubes where the
Eyepiece
eyepieces (oculars) are inserted”. In standard
microscopes this is 160 mm, while in few special The purpose of an eyepiece in a compound
purpose microscopes it is 170 mm. microscope is to enlarge the primary image
In most microscopes tube length cannot be formed by the objective, and to render it visible
altered. Such microscopes are called as finite as a virtual image in the microscope and also to
length microscope. In these microscopes, if correct some of the defects of the objective.
additional filters such as polarizer, analyzer ‘Huygenian’ eyepieces are the simplest form of
fluorescent filters are used, tube length becomes eyepiece in common use; they are cheap, but they
more than 160 mm and aberrations will be are not corrected for chromatic difference of
10 Textbook of Oral Pathology

magnification. Although, Huygenian eyepieces

can be used with low-power achromats, they give
under-corrected curvature of field and lateral
colors with intermediate and higher power
objectives. The other main kind of eyepiece is the Figure 1.10: Measuring graticules used for micrometry
positive eyepiece with a diaphragm below its
lenses, commonly known as the ‘Ramsden’
eyepiece. These eyepieces are corrected for us to point out a certain portion of the specimen.
chromatic aberration of magnification (Fig. 1.9). Such types of eyepieces are called as pointer
eyepieces.
Different Types of Eyepieces Micrometry: Certain types of eyepieces can be
Compensating eyepieces are compound lenses with graduated to have a scale. This type of an
a chromatic difference of magnification which eyepiece is called a ‘micrometer eyepiece’. It is
is equal and opposite to that of high-power specifically intended to measure the size of the
objectives. They are essential for use with object. The micrometer-scale in the eyepiece is
apochromatic objectives, but they also improve calibrated with a stage micrometer before hand
the performance of most high-power achromatic so that the line separation is known. Now, this
objectives. scale could be made to coincide with the object
Eyepieces for binocular microscopes must be image and thus measure its size (Fig. 1.10).
accurately paired, with equal centration, Double demonstration eyepieces: Some types of
magnification, and field in order to reduce eye eyepieces are such that two people can view the
strain. Interocular distance should be accurately same portion of an object at a time. This type is
adjusted, and the microscopist should sit at the called ‘double demonstration eyepieces’.
correct height for the eyepieces to come to the
exact height of the observer’s eyes. Optical Path
Eyepieces, generally, are produced with
different magnifying powers, ranging from Real image: The real image in a microscope is
formed by the objective lens. The image is formed
about 4× to 25×. The most common in use are
at a greater magnification, and is inverted. (This
those with a magnifying power of 10× or 15×.
is when the object is moved nearer the lens).
Pointer Eyepieces: A fine pointer could be
incorporated in the eyepiece in order to enable Virtual image: If the object is placed very nearer to
the lens within the principal focus, the image is
formed on the same side as the object, is enlarged,
the right way up, and cannot be projected onto
the screen. This is the virtual image. The eyepiece
in a microscope forms the virtual image of the
real image projected by the objective.

Image Formation in a Microscope

Two lenses, the objective and eyepiece, are
Figure 1.9: Position of field lens and eye lens in Ramsden responsible for the formation of the image. The
and Huygenian eyepieces object or specimen is illuminated by the light
 Microscope 11

SPECIALIZED MICROSCOPY TECHNIQUES

Dark Field Microscopy

Dark field microscopy is an optical microscopy
illumination technique used to enhance the
contrast in unstained samples. It works on the
principle of illuminating the sample with light
that will not be collected by the objective lens,
so not form part of the image. This produces the
classic appearance of a dark, almost black,
background with bright objects on it.
For the dark field method, the cone of light
normally illuminating the specimen should not
enter the microscope objective, only light that is
scattered or reflected by the specimen is seen by
the objective. This is achieved in the conventional
microscope by use of dark field diaphragm stops
or a special dark field sub stage condenser like
Abbe, paraboloid or cardioid condensers, below
the main condenser. The light rays from the
condenser pass outside the objective and thus
form a hollow cone. Now any object with a
refractive index different from the surrounding
medium, placed within this hollow cone, will
only reflect light into the objective and thus the
Figure 1.11: Image formation in light microscopes object will appear ‘bright’ against the dark
background.
passing through the condenser which forms the The condensers used for this type of micros-
true image of the light source at the specimen copy are:
plane. The illuminated specimen is magnified • Abbe condenser: Used with low power objective.
by the objective to produce a real, inverted • Paraboloid condenser: High power oil immer-
image. A magnified upright virtual image of this sion objective.
real, inverted image is produced by the eyepiece • Cardioid condenser: More refined and
which will be seen by the observer’s eyes corrected for aberrations than the paraboloid
(Fig. 1.11). condenser. It is used with oil immersion
The principal focus or focal point is the single objective (Fig. 1.12).
point where the parallel rays of light entering Two lenses, the objective and eyepiece, are
the lens are brought together by refraction. The responsible for the formation of the image. The
focal point is the point where the clear image of object or specimen is illuminated by the light
an object is formed. Focal length is the distance passing through the condenser which forms the
between the optical centre of the lens and the true image of the light source at the specimen
principal focus. plane. The illuminated specimen is magnified by
12 Textbook of Oral Pathology

Figure 1.12: Condenser used for dark field microscopy

the objective to produce a real, inverted image. Phase contrast microscopy is widely
A magnified upright virtual image of this real, employed in diagnosis of tumor cells and the
inverted image is produced by the eyepiece growth, dynamics, and behavior of a wide
which will be seen by the observer’s eyes. variety of living cells in culture. The phase
Dark field microscope is used to demonstrate contrast microscope is probably the most
spirochetes, trypanosomes, other micro- outstanding contribution to microscopy in recent
organisms, body fluids, cell suspensions, flow years. It can be used to produce excellent contrast
cell techniques, parasites and autoradiographic effects, with a wide variety of otherwise
grain counting. When observed under dark field transparent specimens. Since it permits
microscope these objects appear bright in dark visualization of interior details in cell structures,
background. it has a definite advantage over the dark field
microscope. Probably its widest application is in
Phase Contrast Microscopy the field of tissue culture, where it permits one
Phase contrast microscopy, first described in 1934 to examine and photograph living, growing cell.
by Dutch physicist Frits Zernike, is a contrast- Phase contrast microscope is standard
enhancing optical technique which is utilized to biological microscope and is equipped with
produce high-contrast images of transparent modified objective and condensers (Fig. 1.13).
specimens, such as living cells (usually in Condensers: Condenser has an annular
culture), microorganisms, thin tissue slices, diaphragm made up of opaque glass with a clear
lithographic patterns, fibers, latex dispersions, narrow ring, to produce a controlled, hollow
glass fragments, and subcellular particles cone of light.
(including nuclei and other organelles). Objectives: It requires a different size of annulus,
One of the major advantages of phase contrast an image of which is formed by the condenser
microscopy is that living cells can be examined in in the basic focal plane of the objective as a bright
their natural state without previously being killed, ring of light. The objective has a phase shifting
fixed, and stained. As a result, the dynamics of plate or positive phase plate which is a clear glass
ongoing biological processes can be observed and disk with a circular trough etched in it to half
recorded in high contrast with sharp clarity of the depth of disc. The trough also contains a
minute specimen detail. neutral density light absorbing material to
 Microscope 13

Figure 1.13: Phase plate of phase contrast microscope

showing different objectives

reduce the brightness of the direct rays which

could otherwise obscure the contrast obtained.
The light passing through the trough has a
phase difference of ¼ of wavelength.
Brightfield microscope can be converted into
phase contrast by two specialized accessories. A
specially designed annular diaphragm, which
is matched in diameter and optically conjugated
to an internal phase plate residing in the
objective rear focal plane, is placed in the
condenser front focal plane. Figure 1.14: Image formation in phase contrast
microscope
Principles of Phase Contrast Microscope
The basis of phase contrast microscope is the
exaggeration of minute differences in the The second ray of the incident beam is
refractive indices by advancing or retarding light modified by being scattered and diffracted in
waves, thus converting them into difference of passing around the margin of the object. This ray
amplitude, which are seen as variation in does not pass through the phase shifting ring but
brightness. Thus if two unstained structures of traverses the other areas of the transparent disc,
almost the same refractive index are examined and the wavelength is neither advanced nor
by ordinary illumination it will be found that retarded (Fig. 1.14).
they are indistinguishable from each other. Thus there is an optical difference of ¼th
For each transparent or translucent particle in wavelength, which causes a phase difference
the object, two rays result from an incident light. with the asynchronous waves producing
The direct, or undiffracted ray comes through the reinforced darkness or brightness at certain
‘angular diaphragm’, passes through the object points. Thus ‘phase contrast’ is made visible to
and is focused on the ‘phase shifting ring’ which the observer’s eye with the help of ‘phase shifting
either retards or advances the ray ¼ th wavelength plate’, which enhances the optical effect of the
with respect to the secondary. difference.
14 Textbook of Oral Pathology

Applications: Phase contrast microscope is If no rotating stage is available, the polarizer

valuable in examination of wet mounts and itself can be rotated. Changes in intensity and
hanging drop preparations. It can reveal cellular color are seen during rotation. The color changes
structure of living cell due to difference in the in a rotation of 90°, and back to its original color
refractive index of the components of the cell and in the next 90°. This is due to differential
is of great value in cytology, hematology and absorption of light, depending upon the
microbiology. vibration direction of two rays in a birefringent
substance.
Polarized Light Microscopy
Birefringence is the property by which a light Principle of Polarized Microscopy
ray shows ‘double refraction’ i.e. light entering
into a birefringent crystal splits into two paths A ray of light consists of electromagnetic waves
vibrating in all directions at right angles to the
with different refractive index.
path of the ray of light itself. In polarized light
Birefringence is shown by crystalline
structures, amyloid deposits, proteins, pigments the waves are made to vibrate in one plane only.
This is achieved by the rotating Nicole prisms,
and lipids. When such substances are viewed in
i.e. polarizer and analyzers, which is interrupted
a microscope with polarized filters (Fig. 1.15),
they may appear bright or even colored against in the beam of light.
The specimens are labeled into two categories
a dark background.
isotropic or anisotropic.
In this type of microscope, two ‘polarizers’
made up of Nicole Prisms are used. One is placed Isotropic (Singly refractive): These are substances
beneath the substage condenser and is held in a which are not illuminated by the change in
rotatable graduated mount, and can be removed direction of the beam or 90° rotation of the
from the light path when not required. The other analyzer. The rays transmitted by the lower
called ‘analyzer’ is placed between objective and prism will not pass through the upper, the field
eyepiece and is also graduated for measurements looks dark and the position of the prisms is called
to be taken. crossed Nicoles.
When a birefringent substance is rotated
between two polarizers, which are crossed, the Anisotropic (Birefringent or doubly refractive): These
image appears and disappears alternately at each substances are seen as positive after the changes
45° of rotation. In a complete revolution of 360° in direction of the beam of light, i.e. 90° rotation
the image appears four times. of analyzer, the objects are seen bright against a
dark background. Collagen fibers, bone matrix,
striated muscle, cholesterol, Zenker fixed RBC,
pigments such as formalin pigment crystals such
as talc and vegetable fibers like cotton and linen
are anisotropic.

Applications: The polarizing microscope can be a

useful means of identification of tissue
components and of exogenous an endogenous
Figure 1.15: Different types of polarizing filters for crystal specially when combined with special
polarizing microscope staining techniques and with histochemistry.
 Microscope 15

Fluorescence Microscopy fluorescent wavelengths. The former is

removed by barrier filter to prevent short
Objects invisible by ultraviolet light may become
wavelength light from damaging the
brilliantly luminous if coated with a fluorescent
retina of the eye.
substance, like fluorochromes. Fluorochromes
are the dyes which absorb radiation (e.g. Condensers: Dark ground condensers which do
Ultraviolet light) and become excited; these not allow direct light into the objective, and in
excited molecules are then capable of emitting addition gives a dark contrasting background to
radiation of longer wavelength and which the fluorescence are used. Routine bright field
disappear almost immediately after withdrawal condensers are able to illuminate the object using
of the exciting radiation. This is called all the available energy but they also direct the
‘fluorescence’. Thus fluorescence is the property rays beyond the object into the objective. This is
of some substances, which when illuminated by hazardous to the eyes of the observer.
light of certain wavelength causes them to emit
the rays of different and longer one. In Objectives: For fluorescence microscopy,
objectives with high numerical apertures are
fluorescence microscopy a fluorescent specimen
preferred. The intensity increases with increase
is illuminated with invisible ‘ultraviolet light’
(UV light has wavelength below 400 nm), the in the numerical apertures. Hence apochromat
are generally preferred.
light rays of longer wavelength within the
spectrum of visible light are given off and those Eyepiece: The eyepiece with lower magnification
are seen as various colors on dark background. is desirable as fluorescence is inversely
A completely dark room is desirable. Brilliant proportional to the square of the eyepiece
fluorescence depends upon maximum contrast magnification.
and is reduced if there is background light in
the room. Applications: Fluorescent techniques have
The equipment consists of: become recently widely used in research and
• Light source: Halogen lamps which give off fluorochrome dye methods are routinely
sufficient blue light (9400-500 nm). employed for the demonstration of tissue
Ultraviolet lamps need to be warmed up components, bacteria, fungi and heavy metal
before use and have short life. in sections and for the identification of
• Filters: Two filters are place, one in between malignant cells in exfoliative cytology.
condenser and source (Exciter filter); other is Fluorescent microscopy is the basis for
placed between the objective and eyepiece immunofluorescence techniques for the
(Barrier filter). demonstration of antigens and antibodies in
– Exciter filter: It is made up of tissues and sera.
- Heat filter (heat absorbing filter). Fluorochrome dyes which are used routinely
- Red stop filter: This filter removes red are Thioflavin T (amyloid) acridine orange
light. (Malignant cells, mucin and fungi) Auramine-
- Wavelength selection: This is main Rhodamine (Acid fast bacilli).
exciter filter that allows only the desired
Confocal Microscope
wavelength to pass.
– Barrier Filter: Light on passing through Confocal (“having the same focus”) microscopy
these filters illuminates the specimen; the is one of the most significant advances in
objective collects both exciting and microscopy. The principle of confocal imaging
16 Textbook of Oral Pathology

was patented by Marvin Minsky. With this

technique it is possible to control depth of field,
elimination or reduction of background
illumination, collect serial optical sections from
thick specimens and create 3-D image of the
specimen using microcomputers.
In conventional fluorescence entire specimen
is illuminated by the light from xenon or mercury
bulbs, and light from all areas of the specimen
enter the objective and image is obtained. In
confocal microscope only a pinpoint area is
illuminated and light from this area enters
objective and passes through a pinhole filter to
eliminate ‘out of focus’ light. As only a small
area is focused very bright, light is needed. This
is provided by the laser system. Coherent light
emitted by the laser system passes through a Figure 1.16: Image formation in confocal microscopes
pinhole aperture that is situated in a conjugate
plane (confocal) with a scanning point on the
Applications: The broad range of applications
specimen and a second pinhole aperture
available to laser scanning confocal microscopy
positioned in front of the detector (a
includes a wide variety of studies in
photomultiplier tube). As the laser is reflected
neuroanatomy and neurophysiology, stem cell
by a dichromatic mirror and scanned across the
research as well as morphological studies of a
specimen in a defined focal plane. Secondary
wide spectrum of cells and tissues. In addition,
fluorescence emitted from points on the
the growing use of new fluorescent proteins is
specimen (in the same focal plane) pass back
rapidly expanding the number of original
through the dichromatic mirror and are focused
research reports coupling these useful tools to
as a confocal point at the detector pinhole
modern microscopic investigations.
aperture. The significant amount of fluorescence
emission that occurs at points above and below
The Electron Microscope
the objective focal plane is not confocal with the
pinhole-termed Out-of-Focus Light Rays- is The electron microscope has gained its
eliminated (Fig. 1.16). Refocusing the objective fundamental superiority over the light
in a confocal microscope shifts the excitation and microscope because of its high resolving power
emission points on a specimen to a new plane to produce extreme fine details. In light
that becomes confocal with the pinhole apertures microscopy highest resolution that is possible
of the light source and detector. In this way entire theoretically is half of the wavelength of light.
specimen is covered point by point using a Thus limit of resolution of light microscopy is
scanner, images of individual points are 0.2 microns. With use of ultraviolet rays this can
acquired, processed, analyzed and image is be improved to 0.1 microns. But intracellular
displayed. components, certain bacteria and most of the
 Microscope 17

viruses are smaller than this and cannot be

visualized. So attempts were made to use other
types of radiation. In 1933 Ernst Ruska and Max
Knoll succeeded in this by building electron
microscope. In electron microscope it is possible
to enlarge the image 250,000 times or more. This
image can be photographed for permanent
record and enlarged 4-6 time without undue loss
of details, thus giving pictures in the range of
two million times as large as the object. Greater
resolving power (0.2 nm) makes it possible to
obtain images of protein molecular viruses,
unstained flagella, internal structure of cell etc.
Two types of electron microscope are used
• Transmission electron microscope (TEM)
• Scanning electron microscope (SEM)

Transmission Electron Microscope Figure 1.17: Image formation in light microscopes and
electron microscopes
The transmission electron microscope is similar
to the light microscope in that it uses lenses to electromagnetic coils or solenoids. When
form magnified image. Both have condenser energized these generate a magnetic field that
lenses to concentrate the incident beam upon the forces electrons to a focus. Current and voltage in
specimen. This beam passes through the these coils can be varied to change the focus of
specimen to the objective then to the projector electron beam.
lens and forms an enlarged image onto a Image formation: Focused electrons pass through
fluorescent screen. Difference lies in the radiation object. These electrons are directed to the viewing
used and type of lenses. In electron microscope screen or image recording unit (Fig. 1.17).
beam of electron is used instead of visible light,
Scanning electron microscope: The scanning
and electromagnetic lenses are used in place of
electron microscope provides a topographic
glass lenses. view of the surface contours of the specimen.
For this it uses an incident electron beam and
Components the reflected electrons produce the image which
Electron gun: It generates beam of electrons. It is is three dimensional. As it mimics our own
made of anode and tungsten filament, housed natural perception, the image is instantly
appreciated.
in a wehnelt shield. Filament generates electrons
by thermionic emission. Between tungsten
Maintenance of Microscope
filament and anode high voltage difference is
maintained so that electrons that are emitted Like every precision mechanical instrument,
from filament are accelerated towards object. microscopes will last longer and provide better
performance if cleaned and lubricated at regular
Electron lenses: These electrons pass through a set intervals. The actual work involved is simple and
of electron lenses. These are made up of not time-consuming. After long use of the
18 Textbook of Oral Pathology

instrument, cleaning and lubricating are required. • Loosen dirt with camel-hair brush, and blow
Major defects come from forced movement it off with blower.
especially when dried grease or fixed dirt on the • If oil or other grease film remains, distilled
movable parts causes wearing out the teeth of the water should be sprayed and wiped off with
gears. This occurs commonly between fine lens paper or clean lint-free cloth.
adjustment and coarse adjustment gears.
• If the film persists lens cleaning solution
should be applied and wiped off promptly.
Optical Maintenance
• Circular motion should be applied for
After long use of microscope, lenses become cleaning and polishing.
covered by fixed dust, dirt and film. Under the • The necessity of cleaning inner surfaces may
worst conditions, such as high humidity, fungi be determined by focusing the microscope on
may grow on the inner lens surfaces. This
a specimen and rotating the eyepiece, if dirt
microbial growth may erode the lens surfaces.
spots rotate, cleaning is required. Unscrew
Such lenses cannot be cleaned routinely and
should be returned to the manufacturer. Optical lower and upper lens elements and clean as
glasses are generally softer than window glasses described for outer surfaces.
so gentle touch is required while cleaning such
glasses. Lenses are cemented with adhesive Objectives
materials. The lenses may become loose, if there • Objectives should be taken apart from
is prolonged use of solvent materials for cleaning nosepiece for cleaning.
as these dissolve the adhesive cement. So xylene
• Exposed front surfaces of all objectives
should not be used. Petroleum spirit is
cleaned.
recommended by some manufacturers. The
recommended agent to clean the lenses is xylol. • Because the back lens usually located in the
Commercially available detergent based glass deep position though narrow hole, only
cleaning agents may be used. Alcohol and skilled repairman or the manufacturer can
acetone should be avoided as they may seep into clean it.
the mount and dissolve the cements. • Usually, lens surfaces of the objectives are
smaller than eyepieces, for checking dirt or
Cleaning of Eyepiece crack on the surface it is recommended to use
a magnifying glass.
If eyepieces are observed under good light, dirt
and film that may be present on the outer Condenser
surfaces of lens can be seen readily. Dirt on the
inner lens surface may be seen by looking For the Abbe condenser, take apart iris
through the field lens. Following steps should diaphragm unit from condenser, clean the top
be followed for cleaning: lens surface and back surface of the field lens.
 CHAPTER

2 Tissue Processing
Methods

Histology is the microscopic examination or

study of tissues.
Histopathology refers to the microscopic
examination of tissue to study the manifestations
of the disease.
Specifically, it refers to the examination of a
biopsy of surgical specimen or autopsy by a Figure 2.1: The biopsy specimen received in the laboratory
with details of registration number, date and time of biopsy
pathologist, after the specimen has been removal nature of biopsy and specimen details such as hard,
processed and histological sections are made soft, number of pieces
onto the glass slides.
Preparation of Tissue Specimen for
Specimen Accessioning (Fig. 2.1)
Histological Staining
Tissue specimens received in the surgical
Tissue from the body whether biopsy or tissue
pathology laboratory have a request form that
from autopsy is processed in a series of chemical
lists the patient information and history along
reagents to render it firm and soft enough to be
with a description of the site of origin. The
able to cut into thin sections by special knives.
specimens are accessioned by giving them a
The tissue has to be fixed, following which it
number that will identify each specimen for each
must be processed into a form in which it can be
patient.
made into thin microscopic sections. In usual
way, this is done with paraffin. Tissues
Gross Examination
embedded in paraffin, which is similar in density
Tissues removed from the body for diagnosis to tissue, can be sectioned at anywhere from 3 to
arrive in the Pathology Department and are 10 microns, usually 4μ routinely. The technique
examined. Gross examination consists of; of getting fixed tissue into paraffin is called tissue
describing the specimen for color, shape, size, processing. The main steps in this process are:
number and the time of the tissue received. (Fig. 2.2)
20 Textbook of Oral Pathology

• Coarse and fine abrasive lathe wheel with

water directed onto wheel
• Wooden block 1 × 1 inches in size.
• Adhesive tape
• Camel hair brush
• Mounting medium
• Microscope slide and coverglass

Procedure for Ground Section (Figs 2.3 and 2.4)

• The specimen is cut in mesiodistal or

buccolingual plane into half by means of
carborundum wheel if a longitudinal section
is to be prepared. For the cross-section the
tooth is cut half in horizontal or incisoapical
direction.

Figure 2.2: Steps of tissue processing

• Fixation
• Dehydration
• Clearing
• Embedding and
• Cutting

Study of Hard Tissues

Some tissues that contain calcium salts are
extremely hard and will not section properly
with paraffin embedding owing to the difference
in densities between calcium and paraffin. Such
tissues need special technique. Figure 2.3: The tooth is slowly abraded in one direction
keeping the tooth parallel to the Arcansas stone immersed
The teeth and bone are studied histologically in water
by two methods:
• Ground section
• Decalcification and thereafter regular tissue
processing and staining.

Ground Section
Calcified tissues like teeth and bone may be
ground to a thin section.
Equipment used for making ground section:
• Laboratory lathe Figure 2.4: Ground sections mounted on slides
 Tissue Processing Methods 21

• The wooden block is wrapped with adhesive

tape, sticky side directed outwards to which
the tooth specimen is attached.
• First coarse abrasive wheel of the lathe is used
and then a fine abrasive wheel is used.
• The tooth is ground upto 1-2 mm thickness
on the lathe.
• Then later it is ground on an abrasive stone
(Arkansas stone) manually.
• When the desired thickness is achieved the
section is carefully lifted by camel hair brush
and mounted on the slide.

Decalcification Methods for Hard Tissue

Figure 2.5: The decalcifying solution is changed till the
The ground section can demonstrate the suspended teeth become soft enough for further processing
and cutting
inorganic or calcified portion. For the study of
organic component and structure, it is required Routine Method for Histologic Study
that the hard tissue be made soft enough it to be Fixation (Figs 2.6 and 2.7)
cut by knife into thin sections and then stain it.
The steps for decalcification and staining of Fixation in simple terms is strengthening and
the hard tissue include: prevention of tissue decomposition. The agent
• For fixation, hard tissue is immersed in used for fixation of tissue is called as fixative.
neutral buffered formalin.
Aim of Fixation
• Decalcification is done by immersing the tooth
or bone in acid of specific concentration until The aim of fixation is to preserve tissues
the specimen becomes soft (Fig. 2.5). permanently in a life-like state as much as
• The decalcifying agents are nitric acid 5%, possible. Fixation should be carried out as soon
formic acid 10%, EDTA solution. as possible after removal of the tissues (in the
• The nitric acid decalcifies the tooth faster than case of surgical pathology) or soon after death
any other reagent, i.e. approximately within (with autopsy) to prevent autolysis.
a week. The disadvantage of HNO3 is that the
tissue integrity may not be preserved intact. Purpose of Fixation
• Formic acid and EDTA are slow decalcifiers • To prevent or arrest autolysis and bacterial
but give better results. decomposition and putrefaction.
• The tissue is checked by piercing with needle • To coagulate the tissue so as to avoid or
or a X-ray is taken for remains of calcified prevent loss of diffusible substances.
material. • To make the tissue strong enough to
• The tissue is washed in running water for withstand the tissue processing treatment
24 hours to remove the acid completely. with reagents and wax embedding.
• The procedure after decalcification is same • To prepare the tissue for differential staining
as for other soft tissue specimens. methods with reagents and dyes.
22 Textbook of Oral Pathology

• The diffusion or loss of the components is

prevented.
• The refractive index of tissues is altered in
varying degree.
• Fixation has a marked effect on staining and
it facilitates the action of dyes.

Requisites of Fixation
• The tissue ideally be grossed or cut into 0.5
cm thickness for better penetration of the
fixative.
• The volume of the fixative used must be 20
times that of the specimen.
• The time or length of fixation depends on the
size of the specimen.

Common Types of Fixative

• The fixative commonly used is 10% neutral
formalin.
• Other fixative solutions are:
– Zenker’s fluid (mercuric chloride, and
potassium dichromate solution).
– Lugol’s solution (potassium iodide and
iodine solution)
Figure 2.6: The tissue is placed with a small label of biopsy – Bouin’s fluid (picric acid and forma-
number in this tissue cassette for convenient processing of ldehyde solution)
many samples together
– Carnoy solution (absolute alcohol and
chloroform).

Removal of Fixative
The fixative should be removed by overnight
washing. The tissue cassette is placed in a trough
and is kept under running water overnight with
a small stream of water directed onto the
specimen cassette.

Figure 2.7: Processing of tissue involves dehydration of the Dehydration (Fig. 2.7)
tissue with increasing grades of alcohol and clearing with
two changes of xylene. The tissue is kept for almost one
It is the procedure to remove water from the
hour in each jar specimen, wet fixed tissues (in aqueous
solutions) cannot be directly infiltrated with
Effects of Fixation paraffin. First, the water from the tissues must
• Coagulation of proteins and other coagulable be removed by dehydration. This is usually done
constituents. with a series of alcohols.
 Tissue Processing Methods 23

Ascending grades of alcohol starting from

70%, 80%, 90%, 95% and then absolute alcohol
(two changes) are used to dehydrate the specimen.
The tissue cassette is placed in each of the
solution for minimum 1 hour.

Clearing (Fig. 2.7)

The next step is called "clearing" and consists of
removal of the dehydrant with a substance that
is miscible with the embedding medium
(paraffin). Alcohol is removed by this method.
The commonest clearing agent is xylene.
Toluene works well, and is more tolerant of
small amounts of water left in the tissues, but is
3 times more expensive than xylene. Chloroform
can be used, but is a health hazard, and is slow.
Figure 2.9: Preparation of the tissue paraffin wax blocks
requires two L moulds
Infiltration (Fig. 2.8)
After clearing, the tissue is immersed in melted
paraffin (melting point 58-60ºC). This is done in
an equipment called as Paraffin wax bath for two
hours with a change of half an hour in each
container.

Embedding (Figs 2.9 and 2.10)

After the specimen is completely infiltrated in
melted paraffin it is embedded in paraffin.

Figure 2.10: Tissue infiltrated in paraffin is placed at the

base properly oriented for section cutting

Two L-shaped metal pieces are used to

prepare a rectangular block. The tissue is
properly oriented so as to get the desired plane
of section required.

Sectioning with Microtome (Figs 2.11 to 2.14)

When the tissues have been embedded, they
must be cut into sections that can be placed on a
slide. This is done with a microtome.
Figure 2.8: Tissue is kept in melted paraffin for 2 hours for
complete infiltration of wax. This equipment is called paraffin The microtome is a equipment that holds a
wax bath knife with a mechanism for advancing paraffin
24 Textbook of Oral Pathology

Figure 2.13: Section cutting or tissue sectioning is done

on the microtome

Figure 2.11: Microtome knife is sharpened on a special stone

with machine oil as lubricant. Procedure is called as honing

Figure 2.12: Disposable blades are superior alternative to

knife Figure 2.14: The desired thickness in microns approx 5µ
(arrow) is adjusted and the sections are cut by rotating the
block. The most important necessity for proper wheel
sectioning is a very sharp knife (Fig. 2.11).
they are picked up on a glass microscopic slide
Knives are either of the standard thick metal which is coated with egg albumin.
variety or thin disposable variety (like a
The glass slides are then placed in a warm
disposable razor blade) (Fig. 2.12).
oven for about 15 minutes to help the section
Microtome has a mechanism for advancing the adhere to the glass slide.
block across the knife. Usually this distance can be
set, for most paraffin embedded tissues at 4μ. Frozen Sections
Sometimes during performance of surgical
Picking the Sections (Figs 2.15 and 2.16)
procedures, it is necessary to get a rapid
Once sections are cut, they are floated on a warm diagnosis of a pathologic process. The surgeon
water bath that helps to remove wrinkles. Then may want to know if the margins of his resection
 Tissue Processing Methods 25

Figure 2.17: Tissue blocks are carefully labeled and

preserved in waxblock storing cabinets

Figure 2.15: Semiautomatic microtome

Staining
Frozen sections are obtained with an instrument
called a cryostat. The cryostat is just a
refrigerated box containing a microtome. The
temperature inside the cryostat is about –20 to –
30° Celsius. The tissue sections are cut and picked
up on a glass slide. The sections are then ready
for staining.
The embedding process must be reversed in
order to get the paraffin wax out of the tissue
and allow water soluble dyes to penetrate the
sections. Therefore, before any staining can be
done, the slides are "deparaffinized" by running
them through xylenes (or substitutes) to alcohols
Figure 2.16: After cutting the sections are spread on a hot to water (Fig. 2.18).
water bath and picked up on glue coated slides The staining process makes use of a variety of
dyes that have been chosen for their ability to stain
for a malignant neoplasm are clear before
closing, or an unexpected disease process may various cellular components of tissue. The routine
stain used is hematoxylin and eosin (H and E).
be found and require diagnosis to decide what
Other stains are referred to as "special stains"
to do next, or it may be necessary to determine if
the appropriate tissue has been obtained for because they are employed in specific situations
according to the diagnostic need (Figs 2.19 and
further workup of a disease process. This is
2.20).
accomplished through use of a frozen section.
The piece(s) of tissue to be studied are snap
Mounting
frozen in a cold liquid or cold environment (–20
to –70° Celsius). Freezing makes the tissue solid The stained section on the slide must be covered
enough to section with a microtome. with a thin piece of glass to protect the tissue
26 Textbook of Oral Pathology

Figure 2.18: The tissue section on the slide needs to be

deparafinised in xylene before the actual staining procedure
starts

Figure 2.19: Staining serial set up for H and E staining

Figure 2.21: Slide storing cabinet with slide trays that can
hold at least 100 slides

at which a permanent resinous substance

beneath the glass cover slip, or a plastic film, can
be placed over the section.

Artifacts in Histologic Sections

A number of artifacts that appear in stained
Figure 2.20: Hematoxylin and eosin stained slides slides may result from:
• improper fixation and from the type of
from being scratched, to provide better optical fixative,
quality for viewing under the microscope, and • from poor dehydration
to preserve the tissue section for years to come. • incomplete paraffin infiltration,
The stained slide must go through the reverse • improper reagents
process; that it went through from paraffin • poor microtome sectioning.
section to water. The stained slide is taken • tissues that are insufficiently dehydrated
through a series of alcohol solutions to remove prior to clearing and infiltration with paraffin
the water, then through clearing agents to a point wax will be hard to section on the microtome,
 Tissue Processing Methods 27

with tearing artifacts and holes in the sec- in microtome sectioning artifacts with
tions. chattering and a "venetian blind" appea-
• In humid climates, tissue processing cycles rance.
should allow sufficient time for dehydration • Bubbles under the cover slip may form when
and final ethanol dehydrant solution should the mounting media is too thin, and as it dries
be at 100% concentration. air is sucked in under the cover slip.
• Though alcohols such as ethanol make • Contamination of clearing agents or
excellent fixatives for cytologic smears, they mounting media may also produce a bubbled
tend to make tissue sections brittle, resulting appearance under the microscope.
28 Textbook of Oral Pathology

CHAPTER
Stains and
3 Staining Methods

Antonie Van Leeuwenhoek discovered microscope or structural components makes them clearly
(1719) and started a new era in biological visible or detectable.
sciences. Robert Hook demonstrated that cell is • A staining agent is composed of two
basic unit of life. In following years description components, chromogen and auxochrome.
of cell and its constituents was given. All these • Property of color to a substance comes from
observations were made without any aid except the chromogen group. Most important
the use of microscope. Under microscope, tissues chromogen groups are azo, nitro, nitroso,
and their constituents are usually transparent quinoid and ethylene groups. These groups
and colorless and cannot be easily distinguished do not have the ability to bind to the tissues.
from each other. In order to make different • This ability of binding to the tissues comes
structures easily identifiable two different from auxochrome group, which contains
techniques were developed. ionizable groups that bind to tissues. Sulfates,
• Altering contrast and resolution: Contrast and carboxyls and hydroxyls groups form
resolution can be altered by using micros- auxochrome.
copes such as phase contrast, polarizing and • Picric acid contains nitro and hydroxy
electron microscope. Contrast can also be groups. Nitro group is chromogen as it
altered by the use of silver or gold imparts yellow color. Hydroxy group helps
impregnation techniques. picric acid to bind to the tissues thus acts as
• Alteration of color: Alteration of color is auxochrome. If hydroxy radicals are removed
achieved by using some chemical agents that although the resulting compound has yellow
binds to some structural component of a cell color it cannot bind to the tissues, so it is not
and imparts a color to it, which can be easily a true staining agent. Similarly if nitro groups
identified. This is very cost effective, specific are removed from picric acid it loses its color
and sensitive technique that can be used in although it can bind to the tissues, again not
day today practice of histology. a true staining agent. Thus both chromogen
Chemistry of stains: Stain is any substance which and auxochrome are necessary to call a
when added to living cells or to fixed structures compound staining agent.
 Stains and Staining Methods 29

Classification of Stains Connective Masson’s trichrome, von Geison’s

tissue method.
First—Based on the Source
Bone Trichrome stains, PAS, Schmorl’s
A. Natural • Xanthene picrothionin method, silver staining,
• Hematoxylin - Eosin, Rose Bengal, von Kossa method, solochrome
• Carmine Rhodhamine B, Ery-
cyanine method
B. Synthetic or artificial throsin, Phenolph-
• Nitroso thlein, Pyronin Y Neural Silver impregnation, Bielschowsky’s
- Naphthol Green Anthraquinone silver stain, PTAH method, Cajal’s
• Nitro - Alizarin red method
- Picric acid • Thiazole
• Azo - Titan yellow Amyloid Highman’s congored, Sirius red,
- Congo Red, Orange • Quinolin Toluidine blue, crystal violet,
G, Sudan III and IV, • Pinacyanol Thioflavin T
Bismark brown, • Phthalocyanine
Methyl orange - Alcian blue Mast cell Toluidine blue, azure A, PAS, alcian
granules blue-safranine
Second – Based on Tissue Component Stained Keratine, Performic acid alcian blue method,
keratohyalin Lendrum’s Phloxin-tartazine method,
Protein Ninhydrin Schiff’s method, Millon’s re- granules Ayub-Schlar method
action, diazotization-coupling method
for Tyrosine, performic acid-alcian blue
Based on pH
method for sulfide and disulfide bonds,
DMB-nitrite method, Modified A. Acidic B. Basic
Sakaguchi method for arginine. • Eosin, Erythrosine, • Hematoxylene,
Nucleic acid DNA Feulgen reaction, Fluorescein, Picric acid, Acridine red,
naphthoic acid hydrazine- Aniline
Feulgen method Alizarin, Acid fuchsin, blue, Azure,
RNA Methyl green pyronine Basic fuschin,
method Bismarck brown Crystal violet,
DNA and Gallocyanin-chrome alum, Malachite green,
RNA acridine orange. safranine
Carbohydrate Glycogen PAS, Best’s Carmine,
Hexamine silver method.
Mucins PAS, alcian blue, alcian blue- Theories of Staining
PAS technique, Dialyzed
Iron-Prussian blue tech- Much of the discussion pertaining to the theory
nique, Hale’s Technique, of staining took place in 20’s 30‘s. It was related
Azure A, Toluidine blue, to the doubt whether staining is chemical or
Southgate’s mucicarmine.
physical. Thus there are two theories, chemical
Lipids Routine Oil red O, Sudan black B, and physical.
Bromine-acetone Sudan
black, Nile blue sulfate.
Free fatty Copper rubeanic acid Chemical Theory
acid method.
Cholesterol Perchloric acid- • Certain parts of cell are acidic and others
naphthoquinone (PAN) basic and few are neutral. Similarly staining
Proteoglyce-Gold hexamine method, agents are also acidic, basic or neutral. Thus
rides Filipin method, UV-schiff
method, osmium tetraoxide it is natural to expect that acidic stains form
method. chemical bonds with basic tissue components
Trigly- Calcium lipase method. and basic stains combine with acidic tissue
cerides components, e.g. cytoplasm of most of cells
30 Textbook of Oral Pathology

is basic so it combines with basic stain eosin. and intravital staining. Supravital staining is
Nucleus is acidic as it contains DNA and done for live organism, where cells retains
RNA stains with basic stain hematoxylin. vitality after staining. Intravital staining is done
• Chemical theory explains the specificity of to cells that are removed from the body and cells
staining. But it cannot explain certain facts loose their vitality after application of the stain.
such as absence of new products, which is e.g. Alizarin Red is supravital staining used to
one of properties of chemical reactions. Also demonstrate developing bone. Toludine blue is
a chemical reaction continuous until one of supravital staining that is used to demonstrate
the reactants is exhausted. This is rarely precancerous lesions in the oral cavity. India ink
observed in staining. preparation is intravital staining method used
to demonstrate macrophages.
Physical Theory
According to physical theory one of the three
physical factors are responsible for staining.
• Physical penetration of dyes: Most of the tissue
components are more or less porous. In these
pores staining agents penetrate because of • Direct staining: Dyes such as eosin stain
simple physical forces such as capillarity and tissues perfectly when in alcoholic or aqueous
osmosis. solutions. This is known as direct staining.
• Adsorption of stains: Staining agents are • Indirect staining: Stains such as hematoxylin
adsorbed onto the surface of tissue by require additional substance known as
physical forces such as van der Waals forces. mordant before satisfactorily binding to the
• Absorption of stains: Absorption is a process tissue. This is known as indirect staining.
by which one substance takes another
substance into it. Tissues take up staining
agent in solution form and retain it in the
same form
Any or all of these factors may be responsible
for staining. Physical theory does not explain the • Progressive staining: Different component of
specificity of staining and differential staining. tissues are stained in sequence, so that at the
Now it is believed that reactions involved in end of correct time differential staining is
staining lies in the borderline between the achieved, e.g. eosin.
chemistry and physics, where it is impossible to • Regressive staining: In this technique tissue is
say that a given product is purely physical or first overstained so that all parts of tissue take
purely chemical. Various mechanisms such as up the stain and then excess stain is removed
electrostatic bonding, hydrogen bonding, van der from unwanted parts of tissue by a process
Waal’s forces, covalent bonding, hydrophobic known as differentiation.
bonding and dye aggregation are involved in • Hematoxylin is differentiated using acid
staining. alcohol or prolonged water wash.
• Staining by selective solubility: Certain
Different Techniques Used in Staining substances have ability to dissolve in
Vital Staining: It is the method of demonstrating particular tissue components, such as lipids.
living cells. It is of two types: supravital staining Such substances are known as lysochromes.
 Stains and Staining Methods 31

Usually lysochromes are alcoholic solutions components to differing degrees. Well-retained

that pre ferentially dissolve in lipids than in tissue substances are stained well. For example,
alcohol. This results in staining of lipids, e.g. aldehyde retains proteins well but does not retain
Sudan Dyes. lipids. Alcohols are poor fixatives but retain
• Staining by chemical production of colored lipids well.
substance: Some staining procedures use pale Temperature: Usually staining is done at room
or colorless solution, which react with tissue temperature. But temperature of staining
components to produce colored substances, solution can be changed. It has two effects:
e.g. Feulgen reaction: In feulgen reaction straw 1. It increases diffusion of the dye molecules.
colored or colorless solution of leuco basic 2. It allows greater reactivity between dye and
fuchsin is used. It is converted into purple tissue.
colored substance in presence of aldehyde
pH: Staining depends on pH as it controls the
group in tissues.
• Metallic impregnation: Some metallic ionization of both tissues and stains which is
prerequisite for staining.
substances can be reduced by tissues into
opaque, usually black deposits. Presence of impurities: Impurities present in any
• Certain intestinal cells contain melanin and stain sample have influence on dye solubility and
phenolic substances which reduce they have real effect on the intensity of staining.
ammoniacal silver nitrate into silver and A impurity may alter the pH, it may alter the
appear black such cells are called argentaffin dissociation of dye or tissue or it may act as
cells. Certain cells do not reduce ammoniacal mordant. In same cases impurities are necessary
silver nitrate directly but do so when to assure proper staining, pure stain may be
extraneous reducer is added. Such cells are detrimental to staining. For example, if rose
known as argyrophil cells. Bengal is used with distilled water poor results
• Metallic impregnation techniques are used are obtained. If tap water is used instead of
to demonstrate reticulin, nerve fibers, distilled water excellent results are obtained.
spirochetes and fungi. Ripening of staining solution: Some staining
• Metachromatic staining: Certain tissue solutions are effective only after exposure to air,
substances combine with stains to produce a light or warmth for weeks or months or exposure
color that is different from the original color to chemical oxidizers.
produced in the rest of the tissue. This is Hematoxylin when freshly prepared is
known as metachromasia and substance that useless as a nuclear stain. It becomes active after
produces metachromatic staining. For some weeks of storage. This is because natural
example, Toluidine Blue: Original color of this hematoxylin is not a dye but when oxidized it
dye is blue. But in tissue components such as forms hematin which is a good stain.
cartilage, mucins, mast cell granules and Hematoxylin can be oxidized naturally by
amyloid it takes red color. sunlight which takes months or by chemical
• Other metachromatic stains are azure A and B, oxidizers such potassium permanganate or
methyl violet and safranine. mercuric oxide instantaneously.

Factors Affecting Staining Routine Stains

Fixation: Fixation has profound effects of Hematoxylin and Eosin: The most popular, time
staining. Different fixatives retain different tissue tested and routinely used stain in histology and
32 Textbook of Oral Pathology

histopathology methods is hematoxylin and Contd...

eosin. Their popularity is because of the • Molybdenum
simplicity of staining method and stain – Phosphomolybdic acid hematoxylin
preparation, good contrast, good long lasting • Lead
– For demonstration of endocrine cells of GIT
staining which is beneficial for preserving slides.
• Chromate
The hematoxylin can be prepared in many ways – Weigert-pal’s technique
and can be used to stain dfferent structures. It • Hematoxylin without mordant
mainly stains nucleus with good intranuclear – For demonstration of minerals like iron and
detail. Similarly eosin stains cytoplasm most of copper
connective tissue fibers in varying degrees of
pink, orange, red. Chemical oxidation: Oxidation of hematoxylin to
hematin can be achieved rapidly by the use of
Hematoxylin: It is most successfully used natural chemical oxidizers such as sodium idodate
staining agent. It is extracted from Hematoxylon (Mayer’s Harris, Gill’s), potassium idodate
campuchianum, which is cultivated in West Indies. (Carazzi’s) mercuric oxide (Harris) Iodine
It is extracted from the wood using hot water and solution (Cole’s Hematoxylin) or potassium
precipitated by urea from aqueous solution. permanganate (PTAH). Chemically oxidized
Natural hematoxylin is not a stain as it does not hematoxylin has a very short life as chemicals
bind to the tissues. On oxidation it forms oxidize hematoxylin continuously. Once all
hematin. This hematin binds to the tissues and hematoxylin is converted to hematin, further
gives color to the tissue. oxidation of hematin into a colorless compound
Hematoxylin can be oxidized to hematin by occurs which is unsuitable for staining.
two methods. Hematoxylin is a anionic substance which
1. Natural oxidation and does not have good affinity to tissue. To increase
2. Chemical oxidation. affinity various mordants are used. Mordants
Natural oxidation: Exposure to sunlight, heat or form a complex with hematoxylin forming a
prolonged storage in light can oxidize positive charge which binds to negatively
hematoxylin to hematin. This process takes a very charged tissue components, like nuclear
long time as long as 3-4 months. But once the chromatin. Based on mordants hematoxylin can
solution is ripened in this way it can be stored for be classified into various types:
longer time. And it becomes better with passage Alum Hematoxylin: Alum hematoxylin are most
of time. Ehrlich and Delafield’s hematoxylin commonly used hematoxylin solutions. Mordant
solutions are prepared in this manner. used is either potash alum (aluminum potassium
sulfate) or ammonium alum (aluminum
Classification of Hematoxylin Based on
ammonium sulfate). These stains can be used
Mordants
either progressively or regressively. These
• Alum initially give red color to nucleus. This color is
– Ehrlich’s, Delafield’s, Mayer’s, Harris, Cole’s, converted into blue-black color when section is
Carazzi’s and Gill’s
washed in a weak alkali solution. This is called
• Iron
– Weigert’s, Heidenhain’s, Loyez’s, Verhoeff’s as blueing. Alkaline solutions such as saturated
• Tungsten solution of lithium carbonate, 0.5% ammonia in
– Phosphotungstic acid hematoxylin (PTAH) distilled water, Scott’s tap water substitute or tap
Contd... water can be used for blueing.
 Stains and Staining Methods 33

Alum hematoxylin are sensitive to acidic copper present in tissues can be demonstrated
solutions used in few staining techniques such with hematoxylin without any mordant.
as trichrome staining. So in connective tissue
Eosin: Eosin is a xanthene dye. It is available in
stains alum hematoxylin cannot be used.
various forms such as:
Iron Hematoxylin: Ferric chloride and ferric Eosin Y: Eosin yellow, water soluble
ammonium sulfate are used as mordants in iron Eosin S: Ethyl eosin, alcohol soluble
hematoxylin. These salts are strong oxidizers. So Eosin B: Bluish eosin.
they are not mixed with hematoxylin; they are Of this eosin Y is more popular and is widely
either used before or after application of used. Eosin is most suitable and popular stain
hematoxylin solution, to prevent over oxidation. used with hematoxylin. This is because it gives
In Weigert’s hematoxylin, mordant is applied good contrast with hematoxylin. It stains
after keeping slide in hematoxylin solution cytoplasm of different cells differently in varying
(Postmordanting). In Heidenhain’s hematoxylin shades of pink and red. Similarly it can stain
it is applied before putting the slide in different types of connective tissue fibers and
hematoxylin solution (Premordanting). Mordant matrices differently. In addition to routine
solution is also used for differentiation. staining it is used in pap stain along with azure.
Iron hematoxylin can be used to demonstrate It is usually used in concentration of 0.5% to 1%
wide variety of tissue in addition to nucleus. aqueous solution. To increase staining capacity
Using Heidenhain’s hematoxylin, mitochondria, and to get sharp and crisp staining little acetic
muscle fibers and myelin can be demonstrated. acid is added to this solution.
Verhoeff’s hematoxylin is used to demonstrate
elastic fibers. Special Stains
Iron hematoxylin are technique sensitive, When there is need to identify special structures,
time consuming and have short lifespan. use of hematoxylin and eosin stain is limited. In
Differentiation stage requires microscopic such cases staining agents that can identify
control for accuracy. structure of interest are employed. There are
Phosphotungstic acid hematoxylin is used numerous special stains that are available, but
to demonstrate myelin sheath. Here only a handful are employed regularly. PAS and
phosphotungstic acid acts as a mordant. trichromes are most commonly employed in
Phosphomolybdic and is used as a mordant. identifying glycogen and collagen fibers
Phosphomolybdic acid is used as a mordant in respectively.
place of phosphotungstic acid to demonstrate
collagen, coarse reticulin fibers and argentaffin PAS (Periodic acid Schiff method)
cell granules. Lead salts are used as mordants in This is a technique for the demonstration of
demonstration of granules of endocrine cells of carbohydrates in tissue sections. When basic
GIT. Chromate is used as mordant in Weigert- fuschin is reduced using HCl, it form Schiff’s
Pal technique to demonstrate myelin. In this base. This is a colorless compound, which regains
technique tissues are treated with dichromate its color once it comes in contact with oxidizing
solution before embedding. Later sections are groups in tissue sections. In this technique
dipped in hematoxylin to get brilliant staining periodic acid applied to expose oxidizing groups
of myelin. In few techniques minerals present of glycogen molecule. Later tissue is flooded with
in tissues act as mordants. In this way iron and Schiff’s base. Glycogen molecules are stained into
34 Textbook of Oral Pathology

a bright pink or magenta color. Glycogen is

present in many substances such as basement
membrane, mucins, cartilage, fungi and Russell
bodies. All these structures can be demonstrated
by PAS reaction (Fig. 3.1).

Trichrome Stains
Popularly called as connective tissue stains,
trichrome stains are used in differential
demonstration of connective tissue components
such as collagen, muscle, fibrin, etc. In this
technique two or more acidic dyes of different
Figure 3.1: PAS stained section showing magenta colored
molecular weight and contrasting color are used candidal hyphae (CH) and basement membrane (BM) (E—
in the staining. Epithelium, IC—Inflamed connective tissue)
How exactly differential staining occurs in
trichrome stains is not understood. But various
tissue factors and molecular weight of the dyes
are thought to be responsible. Due to fixation,
tissues form networks, these networks create
pores of different sizes in different structures. In
RBCs, very dense network with small pores is
produced. In muscles pores of intermediates size
are produced. As collagen is loose tissue it
produces least dense network and pores are large
size. Dyes of different molecular weight occupy
these pores. Thus dye of least molecular weight
occupies smallest pore and larger pores are
occupied by large dye molecules. Figure 3.2: von Geison’s stain showing collagen stained red

Von Geison’s method: In this technique nucleus differentiating with phosphomolybdic acid,
is stained first using hematoxylin. Then slides are which also acts as a mordant for the next step of
kept in von Gieson solution which contains the procedure. A dye of large molecular size,
saturated solution of picric acid and acid fuschin. light green is then applied to the section. Nucleus
This results in blue/black nuclei, red collagen is stained by hematoxylin. Thus nuclei and
and other tissues such as muscle, elastin, elastic fibers are stained blue to black, cytoplasm,
reticulin, basement membrane, and fibrin take muscle and acidophilic granules are stained red;
yellow color (Fig. 3.2). Collagen, reticulin, basement membranes,
osteoid and basophilic granules are stained green
Masson’s Trichrome
(Fig. 3.3).
In this method small molecule size dye acid
fuchsin/ponceau is used first and this stains all Staining Procedure
tissue elements in the section. This stain is After making paraffin sections of the tissue block
selectively removed from unwanted areas by routine staining of hematoxylin and eosin and if
 Stains and Staining Methods 35

• Hydration: Xylene is also hydrophobic. This

is removed by a agent that is both miscible
with water and xylene. The ideal candidate
for this is alcohol. Xylene from tissues is
removed by decreasing concentrations of
alcohol in water.
• Nuclear staining: It is done by dipping the slide
in hematoxylin solution.
• Differentiation: In regressive staining excess
hematoxylin in tissues is removed usually by
1% acid alcohol (1% HCl in 70% alcohol).
Differentiation is also brought by prolonged
water wash. In progressive staining this step
is omitted.
• Blueing: Nuclear staining results in red colored
nucleus. This is converted into bluish black
Figure 3.3: Masson’s trichrome stain showing green color by weak alkali solutions such as lithium
collagen fibers and red muscle fibers
carbonate, ammonia water or tap water.
• Counterstaining: This is done by keeping
required, special staining is done. Principles of slides in eosin solution.
staining are same for most of the stains except • Dehydration: After staining slide is mounted
few minor modifications. A section usually goes using DPX (Diastrenedibutyl phthalate
through seven steps during staining. xylene). DPX is hydrophobic. So water from
• Deparaffinization: Hematoxylin and eosin the tissues should be removed first. This is
stains are usually aqueous solutions, not done by increasing concentration of alcohol.
miscible with paraffin. To stain tissues, paraffin Later alcohol is removed by 2 changes of
must be removed from the tissues first. This is xylene. Any remaining water is removed by
usually done by two changes of xylene. warming on the slide warmer.
36 Textbook of Oral Pathology

CHAPTER

4 Diagnostic Pathology and

Caries Activity Test

There are various important investigations Indications

which are required for the diagnosis and • When after careful clinical examination, any
treatment plan of various disorders related to the alter-ation from normal is seen and it is not
oral cavity. Laboratory studies are an extension possible to identify the condition clinically, a
of physical examination in which tissue, blood, histopathological investigation is necessary.
urine or other specimens are obtained from • To evaluate the exact histological nature of
patients and subjected to histological, bio- any soft tissue or intraosseous lesion.
examination, microbiological or immunological • To screen abnormal tissues removed from
examination. Information obtained from these oral cavity including granuloma and cyst.
investigations help in identifying the nature of • To confirm the existence and nature of
the disease. directly apparent malignancy so that the
treatment can be undertaken immediately.
HISTOPATHOLOGICAL EXAMINATION • Diagnostic tests for evaluation of
nonneoplastic lesions such as mucosal
Autopsy: It is the histopathological study of the
nodules, papilloma, erosive lichen planus,
tissues removed after the death of an individual.
erythema multiforme, lupus erythematous
Biopsy: It is the study of tissues removed from a pemphigus, pemphigoid and desquamative
living organism to confirm the diagnosis through gingivitis.
histopathological study.
Contraindications

Biopsy • Benign lesion—Lesions which are diagnosed

clinically and harmless in nature should not
It is a process of surgically removing tissue from be biopsied.
a patient for histopathological examination. It is • Irritation lesion—This lesion usually respond
the removal of sample tissue from living to after removal of irritant, hence biopsy is
individuals. It provides valuable information in not required.
determining the prognosis and type of treatment • Inflammatory lesion—Biopsy is not indicated
required. in inflammatory lesion.
 Diagnostic Pathology and Caries Activity Test 37

• Site near the vital structure—You should be • Adequate tissue—It should have sufficient
very careful while undergoing biopsy where tissue sample for a pathologist to interpret.
vital structure lies. • To facilitate treatment—Biopsy sample should
• Angiomatous lesion—Unless it is needed, you help to facilitate to prescribe treatment and
should not go for the biopsy of angiomatous assess its efficacy.
lesion. • Normal adjacent tissue—To compare and
Biopsy should not be delayed when following strengthen the diagnosis normal adjacent
feature are present: tissue should be included.
• Rapid increase in size of the lesion that can
not be explained by inflammation, edema How to Handle Biopsy Material?
and opening of new vascular channels.
• Biopsy should reach to histopathologist
• Absence of any recognized irritant,
without any damage or autolysis.
particularly when the lesion is chronically
• During biopsy the grasping area of forceps
ulcerated or bleeds spontaneously.
should be away from the site to be removed
• Presence of firm regional lymph nodes,
to prevent trauma to the lesional tissue.
especially when they are seen to be fixed to
surrounding tissues.
Types of Biopsy
• Destruction of tooth roots and loosening of
teeth with evidence of rapid expansion of the Commonly Used
jaw. • Aspiration
• History of cancer elsewhere in the body, • Curettage
previous history of oral cancer and radiation • Excisional
therapy. • Incisional
• Fine needle
Uses
• Punch
• Diagnosis of pathological lesions. • Scrape
• Grading of tumor for diagnosis. • Trephine
• Determining neoplastic and non-neoplastic
lesions. Less Commonly Used
• Diagnosis of metastatic lesions. • Bite
• Evaluation of recurrence. • Brush
• Therapeutic assessment, differentiation • Cone
between benign and malignant lesion. • Core
• Endoscopic
Complications
• Irrigation
• Hemorrhage, infection, poor wound healing. • Pressure
• Spread to adjacent organs and reaction to • Shave
local anesthesia. • Sponge.

Essential Requirement of Biopsy Submission of Specimen

• Less trauma—The tissue taken for biopsy The submission of specimen should be
should have fewer traumas as far as possible. accompanied by the date of biopsy, name, age
38 Textbook of Oral Pathology

and sex of the patient, the area from where biopsy • Pointed surgical scissors, tissue forceps and
specimen is taken and brief description of clinical surgical hemostat.
appearance of lesion and associated symptoms, • Sterile sponge, curved needles and suture,
along with tentative clinical diagnosis. needle holder.
Iodine containing surface antiseptics should • Wide mouthed bottle containing 10% formalin.
be avoided since they have a tendency to stain
certain tissue cells permanently. Intraosseous Biopsy
The portion of the biopsy specimen to be used • Periosteal elevator, bone bur, curette.
for routine histological study should be placed
at once in a suitable fixing solution, usually 10% Aspiration Biopsy
neutral buffered formalin and sent to the
• Large syringe (10-20 cc) with a large bore
pathology laboratory.
needle.
For the histologic examination of teeth, the
apex of tooth should be clipped with a pair of
pliers or a small hole should be drilled into the Excisional Biopsy
radicular pulp with dental burr to allow Total excision of a small lesion for microscopic
penetration of the fixative. examination is called as ‘excisional biopsy’. It is a
The excellent preservation of cellular detail therapeutic as well as a diagnostic procedure.
required is obtained by following methods: Normal tissue on the margins of the lesion
• Cutting the specimen into tiny blocks before should be included.
fixation.
• Use of special fixatives that preserve cellular Indications
detail with minimum disruption from rapid • It is indicated when the lesion is relatively
dehydration or osmotic shock. small and less than 1 cm in diameter, sessile
• Post-fixation and processing of tissues in the or pedunculated and well circumscribed.
laboratory after the initial period of prefixatio • Tissues which are freely movable and located
(Chapter 2. Tissue processing). above the mucosa or just beneath the surface.
As soon as possible after the surgical • It is the preferred treatment if the size of
procedure, carefully section the biopsy specimen lesion is such that it may be removed along
with a fresh scalpel or razor blades into pieces. with the margins of normal tissue and wound
Pieces should not be larger than 0.5 cm in can be closed primarily.
diameter, identifying each fragment in relation
to the overall specimen and lesion. Procedure
Immerse the pieces in Karnovsky’s fixative
• Anesthetize the lesion with 2% local
(4% paraformaldehyde) and 5% glutaraldehyde
anesthetic containing vasoconstrictor. Care is
in 0.1 M solution for 2 to 4 hours and deliver
taken not to inject directly into the lesion that
promptly to the laboratory.
is to be removed.
• With the scalpel make an elliptical incision
Instrument and Materials
on either side of the base of the lesion so that
Excisional and Incisional incision line is intersected.
• Local anesthesia with vasoconstrictor, scalpel • The blade should be at an angle of 45°
holder and blade. towards the center of the lesion.
 Diagnostic Pathology and Caries Activity Test 39

• Outward tension is placed on the lesion by • The incisional lines must be deep enough to
means of suture or with the help of tissue include underlying connective tissue to the
forceps attached at the edge of specimen. level of muscle or bone.
Care must be taken not to crush the specimen. • Suture is inserted through the end, upward
• The specimen is now gently dissected out with tension is applied while tissue sample is
either a scalpel or a pair of surgical scissors. dissected out.
• The tissue must immediately be submerged
in 10% formalin solution. Intraosseous Biopsy
• Surgical site is closed with either silk or
It is less frequently performed. It may be in the
absorbable sutures placed approximately
form of exploratory curettage in which the
5 mm apart.
representative tissue is obtained to determine the
nature of large radiological alterations.
Incisional Biopsy
Incisional biopsy can be performed by removing Procedure
a wedge shaped specimen of the pathological • After correlating the radiographs with
tissue along with surrounding normal zone. overlying anatomical structures, the site at
which the mucoperiosteal flap is to be raised
Indications
is selected.
• If the lesion is large and diffuse and extends • Anesthesia to the area is accomplished by
deeply into the surrounding tissue so that total block injection to the area and with local
removal can not be obtained easily with local infiltration with a 2% solution of local
anesthesia, an incisional biopsy is indicated. anesthetic along with vasoconstrictor.
• Dealing with a large lesion. • With a scalpel, press firmly against the cortical
• Lesions in which diagnosis will determine bone, outline the flap using the periosteal
whether the treatment should be conserva- elevator and strip the tissue from the bone.
tive or radical. • Using a small bone burr in low speed drill,
• The selection of site is important. It is best to make a small square window through cortical
select the site away from an obvious plate; using a water spray as a coolant.
ulceration or area of necrosis, as these are • Remove the cortical plate of bone and with
areas of intense inflammation which make curette, obtain deep sample of the underlying
interpretation difficult. lesion and place the sample in 10% formalin.
• The surgical site is closed by replacing the flap.
Procedure
Aspiration Biopsy
• Carefully observe and palpate the lesion so
that a decision can be made regarding the Indications
appropriate site that will produce a more • This procedure is used to obtain information
preparative specimen. about the nature of the fluid content of a large,
• The tissue around the specimen is infiltrate deep-seated relatively inaccessible, soft tissue
with 2% local anesthetic. mass or an intra-osseous cystic lesion.
• With a scalpel, make an elliptical incision • It may be used either alone or prior to one of
encompassing the selected area of the lesion. the other procedures.
40 Textbook of Oral Pathology

• It is done in cases of lesion either situated in

deep inaccessible sites like in metastasis and
cystic lesions.

Procedure
• Lesion is located and after properly
angulating the needle, it is inserted firmly
into the lesion and negative pressure is
exerted on the syringe until several Figure 4.1: Punch biopsy kit contains punches
of different size
millimeters of fluid is withdrawn.
• If the lesion is composed of desquamated
cells or soft tissue, the barrel will remit
because of negative pressure as; these cells
of soft tissue cannot be aspirated.
• When the lesion is cystic the withdrawn fluid
is yellow in color however occasionally it may
be red due to presence of blood.

Punch Biopsy
It is rarely necessary in the oral cavity as most of
the oral lesions are easily accessible. With this Figure 4.2: Punch of appropriate size is placed on the lesion
technique the surgical defect that is produced is and inserted by rotating until adequate depth is reached
small and does not require suturing.
• The sample can be screened nowadays by a
Procedure
neurally networked computer that is
• In this technique, a sharpened hollow tube; programmed to detect cytologic changes
several millimeters in diameter is rotated associated with premalignancy and
until underlying bone or muscle is reached. squamous cell carcinoma.
• The tissue is then removed in the same • The specimen is reviewed by a pathologist
manner as in incisional or excisional biopsy for final diagnosis. This technique is ideal for
(Figs 4.1 and 4.2) determining the need for scalpel biopsy in
benign-appearing oral mucosal leukoplakias
Oral Mucosal Brush Biopsy
(Figs 4.3 and 4.4).
• The most recent development in oral biopsy
technique is the oral mucosal brush biopsy. Frozen Section Biopsy
It was introduced in 1999. • It is performed in order to get an immediate
• This technique utilizes a disposable brush to histological report of a lesion.
collect a transepithelial sampling of cells. This • It is done to determine whether a lesion is
brush has got two cutting surfaces, i.e. flat malignant or not. It is also used to evaluate
end and circular border. the margins of an excised cancer, to ascertain
• Specimen obtained brushing on the site and that the entire lesion is removed at the time
smeared on clean labeled glass slide. of surgery.
 Diagnostic Pathology and Caries Activity Test 41

smear. Use of cytology in orofacial area was done

in the year 1949 by Morrison.

Principle of PAP Smear

Individual cells can often be diagnosed as such
microscopically by their large size, their
pleomorphism, increased nucleocytoplasmic ratio,
hyperchromatism and prominence of nuclei and
their abnormal mitosis. Cancer cells exfoliate more
easily than normal cells, most likely because their
cohesiveness is lowered as a result of either
decrease in number of tight junctions or lower
calcium content.
Figure 4.3: Oral brush biopsy technique
Indications
• Compromise situation: As a compromise,
when the patient refuses for biopsy.
• Follow-up: As a means of follow up for
recurrence in patients who had radiation
therapy for the lesion that was superficial or
adjacent to bone, periodic recall of high-risk
patient.
Figure 4.4: Oral brush biopsy is technique used to sample
• Debilitated patient: In place of biopsy, when
cells from all the layers of the epithelium
dealing with extremely debilitated patients
posing problems to determine a suitable
• The tissue is obtained from lesion and it is biopsy site.
kept in deep freezer and then frozen tissue is • Aid in the diagnosis: As an aid to the diagnosis
sectioned and stained to get a prompt of some dermatological diseases such as
diagnosis. pemphigus, white sponge nevus, oral
• In this type of biopsy, the slides cannot be malignant and premalignant lesions.
preserved for future reference. • Rapid evaluation: For rapid evaluation of an
oral lesion that on clinical grounds, is thought
Cytology to be malignant or premalignant and for
In this, the surface of the lesion is either wiped which, the dentist is unable to obtain
with some sponge material or scraped to make a permission for a biopsy.
smear. It is the microscopic examination of the • Patient who received radiotherapy: For
cells shed from an epithelium. The appreciation sequential laboratory evaluation of an area
of the fact that some cancer cells are so typical of the mucosa that has previously been
that they can be recognized individually has treated by radiation or by excisional biopsies
allowed the development of this diagnostic to remove malignancy.
technique, which is developed by Dr George • Vesicular lesion: For evaluation of vesicular
Papanicolaou who, is also known as the ‘father lesions where facilities for rapid evaluation
of cytology’ and the technique is called as PAP of Tzanck smears are not available.
42 Textbook of Oral Pathology

Advantages Exfoliative Cytology

• Bloodless procedure: It is quick, simple, It is study of examination of cells which are

painless, bloodless and an inexpensive manually removed from the area and stains
procedure. accordingly.
• Minimum discomfort: It causes minimum
Instruments used
discomfort to the patient and is easily
performed. • Glass microscopic slide, lead pencil, cement
• No anesthesia: No anesthesia is required. spatula or wax carver.
• Check against false negative biopsy: It helps to • Wooden tongue depressor, tooth pick,
check against false negative biopsy. canister of cytospray.
• Recurrent carcinoma: It is especially helpful • 95% isopropyl alcohol or ethyl alcohol.
in a follow up detection of recurrent
Procedure
carcinoma.
• Screening test: It is valuable for screening • Use of two slides for each site to be sampled.
lesions whose gross appearance is such that • With lead pencil print the patient’s name, date
biopsy is not warranted. when the slide is prepared and the site of the
• No complication: It is a safe procedure as lesion on frosted end of glass microscopic
complications are rare. slide.
• Rapid diagnosis: It enables a rapid diagnosis • The instrument selected to remove the
and is economical. superficial cell must have a square edge with
• No delayed wound healing: Less risk of delayed a contour sufficient to scrape off the
wound healing and infection. superficial layer of cells. When the lesion is
• Cost effective: Cost of cytological investigation very small, the edge of tooth pick is effective.
is less as compared to others. • Clear the surface of oral lesions of debris and
• Other advantages: Hundred percent accuracy mucus.
in lymph node aspiration from metastatic • While the tissue is stretched, the squared edge
carcinoma, melanoma, Hodgkin’s and non- of the collection instrument is positioned at
Hodgkin’s lymphoma. the back of the lesion and is firmly held and
brought forward and pressure applied until
Disadvantages visible material is collected.
• Vigorous scraping of the entire surface of the
• Firm tumors: Firm tumors may prevent a lesion several times is done with a metal
proper cyto-diagnosis due to paucity of cells cement spatula or a moistened tongue blade.
in the aspirate. • Collected material is then quickly spread
• Inadequate sampling: Oral cytology can give evenly over the microscopic slide.
false–ve findings due to inadequate sampling. • Fix it in commercial preparation such as
• Poor cellularity: Some specimens can not be spraycyte, 95% alcohol or equal part of
assessed due to poor cellularity. alcohol and ether, immediately before it dries.
• Then allow it to stand for thirty minutes so
Types
that it air dried.
• Exfoliative cytology • Repeat the procedure and prepare a second
• Fine needle aspiration cytology. smear.
 Diagnostic Pathology and Caries Activity Test 43

Reporting Procedure

It is reported by a cytologist as follows into one • Position the needle within the target tissue
of the following five classes: (Fig. 4.5).
• Class I (normal): It indicates that only normal • Plunger is pulled to apply negative pressure.
cells are observed. • Needle is moved back and forth within the
• Class II (atypical): Presence of minor atypia target tissue to obtain a greater field.
but no evidence of malignant changes. • Negative pressure is then released while the
• Class III (indeterminate): This is a stage in needle remains within the target tissue.
between that of class II and IV and separates • Needle is withdrawn and then the defumed
non-cancer cells from cancer cells displaying air drawn in the syringe and the aspirate is
wider atypia that may be suggestive of cancer blown onto the slide.
but they are not clear-cut and may represent • Fixing is done in 95% alcohol for 1 hour for PAP
precancerous lesion or carcinoma in situ and stain and a little prolonged for H & E stain.
a biopsy is recommended in such cases.
• Class IV (suggestive of cancer): Few cells with CARIES ACTIVITY TESTS
malignant characteristic or many cells with
Caries activity can be defined as the occurrence
borderline features. Biopsy is mandatory in
and rate at which teeth are destroyed by the acid
such cases.
produced by plaque bacteria or it can also be
• Class V (positive of cancer): Cells that are
defined as the sum total of new carious lesions
obviously malignant. Biopsy is mandatory in
and the enlargement of existing carious cavities
such cases.
during the given time.
Fine Needle Aspiration Cytology (FNAC)
Uses
It is the microscopic examination of an aspirate
obtained by inserting a fine needle into the lesion. • To determine the need and extent of
preventive measures.
It is a painless and a safe procedure for rapid
• To determine the success of therapeutic
diagnosis. First discovered by Kun in 1847 and
reintroduced in 1930 by Martin and Ellis. measures.
• To motivate and monitor the effect of
Indications education programs related to diet
counseling and oral hygiene procedures.
• FNAC of salivary glands is a useful • To identify high-risk groups and individuals.
procedure for evaluation of salivary gland The various caries activity tests are as follows:
tumors.
• It is indicated in lesions in which open biopsy
require extensive procedures.
• For examination of enlarged clinically
suspicious lymph nodes.
• To check against recurrence or local
extension.
• Detection of metastatic squamous cell Figure 4.5: Fine needle aspiration is used to obtain
carcinoma within cervical nodes. contents of deep seated lesion
44 Textbook of Oral Pathology

Lactobacillus Count Test • Then 0.2 cc of saliva is pipetted into the media
which is incubated at 37°C up to a period of
It was introduced by Hadley in 1933. It estimates
72 hours.
the number of bacteria in the patient’s saliva by
• The media contains bactopeptone (20 gm),
counting the number of colonies appearing on
dextrose (20 gm), sodium chloride (5 gm), agar
tomato Peptone Agar or LBB Agar.
(16 gm) and bromocresol green (0.02 gm).
• Bromocresol green, being an indicator,
Technique
changes the color from blue green to yellow
• Stimulated saliva is collected before breakfast in the range of pH of 5.4-3.8.
by chewing paraffin. This is shaken and 1:10 • The color change is then correlated with the
and 1:100 dilutions are spread on the surface caries activity.
of agar plate.
• These are incubated at 37° for a period of 3-4 Interpretation
days. The number of colonies is then counted
• High—If color changes in 24 hours, caries
in a Quebec counter.
activity are high.
• The count expressed as the average number
• Medium—If the color changes in 48 hours, it
of colonies per milliliter of the original saliva
is medium.
sample.
• Slight—If color changes in 72 hours, it is
slight.
Interpretation
• Immune—If there are no color change,
• Immune: If the count is less than 1000 then patient is immune to caries.
the patient is immune.
• Slight: If the count is between 1000 – 5000, Alban’s Test
caries activity is slight. It is a modification of Snyder’s test. It uses less
• Medium: If the count is between 5000 – 10,000, quantity of agar, i.e. 5 ml per tube. Because of its
caries activity is medium. simplicity and its low cost it is recommended
• High: If it is more than 10000 then caries for all patients prone to caries. The main feature
activity is high. of Alban’s test is the use of a softer medium that
Disadvantages: Although it is quick and easy, the permits the diffusion of saliva and acids without
results are not available for several days and the necessity of melting the medium and use of
counting colonies is a tedious process and is a simpler sampling procedure in which the
complex. Equipments and personals are patient expectorates directly into the tubes that
required. contain the medium.

Snyder Test Technique

This test measures the ability of microorganisms • Sixty grams of Snyder test agar is placed in
in saliva responsible for formation of acids from 1 liter of water and the suspension is brought
carbohydrate media. to boil over a low flame or a hot plate at
medium heat (excessive heating should be
Technique avoided to prevent scorching of medium).
• The saliva sample is collected in a manner • When thoroughly melted, agar is distributed,
similar to the Lactobacillus colony count test. using about 5 ml per tube. The tube should
 Diagnostic Pathology and Caries Activity Test 45

be autoclaved for 15 minutes, allowed to cool added to the sample until a pH of 6.0 is
and stored in a refrigerator. reached. The number of millimeter of lactic
• Two tubes of Alban medium are taken from acid needed to reduce pH from 7.0 to 6.0 is a
the refrigerator and saliva is drooled directly measure of buffer capacity of saliva.
in to the tubes and tubes are incubated for 4
days at 37° Celsius. Interpretation
• The tubes are observed daily for change in color.
• The color change is noted from bluish green • Low buffer capacity: saliva sample requiring
to yellow and the depth to which change has less than 0.45 ml of standard hydrochloric
occurred is noted. acid to reduce the pH to 5 has low buffer
capacity.
Interpretation • High buffering capacity: Saliva sample
requiring 0.45 ml or more has high buffering
• Negative—No color changes. capacity.
• + —Beginning of color change (from top of
the medium towards bottom). Fosdick Calcium Dissolution Test
• ++ —One half color changes.
• +++ —Three fourth color changes. Twenty five milliliters of gum stimulated saliva
• ++++ —Total color change. is collected. Part of this is analyzed for calcium
content and the rest is placed in an eight inch
Streptococcus mutans Level in Saliva sterile test tube with about 0.1 gm of powdered
human enamel. The tube is sealed and shaken
• Saliva samples are obtained by using tongue
for four hours at body temperature after which,
blades (after air drying the tooth for plaque
it is again analyzed for calcium content. If
samples).
paraffin is used, a concentration of about 5%
• These are then incubated on MSB agar (Mitis
glucose is added. The amount of enamel
Salivarius Bacitracin Agar).
dissolution increases as the caries activity
• The number of colonies are then used to
increases which is indicated by an increase in
estimate the caries activity and more than 105
the calcium content of saliva.
colonies per ml of saliva is indicative of high
caries activity.
Dewar Test
Buffer Capacity Test Plaque samples are collected from the gingival
third of buccal tooth surfaces and placed in
Technique
Ringer’s solution. The sample is shaken until
• Ten milliliter of stimulated saliva is collected homogenized. The plaque suspension is streaked
under oil at least one hour after eating; 4 ml across a Mitis-Salivarius agar plate. After aerobic
of this is measured into a beaker. After incubation at 37° Celsius for 72 hours, the culture
collecting the pH meter, the pH of saliva is is examined under a low power microscope and
adjusted to 7.0 by addition of lactic acid or the total colonies in 10 fields are recorded. This
base at room temperature. test is an attempt to semi quantitatively screen
• The level of lactic acid in the graduated the dental plaque for a specific group of caries
cylinder is re-recorded. Lactic acid is then causative organisms including streptococci.
46 Textbook of Oral Pathology

Swab Test is measured after 30 seconds and 15 minutes and

this is taken as measure of caries activity.
The swab test involves sampling of the oral flora
by swabbing the buccal surface of teeth and Interpretation
placing it in Snyder media. This is incubated for • Nonconducive: If it remains blue after
48 hours and the pH changes are read and 15 minutes, it is nonconducive.
correlated with caries activity. • Slightly conducive: If it changes to orchid after
15 minutes, it is slightly conducive.
Reductase Test • Moderately conducive: If it changes to red after
15 minutes, moderately conducive.
Measures the activity of salivary enzyme • Highly conducive: If it changes immediately
reductase. Saliva is collected and the sample is to red, it is highly conducive.
mixed with a diazoresorcinol, which colors the • Extremely conducive: If it changes to pink or
saliva blue. The change in color from blue to red white immediately, it is extremely conducive.
 CHAPTER
Advanced Diagnostic
5 Techniques

As knowledge of pathology increased it was various enzymes and other chemical substances
found that hematoxylin and eosin staining alone in relation to tissues or organelles within a cell.
is not adequate to identify various structures To study the distribution of these chemical
present within tissues. A need for procedures substances it is essential to preserve them
that can identify special structures was felt. This carefully. Certain fixatives cause alteration in
lead to identification of various special dyes and chemical structure, making identification of
techniques that can identify one or few these substances difficult. Thus fixation is one
components that are difficult to identify by of the important aspects during histochemistry.
routine staining. Such techniques involved
identifying definite chemical groups, so they Fixation in Histochemistry
were called as histochemical techniques.
For identification and localization of chemical
Advances in immunology led to replacement of
these chemicals and dyes with antibodies. This substances tissue must be preserved in a such
way that it causes minimal changes to the
improvement led to increased sensitivity and
reactivity of the cytoplasmic and extracellular
specificity in identifying various tissue
components. macromolecules, for example enzymes, proteins,
carbohydrates, lipids and nucleic acids. This is
Advances in genetics brought about a
accomplished by using optimum osmotic
revolution in field of pathology and micro-
biology. Now because of use of various DNA/ conditions, cold temperatures, controlled pH of
the fixing solutions, and the minimum possible
RNA based techniques. It is now possible to
exposure to the fixative.
identify pathology at gene level. Also very rapid
identification of microorganism is possible. Formaldehyde is a widely used fixative. It is
ideal for fixing proteins and enzymes. It causes
HISTOCHEMICAL TECHNIQUES cross linking of proteins without affecting their
reactivity. But it is a poor preservative of lipids.
Histochemistry is study of the chemical It can be used to fix lipids especially phospho-
composition of tissues by means of specific lipids by adding calcium which prevents
staining reactions. It involves identification of dissolution of phospholipids.
48 Textbook of Oral Pathology

For histochemical techniques neutral antibodies labeled with flouorescent dye,

buffered formaldehyde is used as cold solution. enzyme or radioactive isotope or colloidal gold.
Formal calcium is also used. Other substances Albert H. Coons (1941) was the first to label
used are gluteraldehyde and acrolein. antibodies with a fluorescent dye and use it to
For study of glycogen, glycoproteins, identify antigens in tissue sections. With the
proteoglycans and nucleic acids mixtures of expansion and development of
many chemicals are used as fixatives. For immunohistochemistry technique, enzyme
example, Rossmans fluid is used for labels have been introduced such as peroxidase
visualization of glycogen, glycoproteins, and and alkaline phosphatase. Colloidal gold label
proteoglycans. Cornoy’s solution is used as has also been discovered and used to identify
immunohistochemical reactions at both light and
fixative for nucleic acids.
electron microscopy level. Other labels include
Certain tissue groups are highly labile and
radioactive elements, and the immunoreaction
sensitive. Such chemicals cannot be fixed by use
can be visualized by autoradiography. Since
of fixative. They are visualized from fresh frozen
immunohistochemistry involves specific
sections, where tissue is rapidly frozen by used
antigen-antibody reaction, it has apparent
of liquid nitrogen immediately after removal advantage over traditionally used special-
from the body. Other techniques are freeze enzyme staining techniques that identify only a
drying and freeze substitution. limited number of proteins, enzymes and tissue
Various techniques involved in identifying structures. Therefore, immunohistochemistry
different chemicals and structures is given in the has become a crucial technique and widely used
chapter 3 “Stains and staining methods”. in many medical research laboratories as well
as clinical diagnostics.
Enzyme Histochemistry
It involves identifying and locating enzymes Principle
within the cell. The technique involves series of
chemicals to get insoluble colored reaction The basic principle of this technique is isolation
product. By this technique various enzymes have of tissue antigen that is needed to be localized in
tissue. Using this pure protein, antibodies are
been identified (Table 5.1).
formed, such antibodies are labeled with
Table 5.1: Techniques in enzyme histochemistry enzymes. These labeled antibodies are used to
Enzymes Technique cleave a suitable substrate that produces a
Alkaline phosphatase Gomori calcium method, Azo dye insoluble colored precipitate in the tissues.
coupling method, Naphthol AS-BI Animals such as mouse, guinea pig, sheep
method. or rabbit are used to develop antibodies. Such
Acid phosphatase Gomori lead method, Azo dye
coupling method, Naphthol AS-BI antibodies contain different types of antibodies
method derived from different clones of plasma cell. Such
ATP Metal precipitation method antibodies are called polyclonal antibodies. They
Esterase A naphthyl acetate method,
Indoxyl acetate method.
are highly sensitive but specificity is low. To
Cytochrome oxidase Seligman’s technique increase specificity monoclonal antibodies are
β-glucuronidase Naphthol AS-B1 method used. They are manufactured by a special process
G6PD Lead method
known as hybridoma technique. Here plasma
cells producing specific antibody are fused with
Immunohistochemical Methods malignant cells obtained from myeloma, a
Immunohistochemistry is the localization of neoplasm of B-lymphocytes. Monoclonal
antigens in tissue sections by the use of antibodies are highly specific.
 Advanced Diagnostic Techniques 49

Various enzymes are used for labeling of the • Immune complex method
antibodies. More commonly used are alkaline • Avidine biotine method
phosphatase and horse radish peroxidase. These • Streptavidine biotine method
enzymes produce color when substrate is • Avidin-biotine-peroxidase complex method
applied. Different enzymes use different • LSAB method
substrates. Alkaline phosphatase enzyme uses • Polymeric methods
Fast Red and new fuchsin. On the other hand • CSA method.
horse radish peroxidase uses diamino benzidine
and AEC as substrate.
Applications of Immunohistochemistry
There are numerous immunohistochemistry
Diagnostically Challenging Oral Malignant
methods that may be used to localize antigens
Neoplasms
(Fig. 5.1).
• Direct method Immunohistochemistry has been shown to be an
• Indirect method. effective adjunct to H and E diagnosis in a
Direct method: It is also called as single step majority of equivocal tumor cases, through the
method. Here labeled antibody is directly establishment of a definitive diagnosis or
applied to tissues. Later addition of substrate through confirmation of H and E section
causes formation of colored precipitate. impression. It is used to establish origin of a tumor.
For this various markers are used (Table 5.2).
Indirect method: This is two step method where
two antibodies are applied. First, primary
Table 5.2: Various markers used in oral pathology
antibody is applied which reacts with protein of
interest. This is followed by a Second antibody Tissue Markers
which is “antibody of primary antibody”. This Epithelium Keratins
secondary antibody is labeled with enzyme, General mesenchymal Vimentin, desmin, GFAP
marker
which on application of substrate produces
Muscle markers Desmin, actins, myoglobin,
colored precipitate. myogenin.
There are numerous other methods available Neural markers S-100, GFAP, neuro-
for immunohistochemistry which vary in filaments, and CD57
specificity and sensitivity! Endothelial markers CD31, CD34, and factor
• Labeled antibody method VIII–related antigen
• Enzyme bridge method Melanocytic markers HMB45, MART-1 (Melan-
A), and S-100 protein
• PAP method
Lymphoid markers κ and λ, CD3, CD15, CD20,
• APAAP method CD30, CD45, CD68, CD79a,
ALK-1, and TdT.
Neuroendocrine Synaptophysin and
markers chromogranin
Ewing’s tumor marker CD99
Metastatic tumor CK7, CK20, villin
markers
Salivary gland tumor S-100 protein and actins
markers
Odontogenic tumor Shethilin, enamelin,
markers ameloblastin
Figure 5.1: Direct and indirect immunohistochemistry label
50 Textbook of Oral Pathology

Tumor Markers of Patient Course and Outcome

Certain proteins and enzymes are indicators of
prognosis and response to therapy. Such
molecules are used to determine the course and
outcome of the therapy.

Other Applications

Immunohistochemistry is used to identify

Figure 5.2: Direct immunofluorescence method
bacteria and viruses in tumors and other lesions.
It is used to identify human papilloma virus in erythematosus, pemphigus vulgaris, and
ameloblastoma. Similarly, it has been used to mucous membrane pemphigoid. Anti-human
identify various genes that contribute to IgG, IgA, IgM, complement component (C3), and
tumorigenesis, growth and spread of tumors. fibrinogen are used to diagnose these lesions.
These are used on fresh frozen sections or tissues
Immunofluorescent Techniques fixed with Michael’s solution. Based on location
Immunofluorescent techniques are similar to and appearance of immunofluorescence these
immunohistochemistry. Here instead of lesions are diagnosed (Table 5.3).
enzymes antibodies are labeled with a Pemphigus vulgaris: It shows interepithelial
fluorescent dye-fluorescence. There are two distribution of anti-IgG immunofluorescence
techniques in immunofluorescence. (some C3 deposition is also noted). It takes a net
1. Direct technique. like distribution pattern.
2. Indirect technique.
Mucous membrane pemphigoid: It shows a linear
Direct technique: In this technique antigens are pattern of immunofluorescence at the basement
detected in the tissues of patients. Here labeled membrane. All three antibodies, i.e. anti-IgG,
antibodies are directly applied to the tissues. If anti-IgA, and anti-C3 antibodies are deposited
antigen of interest is present in the tissues it will at the basement membrane.
show fluorescence when viewed under Lichen planus: LP shows a characteristic pattern
fluorescent microscope. of fibrinogen deposition outlining the basement
Indirect immunofluorescence technique: This is zone and extending irregularly into the
method of detecting antibodies in plasma. In this superficial lamina propria, described as a
technique plasma containing primary antibodies “shaggy” or “fibrillar” pattern. Another finding
is applied to suitable substrate. Over this typical of but less frequently observed in lichen
secondary antibody labeled with fluorescene is planus is the presence of IgM-, IgA-, IgG-, or C3-
applied and slide is observed under fluorescent positive “cytoid,” “colloid,” or “apoptotic”
microscope for fluorescence. bodies, located in the epithelium and superficial
Applications: Direct immunofluorescence has connective tissue.
more application in dentistry than indirect Lupus erythematosus: DIF reactions in specimens
technique. Direct technique has application in of oral mucosal LE exhibit coarse granular
diagnosis of various vesiculobullous lesions. It deposits of C3, IgM, IgA, and IgG in the
is used in diagnosis of lichen planus, lupus basement zone.
 Advanced Diagnostic Techniques 51

Figure 5.3: Indirect immunofluorescence method

Table 5.3: Immunofluorescence appearance of various

vesiculobullous lesions
Lesion Serum Appearance Figure 5.4 : Parts of a flow cytometer
component
stream passes through the light beam; one in line
Pemphigus IgG Net like ditribution
vulgaris with the light beam and several perpendicular
Mucous membrane IgG, IgA, C3 Linear distribution to it. Each cell passing through the beam scatters
pemphigoid along basement
the light in some way. This scattered light is
membrane
Lichen planus IgG, IgA, C3 Shaggy or fibrillar picked up by the detectors, and analysed by
pattern using computers. By detecting variations in the
Lupus C3, IgM, IgA, Coarse granular
erythematosus and IgG deposition
brightness at each detectors it is then possible to
extrapolate various types of information about
the physical and chemical structure of each
Flow Cytometry
individual cell. To aid in detection cells are
Flow cytometry is the process in which usually stained immunocytochemically or by
measurements are made while cells in a liquid immunofluorescent stains.
suspension are forced to flow one at a time The main limitation of flow cytometry is need
through a measuring device. It is a technique for for single cell suspension. This is not a problem
counting, examining and sorting cells suspended for samples such as blood and other body fluids.
in a stream of fluid. It allows simultaneous It is difficult to employ this technique for solid
analysis of the physical and/or chemical tumors. But now various techniques have been
characteristics of single cells flowing through an developed to overcome this difficulty. Tissue
optical and/or electronic detection apparatus. from paraffin embedded tissue can also be used
A beam of laser light is directed onto a stream for flowcytometry. The section is dewaxed
of fluid containing suspension of cells. A number hydrated by passing through xylene and graded
of detectors are aimed at the point where the alcohols. Then tissue is minced with sharp
52 Textbook of Oral Pathology

scissors in a watch glass. Following this tissue is act as template for next cylce. By the end of next
digested using 0.5% pepsin and a liquid cycle 2 × 2 DNA are formed. In next step 4 × 2
suspension is prepared. This releases the DNA DNA chains are formed. This process continuous
which is stained using appropriate agent and exponentially.
then fed into the flow cytometer. This whole step is repeated number of times
Flow cytometer has a wide application in to amplify the DNA. The reaction is terminated
pathology. (i) It is used to support a diagnosis of after sufficient number of DNA fragments are
malignancy when the morphological changes are formed. The DNA amplified is separated by
equivocal; (ii) It can be used to classify tumors electrophoresis and analysed.
of borderline malignancy; (iii) It provides There are various modifications of this
prognostic information independent of stage and technique:
grade of the tumor; (iv) It helps to identify tumor 1. Hot-start PCR is a technique that reduces
relapse; (v) It helps to detect tissue of region of nonspecific amplification during the initial
a tumor. set up stages of the PCR.
2. Multiplex-PCR—The use of multiple,
Polymerase Chain Reaction (PCR) unique primer sets within a single PCR re-
action.
PCR is the enzymatic amplification of a specific
DNA sequence in vitro to obtain large number 3. Nested PCR—Nested PCR increases the
specificity of DNA amplification, by reduc-
of DNA copies. This techniques amplifies DNA
ing background due to nonspecific ampli-
without involving any live organism, so it can
be repeated any number of times. As it is a in fication of DNA.
4. Quantitative PCR is used to measure the
vitro procedure, it can be subjected to different
quantity of a PCR product.
modifications.
PCR is used to amplify specific regions of a 5. RT-PCR (Reverse Transcription PCR) is a
method used to amplify, isolate or identify
DNA strand. Following steps are followed in
PCR:
Denaturation: The DNA which needs to be
amplified is isolated. It is added to reaction
mixture which contains nucleotides, primer,
which closely resembles gene of interest, and
polymerase enzyme in a micropipette and
heated to high temperature of 90-95° C. At this
temperature two strands of DNA separate.
Annealing: Temperature is lowered to 50-60°C.
At this temperature primer attaches to gene of
interest.
Amplication: Once primary attaches to the DNA
polymerase starts synthesizing the DNA from
nucleotides present in the reaction mixture. In
this way two DNA chains are formed at the end
of one cycle. In next step these two DNA chains Figure 5.5: Steps involved in PCR
 Advanced Diagnostic Techniques 53

a known sequence from RNA. In this tech- Agar gel. Once these fragments are separated,
nique DNA is synthesized first from RNA they are blotted on to a paper by placing the
using the enzyme reverse transcriptase. paper on agar gel. Once the DNA fragments are
This DNA acts as an template for further separated and blotted, probe is applied. After
amplification. washing it may be observed under UV light. If it
shows positive reaction, it means that it contains
Application of PCR in Dentistry DNA under investigation.
Microbial identification: The use of PCR has Northern Blotting: It is similar to Southern
revolutionized the diagnosis and study of blotting. The only difference is in this technique
infectious diseases and malignancies associated instead of DNA, RNA is detected. The procedure
with microorganisms. PCR is used to identify is similar to Southern blotting.
bacteria and viruses in infections. It has been
Western Blotting: Western blotting is not a DNA/
used to study microorganisms causing
periodontal, endodontic infection and to identify RNA blotting method. It is a method used to
separated proteins. It is also similar to southern
microorganisms of dental caries.
blotting.
Human genetics: PCR plays an important role in
In situ hybridization: It is a method of identifying
the identification of chromosomal disorders and
DNA and RNA in tissue samples. It is similar to
hereditary diseases, including cystic fibrosis,
Gaucher’s disease, alpha-1-antitrypsin Southern blotting method. Here a probe
containing complementary copy of DNA/RNA
deficiency, hemophilia, and sickle cell anemia.
under investigation is prepared. The probe is
PCR can also be used to analyze fetal DNA for
aneuploidy the presence of extra chromosomes either labeled with radioactive isotope or
fluorescent dyes. In latter case it is called as
or the absence of chromosomes, trisomy 21,
fluorescent in situ hybridization (FISH). The
Turner’s syndrome, Klinefelter’s syndrome, and
for sex determination. labeled probe is placed on tissue and allowed to
hybridis. This is detected by observation under
Forensic odontology: PCR is used for identification fluorescent microscope or by exposing X-ray film
of mutilated or decomposed human tissues, for sex in case of radioactive labeled probes.
determination, and for disputed paternity cases. In situ hybridization has wide applications
Tumor biology: PCR is used to identify various in dentistry. It is used to detect various viruses
cancer associated genes. in the tissues. Similiarly, it is used to identify
various genes involved in tumor biology.
Hybridization Methods
Hybridization refers to pairing of complimentary
copies of DNA. As pairing between two DNA
fragments is very specific, one copy can be used
to detect other copy. This detector copy is known
as a probe. It is labeled with either fluorescent
material or radioactive isotopes.
Southern blot: This is a detection method for DNA.
The DNA under question is digested using
enzymes to produce small fragments. These
fragments are separated by electrophoresis on a Figure 5.6: In situ hybridization
54 Textbook of Oral Pathology

Laser Capture Microdissection

Tissue obtained by biopsy contains mixture of
different cell populations. In such case it becomes
difficult to obtain homogenous population of
cells for further study. In a new technique it is
possible to isolate and obtain a homogenous
population of cells using infrared laser light. In
this technique a plastic sheet is placed on the
slide. The area of interest is identified and it is
radiated with laser light. This caused plastic film
in that area to melt and adhere to tissue. The
adherent tissue can be lifted and further
processed. Thus, in this technique it is possible
to obtain specific area of tissue and homogenous
population of cell without distorting the
structure and contents. This method is fast,
precise, and adaptable to a wide range of tissues
and molecules to be studied. Both the tissue left
behind and the tissue retrieved can be identified, Figure 5.7: Laser capture microdissection
and the morphology of both is excellent. Large
numbers of well-characterized cells can be
obtained within a few minutes (Fig. 5.7). tumor mRNA by the process of reverse
The applications of microdissection in transcription. These probes are attached or
pathology increasing. It is used to obtaining pure “printed” on a solid phase or a “chip”. The tumor
cell populations from fresh, frozen, or fixed to be tested is applied on this chip.
tissues and cytology samples for DNA molecular Complementary DNA hybridize with the
genetic analysis; gene expression studies corresponding probes. This hybridization results
involving mRNA such as RT-PCR. in varying green/red and yellow fluorescent
emisssions. These emissions are then scanned by
Proteomics a argon laser reader and a scanning confocal
It is also called as microarray technology. Here it microscope. This is analyzed by computer for
is possible to simultaneously analyze tens of quantification of intensity of thousands of
thousands of genes in single step. The main different genes on the array. This is compared
applications of this technology is study of gene with normal tissues.
expression analysis, genotyping for detection of Thus, in recent times diagnostic pathology
point mutations, single nucleotide pleomorphism, has witnessed a range new technologies to help
etc. Conceptually, DNA microarray technology in diagnosis, prognosis, and treatment of
is similar to the underlying principles of Northern diseases. Some of these methods are based on
and Southern blotting. Here first step is antigen and antibody reactions, some are based
preparation of probes. Probes are labeled genomic on DNA hybridization, and some are based on
DNA, cDNA and oligonucleotides produced from laser technology.
 CHAPTER
Embryology and Anatomy
6 of Oral Structures

EMBRYOLOGY OF THE OROFACIAL REGION Second Week

First Week (Fig. 6.1) • The embryo at this stage is enclosed by the
• Morula: The fertilized eggs initially undergoes extraembryonic coelomic cavity which is
a series of rapid divisions that lead to the lined by extracoelomic membrane.
formation of a ball of cells called the morula. • Bilaminar disc: After eighth day of gestation,
• Blastocyst: The morula is transformed into a the cells of the embryoblast differentiate into
fluid filled cystic cavity with realignment of a two layered disc called the bilaminar disc.
the cells leading to the formation of blastocyst. • Amniotic cavity: The dorsal aspect cells of the
• Two cell populations formed in the bilaminar disc are columnar and they are
blastocyst, are a cluster of cells or inner mass called as ectodermal cells. These ectodermal
of cells called ‘Embryoblast’ and cells lining cells enclose a cavity called as amniotic cavity
the cavity are called the ‘trophoblast’. (Figs 6.2 to 6.4).
• The embryoblast cells form the embryo • Ventral aspect cells: These cells are cuboidal
proper whereas the placenta formation and and form the roof of the second cavity called
implantation of embryo is done by the as secondary yolk sac. The ventral aspect cells
trophoblast. develop from migration of peripheral cells

Figure 6.1: Formation of blastocyst (Embryoblast –red, trophoblast – black)

56 Textbook of Oral Pathology

of the extra embryonic layer. So this layer of

cells is called as endodermal layer.
• After formation of endoderm and ectodermal
layer the ‘axis of the embryo’ is determined.
This is represented as enlargement of the
ectodermal and endodermal cells at the head or
‘rostral end’ of the embryo in a region called
prochordal plate.

Third Week
During this week the bilaminar embryonic disc
is transformed into trilaminar disc. The
ectodermal layer forming the floor of the
embryonic cavity becomes well differentiated
Figure 6.2: 12 day blastocyst showing A-amniotic cavity, and columnar.
SYS-secondary yolk sac, PYS-primary yolk sac, amniotic • Primitive streak: It is a development of a
cavity is lined by epiblast which further form ectoderm, SYS
is lined by hypoblast, epiblast and hypoblast form the narrow groove along the midline of the
bilaminar disc ectoderm which has slight bulging areas on
either side.
• Primitive node: The head end (rostral end) of
the primitive streak end in a small depression
called the primitive node or pit.
• Notochord: The primitive embryo up to this
stage is a delicate ball of cells. It is at this stage
that the ectodermal cells of the primitive node
divide and migrate in between the endoderm
and ectoderm to form a solid column that
pushes forward in the midline up to the
Figure 6.3 prochordal plate. This solid column or cord
of cells is canalized to form the notochord.
• The notochord is the prime support for the
primitive embryo.

Central Portion of the Primitive Streak (Fig. 6.5)

Along the central portion of the primitive streak
the surface ectodermal layer divides and
migrates toward the streak or groove and
invaginate it to occupy space between ectoderm
and endoderm.
Figure 6.4 These cells between ectoderm and endoderm
Figures 6.3 and 6.4: Bilaminar disc embryo; Amniotic cavity
or cyst (pink) is lined by ectodermal cells after differentiation,
are called as mesoblasts. The mesoblasts infiltrate
secondary yolk sac (blue) is further lined by endoderm further to push away the extra embryonic
 Embryology and Anatomy of Oral Structures 57

Figure 6.6: The three germ layer differentiation; Notochord is

separated from surface ectoderm. Neural crest cells migrate
alongside the surface ectoderm between the mesoderm
(green), adjacent to the nucleus

organs differentiate which include the head and

face and development of teeth.
Figures 6.5: Initial formation of buccopharyngeal membrane Other events which occur in fourth week are:
(BM) at the cranial end of the embryo (Third week). Arrows • Differentiation of nervous system
indicate the direction of migration of ectodermal cells • Neural crest tissues form ectoderm
• Differentiation of mesoderm
endodermal cells which are placed laterally. The
• Folding of the embryo into two planes along
migration progress laterally as well as towards
the central axis i.e. the head-tail and lateral axis.
the prochordal plate. These mesoblasts cells
accumulate anterior to the prochordal plate and Neural Plate and Neural Folds
give rise to cardiac plate (a structure in which
heart forms). The ectodermal thickenings that form raised
The mesoderm which has invaginated and margins form the neural folds. The thickening of
formed a layer of cells in the central position of the ectodermal layer at the head end of the embryo
the primitive streak forms the ‘true embryonic forms the neural plate. The two neural folds are
mesoderm’. Further migration of the mesoblasts marked by a depression called as neural groove.
between the embryonic endoderm form the third Neural tube: This is formed by fusion of the neural
layer of cells called mesoderm. folds in the center. A unique population of cells
The endoderm and ectoderm are now develops from the ectoderm along the lateral
completely separated by notochord and margins of the neural plate. These are the neural
mesoderm (Fig. 6.6). crest cells. They undergo extensive migration and
At the same time, caudal end or the tail end is give rise to a variety of different cells and form
formed by area of fusion of cells marked by components of many tissues.
formation of coecal plate. Neural crest cells migrate in the trunk and
also towards the cephalic end. While migrating
Fourth Week towards the head end it follows a route beneath
During first 3 weeks of development the the surface ectoderm as a sheath of cells.
formation of 3 layered triploblastic embryo is Mesenchyme: They form the mesenchyme in the
complete. In the 4th week the major tissues and upper facial region. The mesenchyme is defined
58 Textbook of Oral Pathology

as loosely organized embryonic tissue in contrast

to epithelia which are completely arranged. The
neural crest cells in the facial region surround
mesodermal cores already present in the visceral
arches.
Somites formation: Mesodermal thickening
adjacent to the neural tube form the somites.
Each somite has three components namely:
• Myotome: It gives origin to segmented mass
of muscles.
• Sclerotome: It forms vertebrae and their discs. Figure 6.7: Buccopharyngeal membrane is formed by
• Dermatome: It gives rise to connective tissue ectoderm of the stomodeum and endoderm of the foregut
segment of the skin.
Somatomeres: There are a series of somites found
in the head region which form the head
musculature.
Intermediate and lateral plate mesoderm: These give
rise to urogenital system, connective tissue
associated with muscles and viscera, blood and
lymphatic cells, pericardium, peritoneum,
membranes of the pleura (3 p’s), adrenal cortex
and spleen. Further the neural tube under goes
massive expansion to form forebrain, midbrain Figure 6.8: Pharyngeal arches, Er- developing ear, iP-inferior
and hindbrain. parathyroid, th-thymus, sP- superior parathyroid, manP-
mandibular process, maxP-maxillary process
Folding of the embryo: The most crucial event is
the folding of the embryo in two planes. The
head fold forms the primitive stomatodium or
the oral cavity.
At about 27th day of gestation the
buccopharyngeal membrane ruptures and the
connection between the primitive oral cavity and
the foregut is achieved (Figs 6.7 to 6.10).

Development of Frontonasal Process

The migration and repair persisting high rate of
cell proliferation leads to formation of fronto-
nasal process in the upper and midface. The
frontonasal process shows further development
Figure 6.9: Cartilages of the corresponding arches, Meckels
into nasal pits, nasal placodes, lateral nasal cartilage and maxillary process are replaced by the mandible
prominence, medial nasal prominence. ad maxilla, they develop by membranous ossification
 Embryology and Anatomy of Oral Structures 59

and give rise to labial component, upper jaw

component and primary palate.

Secondary Palate
Secondary palate is derived from two shelves
like outgrowths called as palatine shelves form
the maxillary processes. Certain areas of basal
cells of oral ectoderm lining the oral cavity
proliferate more rapidly than the adjacent cells.
This leads to the formation of dental lamina
which is a band of epithelium that has involved
in the underlying ectomesenchyme along each
of the horseshoe shaped future dental arch.
The dental lamina is present along the length
Figure 6.10: The buccopharyngeal membrane ruptures of upper and lower jaws. Subsequently it gives
when embryo is six weeks old
rise to primordium of the teeth. This primordium
These nasal processes grow in a more forward further develops into morphogenic stages of bud,
position and simultaneously are associated by cap, bell and advanced bell stage.
formation of maxillary and mandibular Each stage develops into different layers of
prominences in the midface. cells by initiation, proliferation, histodifferentia-
Maxillary prominence is present lateral to the tion, Morphodifferentiation and apposition of
stomatodium and mandibular prominences are calcified dental tissues for each tooth bud.
caudal to the stomodeum.
Seventh Week
Fifth Week
The palatine shelves attain a horizontal position
During the 5th week the medial nasal prominences,
above tongue and fuse. Nasal septum grows
lateral nasal prominences and maxillary
down and fuses with the palate. This completes
prominences grow medially compressing medial
formation of the palate.
nasal process towards the midline.
The maxillary prominence of each side fuse.
Thus upper lip is formed by 2 medial nasal DEVELOPMENT OF TONGUE
prominences and 2 maxillary processes. Lateral
The tongue appears in the 4th week in the form
nasal process does not participate in upper lip
of two lateral lingual swelling and one medial
formation. Simultaneously the nasolacrimal duct
swelling (tuberculum impar).
and lacrymal sac is formed as ectodermal groove
These three swellings are derived from first
between lateral nasal process and maxillary
pharyngeal arch. A second medial swelling the
process.
copula (hypobranchial eminence) is formed by
Sixth week mesoderm of 2nd, 3rd and part of 4th arch. The
lateral swelling increases in size and merge in
Primary Palate Formation
midline to form the anterior 2/3rd or body of
As a result of medial growth of maxillary the tongue. During this the tuberculum impar
processes the two medial nasal processes merge regresses and is marked by a pit (foramen
60 Textbook of Oral Pathology

caecum). This marks the junction between

anterior 2/3rd ectodermal origin) and posterior
1/3rd of tongue (endodermal origin) (Figs 6.11
and 6.12).
The thyroglossal duct is formed at the site of
foramen caecum as a downward duct formation
in the floor of pharynx.

DEVELOPMENT OF MANDIBLE

In primitive vertebrates Meckel's cartilage forms

the lower jaw but in human beings it has close
positional relationship with mandible but do not
contribute to its formation.
• At the sixth week of development this cartilage
extends as solid hyaline cartilage rod. Figure 6.11: Development of tongue two lingual swellings
• Mandibular nerve has a close relationship (blue) tuberculum impar and foramen cecum (black) cranial
with cartilage; about 2/3rd of its beginning hypobranchial eminence (green), caudal hypobranchial
eminence (red)
part runs along the cartilage.
• During the sixth week on lateral aspect of the
cartilage, condensation of mesenchyme
occurs in the angle formed by the division of
inferior alveolar nerve in its incisal and
mental branches.
• At the seventh week intramembranous
ossification takes place in this condensation.
• This spread of new bone formation occurs
anteriorly along the lateral aspect of Meckel’s
cartilage.
• This occurs in form of a groove which later
gets transformed into a canal as bone forms
over nerve.
• Similarly on backward extention of ossifica-
tion along the later aspect of Meckel’s carti-
lage forms a gutter which get transformed
into canal containing inferior alveolar nerve.
• From this canal (which extends from midline
to divisional point of mandibular nerve,
medial and lateral plates of alveolar process
develop in relation to developing tooth germ.
• Finally bone grows over tooth germ and Figures 6.12: Complete development of tongue in relation
closes it. of pharyngeal arches
 Embryology and Anatomy of Oral Structures 61

Development of Maxillary Sinus Facial growth continues by surface

deposition on the face, alveolar processes and
During 4th week of embryonic life the dorsal
palate, it is accompanied by the resorption of the
portion of the 1st pharyngeal arch form the
internal structure of the maxillary sinus.
maxillary process. It extends forwards beneath the
developing eye and gives rise to the maxilla, the
Congenital Anomalies of the Face
zygomatic bone and part of the temporal bone.
The maxillary sinus starts to develop at about Macrostomia
12 weeks as an evagination from the primitive
Arrest in the medial shift and fusion of the
ethmoid infundibulum in the lateral wall of the maxillary and mandibular process leads to
middle meatus of the corresponding nasal cavity
formation of the condition called as the
when the nasal epithelium invades the maxillary
macrostomia or large mouth.
mesenchyme.
At birth manilla is filled with deciduous tooth Microstomia
germs which are very close to orbital floor. The
superior dental nerves and vessels (branches of An extensive fusion between the maxillary and
orbital nerve of vessels), thus have close the mandibular process arch makes the oral
appronimation with the maxillary teeth. The cavity too small.
alveolar and orbital aspects of the maxilla
gradually become separated by the cancellous Cleft Lip
bone which is then resorbed as the sinus enlarges. It may be unilateral or bilateral. It is caused by
Pneumatization of the maxilla commences failure of fusion between globular swellings of
just below the orbital floor. Downgrowth of the the median nasal process and maxillary process.
maxillary sinus leaves the ostium in a position
unfavorable for gravitational drainage.
Cleft Palate
The maxillary sinus expands not only
downward but also forward and backward from It may be complete or incomplete. The complete
its initial evagination, the site which persists as cleft palate may be unilateral or bilateral. When
the antronasal duct. It undergoes concurrent unilateral, the palatine process fuses with the
lateral expansion and by the end of the first year primitive palate on one side only whereas there
extends beneath the orbit as far as the infraorbital is an arrest in the fusion on the opposite side.
canal. By the end of 2nd year the sinus reaches When bilateral, the primitive palate and nasal
above half its adult size. septum fusion is failed on both the sides.

Table 6.1: Derivatives of pharyngeal arch

Pharyngeal arch Nerve Muscles Skeleton
I Mandibular Trigeminal Masticatory muscles, Meckel’s cartilage, incus, malleus,
anterior digastric, tensor maxilla and premaxilla,
palati and tympani sphenomandibular ligament
II Hyoid Facial Muscles of facial expression, Stapes, styloid process,
post. digastric, stylohyoid, stapedius stylohyoid ligament.
III Glossopharyngeal Stylopharyngeus Greater and lesser horn of hyoid
IV Vagus Cricothyroid, laryngeal muscles Laryngeal cartilages
62 Textbook of Oral Pathology

First Arch Syndrome

Congenital anomalies of the eyes, ears, mandible
and palate occurs due to insufficient migration
of the neural crest cells into the first arch.

Treacher-Collins Syndrome
This is mandibulo-facial-dysostosis occurring
due to incomplete formation of zygomatic bone,
lower eyelids, and deformed ears. The defects
are caused by the abnormalities of the first and
second branchial arch which is due to hereditary
pattern.
Figures 6.13: Maxilla showing frontal process (red arrow),
alveolar process (green arrow) and zygomatic process (brown
Anatomy of Face arrow)

Bones
The skull consists of the 22 bones out of which 8
are paired and 6 are unpaired. Paired bones are
parietal, temporal, maxilla, zygomatic, nasal,
lacrimal, palatine and inferior nasal concha.
Unpaired bones are frontal, occipital, sphenoid,
ethmoid, mandible and vomer. From dental
point of view maxilla and mandible are most
important and they are described below.

Maxilla
The maxilla consists of a central body, which is
hollowed out forming the maxillary sinus and
four processes.
Frontal process—it ascends from the
anteromedial corner of the body, serves as the
connection with the frontal bone (Fig. 6.13).
Zygomatic process—it forms in the lateral
corner of the body, connects with the zygomatic
bone.
Palatine process—it is horizontal and arises Figure 6.14: Maxillary showing incisive foramina,
pterygoid plate zygomatic arch
from the lower edge of the medial surface of the
body. with its base facing the nasal cavity. It lies in an
Alveolar process—it extends downwards and almost horizontal axis with its apex being
carries the socket for the maxillary teeth (Fig. 6.14). elongated into the zygomatic process. The three
The body of maxilla is a three sided pyramid sides are superior or orbital (it forms greater part
 Embryology and Anatomy of Oral Structures 63

of the orbital floor), an anterolateral or malar Mandible

(surface forming part of the skeleton of the cheek It is the largest and strongest bone of the face. It
and face) and posterolateral or infra-temporal consists of a horseshoe shaped body continuous
(surface turned towards the infra-temporal fossa) upward and backward on either side with the
(Fig. 6.15). mandibular rami. The body is thick, has a
The base is rimmed on its inferior edge by rounded lower border and carries the alveolar
the alveolar process housing the teeth row. process on its upper border. It extends backward
Alveolar process consist of two roughly parallel from the chin at the midline symphysis to the
plates of bone that unite behind the last tooth to anterior limit of the ramus (Fig. 6.16).
form a small rough prominence, the alveolar
tubercle, which often contains a single large Ramus: It is a thick quadrilateral plate which
marrow process. The lateral and external extends backward from the groove for the facial
alveolar plate continues upward into the artery (antegonial notch) to include the region
anterolateral and posterolateral surface of the called the mandibular angle.
maxillary body. Mental protuberance: In the midline of anterior
The internal alveolar plate continues into the surface of the body projects a triangular
palatine process and behind the posterior end prominence called mental protuberance.
of the latter into the nasal surface of the maxillary
body. The deep furrow between the two alveolar Symphysis menti: It is the line at which the right
plates is divided by radial bony plates into the and left halves of the bone meet each other.
sockets of the individual teeth. The mental foramen, through which the mental
At the boundary between two portions of the nerve and blood vessels pass, is located on the
nasal crest a canal commences in the nasal floor lateral surface of body between the roots of the
close to the midline and extends downwards, 1st and 2nd premolars. In the vertical dimension
anteriorly and medially to unite with the canal the foramen lies halfway between the lower
of the other side in a common opening which is border of mandible and the alveolar margin.
called as incisive or nasopalatine canal. On the
anterior surface of maxilla there is canine fossa
situated lateral to the canine eminence.

Figure 6.15: Front view of maxilla Figure 6.16: Overview of mandible

64 Textbook of Oral Pathology

The anterior border of the ramus continues by the inter-radicular septa, thus forming the
along the body lateral to the alveolar process as socket for the teeth much in the same manner as
a blunt ridge, the oblique line, running in the upper jaw.
downward and forward to disappear at about The lower border of the mandible is also
the level of the 1st molar (Fig. 6.17). called as base. Near the midline, the base shows
an oval depression called the digastric fossa.
Genial tubercle: Slightly above the lower border
on its inner surface the mandibular symphysis Muscles of Mastication
is elevated in more or less sharply defined
The muscles of mastication move the mandible
projection called as genial tubercle.
during mastication and speech. They are the
Mylohyoid line: It is a prominent ridge that runs masseter, the temporalis, the lateral pterygoid
obliquely downward and forward from below 3rd and the medial pterygoid. They develop from
molar tooth to the medial area below the genial the mesoderm of the 1st branchial arch.
tubercle. Below the mylohyoid line the surface is
slightly hollowed out to form the submandibular Masseter Muscle
fossa, which lodges the submandibular gland. It is the most superficial of the masticatory
The mandibular canal which houses the muscle, stretches as a rectangular plate from the
inferior alveolar nerve and blood vessels begins zygomatic arch to the outer surface of the
at the mandibular foramen, curves downward mandible. It has three layers, i.e. superficial,
and forward and turns into a horizontal course middle and deep.
below the roots of the molars.
Superficial layer: It arises by thick aponeuroses
In the region of the premolars the mandibular
from the zygomatic process of the maxilla and
canal splits into two canals of unequal width; the
from the anterior two-third of the lower border
narrower incisive canal continues the course the
of zygomatic arch. Its fibers pass downward and
mandibular canal toward the midline and the
backward to be inserted into the angle and lower
wider branch, the mental, turns laterally, superiorly
half of lateral surface of ramus of mandible.
and posteriorly to open at the mental foramen.
The alveolar plates consist of two compact Middle layer: It arises from the deep surface of
bony plates, the external and internal alveolar the anterior two-third of the zygomatic arch and
plate. These two plates are joined to each other posterior one-third of lower border of zygomatic
by the radial interdental and in the molar region, arch and is inserted into middle of ramus of
mandible.
Deep layer: It arises from deep surface of the
zygomatic arch and is inserted into upper part
of the ramus of the mandible and into the
coronoid process.
Nerve supply: It is supplied by masseteric nerve
which is a branch of anterior division of the
mandibular nerve.
Blood supply: The masseteric artery which is a
branch of internal maxillary artery and the
Figure 6.17: Side view of mandible masseteric vein follow the course of the nerve.
 Embryology and Anatomy of Oral Structures 65

The Temporalis Muscle tuberosity of the maxilla and adjoining bone. Its
deep head originates from the medial surface of
Origin and insertion: It is fan shaped and arise
medial pterygoid plate and the lateral surfaces
from whole of the temporal fossa and from the
of pyramidal process of palatine bone. Its fibers
deep surface of temporal fascia. Its fibers
converge and descend into tendon which passes pass downward, laterally and backward and are
through the gap between the zygomatic arch and attached by strong tendinous lamina to the
posterior inferior part of the medial surfaces of
the side of the skull to be attached to the medial
the ramus and the angle of mandible as high as
surface, apex, anterior and posterior borders of
mandibular foramen and as forward as
the coronoid process and the anterior border of
mylohyoid groove.
the ramus of mandible nearly as far as the last
molar teeth. Nerve supply: It is supplied by branch of the
Nerve supply: It is supplied by the two deep mandibular nerve.
temporal branches of anterior trunk of the Blood supply: By the branch of maxillary artery.
mandibular nerve.
Blood supply: It is supplied by middle and deep Oral Cavity
temporal arteries. The middle temporal artery The oral cavity is incompletely bounded by
is a branch of the superficial temporal artery. The bones. Its lateral and anterior walls are formed
deep temporal artery is a branch of internal by the inner surface of the alveolar processes,
maxillary artery. which join at the midline. The lingual surface of
the teeth completes these walls.
Lateral Pterygoid
Origin and insertion: It is a short thick muscle with Oral cavity is divided into:
two heads. Upper arises from the infratemporal • Vestibule
surface and infratemporal crest of the greater – It is outer smaller portions.
wing of sphenoid bone and lower from the – Vestibule of the mouth is a narrow space
lateral surface of lateral pterygoid plate. bounded externally by lips and cheeks and
Its fibers pass backward and laterally to be internally by teeth and gums.
inserted into the pterygoid fovea on the anterior • Oral cavity proper
surface of the neck of the mandible and into the – It is inner larger part.
articular capsule and disc of temporomandibular – It is bounded anterolaterally by the teeth,
joint. the gums and the alveolar arches of the
jaws.
Nerve supply: It is supplied by a branch of the
– The roof is formed by the hard and soft
anterior trunk of mandibular nerve.
palate.
Blood supply: Branch of maxillary artery. – The floor is occupied by the tongue
posteriorly and presents sublingual region
Medial Pterygoid anteriorly, below the tip of tongue.
Origin and insertion: It is a thick quadrilateral – Posteriorly the cavity communicates with
muscle attached to the medial surface of lateral the pharynx through the oropharyngeal
pterygoid plate and the grooved surface of the isthmus which is bounded superiorly by
pyramidal process of the palatine bone above. It the soft palate, inferiorly by the tongue and
has a superficial head which originates from the on each side by the palatoglossal arches.
66 Textbook of Oral Pathology

Vestibule teeth are replaced only once (diphyodont) in

contrast with non-mammalian vertebrates where
Lips
teeth are constantly replaced throughout life
It is described in the chapter of disease of lip. (polyphyodont). Each tooth has three parts, i.e.
crown (projection above the gums), root
Cheeks
(embedded in the jaw beneath the gum) and neck
Cheeks are the fleshy flaps, forming a large part (between the crown and root and surrounded
of the sides of the face. It is formed in its mobile by the gums).
parts by the buccinator muscle, covered on inside
by the mucous membrane, on the outside by the Nerve Supply
skin.
In the posterior part the masseter muscle and • Anterior superior alveolar nerve — It supplies
the parotid gland are interposed between the the upper incisor and canine teeth.
mucous membrane and buccinator muscle on • Middle superior alveolar nerve — It supplies
one side and the skin on the other. In the oral upper premolar teeth.
cavity the cheek is bounded above and below • Posterior superior alveolar nerve — It supplies
by the reflection of its mucous membrane onto molar teeth.
the alveolar process. • Inferior alveolar nerve — It supplies the lower
The mucous membrane of the cheeks is fixed teeth.
to the inner fascia of the buccinator muscle by tight
strands of connective tissue. They are continuous Blood Supply
in front with the lips and the junction is indicated • Posterior superior artery — It supplies the
by the nasolabial sulcus which extends from the molar and premolar maxillary teeth.
side of nose to the angle of the mouth. • Anterior superior alveolar artery — It is branch
The cheek contains a peculiar body of fat of infraorbital artery and supplies incisor and
tissue called as buccal fat pad of Bichet. It is a
canine maxillary teeth.
rounded biconvex structure limited by a thin but
• Inferior alveolar artery — It enters the
distinctive capsule.
mandibular canal and gives branches to the
Blood supply: It is supplied by the branches of mandible and to the roots of each teeth
the maxillary artery. attached to the bone.
Lymphatic drainage: Drain into the submandibular
Hard Palate
and pre-auricular lymph nodes and partly to the
buccal and mandibular nodes. It is a partition between the nasal and oral
cavities. Anterolateral margins are limited by
Oral Cavity Proper alveolar arches and gingivae. Posterior margin
Gingiva is continuous with the soft palate. Superior
surfaces forms the floor of the nose and inferior
It is described in the chapter of gingival and
surfaces form the roof of the oral cavity.
periodontal diseases.
Blood supply: Greater palatine branch of the
Teeth maxillary artery and nasopalatine artery.
The teeth form a part of the masticatory Venous drainage: Palatine vessels drain to the
apparatus and are fixed to the jaws. In man the pterygoid plexus of veins.
 Embryology and Anatomy of Oral Structures 67

Lymphatic drainage: Drains mostly to the upper palati is supplied by mandibular nerve. General
cervical and partly to the retropharyngeal groups sensory nerves are derived from lesser palatine
of nodes. nerve.
Nerve supply: Greater palatine nerves from the
greater palatine foramen and nasopalatine nerve Tongue
from the incisive foramen. It is described in the chapter of disease of tongue.

Soft Palate Floor of Mouth

It is a movable fold suspended from the posterior It is a crescent shaped area between the lower
border of the hard palate. It separates the gingiva and undersurface of the tongue which
nasopharynx from the oropharynx. composes the inferior most portion of the oral
It has two surfaces, i.e. anterior and posterior cavity overlying the mylohyoid and thyroglossal
and two borders, i.e. superior and inferior. muscles.
Anterior surface—It is concave and is marked by Arterial supply—It is supplied by facial artery.
median raphe.
Venous drainage—Drains into facial or lingual
Posterior surface—It is convex and is continuous vein.
superiorly with the floor of the nasal cavity.
Lymphatic drainage—From the anterior portion of
Superior border—It is attached to the posterior mouth, lymphatics may pass into the deep
border of the hard palate, blending on each side cervical nodes or laterally to the periosteal
with pharynx. lymphatics and then to the submandibular nodes
Inferior border—It is free and bounds with and to the deep internal jugular nodes.
pharyngeal isthmus. Nerve supply—It is supplied by the branches of
Muscle of the soft palate—these are tensor palati, trigeminal nerve.
levator palati, musculus uvulae, palatoglossus
and palatopharyngeus. Maxillary Sinus

Blood supply—Greater palatine branch of the Paranasal sinuses are the air filled spaces present
maxillary artery, ascending palatine branch of with some bone around the nasal cavities. The
facial and palatine branch of ascending sinuses are frontal, maxillary, sphenoidal and
pharyngeal arteries. ethmoidal. Because of the close proximity of the
maxillary teeth with the maxillary sinuses, they
Venous drainage—Veins pass to the pterygoid and are the most important paranasal sinus in dental
tonsillar plexus of veins. point of view. They are the largest air filled
Lymphatic drainage—Lymphatics drain into sinuses surrounding the nose.
upper cervical and retropharyngeal lymph It also called as the ‘Antrum of Highmore’. They
nodes. are the paired structures located largely in the
body of maxilla and are mirror image of each
Nerve supply—All muscle of the soft palate except
other (Fig. 6.18).
the tensor palati are supplied by the pharyngeal
plexus. The fibers of the plexus are derived from Shape—It is pyramidal in shape with its base
the cranial part of the accessory nerve. The tensor directed medially towards the lateral wall of the
68 Textbook of Oral Pathology

proximity with the maxillary sinus followed by

the premolars and 3rd molar.
Medial wall—It is bounded by the nasal cavity.
Medial wall is generally slightly convex toward
the sinus.
Posterior wall—It is related to the pterygopalatine
fossa. The posterior wall bulges posteriorly
towards the infratemporal fossa.
Lateral wall—It is related to zygoma and cheek.
Anterior wall—It is related to the cheek. It is
depressed by canine fossa on the anterior surface
Figure 6.18: Location of paranasal sinus
Table 6.2: Lymphatic drainage of head and neck (Fig. 6.19)
nose and the apex directed laterally in the Nodes Sites
zygomatic process of the maxilla. Occipital Scalp, posterior to the ear and
occipital region
Size—Size is variable varying up to 3.5 cm in Posterior auricular External ear, scalp above and behind
height and 2.5 cm in width and 3.2 cm the ear.
Anterior auricular Skin anterior to the temple, external
anteroposteriorly.
(pre-auricular/ meatus,
It opens into the middle meatus of the nose parotid) lateral forehead, lateral eyelids,
in the lower part of the hiatus semilunaris. infraorbital
nodes, posterior cheek, part of the
Maxillary antrum contains air and is lined outer ear, parotid gland.
by mucoperiosteum with a pseudostratified Inferior auricular Pre and postauricular nodes.
ciliated columnar epithelium. (infra auricular)
Infra-orbital Skin of inner corner of the eye, skin
Roof—It is formed by the floor of the orbit and is of anterior face, and superficial aspect
of the nose.
transversed by the infraorbital nerve. The roof Buccal Skin over the anterior face, mucous
is flat and slopes slightly anteriorly and laterally. membrane of the lips and cheeks,
The most medial part of the roof forms the occasionally mandibular and
maxillary teeth and gingivae.
sloping wall of the maxilloethmoidal sinuses, Submental Tip of the tongue, mid portion of the
from which disease may spread to the maxillary lower lips, chin, lower incisors and
sinus. Antral infection may involve infraorbital gingivae.
Mandibular Skin over the mandible, mucous
vessels and nerve and malignant tumors (supra- membrane of
growing in the sinus may involve the orbit. mandibular) the lips and cheeks. Occasionally
maxillary and
Floor—It is curved rather than flat and is formed mandibular teeth and gingivae.
by the alveolar processes of maxilla and lies Submandibular Upper and lower teeth and gingivae
(sub maxillary) except
about 1 cm below the level of the floor of the mandibular incisor anterior nasal
nose. Roots of upper molars and premolar may cavity, palate, body of tongue upper lip,
ridge the floor or project into it. Floor may be lateral angle of eye submental nodes
Superficial Pinna and adjacent skin, pre and
subdivided by incomplete bony septa lying cervical postauricular
between the roots of the teeth, especially in the nodes.
posterior part of the sinus. The 1st and 2nd Deep cervical Submandibular, submental, inferior
auricular tonsillar and tongue nodes
molar’s roots are most commonly in close
 Embryology and Anatomy of Oral Structures 69

of the maxilla and is convex toward the interior

of the sinus.
Arterial supply—Facial, infraorbital and greater
palatine arteries.
Venous drainage—Into the facial and pterygoid
plexus of veins.
Lymphatic drainage—Drains into the
submandibular nodes.
Nerve supply—it is supplied by infraorbital and
anterior, middle and posterior superior alveolar
nerves.
Figure 6.19: Different types of nodes seen in orofacial region
Salivary Glands
It is described in the chapter of disease of salivary ascending palatine and tonsillar branch of the
glands. facial artery, dorsal lingual branch of lingual
artery and greater palatine, pharyngeal and
Pharynx pterygoid branch of the maxillary artery.
It is a wide muscular tube situated behind the Venous drainage—It is by a plexus which receives
nose, mouth and larynx and clinically it is a part blood from the pharynx and soft palate and
of upper respiratory tract. It is divided into three prevertebral region and drains into the internal
parts, i.e. nasopharynx (nasal part of pharynx), jugular and facial veins.
laryngopharynx (laryngeal part of pharynx) and
oropharynx (oral part of pharynx). Oropharynx Lymphatic drainage—Drains into the retro-
is the middle part of the pharynx situated pharyngeal and deep cervical lymph nodes.
behind the oral cavity. Nerve supply—It is supplied by the pharyngeal
Blood supply—It is supplied by ascending plexus of nerves which lies chiefly on the middle
pharyngeal branch of the external carotid artery, constrictor.
70 Textbook of Oral Pathology

CHAPTER
Hyperplasia, Hamartoma
7 and Neoplasm

DYSPLASIA Characteristic features of hyperplasia are

• It does not have autonomous growth.
It is a loss in the uniformity of the cells, as well • It ceases growth at some point like hamartoma.
as a loss in the architectural orientation. • Clinically hyperplasia may be seen as an
Dysplasia is usually referred to change seen in overgrowth, or mass, or tumorous swelling.
the epithelium, e.g. epithelial dysplasia in • Histologically it’s increased cellularity by
premalignant and malignant lesions. virtue of their proliferation. It makes them
resemble neoplastic proliferations, difference
METAPLASIA being absence of dysplastic features.
• Hyperplasias are usually reactive lesions.
Metaplasia is defined as a reversible change of
• They are initiated by an identifiable stimulus,
one type of mature epithelial or mesenchymal
e.g. Irritation of mucosa –fibrous hyperplasia
cells, usually in response to abnormal stimuli,
(Fig. 7.1).
and often reverts back to normal after removal
of the stimulus.

HYPERPLASIA

Hyperplasia represents overgrowth of normal

mature tissues with a limited cellular
proliferation or it is an increase in the number of
parenchymal cells resulting in enlargement of
the organ or tissue. Hyperplasia can be:
• Physiologic hyperplasia e.g. Due to hormonal
changes as in pregnancy.
• Pathologic hyperplasia may occur as
Figure 7.1: Localized fibrous hyperplasia due to irritation of
– Inflammatory removable prosthesis (Courtesy: Dr Roshna, Dept of
– Non inflammatory growth. Periodontia, Peoples Dental Academy, Bhopal)
 Hyperplasia, Hamartoma and Neoplasm 71

• The process or growth stops when stimulus – Oral and labial melanotic macule
is removed. – Pigmented cellular nevus
• Inflammatory lesions may be initiated and • Vascular origin
controlled by same stimulus repeatedly, e.g. – Hemangioma
Peripheral giant cell granuloma, pulp polyp – Lymphangioma
(chronic hyperplastic pulpitis). – Glomus tumor
• The process or growth stops but the size of • Osseous origin
the lesion does not usually regress on its own. – Torus palatinus
– Torus mandibularis
HAMARTOMA • Adipose tissue
– Lipoblastomatosis
Hamartoma is an overgrowth or abnormal
• Neural tissue
proliferation of mature cells and tissues or
– Neurofibromatosis
structures native to that part.
• They are dysmorphic proliferation of tissue Unknown or Doubtful Origin
often with one element predominating.
• Granular cell myoblastoma
• These tissues do not have inherent capacity
• Congenital epulis of the newborn
for continuous growth but they nearly
• Melanotic neuroectodermal tumor of infancy
parallel the growth of host.
• Fibromatosis gingivae.
• Because of this property the distinction
Characteristic features of hamartoma are:
between hamartoma and benign tumors is
• Growth of hamartoma ceases with general
often subjective as most benign tumors of
body growth.
infancy and childhood are actually
• They do not infiltrate into surrounding tissue.
developmental hamartoma.
Treatment
CLASSIFICATION OF HAMARTOMA
In bone they are treated by enucleation. If they
Odontogenic Hamartoma are present in soft tissue they are removed by
pericapsular incision.
• Those involving teeth:
– Dens invaginatus
Choriostoma
– Dens evaginatus
– Talon’s cusp Choriostoma are tumor like proliferation of
• Those not involving teeth (as these lesions tissue not native to the site. Histologically it is
do not have uncoordinated uncontrolled normal tissue found at abnormal site. They are
growth they can be called hamartoma than similar to hamartoma with basic difference is
neoplasm.) that proliferating tissue is not usual or native to
• Enameloma the site.
• Odontoma (complex and compound) • Heterotrophic gastrointestinal cyst — it may
• Gigantiform cementoma occur in tongue or floor of the mouth of
• Dental lamina cyst of the newborn infants and contains gastrointestinal
glandular structures.
Non Odontogenic Hamartoma • Rarely bone and cartilage may be found in
• Epithelial origin the tongue, known as osseous choriostoma
– Epstein pearls and Bohn’s nodules and cartilaginous choriostoma respectively.
72 Textbook of Oral Pathology

Lingual thyroid nodule—presence of thyroid tissue where DNA synthesis is absent but the synthesis
in the posterior part of tongue. of RNA and protein continues. At the end of G1
phase unknown signals continuously institute a
Fordyce’s granules—ectopic sebaceous glands in
burst of RNA synthesis which is followed by
the oral cavity.
DNA synthesis (S phase). Then the cells undergo
Presence of salivary gland within lymph
replication or remain polypoid and eventually
nodes.
die. The cells cease DNA synthesis during the
G2 phase and DNA content is fairly constant in
Teratoma
the growing normal cells. The proportion of cell
These are tumor like proliferation of tissues population undergoing active proliferation in the
derived from all the three germ layers of the body. cycle is termed as the growth fraction.
• Characteristic features: These arise from all
three germ layers
• Produce tissues that are foreign or not native
tissue in which they develop
• They are most commonly found in testes and
ovary. But they are rarely found in head and
neck region.

NEOPLASM

Definition
It is an abnormal mass of tissue, growth of which
exceeds and is uncoordinated with that of normal
tissue and persists in the same excessive manner
after cessation of the stimuli which evoked the
change.
Figure 7.2: Normal cell cycle
Nomenclature
ETIOLOGY
Mainly of two types: benign and malignant.
Benign tumors are designated by attaching Hereditary Predisposition
‘oma’ to cell of the origin. Tumor from fibrous The risk of developing cancer in relatives of a
tissue is called as fibroma. known cancer patient is 3 times higher than
Malignant tumors arising from mesenchymal
control population. Genetic cancers comprise not
tissues are known as ‘sarcomas’, like osteosarcoma.
greater than 5% of all cancers, e.g. retinoblastoma,
Malignant tumors of epithelial origin are
familial polyposis coli, cancer of breast, etc.
called ‘carcinoma’, like adenocarcinoma and
squamous cell carcinoma. Racial and Geographic Factors
Normal Cell Cycle (Fig. 7.2) Cancers are largely due to the influence of
All renewing cells go through a series of events environment and geographic differences
known as cell cycle. After mitosis (M phase), cells affecting the whole population such as climate,
spend a variable period of resting (G1 phase) water, diet, habit. For example
 Hyperplasia, Hamartoma and Neoplasm 73

• Black Africans commonly have cancers of Acquired Preneoplastic Conditions

skin, penis, cervix, and liver.
These may be inflammatory, hyperplastic
• Europeans and Americans commonly
conditions or may be certain benign tumors. For
develope malignancies of lung, breast, and
example
colon.
• Chronic atrophic glossitis.
• Carcinoma of stomach is five times higher in
• Leukoplakia of oral cavity, vulva and penis.
Japanese than in Americans.
• Cirrhosis of liver.
• Nasopharyngeal cancer is common in south
• Chronic irritation.
East Asians.
• Multiple neurofibromas.
Environmental and Cultural Factors
Carcinogenesis
We are surrounded by an environment of
carcinogens which we eat, drink, inhale and Carcinogenesis or oncogenesis or tumorigenesis
touch. For example means induction of tumors; agents which can
• Cigarette smoking is the etiology of cancer induce tumors are called carcinogens. The
of oral cavity, pharynx, larynx, esophagus, difference between cancer and bening and mass
lung, pancreas and urinary bladder. is shown in Table 7.1.
• Alcohol causes cancer of esophagus and liver. Carcinogens are broadly divided into 4
• Alcohol and tobacco together accelerate the groups:
risk of developing cancer of upper • Chemical carcinogens
aerodigestive tract. • Physical carcinogens (radiation)
• Betel nut chewing causes cancer of cheek and • Hormonal carcinogens
tongue. • Biologic carcinogens (virus)
• Industrial and environmental materials are
Chemical Carcinogenesis
carcinogenic. This includes exposure to
substances like arsenic, asbestos, benzene, Chemical carcinogens are divided into 2 broad
and naphthylamine. groups:
• Overweight individuals, deficiency of
vitamin A, people consuming foods rich in Initiators of Carcinogenesis
animal fats and low in fiber content have
Direct-acting carcinogens: These require no
more risk of developing cancers like colon
metabolic conversion to become carcinogens.
cancer.
• Alkylating agents: It includes various
Age chemotherapeutic drugs that have
successfully cured, controlled or delayed
Generally it occurs in older individuals past fifth recurrence of certain types of cancers only to
decade of life but there is variation in age groups. later evoke a second form of cancer usually
For example acute leukemia occurs in children, leukemia.
neuroblastoma in infancy. • Various agents used are cyclophosphamide,
chlorambucil, busulfan, melphalan,
Sex
nitrosourea, β-propiolactone and epoxides.
Generally more common in men except cancer This tragic consequence is called as “Pyrrhic
of breast, gallbladder, and thyroid. victory” which becomes less of a victory when
74 Textbook of Oral Pathology

Table 7.1: Differences between benign and malignant tumors

Macroscopic features
Features Benign tumors Malignant tumors
Boundaries Encapsulated or well circumscribed Poorly circumscribed and irregular
Surrounding tissues Often compressed Usually invaded
Size Usually small Often large
Growth Slow and expanding Rapid and infiltrating
Capsule Present Absent
Degeneration Rare Common
Recurrence Not common Common
Fixity Absent Present
Secondary changes Occur less often Occur more often
End of growth May come to standstill Growth rarely ceases
Microscopic features
Features Benign Malignant
Pattern Usually resemble tissue of origin Have poor resemblance to tissue of origin
Basal polarity Retained Lost
Pleomorphism Usually not present Often present
Nucleo-cytoplasmic ratio Normal Increased
Anisonucleosis Absent Present
Hyperchromatism Absent Often present
Mitosis May be present but always with typical mitosis Mitotic figures increased and are generally
atypical and abnormal
Tumor giant cells May be present but without nuclear atypia Present with nuclear atypia
Cytoplasm May show normal constituents Normal cytoplasmic elements are decreased
or lost
Function Well maintained May be retained, lost or become abnormal
Growth rate Usually slow Usually rapid
Local invasion Often compresses the surrounding tissue Usually infiltrates and invades the adjacent
without invading or infiltrating tissue
Metastasis Absent Present frequently

their initial use has been converted to cause • Aromatic amines and azo dyes: These are b-
later on second form of cancer. naphthylamine, benzidine, azo dyes used for
• Acylating agents: Substances like acetyl coloring foods, and acetyl aminofluorene.
imidazole. They may cause bladder cancer and
• Indirect-acting carcinogens or procarcinogens- hepatocellular carcinoma.
these are chemical substances requiring • Naturally occurring products: Aflatoxin,
metabolic activation for becoming potent actinomycin-D, mitomycin-C, safrole, and betel
initial carcinogens. nut. They can cause hepatocellular carcinoma.
• Polycyclic aromatic hydrocarbons (in tobacco, • Miscellaneous: Nitroso compounds, vinyl chloride
smoke, animal foods, industrial oil, and monomer, asbestos, arsenical compounds, metals
atmospheric pollutants). Important chemical like nickel, lead, chromium, and insecticides,
compounds included are benzanthracene, fungicides can cause gastric carcinoma,
benzapyrene, methylcholanthrene. They may hemangiosarcoma of liver, bronchogenic
cause lung cancer, skin cancer, cancer of oral carcinoma, epidermal hyperplasia, basal cell
cavity, and sarcoma. carcinoma, and lung cancer.
 Hyperplasia, Hamartoma and Neoplasm 75

Promoters of Carcinogenesis • Target molecules: The primary target is DNA,

Certain chemical substances lacking the intrinsic producing mutagenesis.
carcinogenic potential but helping the initiated • Initiated cell: The unrepaired damage
cell to proliferate further are called promoter of produced in the DNA of the cell becomes
carcinogenesis. For example phorbol, phenols, permanent only if the altered cell undergoes
drugs like phenobarbital, and artificial at least one cycle of proliferation.
sweeteners likes saccharine.
Promotion
Mechanism of Action and It does not damage the DNA but enhances the
Stage of Chemical Carcinogen
effect of direct-acting carcinogen or
The great majority of chemical carcinogens are procarcinogens. The ultimate effect is further
mutagens. They bind directly to DNA and RNA clonal proliferation of the initiated cell.
or cytoplasmic proteins to specific sites within Sometimes two or more initiators be the
molecule inducing miscoding error during chemical, oncogenic virus or radiant energy may
transcription and replication. The carcino- act in concert to induce malignant transfor-
genicity of chemical agents is dose dependent mation referred to as cocarcinogens.
and multiple traditional doses have same
oncogenecity as a single comparative dose. The Physical Carcinogenesis
carcinogenicity of chemical agents can be Divided into two groups.
significantly enhanced by the subsequent
administration of promotors. To be effective the Radiation Carcinogenesis
promotor must follow the initiator. The
phenomenon of cellular transformation by Radiation whenever in the form of UV light from
chemical carcinogenesis is a progressive process sunlight, UV lamp, welder’s arc, or ionizing
involving 2 different stages. They are initiation radiation like X-ray, α, β and γ ray, radioactive
and promotion. isotopes, protons and neutrons are established
carcinogens. Most frequent radiation induced
cancers are leukemia, cancer of thyroid, skin,
Initiation
breast, lung, and salivary gland. Therapeutic
In this initiator, interacts with DNA of target cell irradiation can also induce carcinogenesis.
to induce mutation that is more or less Radiant energy has potential of producing
irreversible to transform it into initiated cell: mutation and even killing the cells. It can affect
• Metabolic activation: Only indirect acting carcinogenesis by the following facts:
carcinogen or procarcinogens require • Few tumors appear only after long latent
metabolic activation, chiefly by mixed period during which successive generation
oxidases of cytochrome P-450 system located of clones are developed.
in microsomal compounds of the endoplasmic • The radiation initiation is generally
reticulum or in the nucleus. irreversible, but at a low dosage level is
• Reactive electrophiles: They are electron amenable to repair.
deficient protons, which bind to electron rich • The effect of radiation depends upon a
portions of other molecules of cell such as number of factors such as type of radiation,
DNA, RNA or other proteins. dose, length of interval between the doses,
76 Textbook of Oral Pathology

capability of cells to repair in intervals and in mice by estrogens are squamous cell
various host factors such as age, individual carcinoma of cervix, connective tissue tumor of
susceptibility, immune competence, myometrium, tumor of kidney in hamsters, and
hormonal influence and type of cell benign and malignant tumors of liver in rats.
irradiated. In humans: Women receiving estrogen
The ultimate mechanism of radiation may therapy and women with estrogen secreting
directly alter the cellular DNA and it may granulosa cell tumor of the ovary have increased
dislodge ions from water and other molecules risk of developing endometrial carcinoma.
of cell and result in the formation of highly Adenocarcinoma of the vagina is seen with
reactive free radicals that may bring about the increased frequency in adolescent daughter of
damage. Radiation mutation may render cell mother who had received estrogen therapy
vulnerable to other carcinogenic influence, i.e. during pregnancy.
acting as co-carcinogen, inhibition of cell division
and inactivation of enzymes, and radiation Contraceptive Hormones
might cause cell killing; permitting survivors to
Increased risk of developing breast cancer,
proliferate and thereby become vulnerable to
benign tumors of the liver and few patients have
oncogenic influence.
developed hepatocellular carcinoma.
Non Radiation Physical Carcinogenesis
Anabolic Steroids
Mechanical injury to tissues such as from stones
Consumption of anabolic steroids by athletes to
in the gallbladder, stones in the urinary tract, and increase the muscle mass also increases the risk
healed scars following burns or trauma have of developing benign and malignant tumors of
been suggested as causes of increased risk of the the liver.
carcinoma. Implants of inert materials such as
plastic, glass, etc in prostheses and foreign bodies Hormone Dependent Tumors
like metal foils observed to cause tumor It has been shown in experimental animals that
development in experimental animals. induction of hyperfunction of adenohypophysis
is associated with increased risk of developing
Hormonal Carcinogenesis neoplasia of the target organs following
preceding functional hyperplasia.
Carcinoma is most likely to develop in organs
and tissues which undergo proliferation under
Biologic Carcinogenesis
influence of excessive hormonal stimulation.
Hormone sensitive tissues developing tumors The epidemiological studies on different types
are breast, endometrium, myometrium, vagina, of cancer indicate the involvement of
thyroid, liver, prostate, and testis. transmissible biologic agents in their
development, chiefly viruses. It has been
estimated that about 20% of all cancers
Estrogen
worldwide are virus associated cancers.
In experimental animals: Induction of breast cancer Therefore biological carcinogenesis is largely
in mice by administration of high doses of viral oncogenesis. A large number of viruses
estrogen. Other cancers which can be induced have been proved to be oncogenic in wide variety
 Hyperplasia, Hamartoma and Neoplasm 77

of animals and in certain types of cancers in virion core proteins from gag gene,
humans. The association of oncogenic virus with enveloped glycoprotein from env gene and
neoplasia was observed by an Italian physician reverse transcriptase from pol gene.
Sanarelli in 1889 who noted association between • The three components of virus particles are
myxomatosis of rabbit with poxvirus. Oncogenic then assembled at the plasma membrane of
viruses fall into 2 broad groups, i.e. those host cells and virus particles released by
containing ribonucleic acid are termed as RNA budding off from plasma membrane, thus
oncogenic viruses and those containing completing the process of replication.
deoxyribonucleic acid are termed as DNA
oncogenic viruses. DNA Oncogenic Viruses

RNA Oncogenic Viruses They are divided into five groups:

• Papovavirus group: Human papilloma virus,
These are retroviruses, i.e. they contain the polyoma virus, SV-40 (simian vacuolating)
enzyme reverse transcriptase, which is required virus.
for reverse transcription of viral RNA to • Herpes virus: Epstein Barr virus, Human
synthesize viral DNA strands. Based on their herpesvirus, cytomegalovirus, lucke’s frog
activity to transplant target cells into neoplastic virus, Marek’s disease virus.
cells, they all are divided into three subgroups: • Adenoviruses: It can cause upper respiratory
• Acute transforming viruses — It includes Rous infections and pharyngitis. In man, they are
sarcoma virus in chickens, leukemia-sarcoma not known to be involved in tumors but in
viruses of avian, feline, bovine and primate. hamsters they may induce sarcomas.
• Slow transforming tumor viruses — Mouse • Poxvirus: In rabbits it can cause myxomatosis
mammary tumor virus (MMTV) that causes and in humans it can cause molluscum
breast cancer in daughter mice. contagiosum and may induce squamous cell
• Human T-cell lymphotropic viruses (HTLV) — papilloma.
It can cause adult T-cell leukemia-lymphoma • Hepadnaviruses: Hepatitis B virus is a member
syndrome and AIDS. of this family and it can cause acute hepatitis
• Mechanism of RNA viral oncogenesis — Reverse and is responsible for carrier state which can
transcriptase acts as a template to synthesize result in some cases to chronic hepatitis
a single strand of matching viral DNA. progressing to hepatic cirrhosis and onto
• Single strand of viral DNA is then copied by hepatocellular carcinoma.
DNA dependent DNA synthetase to form
another strand of complementary DNA Mechanism of DNA Viral Oncogenesis
• Replication: The virus may replicate in the
resulting in double stranded viral DNA or
host cell with consequent lysis of infected cell
provirus.
• The provirus is then integrated into the DNA and release of virions.
• Integration: The viral DNA may integrate into
of the host cell genome and may transform
the host cell DNA. This results in neoplastic
the cell into a neoplastic cell.
• Virus replication begins after integration of transformation of the host cell.
provirus into host cell genome. Integration
Oxidative Mechanism of Carcinogenesis
results in transcription of proviral genes or
progenes into messenger RNA which then Active oxygen species and other free radicals are
forms components of the virus particle, i.e. known to be mutagenic. Further these agents
78 Textbook of Oral Pathology

have emerged as mediators of the other to produce 109 cells weighing approximately
phenotypic and genotypic changes that lead to 1gm, which is the smallest clinical detectable
form mutation to neoplasia. Free radical mass. To produce a tumor of 1012 cells, weighing
production is ubiquitous in all respiring 1 kg approximately, which is usually the
organism and is enhanced by many disease maximum size compatible with life, the tumor
states, by carcinogen exposure and under cells have to undergo 10 further population
conditions of stress. Free radicals may therefore doublings. So by the time the tumor is clinically
contribute widely to cancer development in detectable, it has already complete a major
humans. Free radicals scavenging vitamins C portion of its life cycle. In tumor cells, there is an
and E have been shown to protect against cancer imbalance between cell production and cell loss,
development in animal models. therefore the tumor grows progressively. The
rate of tumor growth depends upon the growth
BIOLOGY OF TUMOR GROWTH fraction and the degree of imbalance between
cell production and cell loss.
The life cycle of malignant tumors can be divided
into four phases. Mechanism of Local Invasion and Distant
Metastases
Induction of Malignant Changes in the Target
Cell (Transformation) There are three routes through which metastases
Large number of carcinogen agents induce of tumor cells occur, i.e. local invasion, via blood
neoplastic transformation of cells in vivo and in vessels and via lymphatics. The local invasion
experimental animals. All etiologic factors takes the path of least resistance and the tumor
ultimately affect the function of two sets of genes, cells invade the surrounding tissue spaces. In
one is proto-oncogenes or oncogenes and another case of oral malignancies distant metastasis is
one is anti-oncogenes or cancer suppressor genes. mainly via lymphatics, either by lymphatic
The majority of carcinogens are mutagenes permeation or by lymphatic embolism. Rarely it
which bind the DNA directly or indirectly by spreads through blood vessels and if this occurs,
undergoing enzymatic activation, inducing the tumor cells invade the lumen of blood
miscoding errors during transcription and vessels, the tumor emboli form, which are
replication. Oncogenes may code for growth fragmented and the tumor cells are lodged into
promoting factors and as a result the tumor cells distant tissues.
produce large amount of growth factors to
which, only they can respond. Oncogenes may Theories of Carcinogenesis
encode a defective receptor that sends stimulating The Epigenetic Theory
signals to the cells, even in the absence of growth
According to this theory, the carcinogenic agents
factors. Thus cancer is a genetic disease that results
when multiple mutations accumulates in the act on the activators or suppressors of genes and
not on the genes themselves and result in the
DNA of a cell and specific chromosomal
abnormal expression of genes.
abnormalities predispose to cancer.

Growth of Transformed Cells (Kinetics of Genetic Theory

tumor cell growth) This is the most popular theory which suggests
The monoclonal cancer cell (10 μm in diameter) that cells become neoplastic because of alteration
has to undergo about 30 population doublings in the DNA. It is suggested that the secret of
 Hyperplasia, Hamartoma and Neoplasm 79

cancer lies within the normal cells themselves stimulates extreme hyperplasia without affecting
in the form of proto-oncogenes (C-oncs). The the genome cell. It acts as a biologic carcinogen
mutated cells transmit their characters to the next on some cellular constituents to release or
progeny of cells. Inappropriate over expression activate neoplastic potentialities normally
of the gene or point mutation cause the cell to present in cells. Carcinogens of all kinds
produce stimulating growth factors or in some ultimately act by creating some new auto-
way damages normal regulatory control. The synthesizing cytoplasmic constituents, probably
qualitative and quantitative changes in the an auto-catalytic protein, which can excite the
expression of genome may be brought about by cell to unlimited growth.
carcinogenic influence, i.e. chemicals, viruses,
Immune Surveillance Theory
radiation or spontaneous random mutations.
Oncogenes: Oncogenes are the transforming It suggests that an immune-competent host
genes present in many tumor cells. Closely mounts an attack on developing tumor cells so
related genes are detected on normal animal and as to destroy them while an immune-
human cells and are called as ‘proto-oncogenes’ incompetent host fails to do so. According to
or ‘cellular oncogenes’, abbreviated as ‘c-oncs’. original immunological theory, normal cells
‘Cellular oncogenes’ of the host cells can contain specific ‘self-marker’ (identity proteins)
transcribe its copies in the viral genome of acute which is recognized by the normal growth
transforming oncogenic retroviruses called as regulating mechanism. These proteins serve as
viral oncogenes or ‘v-oncs’. An alternate receptor for chemical carcinogens (hapten) and
mechanism is by anti-oncogenes in which there the resulting complex is self-replicating. The
complex (complex antigen) triggers off an
is inactivation or deletion of genes that normally
immune response and the antibody (free or cell
perform the function of suppressing cell
bound) combines with the self-marker
proliferation, thus allowing them to proliferate.
carcinogen complex and eliminates it. The new
According to genetic regulatory mechanism
race of cells produced is with self-markers
theory, primary change in the cell consists of a
deleted and goes unrecognized by growth
modification of repressor molecule which
regulatory mechanism. The high incidence of
controls the functions of the gene. The repressor
molecules are either RNA or protein. The cancer in AIDS patients is in support of this
modification of repressor molecules removes theory.
their orderly inhibitory control, which is
responsible for normal morphogenesis and Monoclonal Hypothesis
differentiation, and unearths the cell genetic Currently, there is strong evidence on studies of
potentiality for unrestricted growth. This concept human and experimental animals that most
of loss of growth control is described as ‘feed cancers arise from single clone of transformed
back deletion’. cell. The best documentation of monoclonal
origin of cancer cells comes from the study of
Virus Theory G6PD in women who are heterozygous for its
Viruses participate at some stage in the two isoenzymes A, and B. It is observed that all
development of cancer. The concept of mode of tumor cells in benign uterine tumor (leiomyoma)
action of virus has taken many forms: contain either A or B genotype of G6PD – i.e. the
Virus is present as a parasite in all tumor cells tumor cells are derived from a single progenitor
and it is transmitted from cell to cell and cell.
80 Textbook of Oral Pathology

Multistep Theory (sarcoma), penetration of the basement

membrane and invasion of connective tissue
According to this theory, carcinogenesis is a
(carcinoma) → Entering the wall of blood and
multistep process which is substantiated by in
lymphatic vessels → Survival of malignant cells
vitro changes in experimental animals as well as
in the blood stream → Emergence of the
in vivo changes in human cancers. In chemical
malignant cells from the blood vessels in the
carcinogenesis, there are two essential features,
form of the emboli and lodgment in other tissues
i.e. initiation and promotion. Many tumors arise
→ Survival in the compatible tissue environment
from combination of activation or growth
and induction of growth factor to stimulate new
promoting oncogenes and inactivation of growth
vessel formation to obtain nutrition →
suppressing anti-oncogenes. In some cancers,
Multiplication of neoplastic cells and growth to
there is initial dysplastic change that may
form secondary neoplasm at the new site → Each
progress into carcinoma in situ and then into
of these steps is probably controlled by different
invasive carcinoma.
molecular mechanism and this may explain the
Metastasis differences in the behavior with reference to
tumor metastasis. Neoplastic cells within a single
Metastasis is defined as spread of tumor by tumor might differ in their ability to metastasize.
invasion in such a way that discontinuous A sub-population of cells preexists within the
secondary tumor mass/masses are formed at the heterogeneous primary tumor. The relative size of
site of lodgment. This metastasis is the transfer this sub-population in the primary tumor may vary
of the disease from one organ or part to another with time between the neoplasms.
not directly connected with it.
If malignant cells do not metastasize, the Routes of Metastasis
surgical removal of primary neoplasm would
Lymphatics: Particularly for carcinoma and
completely cure the patient. Metastasis is
lymphosarcoma. For example- mouth to neck
fundamentally an embolic process. The
nodes and breast to axillary nodes.
invasiveness of malignant cells involves motility,
which requires changes of shape and Blood stream: Particularly veins from gut via
adhesiveness and ability to degrade the matrix portal circulation to liver, from systemic sites
in order to penetrate it. through right heart to lung, from left heart to
Thus, a definition of the behavior of the any systemic sites.
metastatic tumor cells is the tendency to cross Cavities: Along epithelium lined cavities, for
the tissue compartment/boundary and intermix example- respiratory tract, gut, urogenital tract, etc.
with other cell types. The metastatic process can
Others: Transcelomic spread, cerebrospinal fluid,
be divided into several sequential steps although
tissue planes and through nerve sheath.
these steps are interconnected.
Factors which control metastasis are
Pattern of Metastatic Spread
proteolysis, cell adhesion, tumor angiogenesis,
cell mediated immunity and genetic factor. Mechanistic theory: The capillary bed of the first
organ which encounter viable neoplastic cells is
Steps of Metastasis the preferred site of metastasis.
The breaking of loose neoplastic cells from the Seed and soil hypothesis: It suggests that
parent tumor → Invasion of the matrix availability of fertile environment (the soil) in
 Hyperplasia, Hamartoma and Neoplasm 81

which compatible tumor cells (the seed) can as epithelial origin on the basis of histology
grow is important. Ewing suggested that varying alone, nuclear aberrations are abundant and
pattern of metastasis is due to fact that different no keratinization is found.
tumor cells thrive in certain biological sites (soils)
but not in the other sites. CIN Grading

Cell interaction: Interaction between cell surface Alternative classification for grading of dysplasia
protein of malignant cells and organ specific and carcinoma in situ together is cervical
protein, e.g. fibronectin receptor. intraepithelial neoplasia (CIN). According to this
grading, the criteria are as follows:
GRADING AND STAGING OF TUMORS • CIN I – it represent less than one-third
involvement of the thickness of the
Grading epithelium.
The grading features are those indicative of • CIN II – in it there is one-third to two-third
proliferation and differentiation. It is defined as involvement.
macroscopic and microscopic degree of • CIN III – it is full thickness or equivalent to
differentiation of tumor. Grading depends carcinoma in situ.
mainly on two histologic features, the degree of Depending on thickness of squamous
anaplasia and the rate of growth. Different types epithelium involved by atypical cells
of grading systems are as follows: According to this grading of dysplasia is as
follows
Broder’s Classification System • Mild
• Moderate
• Grade I (well differentiated i.e. less than 25%
• Severe
anaplastic cells): It is characterized by the pres-
ence of relatively mature cell with few Staging
nuclear aberration and with the presence of
keratin pearls and individual cell keratiniza- It is the extent of spread of tumor within patients.
tion. It is assessed by following ways:
• Grade II – (Moderately differentiated i.e. 25 to • By clinical examination - Size and extent of
50% anaplastic cells): It is characterized by the primary lesion.
presence of tumor cell exhibiting a wide • By investigations.
range of differentiation, keratinization is • By pathological examination.
occasionally present, and nuclear aberrations • Degree of infiltration of primary lesion.
are moderately abundant. Usually the • Presence or absence of metastasis to regional
invasion is poorly delineated from the stroma. lymph nodes.
• Grade III – (Moderately differentiated i.e. 50 to • Presence and absence of distant metastasis.
75% anaplastic cells): It is characterized by • Involvement of contralateral or ipsilateral
disorderly and poorly differentiated cells node.
with no tendency towards keratinization, • Whether node are fixed or not.
nuclear aberrations are abundant.
Objectives
• Grade IV – (Poorly differentiated i.e. more than
75% anaplastic cells): In it cells are so poorly • To aid clinician in the planning of treatment.
differentiated that they can not be identified • To give some indication of prognosis.
82 Textbook of Oral Pathology

• To assist in evaluation of the result of – N3: metastasis in lymph node more than 6
treatment. cm and it is fixed.
• To facilitate the exchange of information - N 3 a – ipsilateral nodes at least one
between treatment centers. greater than 6 cm.
• To contribute to the continuing investigations - N3b – bilateral nodes greater than 6 cm.
of human cancer. - N3c – contralateral nodes at least one
greater than 6 cm.
TNM Staging • Distant metastasis (M)
It is universally accepted system which is – Mx: Distant metastasis can not be assessed.
– M0: No distant metastasis.
developed by UICC (union international control
– M1: Distant metastasis.
of cancer).
• Primary tumor (T) - local extent is major – Category M 1 may be further specified
according to the notation.
factor contributing to prognosis.
- Pulmonary – PUL
– Tx: primary tumor can not be assessed.
– T0: no evidence of primary tumor. - Osseous – OSS
- Hepatic – HEP
– Tis: carcinoma in situ.
- Brain – BRA
– T1: tumor 2 cm or less in diameter.
– T2: tumor 2-4 cm in diameter. - Lymph nodes – LYM
- Bone marrow – MAR
– T3: tumor more than 4 cm in greatest
- Pleura – PLE
diameter.
– T4 – tumor of any size in which tumor - Peritoneum – PER
- Skin – SKI
invades adjacent structure (e.g.: cortical
- Other – OTH
bone, inferior alveolar nerve, floor of
mouth, skin of face, etc).
AJC (American Joint Committee)
• Regional lymph nodes (N)
– N x : regional lymph node can not be It divides all cancers into stage 0 to 4, and takes
assessed. into account all three previous TNM systems.
– N0: no regional lymph node metastasis. • Stage 0 – Tis N0 M0
– N1: metastasis in single ipsilateral lymph • Stage 1 – T1 N0 M0
node less than 3 cm in diameter. • Stage 2 – T2 N0 M0
- N1a: nodes considered not to contain • Stage 3 – T3 N0 M0, T1 N1 M0, T2 N1 M0, and
tumor growth. T3 N1 M0
- N1b: nodes considered to contain growth • Stage 4A- T4 N0 M0, T4 N1 M0, any T N2 M0
– N2: single lymph node, no more than 6 cm • Stage 4 B- Any T N3 M0
in greatest dimension, of bilateral/con- • Stage 4 C- Any T, Any N, M1
tralateral lymph node, none more than
6cm. Dukes ABC Staging
- N2a: Single ipsilateral lymph node more It is used in cancers of bowel:
than 3 cm but less than 6 cm. • Stage A – when tumor is confined to
- N2b: multiple ipsilateral lymph nodes submucosa and muscle and cure rate is 100%.
less than 6 cm. • Stage B – tumor penetrates the entire
- N2c: Bilateral or contralateral lymph node thickness of bowel wall into pericolic or
less than 6 cm in greatest dimension. perirectal tissues and cure rate is 70%.
 Hyperplasia, Hamartoma and Neoplasm 83

• Stage C – it is characterized by lymph node – N1- equifocal node enlargement.

metastasis and reduces the cure rate to 30%. – N 2 - clinically palpable homolateral
regional nodes, not fixed.
STNMP Staging System – N3- same as N2 but fixed.
• S- site of primary tumor – N4- clinically palpable contralateral or
– S1- lip and skin. bilateral nodes, not fixed.
– S2- lip mucosa. – N5 – same as N4 but fixed.
– S3- tongue. • Metastasis
– S4- cheek. – M0- no distant metastasis
– S5- palate. – M1- clinical evidence of distant metastasis
– S6- floor of mouth. with out definite histological or
– S7- alveolar process. radiographic conformation.
– S8- antrum. – M2- proven evidence of metastases beyond
– S9- central carcinoma of bone. regional nodes.
• Size of tumor – it is denoted by T • Pathology of lesion
– T1- less than 2 cm in diameter. – P0- hyperkeratotic lesion showing atypia.
– T2- between 2 cm and 4 cm in diameter. – P1- carcinoma in situ.
– T3- between 4 cm and 6 cm in diameter – P2- basal cell carcinoma.
and extending beyond the primary region – P3- verrucous carcinoma.
and extending through adjacent perios- – Well differentiated squamous cell
teum. carcinoma.
– T4- greater than 6 cm in diameter and – Moderately differentiated squamous cell
extending to involve adjacent structures. carcinoma.
• Regional nodes were grouped as – Poorly differentiated squamous cell
– N0- no palpable nodes. carcinoma.
84 Textbook of Oral Pathology

CHAPTER
Healing of
8 Oral Wounds

Wound healing is one of the primary survival Physical Factors

mechanisms from the birth. It is not an isolated Trauma: Severe trauma to tissue delays wound
solitary phenomenon but a very complex series healing and mild traumatic injury favors healing.
of biological events. Oral wounds are common,
some being sustained accidentally (jaw Local temperature: It influences healing probably
fracture), others being produced by the dentist through the effect on local circulation and cell
for specific purposes (extraction wounds, biopsy multiplication. In environmental hyperthermia,
wounds). wound healing is accelerated; while in
Unusual anatomic situation of the oral cavity hypothermia it is delayed.
like teeth protruding through bone, constant
Effect of X-ray radiation: Low doses of radiation
inflammation of gingiva, presence of countless
tends to stimulate healing while large doses of
microorganisms in the warm moist environment
radiation or total body radiation tends to
of saliva contribute to the modification of healing
suppress it.
reactions of various wounds.
Circulating Factors
GENERAL FACTORS AFFECTING THE
Anemia: It is reported to be responsible for
HEALING OF ORAL WOUNDS
causing delay in wound healing.
Location of Wound
Dehydration: It has been found to affect healing
Wound in an area which has a good vascular bed adversely.
heals more rapidly than wounds in an area which
Nutritional Factors
is relatively avascular. Immobilization is
important in healing of a fracture. If the wound Proteins: It influences the rate at which healing
is in an area that is subjected to constant takes place. Poorly nourished patients, due to
movement and so the formation of new low protein intake manifested as hypo-
connective tissue is disrupted (in the corner of proteinemia exhibits delay in appearance of
mouth) thus delaying the healing process. fibroblasts as well as decreased rate of

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 Healing of Oral Wounds 85

multiplication of fibroblasts in the wound, thus Miscellaneous Factors

delaying healing. Conversely, it has been shown
Various factors like enzymes such as trypsin,
that feeding a high protein diet to animals will
streptokinase, mucopolysaccharide, therapeutic
increase the rate of fibroblastic proliferation and
agents like dilantin sodium, sulfonamides,
cause the wound to heal more rapidly. Effect is
antibiotics, anti-coagulants like heparin,
related to dietary compounds containing free
dicumarol affect wound healing.
sulfahydryl groups. Of all the essential amino
acids only methionine furnishes such group. Suture materials: Wounds sutured with non-
Vitamins: Vitamin-C affects wound healing. It acts absorbable sutures are weaker than those
through regulation of collagen formation and sutured with absorbable ones.
formation of intercellular ground substances of the Cyanoacrylate adhesives: It permits uncomplicated
connective tissue. Vitamin-A deficiency retards healing and also does hasten the healing process.
healing. Riboflavin and pyridoxine deficiency
also results in a delay of the healing process.
HEALING OF BIOPSY WOUNDS
Age of the Patient Biopsy: It is the removal of tissue for the purpose
A wound in younger patients heals more rapidly of microscopic examination and diagnosis.
than a wound in elder patients due to general
reduction of rate of tissue metabolism as the Primary Healing
person ages.
Healing by primary intention or healing by first
Infection intention is that type of healing which occurs
after excision of a piece of tissue with close
Wounds completely protected from bacterial
apposition of the edges of the wound. When the
infections heal considerably slower than wounds
edges of the wound are brought together into
which are exposed to bacteria or other mild
contact and held in place by sutures, the blood
physical irritation. It has been shown that severe
clots, and in a matter of hours, numerous
bacterial infection slows the healing process.
leukocytes are mobilized in the area. Connective
tissue cells in immediate vicinity undergo
Hormonal Factors
transformation into fibroblasts which in turn
ACTH (adrenocorticotropic hormone) and undergo mitotic division. New fibroblasts begin
cortisone administration result in a delay in to migrate into and across the line of incision.
wound healing. It is due to the fact that ACTH These cells form thin, delicate collagen fibrils
and cortisone inhibit the growth of granulation which intertwine and coalesce in a general
tissue due to inhibition of proliferation of new direction parallel to the surface of the wound.
fibroblasts and depression of inflammatory Endothelial cells of capillaries begin to proliferate
reaction. In diabetes mellitus, clinically evident and small capillary buds grow out and across
retardation in repair of wounds occurs and also the wound. These buds eventually form new
there is complication in the healing process due capillaries which fill with blood and a rich
to disturbances in the carbohydrate metabolism network of young capillaries and capillary loops
at the cellular level in the local area of the wound. are formed.

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86 Textbook of Oral Pathology

Secondary Healing 48 hours, there is vasodilation and engorgement

of blood vessels in the remnants of periodontal
It occurs when there is a loss of tissue and the
ligament and there is mobilization of leukocytes
edges of the wound can not be approximated
to the immediate area around the clot. Surface
like in palate or on the alveolar mucosa. The
of the blood clot is covered by a thick layer of
material which fills the defect in secondary
fibrin. It is important to recognize that the
healing is called as granulation tissue. After the
collapse of unsupported gingival tissue into the
removal of the lesion, blood clots and the repair
opening of fresh extraction wound is of great aid
process begins. It is basically identical with the
in maintaining the clot in position.
healing by primary intention except that the
fibroblasts and capillaries have a greater distance
First Week Wound
to migrate, more granulation tissue must form
and healing is slower. Cellular proliferation Proliferation of fibroblasts from connective tissue
begins around the periphery of the wound and into the remnants of periodontal ligament is
fibroblasts and endothelial cells grow into the evident and these fibroblasts begin to grow into
clot along the fibrin strands. In addition, the clot around the periphery. The clot is
polymorphonuclear leukocytes, mononuclear gradually replaced by granulation tissue.
phagocytes and later the lymphocytes migrate Epithelium at the periphery of the wound
into the granulation tissue from the adjacent exhibits evidence of proliferation seen in the
vessels and tissues. Large numbers of leukocytes form of mild mitotic activity. Crest of the alveolar
also accumulate on the surface of the wound. As bone, which marks up the margin or neck of the
granulation tissue matures, it becomes more socket exhibits beginning of osteoclastic activity.
fibrous through condensation of collagen Endothelial proliferation signals the beginning
bundles and the surface of the lesion becomes of capillary growth. During this period, blood
epithelialized. Lesions become somewhat clot begins to undergo organization by in-growth
avascular. of fibroblasts and occasionally by small
capillaries from the residual periodontal
HEALING OF EXTRACTION WOUNDS ligament. An extremely thick layer of leukocytes
gathers over the surface of the clot and the edges
The healing of extraction wound does not differ of the wound continue to exhibit epithelial
from the healing of other wounds of the body proliferation.
except as it is modified by the peculiar anatomic
situation which exists after the removal of tooth. Second Week Wound
During the second week, after extraction of the
Immediate Reaction following an Extraction
tooth, the blood clot is becoming organized by
After the removal of a tooth, blood which fills fibroblasts growing into the clot and forming
the socket coagulates, red blood cells get fibrin meshwork. At this stage, new delicate
entrapped in the fibrin meshwork and the ends capillaries have penetrated to the center of the
of blood vessels in the periodontal ligament clot. Remnants of periodontal ligament have
are sealed off. Hours after the tooth extraction, been gradually undergoing degeneration. Walls
if blood clot is dislodged, healing may be greatly of the bony socket appear slightly frayed. In
affected and may be extremely painful. some cases, trabeculae of osteoid can be seen
However, if the healing is normal, within 24 to extending outward from the walls of the

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 Healing of Oral Wounds 87

alveolus. Epithelial proliferation over the surface the adjacent bone. Most healed socket reveal
of the wound is extensive. Margins of the slight or marked cortical bone formation at the
alveolar socket exhibits prominent osteoclastic surface of the alveolar process. Following the
resorption and fragments of necrotic bone are removal of teeth, the alveolar margins undergo
seen in the process of resorption or sequestration. some resorption. The surface usually becomes
flat or slightly curved but smooth.
Third Week Wound
HEALING OF FRACTURES
Original clot appears almost completely
organized by maturation of granulation tissue. Immediate Effect of a Fracture
Very young trabeculae of osteoid or uncalcified
After fracture, haversian vessels of the bone are
bone are forming around the entire periphery of
torn at the fracture site so are the vessels of
the wound from the socket wall. Early bone is
periosteum and marrow cavity that happen to cross
formed by osteoblasts derived from
the fracture line. Due to disruption of vessels, there
pluripotential cells of the original periodontal
is considerable extravasation of blood in that area,
ligament which assume osteogenic function.
but at the same time, there is loss of circulation and
Original cortical bone of alveolar socket
lack of local blood supply. The bone cells or
undergoes remodeling so that it no longer
osteocytes of haversian system die due to tearing
consists of such a dense layer. Crests of alveolar
of vessels at the fractured site. Concomitant with
bone have been rounded off by osteoclastic
the disruption of blood supply and the tearing of
resorption. By this time, surface of the wound
the blood vessels, there is death of bone marrow
may have become completely epithelialized.
adjacent to the fracture line. The blood clot which
forms, play an important role in healing of the
Fourth Week Wound
fracture through replacement by granulation tissue
There is continuous deposition and remodeling, and its subsequent replacement by bone.
resorption of the bone filling the alveolar socket.
Due to this, crest of the alveolar bone undergoes Callus Formation
considerable amount of osteoclastic resorption
during the healing process and because of this, It is a structure which unites the fractured ends
bone filling the socket does not extend beyond of bone and is composed of varying amounts of
alveolar bone crest. It is obvious that crest of the fibrous tissue, cartilage and bone.
healing socket does not extend above the alveolar There are two types of callus:
crest. • External callus: It consists of new tissue which
forms around the outside of the two
fragments of bone.
Radiographic Changes in Healing Sockets
• Internal callus: It consists of new tissue arising
A tooth have been removed and after variable from the marrow cavity.
and unspecified length of time, there is gradual Periosteum is an important structure in callus
loss of density of the lamina dura and at the same formation and ultimate healing of fracture. Cells
time bone develops at the base and sides of the of periosteum torn at the fracture line usually
socket. By the time that the socket is filled with die, but peripheral to the area is found a flurry
bone, all traces of lamina dura is gone. Later the of cellular activity within hours of injury. Outer
new bone consolidates and comes to resemble or fibrous layer of periosteum is relatively inert

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88 Textbook of Oral Pathology

and actually lifted away from the surface of bone For successful osseointegration, it is required
by proliferation of cells in osteogenic or inner layer that the implant should be totally free of any
of periosteum which assumes features of small amount of load or movement.
osteoblasts which in turn begins the formation of Healing process consist initially of deposition
small amount of new bone at some distances from of the granulation tissue and woven bone.
the fractured site. There is continuous proliferation Complete healing requires a period of several
of these osteogenic cells forming a collar of callus months together. The process is completed with
around or over the surface of fracture. the deposition of cancellous or compact bone
New bone which begins to form in the around the implant. Currently the implants used
external callus usually consists of irregular for support for the restorative or rehabilitative
trabeculae laid down at right angles to the oral procedures are biocompatible.
surface. This differentiation of cells into Biocompatible implants serve as a normal
osteoblasts and subsequent formation of bone interface of the connective tissue and the implant
occurs in the deepest part of the callus collar. In as is with the dentogingival junction.
the rapidly growing area of collar, varying Peri-implant disease is the term used for the
number cells of the osteogenic layer differentiate pathologic changes seen around the implant. It
into chondroblasts rather than osteoblasts and involves peri-implant mucositis peri-implantitis.
actually form cartilage. The inflammation is seemed more at the coronal
This cartilage fuses with bone and then begins aspect; the apical osseointegration is maintained
to calcify by endochondral bone formation. The under favorable conditions.
calcified cartilage is gradually resorbed and
replaced by bone. Internal callus forms from HEALING OF THE WOUNDS OF SKIN AND
endosteum of haversian canals and undifferen- THE ORAL MUCOSA
tiated cells of bone marrow. Shortly after the
fracture, endosteum begins to proliferate within Healing of the wounds of skin and the oral
a week to form new bone, which gradually unite mucosa is different on the basic aspect that the
and establish the continuity of bone. After this, oral tissues heal usually without scar formation.
both external and internal callus remodel to form Collagen that is laid down by the ‘contractile
indistinguishable bone. fibroblast’ and ‘myofibroblast’ during healing of
the cutaneous wounds lead to rigidity.
HEALING OF OSSEOINTEGRATED It also leads to immobilization of the area
IMPLANTS with impairment of function at times. This
Implants are of three types: fibroblast has abundant actin and myosin which
• Endodontic are responsible for the wound contraction. The
• Endoosseous exact difference in the healing process between
• Subperiosteal skin and the oral mucosa is still under research,
Osseointegration is the term used for healing the main difference is the type of fibroblasts. The
of bone around an endo-osseous implant. This fibroblasts in the mucosa are said to be
results in an intimate interface between the bone phenotypically different from the fibroblasts in
and the implant. The healing process in the initial the skin. The fibroblasts in oral mucosa closely
phases is same as of the healing of the extraction resemble fetal fibroblasts. The differences are in
wound. Osteogenesis and remodeling are the the synthesis of the glycosaminoglycans and in
prime features seen during the process. response to the cytokine TGF-β.

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 CHAPTER
Teeth
9 Anomalies

DEVELOPMENTAL DISTURBANCES OF TEETH • Localized—It involves only single tooth. It

occurs with congenital heart diseases,
The scale of human tooth development Down’s syndrome and progeria
• 6 weeks (40-42 days)—Rupture of bucco- (Hutchisons–Gilford syndrome characteri-
pharyngeal membrane. zed by dwarfism and premature senility).
• 42-48 days—Dental lamina formation.
• 55-56 days—Bud stage—deciduous incisor, Clinical Features
canine and molar.
• 14th week—Bell stage for deciduous, bud stage Localized Microdontia
for permanent. Most commonly affected teeth are maxillary
• 18th week—Dentin and functional ameloblast. lateral incisors and 3rd molars. Supernumerary
• 32nd week—Dentin and functional amelo- teeth are frequently smaller than normal. One
blasts of permanent 1st molar. of the common form of localized microdontia is
peg shaped laterals in which the mesial and distal
ANOMALIES AFFECTING SIZE OF TEETH sides converges or taper incisally, forming peg
shaped or cone shaped crown. When molars are
Microdontia
involved, they may also undergo a change in
It refers to teeth that are smaller than normal. shape from five to four cusps in case of mandibular
There are three types of microdontia: molar and from four to three cusps in upper molars.
• True generalized—All the teeth are smaller
than normal. It occurs in pituitary dwarfism, Management
Down’s syndrome and congenital heart
Crown and bridge work is required for esthetic
disease.
rehabilitation of teeth.
• Relative generalized—Normal or slightly
smaller than normal teeth; are present in jaws
Macrodontia
that are somewhat larger than normal. It is
hereditary. It often exhibits spacing between It is also called as ‘megadontia’. These are the teeth
the teeth. which are larger than normal. It is of three types:

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92 Textbook of Oral Pathology

• True generalized—All the teeth are larger than

normal. It is commonly associated with
pituitary gigantism.
• Relative generalized—Teeth are normal or
slightly larger than normal, but present in a
smaller jaw.
• Localized—One or more large teeth exist in
relation to an otherwise normal dentition and
body size.

Causes
It is occasionally seen in facial hemi-hypertro-
phy, in which half of the teeth in unilateral dis-
tribution are affected. Angioma of face, pituitary
gigantism and genetic abnormalities may be
seen. Figure 9.1: Gemination is a macrodont formed when
there is partial division of tooth germ
Clinical Features
Clinical Features
Teeth are larger than normal. There is crowding,
Males and females are equally affected. The
which may result in malocclusion. As space is
commonly affected teeth are deciduous
less, there is impaction of teeth. It should not be
mandibular incisors and permanent maxillary
confused with fusion.
incisors. It appears clinically as bifid crown on
single root. It does not increase or decrease the
Management
number of teeth present.
If necessary, orthodontic treatment is done. If There are common pulp canals and either
impacted, extraction is indicated. single or partially divided pulp chambers.
Crown is wider than normal with shallow
ANOMALIES AFFECTING SHAPE OF TEETH groove extending from incisal edge to cervical
region. Enamel or dentin of crown of geminated
Gemination teeth may be hypoplastic or hypocalcified.
It refers to the process whereby, single tooth Invagination of crown occurs with complete and
germ invaginates resulting in incomplete incomplete division.
formation of two teeth that may appear as bifid Management
crown on single root. It occurs during the
Affected tooth structure should be removed and
proliferation stage of the growth cycle of tooth.
crown may be restored and reshaped. Reduction
of mesiodistal width with periodic disking. Final
Etiology
jacket crown preparation.
Hereditary and familial tendency. It results from
the splitting of a tooth germ during development Complication
or from the fusion of a normal tooth bud with a • Pulpal infection—Areas of hypoplasia and
developing supernumerary tooth (Fig. 9.1). invagination lines or areas of coronal

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 Teeth Anomalies 93

separation represent caries susceptible area,

which may lead to pulpal infection.
• Others—It may also cause malocclusion and
periodontal pathosis.

Twinning
It indicates cleavage of tooth germ which results
in formation of supernumerary teeth that is
mirror image or near image of tooth from which
it has developed.

Fusion
It is also called as ‘synodontia’. It represents the
embryonic union of normally separated tooth
germs. It represents junction at the level of dentin
between juxtaposed normal tooth germs.

Etiology
Figure 9.2: Fusion is a process in which two adjacent
It is transmitted as autosomal dominant trait
tooth germ fuse to form a macrodont
with reduced penetration. Physical force or
pressure generated during development causes Tooth is almost twice in size than normal,
contact of tooth germs. Two separate developing with or without bifid crown. Tooth may have
tooth germs being initially close together as they separate or fused root canals. Clinically there
grow and expand they contact with each other may be spacing and periodontal conditions.
and the germs fuse to varying degrees (Fig. 9.2). Dental caries is common in fused teeth. It
may result in reduced number of teeth in the
Classification jaws. When deciduous teeth fuse, the
• Complete—If fusion takes place before corresponding permanent teeth may be absent.
calcification begins, the two teeth may be
Management
completely united to form a single large
tooth. Morphology of teeth should be determined ra-
• Incomplete—If contact of teeth occurs later, i.e. dio-graphically for endodontic treatment. After
when the portion of crown has completed its endodontic treatment, tooth may be reshaped
formation then there is union of root only. with a restoration that will mimic independent
crown.
Clinical Features
Concrescence
Male to female ratio is 1:1. It is seen more
commonly in anterior teeth. It is more common It is a form of fusion that occurs after the root and
in deciduous dentition than in permanent other major parts involved in teeth are formed or
dentition. It may occur between a normal tooth when the roots of two or more teeth are united by
and a supernumerary tooth such as ‘mesiodens’ cementum, below the cementoenamel junction.
or ‘distomolar’. It is also called as ‘false gemination’.

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94 Textbook of Oral Pathology

Etiology Talon’s Cusp

It may occur due to traumatic injury, over- It projects lingually from cingulum area of
crowding of the teeth with resorption and maxillary and mandibular teeth or it is an
interdental bone loss, distal inclination of crown anomalous hyperplasia of cingulum on the
of molar, space restriction during development, lingual of maxillary and mandibular incisors,
excessive occlusal trauma and local infection resulting in the formation of supernumerary cusp.
after development.
Pathogenesis
Types
A focal proliferation of tissue during development
• True concrescence—If roots are bound during
and exuberant development of the fourth lobe
development.
(cingulum) leads to development of Talon cusp.
• Acquired concrescence—If the condition occurs
after development. Clinical Features (Fig. 9.4)
Clinical Features It may be found in both sexes and common in
It has got no sex predilection. It is common in both dentitions. It resembles like an ‘eagle’s talon’.
maxillary 2nd and 3rd molar area. Either primary It blends smoothly with the erupted tooth, except
or secondary teeth are affected. Usually involved that there is a deep developmental groove where
are only two teeth, roots are fused by cementum. the cusp blends with sloping lingual tooth surface.
Teeth may fail to erupt or incompletely erupt. It is composed of normal enamel, dentin and
There may be malocclusion or the teeth may be contains a form of pulp tissue. Cusp may or may
impacted (Fig. 9.3). not contain pulp horn and is usually ‘T’ shaped.
Patients face problems with high incidence of caries.
Management In some cases occlusal interference may be there.
Dentist must be careful while doing extraction. It is associated with Rubinstein-Taybi syndrome.

Figure 9.3: Concrescence is fusion between two teeth by Figure 9.4:Talon’s cusp (Courtesy: Dr Alka Kale, Prof and
means of cementum (Courtesy: Dr Alka Kale, Prof and Head, Head, Oral Pathology, KLES’s Institute of Dental Sciences,
Oral Pathology, KLES’s Institute of Dental Sciences, Belgaum) Belgaum)

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 Teeth Anomalies 95

Management Etiology
Removal of cusp followed by endodontic There is relative retardation in growth of a
therapy should be carried out. portion of the enamel organ, with the result that
this part remains stationary and the remainder
Dilaceration grows around it. Another view is that
It refers to angulations or sharp bends or curves invagination is due to active proliferation of an
area of enamel organ which then grows into
in the roots and crowns of the teeth.
dental papilla.
Etiology Study shows that there is presence of
extravascular fluid in the soft tissue that fills the
Mechanical trauma to calcified portion of potential invagination cavity of the tooth, before
partially formed teeth results in dilaceration. The it erupts, suggesting that there is increased
portion formed after accident is in different venous pressure within the invagination. It could
direction causing the dilaceration. Develop- be due to pressure on the blood vessels as they
mental defect and obstacle to the normal pass through the entrance channel of the
direction of growth can cause dilacerations. invagination cavity where enamel is forming
concentrically and centripetally, thus tending to
Clinical Features progressively narrow the entrance. Expansion of
It is found equally in both sexes. It is most the invagination could then result from the
commonly found in maxillary incisors. Curve or increased venous pressure and transudation
bending occurs anywhere along the length of (Fig. 9.5).
tooth, sometimes at cervical portion or midway
along the root or even just at the apex of root.
Sometimes, angles are so acute that a tooth does
not erupt. If the defect is in the crown of an
erupted tooth, the angular distortion will be
recognized.

Management
There is difficulty at the time of extraction.
Dilacerated crown has to be restored with crown
to improve esthetics and function and to
preclude dental caries and periodontal disease.

Dens in Dente
It is also called as ‘dens invaginatus’ or ‘dilated
composite odontome’ or ‘gestant odontome’.
Infolding of the outer surface of the tooth into
its interior surface occurs. It is a developmental
variation which is thought to arise as a result of
an invagination in the surface of crown before
calcification. Figure 9.5: Pathogenesis of dense invaginatus
96 Textbook of Oral Pathology

It can occur due to infection of the deciduous In moderate type pocket of enamel is formed
predecessor or trauma. Pressure on the growing within tooth, with dentin at periphery. Opening
teeth can also cause invagination. to the surface is constricted or remains open.
Food debris may become packed in this area with
Classification resultant caries and infection of pulp.
• Coronal dens invaginatus—It is anomalous In severe type it may exhibit an invagination
infolding of enamel organ into dental papilla. extending nearly to the apex of the root (Fig. 9.7).
It results in fold of hard tissue within the tooth, The crown may or may not be enlarged. The
characterized by enamel lining the fold and shape of crown may be conical or it may be of
covering the dentin peripheral to it (Fig. 9.6). irregular shape. In some cases there appears to
• Radicular dens invaginatus—It is a result of be a grossly magnified cingulum rising to the
invagination of Hertwig’s epithelial root level of the incisal edge of the tooth, but lacking
sheath resulting in accentuation of normal the normal contour of a cingulum. The labial face
longitudinal root grooves. It is lined by of the tooth is often bulbous. Some teeth with
cementum. Root sheath may bud off sac like these abnormalities are so misshapen as to defy
invagination that results in a circumscribed verbal description.
cementum defect in root.
Radicular Dens Invaginatus (Fig. 9.8)
Clinical Features
Coronal Dens Invaginatus It is more common in mandibular 1st premolar,
upper lateral incisor and second molar.
Commonly affected tooth is permanent maxillary Abnormality is usually unilateral. It occurs most
lateral incisor and maxillary central incisor. The frequently in at the site of anatomical defect.
lower incisor or cuspid is the next common site. Crown is small, short and conical with small
No sexual predilection. It is diagnosed in children orifice at the extreme summit of the convexity.
or in adolescents. It is more common in females. Lingual marginal ridge is prominent. Cavity is
This condition is frequently bilateral. separated from the pulp chamber by a thin wall
In mild type (Fig. 9.7) form there is a deep pit and opens in oral environment through very
in cingulum. narrow constriction.

Figure 9.6: Dense invaginatus (Courtesy: Dr Alka Kale, Prof

and Head, Oral Pathology, KLES’s Institute of Dental
Sciences, Belgaum) Figure 9.7: Severity of dense invaginatus
 Teeth Anomalies 97

Clinical Features
It occurs on premolar and molar teeth and
usually occurs unilaterally or bilaterally. It
develops in persons of Mongoloid ancestry. It
consists of all three dental tissues, i.e. enamel,
dentin and pulp.
It appears as a tubercle of enamel on occlusal
surface of the affected tooth. Polyp like
protuberance in central groove, on lingual ridge
of buccal cusp is seen.
There may be incomplete eruption.
Displacement of teeth with pulp exposure and
subsequent infection may present, following
Figure 9.8: Radicular dense invaginatus
occlusal wear or fracture.
Histopathological Features
Management
The lining consists of enamel and at the opening If tubercle is a cause of occlusal interference, it
of the cavity this is continuous with the enamel should be removed under aseptic conditions.
that covers the exterior of the tooth. In
invagination of severe type, pulp cavity is grossly Taurodontism
encroached upon and may be represented by a It is described in 1913 by Sir Arthur Keith. In this,
mere slit in the dentin on each side of the body of tooth is enlarged at the expense of root.
invagination cavity. Enamel lining is defective It is characterized by clinical and anatomical
owing to poor mineralization and may be totally crown of normal shape and size, an elongated
absent in the area. body and short roots with longitudinally
enlarged pulp chambers.
Management
Tooth should be restored prophylactically. Etiology

Dens Evaginatus It is hereditary in origin and usually caused by

genetically determined trait. Failure of Hertwig’s
It is also called as ‘Leong’s premolar’, ‘evaginated epithelial root sheath to invaginate at proper
odontome’ or ‘occlusal enamel pearl’. Dens horizontal level and mutation resulting from
evaginatus is a developmental condition that odontoblastic deficiency during dentinogenesis
appears clinically as an accessory cusp or globule of the root can leads to taurodontism.
of enamel on occlusal surface, between buccal
and lingual cusps of premolar. Classification by Shaw (Fig. 9.9)
• Hypotaurodont—In this condition bi or
Pathogenesis
trifurcation is near the cervical area of the
It is caused by proliferation and evagination of root. This is mild form.
an area of inner enamel epithelium during tooth • Mesotaurodont—In this bi or trifurcation occur
development process. Subsequently there is at the middle area of the root.
proliferation of odontogenic mesenchyme into • Hypertaurodont—In this condition bi-or
the dental organ. trifurcation occur at the apices of the root.
98 Textbook of Oral Pathology

Figure 9.9: Types of taurodontism

Clinical Features (Fig. 9.10)

Figure 9.10: Taurodontism showing enlarged root trunk.
It is common in early aged men but has gradually Radiograph showing enlarged pulp chamber (Courtesy:
Dr Alka Kale, Prof and Head, Oral Pathology, KLES’s Institute
decreased in incidence over last 3 millions years. of Dental Sciences, Belgaum)
It may affect either deciduous or permanent
dentition and teeth involved are invariably Enamel Pearls or Droplet: Nodules
molars. It may be unilateral or bilateral, or may It is also called as ‘enameloma’. Pearls or droplets
exhibit any combination of quadrant involve- are described as small buttons or nodules of
ment. enamel, usually about 1 mm or 2 mm in
Involved teeth tend to be of rectangular shape diameter, that form on the root, or at bifurcation
rather than the normal tapering towards root. or trifurcation of multirooted teeth.
It may associate with certain dermatological
condition like epidermolysis bullosa, otodental Etiology
dysplasia and dyskeratosis congenita. It arises from local activity of remnants of
Syndromes which are associated with this Hertwig’s epithelium, before it reduces to rests
disease are Klinefelter’s syndrome and of Malassez. It is also suggested that it may form
Trichodentoosseous syndrome. due to proliferation of group of displaced
ameloblasts or localized activity of odontoblastic
Management layer located between the Hertwig’s root sheath
No specific treatment is necessary. and developing dentin which also induces
enamel formation.
Supernumerary Roots
Clinical Features
Clinical Features
It is common in trifurcation of maxillary molars,
Teeth that are normally single rooted exhibit two followed by bifurcation of mandibular molars.
roots. Both, maxillary and mandibular molars Those found on maxillary molars are on, usually,
particularly 3rd molars, are affected showing mesial or distal aspect in contrast to those on
supernumerary roots. They develop as slender mandibular molars, which are most often on
outgrowths at the center of furcation area of buccal or lingual aspect.
molar teeth. Rarely, small pulpal extensions may reach
into center of nodule and thereby constitute
Management additional hazard. Rarely, tiny nodule may be
It assume significance only during exodontia as sufficiently close to gingival margin to become
these roots may fracture during extraction. involved in the periodontal problems.
 Teeth Anomalies 99

Histopathological Features NUMBER OF TEETH

Overgrowth may be simply a cap of enamel sitting Anodontia
on dentin and replacing cementum in that area.
It is congenital absence of teeth.
Management
Etiology
If it is causing periodontal problems, mass can
Hereditary ectodermal dysplasia, cleidocranial
be removed.
dysplasia, craniofacial dysostosis, cleft lip, and
cleft palate can cause anodontia. Genetic factors,
Globodontia
evolutionary trend towards few teeth also lead
It is common in crown of premolar and molar to anodontia. In some cases X-ray radiation can
teeth. Teeth have a round globular or clove leaf be causative factors.
appearance.
It is characteristic of otodental syndrome in Classification
which there is also deafness. Other anomalies • True
associated with it are fusion of molar and – Total (oligodontia)
premolar teeth and double pulp chamber.
– Partial (hypodontia)
• False
Mulberry Molar/Moon’s Molar/Fournier’s Molar
• Pseudo.
It is a characteristic syphilitic lesion of posterior
teeth in which hypoplastic enamel develops with Clinical Features (Fig. 9.11)
spherical aggregates or globules on the occlusal
It is higher in women and most probably in
surface.
Mongoloid, than whites. Absence may be
1st molar is commonly involved. Cusp of
unilateral or bilateral.
teeth show exaggerated rounded or nodular
In true total anodontia all teeth are missing and
shapes. It is often grossly distorted, usually
it involves both deciduous as well as permanent
narrower occlusally than cervically.
dentition.
In true partial anodontia there is absence of
Hutchinson’s Incisor
one or more teeth. Commonly missing are 3rd
Center of the incisal edge shows typical notching. molar, maxillary lateral incisor, maxillary or
An affected tooth is screwdriver shaped, with mandibular 2nd premolar.
tapering marginal ridges converging towards the
incisor edge. Teeth may show barrel shaped
outline.

Shovel-Shaped Incisors
It is morphologically an anomaly of the crowns
of incisor teeth. It is more common in the
maxillary teeth. The shovel shape is manifested
by the prominence of the mesial and distal
marginal ridges which enclose a central fossa on
the lingual surface of incisor teeth. It has Figure 9.11: Patient showing missing teeth in partial
frequently a short root. anodontia
100 Textbook of Oral Pathology

False anodontia results due to extraction of

teeth.
Pseudoanodontia refers to multiple unerupted
teeth.

Management
Patient can be managed by orthodontic
treatment and by restoration or prosthesis.

Ectodermal Dysplasia
It is also called as ‘hereditary hypohidrotic Figure 9.12: Patient having saddle nose appearance seen
(anhidrotic) ectodermal dysplasia’. It is a X-linked, in case of ectodermal dysplasia
recessive mendelian character.

Etiology
It results from aberrant development of
ectodermal derivation in embryonic life.

Clinical Features
Males are affected more frequently than females.
It is characterized by hypotrichosis, hypohidrosis
and anhidrosis with saddle nose appearance. The
hair of scalp and eyebrows tend to be fine, scanty
and blond. Supraorbital and frontal bosses are Figure 9.13: Missing teeth seen in patient with ectodermal
dysplasia
pronounced (Fig. 9.12).
Skin is often dry, soft, smooth and scaly with above reason. The palatal arch is frequently high
partial or complete absence of sweat glands. Such and cleft palate may be present. Along with all
patient can not perspire and they usually suffer this, since the salivary gland, including the
from hyperpyrexia and inability to endure warm intraoral accessory gland, are sometimes
temperature. Facial appearance of these hypoplastic, they result in xerostomia and dry
individual resemble to each other, enough to be cracked lips with fissuring at the corners of mouth.
mistaken for siblings. In some cases there may be hypoplasia of
accessory gland which will results in xerostomia.
Oral Manifestations (Fig. 9.13) There is also hypoplasia of nasal and pharyngeal
mucus gland which lead to chronic rhinitis and
Patient with this abnormality invariably manifest
pharyngitis.
oligodontia or partial absence of teeth, with
frequent malformation of any present tooth in
Histologic Finding
deciduous and permanent dentition. Teeth
which are present are usually cone shaped. There is reduction in the number of sweat glands
There is reduction of normal vertical and hair follicles. Epidermis is thin and flattened.
dimension of alveolar process as there is absence Mucous gland in upper respiratory tract are
of teeth. There is also protuberance of lip due reduced in number.
 Teeth Anomalies 101

Management
From dental point of view partial and complete
dentures should be constructed for both
functional and cosmetic purpose.

Supernumerary Teeth
It is also called as ‘hyperdontia’.

Etiology
A supernumerary tooth develops from 3rd tooth
Figure 9.14: Supernumerary teeth seen in upper anterior
bud arising from dental lamina near the region
permanent tooth bud or possibly from splitting
of the permanent bud itself. It is inherited as an Paramolar: It is supernumerary molar, usually
autosomal dominant trait, when associated with small and rudimentary and is situated buccally
syndromes. It is inherited as an autosomal or lingually to one of the maxillary molars or
recessive trait when associated with only inter-proximally between 1st, 2nd and 3rd
supernumerary teeth. maxillary molars.
Peridens: Supernumerary teeth that erupt
Types
ectopically, either buccally or lingually to the
• Supplemental supernumerary teeth—These normal arch are referred as peridens.
teeth duplicate the typical anatomy of
posterior and anterior teeth. Clinical Features
• Rudimentary supernumerary teeth—These are
Males are affected more than females. Male to
dysmorphic and can assume conical forms.
female ratio is 2:1. It may occur in both dentitions,
Most Common Supernumerary Teeth are the but frequently found in permanent dentition and
follows: more often in mandible. It may be erupted or
impacted and occurs in 1% of the population.
Mesiodens: It is located at or near the midline in
Supernumerary teeth occurs most often with
the incisal region of maxilla between central
cleft palate. Supernumerary teeth which occur
incisors. It may occur singly or paired, erupted
in the bicuspid region, most frequently in the
or impacted or even inverted. It is a small tooth
lower jaw, are commonly well formed and
with cone shaped crown and short root. It may
resemble the normal teeth.
cause retarded eruption, displacement or
Their form is normal or conical or can be just
resorption of adjacent root. It frequently causes
arranged as masses of dental tissue.
improper alignment (Fig. 9.14).
Supernumerary teeth frequently prevent
Distomolar: It is found in molar region permanent teeth from erupting or cause them to
frequently located distal to 3rd molar. erupt in an abnormal direction or site, even
Generally, these teeth are smaller than normal without being any contact between them. It is
2nd and 3rd molar. General crown morphology associated with cleidocranial dysplasia, orofacial
is highly abnormal. digital syndrome and Gardner’s syndrome.
102 Textbook of Oral Pathology

Management Histological Features

It depends on potential effect on normal denti- Most of the crowns of natal and neonatal teeth
tion, their position, number and complications are covered with hypoplastic enamel with
that may result from surgical removal. If re- varying degrees of severity. Absence of root
quired, they should be extracted. formation, ample vascularized pulp, irregular
dentin formation and lack of cementum
Predeciduous Dentition formation are characteristic features.
It is also called as ‘congenital teeth’, ‘fetal deciduous
teeth’ and ‘natal and neonatal teeth’. Management
Extraction of the teeth, if it is causing inconve-
Etiology
nience during suckling, interference with
Eruption accelerated by febrile incident or breastfeeding and causing traumatic injury.
hormonal stimulation. Extraction should be done after 10 days of life.
The other option that may be used is rounding
Classification of the sharp angle of incisal edges of teeth. If not
• Mature: They are fully developed in shape necessary, tooth should not be removed.
and comparable in morphology to the
primary teeth. Prognosis is relatively good. Riga-Fede Disease
• Immature: Their structure and development It is a complication from natal and neonatal teeth.
is incomplete. Poor prognosis of teeth. There is ulceration of the ventral surface of the
tongue caused by the sharp incisal edges. It leads
Clinical Features to interference with proper suckling and feeding
Natal Teeth: There is premature eruption of teeth and thus the neonate is at risk of nutritional
or teeth like structures that are present at birth. deficiency.
They are hyper mobile because of their limited
Post-permanent Dentition
root development. Within relatively short time,
premature erupted tooth will become stabilized Person who have all the permanent teeth
and other teeth of the arch are erupted. Teeth extracted and yet have subsequently erupted
may be conical or may be normal in size and teeth, particularly after insertion of complete
shape and opaque yellow-brownish in color. denture come in this category. They possibly
Teeth appear to be attached to a small mass of develop from bud of dental lamina beyond the
soft tissue. permanent tooth germs.
Some teeth are so much mobile that there is
STRUCTURE OF TEETH
danger of displacement and possible aspiration
and in this case, removal is indicated. Sharp Amelogenesis Imperfecta
incisal edges of tooth may cause laceration of
lingual surface of tongue or may interfere with It is also called as ‘hereditary enamel dysplasia’,
‘hereditary brown enamel’ and ‘hereditary brown
nursing. It may associate with syndromes like
opalescent teeth’.
Ellis-Van Creveld syndrome and cleft palate.
It represents group of hereditary defects of
Neonatal Teeth: They are teeth or teeth like enamel associated with any other generalized
structures that erupt prematurely during defect. It is an ectodermal disease and
neonatal period, from birth to 30 days. mesodermal component is normal.
 Teeth Anomalies 103

Classification Local: Teeth in both the dentitions are affected.

Horizontal rows of depressions or one large
Classification depends upon the stage of at which
hypoplastic area with hypocalcification adjacent
the disease occurs. This classification is given by
to and below the hypoplastic area is found.
Witkop (J Oral Pathol 1989;17:547-53). Accord-
Defects are most prominent on buccal surfaces
ing to it, there are mainly four type of
of the teeth, involving middle 3rd of enamel.
amelogenesis imperfecta.
Incisal or occlusal surfaces of the teeth are usually
• Hypoplastic type: There is defective formation
not involved.
of enamel matrix.
– Autosomal dominant Smooth: Enamel is thin, hard and glossy with
- Pitted smooth surface. Enamel is 1/4th to 1/8th of its
- Local normal thickness. On newly erupted teeth they
- Smooth have yellow color, but may vary from opaque
- Rough white to translucent brown. Some of the enamel
– Autosomal recessive may be missing on newly erupted teeth, especially
- Local on the incisal and occlusal surface and may be
- Enamelogenesis chalky in inter-proximal areas. Delay in eruption
– X-linked dominant occurs with resorption of teeth in the alveolus.
• Hypocalcification type: There is defective Rough: Both primary and secondary teeth are
mineralization of formed matrix. affected. Enamel is hard with rough granular
– Autosomal dominant surface that may be chipped from underlying
– Autosomal recessive dentin, rather than abrade away as seen with
• Hypomaturation type: In this enamel crystal smooth type. Enamel is 1/4th to 1/8th in
lattice remains immature. thickness. Teeth are white to yellow-white when
– X-linked recessive newly erupted. Teeth do not meet at contact
– Autosomal recessive – pigmented points but retain normal tooth outline than the
– Snow-capped teeth autosomal dominant smooth type of imperfecta. May have thicker
• Hypomaturation/ hypoplastic with taurodontism. enamel at cervical areas. There is also anterior
open bite present.
Clinical Features
Autosomal Recessive Type
Hypoplastic Type
Teeth which are erupted have distinct yellow
It includes localized portions of enamel that do
color like of normal dentition. Surface is rough
not reach normal thickness during development.
and granular, resembling ground glass. Nearly
complete lack of enamel formation. Teeth are
Autosomal Dominant
widely spaced and do not meet each other at
Pitted: Both the dentitions are affected. It appears contact point. Patient may have anterior open
as thin enamel on teeth that do not contact with bite. Numerous teeth are missing in the dentition
each other mesiodistally. Pinpoint to pinhead and represent radiographically as unerupted
pits randomly distributed over the surface. teeth undergoing resorption.
Enamel on newly erupted teeth is hard with
normal yellow white color. Staining of teeth Hypocalcified
occurs after exposure to oral environment, giving The enamel is so soft that it can be removed by a
teeth a black appearance. prophylaxis instrument.
104 Textbook of Oral Pathology

Autosomal Dominant
Enamel is of normal thickness, although
occasional areas of hypoplasia are seen on
middle 3rd of labial surface. The enamel is so
soft that it may be lost soon after eruption,
leaving crown composed of only dentin. Enamel
has cheesy consistency and can be scraped from
dentin with an instrument or penetrated easily
by dental explorer. Newly erupted teeth are
covered with dull lusterless opaque, white, honey
colored or yellowish orange or brown enamel. Figure 9.15: Amelogenesis imperfecta showing exposed
Exposed dentin may be hypersensitive. dentin and yellow enamel
Anterior open bite may be present. Patients with
this condition are prone to form calculus rapidly. tends to form large amount of calculus which may
contain pigment forming agents. Teeth may be
Hypomaturation Type seen undergoing resorption within alveolus.

The enamel can be pierced by an explorer point Snow Capped Teeth

under firm pressure and can be lost by chipping
away from the underlying, normal appearing Maxillary teeth are affected more commonly
dentin. than mandibular teeth. Both primary and
secondary dentitions are affected. In this
Autosomal Dominant condition varying amount of enamel on incisal
or occlusal aspect of crown is present and has
It is more commonly found in males. Both opaque white appearances. Opacity may be solid
primary and permanent dentitions are affected. or flecked and may involve enamel surface.
Permanent teeth are mottled yellow white in Junctional line of opaque white and translucent
color, but may be darkened with absorption of enamel is sharp. Pattern of defect on teeth
stains. Primary teeth of affected males have anterior to the posterior teeth resemble that
ground glass opaque white appearance. Patient which would be obtained when ‘dipped into white
occasionally shows slight yellow cast to enamel paints’.
surface. Teeth meet at contact points and have
normal contour. Enamel approaches normal Histopathological Features
thickness, but it may be thinner. Point of explorer
Hypoplastic type: There are disturbances in
can be forced into enamel.
differentiation or viability of ameloblasts in
Autosomal Recessive hypoplastic type.

Both primary and permanent dentitions are Hypocalcification type: In hypocalcification, There
affected. Enamel has milky to shiny, agar brown is defect in matrix structure and mineral
deposition.
color on newly erupted teeth. It may become
more deeply stained on contact with exogenous Hypomaturation type: In hypomaturation type,
agents. Teeth are of normal thickness and tend to there is alteration in enamel rods and rod sheath
chip away, especially around restoration. Patient structure.
 Teeth Anomalies 105

Management resulting from lack of proper calcification of

enamel matrix. Horizontal pitting occurs in rows,
Cosmetic improvement should be done.
on the teeth undergoing matrix formation at the
Enamel Hypoplasia time of dietary deficiency or during course of
febrile episode. Pitting characteristically picks up
It is an incomplete or defective formation of stain and discoloration occurs (Fig. 9.16).
organic enamel matrix. Local and systemic
factors that interfere with the normal matrix Hypoplasia due to exanthematous disease: It includes
formation can cause enamel surface defects and measles, chickenpox and scarlet fever. There is
irregularities. temporary elevation of body temperature.
Temperature may remain elevated for prolonged
Classification period of time and under these circumstances,
ameloblasts may be adversely affected.
1st
• Hereditary: Same as amelogenesis imperfecta. Hypoplasia due to congenital syphilis: It involves
• Environmental: It is caused by external agents. maxillary and mandibular permanent incisors
and 1st molars. Incisors affected are called as
2nd
‘Hutchinson incisors’ and molar are called as
• Mild: There may be only few small grooves, ‘mulberry molar’s (Moon molar, Fournier’s molar).
pits and fissures on enamel surface.
Hutchinson’s incisors are upper central incisor is
• Moderate: Enamel exhibits rows of deep pits
screw driver shaped. Mesial and distal surfaces
arranged horizontally across the surface. of crown are tapering and converging towards
• Severe: Considerable portion of enamel may
incisal edge of the tooth, rather than towards
be absent.
cervix. In addition, incisal edge is also notched.
The cause behind this is the absence of the central
Types of Environmental Hypoplasia
tubercle or calcification center. In mulberry molar
• Hypoplasia due to nutritional deficiency crown of 1st molar in congenital syphilis is
• Hypoplasia due to exanthematous disease irregular. Enamel of the occlusal surface and
• Hypoplasia due to congenital syphilis occlusal third of the tooth appears to be arranged
• Hypoplasia due to hypocalcemia in agglomerate mass of globule, rather than in
• Hypoplasia due to birth injury
• Hypoplasia due to local infection or trauma
• Hypoplasia due to tetracycline
• Hypoplasia due to chronic lead poisoning.

Clinical Features
Hypoplasia due to nutritional deficiency: It occur
due to deficiency of vitamin A, C, D, calcium and
phosphorus. Two-thirds of this occurs during
infancy period or early childhood. Frequently
involved are those teeth which are formed
within the first year after birth. Vitamin D Figure 9.16: Hypoplasia occur due to nutritional
deficiency causes rickettsial phenomenon, deficiency presented as horizontal pitting
106 Textbook of Oral Pathology

well formed cusp. The crown is narrower on Hypoplasia associated with tetracycline ingestion: It
occlusal surface than at the cervical margin. may be incorporated in calcifying enamel matrix
Hypoplasia due to hypocalcemia: Tetany induced by formation of a tetracycline calcium
orthophosphate complex. After teeth eruption
by decreased level of calcium in the blood, which
and exposure to sunlight, discoloration may
is as low as 6 to 8 mg per ml. As calcium is
required for normal tooth formation, there is result, ranging from light yellow to brown.
Varying degree of hypocalcification may also
defective formation of the enamel. Enamel
exist. Tetracycline should not be administered
hypoplasia in it is usually of ‘pitting’ variety.
during pregnancy and until the child become 8
Hypoplasia due to birth injury: In prenatal type years old.
marked enamel hypoplasia affects incisal 2/3rds
of enamel on maxillary primary incisors. It is due Hypoplasia associated with chronic lead poisoning:
to gastrointestinal tract disturbances or metabolic It is more common in children with low
disorders in the fetal life, probably during 2nd economic status. Fetus of lead poisoned mother
and 3rd trimester of pregnancy. In neonatal type can be affected because lead readily crosses the
a wide band or line of enamel affects the primary placenta during pregnancy. Pitting type of
teeth of children associated with premature birth hypoplasia is more common in cases of lead
or low birth weight. In traumatic birth, it may poisoning.
affect the process of amelogenesis. Management
Hypoplasia due to local infection or trauma: Most
The hypoplastic teeth are more susceptible to
commonly affected teeth are permanent
dental caries than the normal teeth. The
maxillary incisor or maxillary or mandibular
restoration is usually confined to area of
premolar. Localized type of hypoplasia caused
involvement. Chrome steel crown is given in case
by local infection or trauma is called as ‘turner’s
of severe hypoplasia. Eight percent stannous
hypoplasia’ and that tooth is called as ‘Turner’s
fluoride has been found to decrease the sensitivity
tooth’. There may be any degree of hypoplasia,
of teeth which may be due to exposed dentin.
ranging from mild brownish discoloration of
enamel to severe pitting and irregularity of the Mottled Enamel or Dental Fluorosis
crown. If deciduous teeth become carious during
Drinking water that contains in excess of 1 PPM
the period when the crown of succeeding
(part per million) fluoride can affect the
permanent tooth is formed, bacterial infection
involving periapical tissues may occur and this ameloblasts during the tooth formation stage and
can cause the clinical entity called as ‘mottled
may disturb the ameloblastic layer of permanent
enamel’. It is due to disturbance in tooth
tooth bud, resulting in hypoplastic crown. When
deciduous teeth have been driven into alveolus formation caused by excessive intake of fluoride,
during the formative period of dentition.
and have disturbed the permanent tooth bud
and if this permanent tooth bud is still being
Pathogenesis
formed, the resulting injury may be manifested
as yellowish or brownish stains or pigmentation • Formative stage: Disturbance of ameloblasts
of enamel, usually on labial surface or as true during the formative stage of tooth
hypoplastic pitting defect. Hypoplastic defect development and higher level of fluorides
may contain cementum, which may be stained interfere with the calcification process of
yellowish brown. matrix.
 Teeth Anomalies 107

• Matrix formation stage: There is diminished

matrix production, change of matrix
composition and change in ion transport
mechanism.
• Maturation stage: In maturation phase, there
is diminished withdrawal of protein and
water.

Clinical Features
Dental fluorosis in primary dentition is less
severe as compared to permanent dentition. It
frequently becomes stained as unsightly yellow
to brown color, which is caused by coloring Figure 9.17: Hypoplasia occur due to fluoride toxicity
showing corroded appearance
agents from food, medicine and by
disintegration of the increase protein contain in
• Score 2—The opaque whites flecks are more
the hypomineralized parts of the enamel.
Sometimes, white patches in enamel may pronounced and frequently merge to form
become striated, pitted and mottled. small cloudy areas scattered over the whole
The range of severity and appearances surface.
changes: • Score 3—Merging of the white lines occurs
• Questionable changes: It is characterized by and cloudy areas of opacity occur to spread
occasional white flecking or spotting of into many parts of the surface. In between
enamel. the cloudy area, white lines can also be
• Mild changes: It is manifested by white seen.
opaque areas involving more of the tooth • Score 4—The entire surface exhibits a marked
surface. opacity or appears chalky white.
• Moderate and severe: Changes showing pitting • Score 5—The entire surface is opaque and
and brownish staining of the surface and there are round pits that are less than 2 mm
sometimes even corroded appearance. Teeth in diameter.
which are moderately or severely affected • Score 6—The small pits may frequently be seem
may show tendency for wear or even fracture merging in the opaque enamel to form bands
of enamel (Fig. 9.17). that are less than 2 mm in vertical height.
• Score 7—There is loss of the outermost enamel
Clinical Classification for Fluorosis in irregular areas and less than half of surface
• Score 0 —The normal translucency of the is involved.
glossy creamy-white enamel remains after • Score 8—The loss of the outermost enamel
wiping and drying of the surface. involves more than half of the enamel. The
• Score 1—Thin white opaque lines are seen remaining intact enamel is opaque.
running across the tooth surface. The lines • Score 9—The loss of the major part of outer
correspond to the position of perikymata. In enamel results in change of the anatomic
some cases ‘snow-capping’ of cusps may also shape of the surface. A cervical rim of opaque
be seen. enamel is often noted.
108 Textbook of Oral Pathology

Histopathological Features Features of this condition are multiple bone

fractures, hyperextensible joints, blue sclera and
Enamel of these teeth has been described as
progressive deafness. Deciduous teeth are more
hypomineralized with an irregular prism pattern
severely affected than permanent teeth. Color of
and with scalloped or arcading pattern of
teeth may vary from brownish violet to yellowish
enamel. DE junction is more pronounced than
brown. Amber translucency of both primary and
in normal teeth.
permanent dentition may be seen. Enamel may
be lost and dentin undergoes rapid attrition.
Management
Usual scalloping of dentinoenamel junction is
Bleaching with 30% H2O2 (hydrogen peroxide)— absent. The teeth are short than normal often
This technique is enhanced by microabrasion or markedly, in respect to the roots and crowns. In
grinding of the surface layer. the incisor region the crowns tend to more nearly
Calcium sucrose phosphate gel—Treatment involves square, but the mesial and distal borders are
cleaning the affected teeth with pumice and glyc- sometimes curve. The bicuspids and molars are
erin, rinsing with water and applying 37% phos- flatter than normal and the normal
phoric acid for 1½ or 2 minutes. The treatment is circumferential curves are accentuated so that the
repeated followed by application of 2% sodium teeth present bulbous appearance. The neck of
fluoride for 4 minutes. Finally, a thick layer of 40% teeth suddenly narrows down. The appearance
calcium sucrose gel is placed on the affected teeth. of crowns may be described as ‘dumpy’.
The patient was instructed not to rinse or eat for Shield type II —It is inherited as an autosomal
30 minutes. dominant trait. Both dentitions are affected.
Other clinical features are same, except they are
Dentinogenesis Imperfecta somewhat of severe form and it is not associated
There are various names for dentinogenesis with osteogenesis imperfecta.
imperfecta like ‘hereditary opalescent dentin’ and Shield type III —It is also inherited as an
‘odontogenesis imperfecta’. Affected teeth are of tulip autosomal dominant trait. Both the dentitions are
shape (broad crown with constriction are the cervical affected. Opalescent color, bell shaped crown
area). and multiple pulp exposure.

Classification
• Shield type I—Dentinogenesis imperfecta
always occurs with osteogenesis imperfecta.
• Shield type II—It is also called as ‘hereditary
opalescent dentin’. It does not occur in
association with osteogenesis imperfecta.
• Shield type III—It is also called as ‘Brandywine
type’. It has got shell teeth appearances and
multiple pulp exposure.

Clinical Features (Fig. 9.18)

Shield type I—It segregates as an autosomal Figure 9.18: Exposed dentin, stained dark brown
dominant trait with variable expressivity. suggestive of dentinogenesis imperfecta
 Teeth Anomalies 109

Histopathology Features and translucent. There is malalignment and

malpositioning due to extreme mobility. Minor
• Enamel is Normal
trauma may result in exfoliation.
• Features of dentin—It is composed of irregular
dentinal tubules, with large areas of Shield type II—Significant differences in color in
uncalcified matrix. Cellular inclusions like both the dentitions. Primary teeth with yellow,
odontoblasts are common with obliteration brown, bluish, grey-amber translucent
of pulp chamber. Odontoblasts degenerate appearances. Permanent teeth of normal color.
rapidly, becoming entrapped in the matrix. Obliteration of pulp chamber does not occur
Tubules are larger in diameter and less before eruption.
numerous than normal, in a given volume of
dentin. Atypical odontoblast cells occur at the Histopathological Features
surface of pulp.
Shield type I — Coronal dentin is normal and
apically there are areas of tubular dentin which
Management
obliterates the pulp. Normal dentinal tubule
Cast metal crown in posteriors and jacket crown formation appears to be blocked so that new
in anterior can be given. dentin forms around obstacle and takes the
characteristic appearance described as ‘lava
Dentin Dysplasia flowing around boulder’. Electron studies (Sauk)
It is a rare disturbance of dentin formation, this pattern results due from repetitive attempts
characterized by normal but atypical dentin to form root structure.
formation, with abnormal pulp morphology. Shield type II — Deciduous teeth exhibit defective
dentin and permanent teeth show relatively
Classification normal dentin. In radicular portion dentin is
According to Shield (clinical) amorphous and atubular and in coronal portion
• Shield type I – dentin dysplasia dentin is relatively normal. In permanent teeth
• Shield type II – anomalous dysplasia. pulp contain multiple pulp stones or denticles.

According to Witkop (radiological) Management

• Radicular dentin dysplasia
• Coronal dentin dysplasia. Prosthetic replacement should be done for
esthetic point of view.
Etiology
Regional Odontodysplasia
Hereditary and autosomal dominant trait can
lead to dentin dysplasia. It is also called as ‘odontogenic dysplasia’ or ‘ghost
teeth’. It is a localized arrest in tooth
Clinical Features development.

Shield type I—It is also called as ‘rootless teeth’,

Clinical Features
‘non-opalescent and opalescent dentin’ and ‘radicular
dentin dysplasia’. Permanent and primary teeth Deciduous and permanent teeth are involved.
are of normal size, shape and consistency. Maxillary involved more than mandibular. Most
Affected teeth are occasionally slightly amber frequently affected are permanent central incisor,
110 Textbook of Oral Pathology

lateral incisor and cuspid. Single tooth or several Histological Features

teeth in one quadrant are affected. Affected teeth
It is easily detected in both, ground section and
are small and mottled brown. Shape is irregular
decalcified histological matrix.
with evidence of defective mineralization.
Anomalies associated with eruption of the
Affected teeth are susceptible to caries, local
teeth.
infection and prone to fracture. There is either
delay or total failure of eruption. All the elements
Eruption of Teeth
of tooth are hypocalcified and hypoplastic.
The axial or occlusal movement of tooth from its
Histopathological Features developmental position within the jaw to its
functional position in the occlusal plane is known
There is marked reduction in amount of dentin
as eruption of teeth.
with widening of predentin layer. Large areas
There are three types of movements:
of interglobular dentin and irregular tubular
pattern of dentin is also evident. Reduced enamel
Pre-eruptive
epithelium of unerupted teeth shows irregular
calcified bodies. When deciduous tooth germ first differentiates,
there is good deal of space between them. But
Management due to their rapid growth, this available space is
Extraction of teeth followed by prosthetic utilized and developing teeth become crowded
together, especially in incisor and canine region.
appliances should be done.
This crowding is relieved by growth in length of
Dentin Hypocalcification infant jaws, which provides room for second
deciduous molars to drift backward and anterior
In this condition normal dentin is calcified by teeth to drift forward. At the same time, the tooth
deposition of calcium salts in the organic matrix germ also moves outward as jaw increases in
in the form of globules, which increases in size width and height. Permanent teeth with
by further peripheral deposition of salts, until deciduous predecessors also undergo complete
all the globules are finally united into a movement before they reach the position from
homogenous structure. which they will erupt.
As their deciduous predecessors erupt, they
Etiology
move to a more apical position and occupy their
Etiological factors responsible for dentin own bony crypt. Premolars begin their
hypocalcification are same as that of environmen- development lingual to their predecessors at the
tal hypocalcification of enamel. level of occlusal surface and in same bony crypt.
They are situated beneath the divergent roots of
Pathogenesis deciduous molars. The permanent molars which
In dentinal hypocalcification, there is failure of do not have predecessors also move from the site
union many of these globules, leaving of their initial differentiation.
interglobular areas of uncalcified matrix.
Eruptive
Clinical Features
There is axial or occlusal movement of tooth from
There is no alteration in their clinical appearance. its developmental position within the jaw to its
 Teeth Anomalies 111

final functional position in the occlusal plane. It cushion–hammock ligament, straddling the base
is important to recognize that jaw growth is of the socket from one bony wall to the other
normally occurring while most teeth are like a sling. But the structure described as the
erupting, so that movement in plane other than cushion-hammock ligament is the pulp
axial is superimposed on eruptive movement. delineating membrane that runs across the apex
of the tooth and has no bony insertion. So it can
Posteruptive not act as a fixed base.
These movements are those that maintain the
Vascular Pressure
position of the erupted tooth while the jaw
continued to grow and compensates for proximal It states that there is a higher pressure system
and occlusal wear. either within or around the base of tooth. It is
known that teeth move in synchrony with
THEORIES OF TOOTH ERUPTION arterial pulsation, so local volume changes can
produce limited tooth movement. Whether such
Bone Remodeling pressure is prime for movement of teeth is
It supposes that selective deposition and debatable because surgical excision of the root
resorption of bone brings about eruption. In and therefore the local vasculature does not
experiments, where tooth germ is removed and prevent tooth eruption.
the follicle is left in position the eruptive pathway
still forms in bone. Thus, this indicates the dental Periodontal Ligament Traction
follicle and not bone as major determinant in There is good deal of evidence that eruptive force
tooth eruption.
resides in the dental follicle-periodontal ligament
complex. As long as periodontal tissue is
Root Growth
available tooth movement occurs. Tissue culture
Root formation is also unlikely to be the cause of experiments have shown that ligament fibroblast
tooth eruption; as the onset of root formation is is able to contract a collagen-gel which in turn
not synchronous with onset of axial tooth brings about movement of a disc of root tissue
movement. It causes overall increase in length attached to that gel.
of tooth that must be accompanied by root Thus, there is no doubt that periodontal
growing in the bone of jaw by an increase in jaw ligament fibroblasts have the ability to contract
length or by crown moving occlusally. But it is and transmit a contractile force to the
not accepted. For example, if erupting tooth is extracellular environment and in particular to
prevented from erupting by pinning it to the the collagen fiber bundles in vitro. The entire
bone, root growth continues and is surrounded morphological features exist in vivo to permit
by resorption of bone at base of socket. Thus, similar movement. In summary, eruptive
although it can produce force, root growth can movement is brought about by a combination of
not be translated into eruptive tooth movement events involving a force initiated by the
unless there is some structure at the base of tooth, fibroblasts. This force is transmitted to the
capable of withstanding this force. But no such extracellular compartment via fibronexuses and
structure is exists. to collagen fiber bundles; which aligned in an
Advocates of the root growth theory appropriate inclination brought about by root
postulated the existence of a ligament, the formation, bring about tooth movement. These
112 Textbook of Oral Pathology

fiber bundles must have the ability to remodel for Management

eruption to continue and interference with this
Extract the primary teeth and use space
ability affects the eruptive process. The removal
maintainers until the permanent tooth erupts.
of bone to create the eruptive pathway is also
dictated by the tissue surrounding the tooth.
Embedded and Impacted Teeth

Premature Eruption Embedded teeth are those which are unerupted,

usually because of lack of eruptive force.
The teeth erupted in the oral cavity at the time
of birth are called as ‘natal teeth’ and those teeth Impacted teeth are those prevented from erupting
erupting prematurely in 1st 30 days of life are by some physical barrier in eruption path.
called as ‘neonatal teeth’.
Etiology
Etiology
Lack of space due to crowding of dental arch and
There is familial pattern is seen. Secretion of premature loss of deciduous teeth with
several endocrine organs like thyroid, adrenals subsequent partial closure of the spaces they
and gonads may alter the eruption rate of teeth. occupied. Rotation of tooth bud results in teeth
Endocrinal disturbances and adrenogenital which are aimed at wrong direction because their
syndrome. axis is not parallel to normal eruption path. Some
systemic disease like osteopetrosis, ectodermal
Clinical Features dysplasia, cleidocranial dysostosis, rickets and
cretinism can be associated with impactions.
Usually only one or two teeth erupt early and
most often deciduous and mandibular central
Clinical Features
incisors. They are often well formed and normal
in all aspects, except they may be mobile. Most commonly affected teeth are maxillary and
Premature loss of deciduous teeth may give rise mandibular third molars and maxillary cuspids,
to premature eruption of permanent teeth. followed by the premolars and supernumerary
teeth. Teeth may be impacted distally, mesially,
Delayed Eruption horizontally, etc.
Dentigerous cyst may be associated with
Etiology
impacted teeth and may cause displacement and
Systemic disease like rickets, cretinism and destruction of bone. Periodontal pocket forma-
cleidocranial dysplasia and local factors like tion and subsequent infections may occur. Be-
fibromatosis gingiva in which dense connective cause of location, impacted tooth may cause re-
tissue does not permit the eruption of teeth. sorption of roots of adjacent teeth. There may be
periodic pain and trismus when infection occurs
Clinical Features around the partially impacted teeth. Referred
Individual permanent teeth are observed to be pain from impacted teeth is also been described.
delayed in eruption. There may be partially
Management
impacted permanent teeth. There is deviation in
the eruption path of teeth. Patients may suffer It depends upon the tooth involved. In some
from pseudoanodontia. cases, like in maxillary cuspids, orthodontic
 Teeth Anomalies 113

treatment with surgical exposure can be done to arch, i.e. two teeth apparently exchanging their
bring the tooth in normal occlusion. Surgical position.
removal can be done.
Clinical Features
Ankylosis or Submerged Teeth
Teeth often exchange their positions. Permanent
Submerged teeth are deciduous teeth that have canine is most often involved, with its position
undergone variable degree of root resorption and interchanged with lateral incisor. Second
then have become ankylosed to bone. Ankylosis premolar is infrequently found between first and
of the teeth should be considered as interruption second molar. Transposition of central and
in rhythm of eruption. Unerupted permanent lateral incisor is rare. Transposition does not
teeth may become ankylosed by enostosis of occur in primary dentition.
enamel.
Management
Etiology
These teeth can be prosthetically altered to
Observations of ankylosed teeth in the same
improve function and esthetics.
family are very frequent. In some cases infections
and disturbed local metabolism.
Eruption Sequestrum
Clinical Features
Etiology
Most commonly affected are mandibular
As the molar teeth erupt through bone, they will
deciduous second molars, followed by anterior
occasionally separate small osseous fragments
teeth. Exfoliation and subsequent replacement
by permanent teeth is prevented due to of bone like corkscrew. If bony spicule is large
or eruption is fast, complete resorption can not
ankylosis. Patient who has one or two ankylosed
occur.
teeth is more likely to have other teeth ankylosed.
Gradual loss of occlusal plane as the tooth is
submerged below the level of occlusion. Teeth Clinical Features
affected lack mobility even after root resorption. It is a tiny irregular spicule of nonviable bone
On percussion it produces characteristic solid overlying the crown of an erupting permanent
sound in contrast to dull, cushioned sound of molar. Spicule directly overlies the central
normal teeth on percussion. occlusal fossa, but is contained within the soft
There may be development of malocclusion, tissues. As the tooth continues to erupt, the cusps
local periodontal disturbances and dental caries emerge and the fragments of bone completely
occurs. sequestrate through mucosa and are lost.
Child may complain of slight soreness
Management
produced by compression of soft tissues over the
Keep tooth under observation. If required, spicule, by the movement of the spicule in the
surgical excision is carried out. soft tissue crypt during mastication and
following eruption through mucosa. For few
Transposition days, fragment of bone may be seen lying on
Tooth may be found occupying an unusual crest of ridge in a tiny depression which can be
position in relation to other teeth, in the dental easily removed.
114 Textbook of Oral Pathology

Management Management
Removal of spicule should be done, if it is needed. No treatment, except to correct the etiological
factors.
Ectopic Eruption
REGRESSIVE CHANGES OF THE TEETH
Etiology
If the tooth is larger than the normal mean size Attrition
of all maxillary primary and permanent teeth It is the physiologic wearing away of teeth
and smaller maxilla or posterior positioning of because of tooth to tooth contact, as in
maxilla in relation to the cranial base can lead to mastication. It plays an important physiological
ectopic eruption. Some local factors like role as it helps to maintain an advantageous
abnormal angulation and delayed calcification crown-root ratio and gains intercoronal space of
also lead to ectopic eruption. 1 cm, which facilitates third molar eruption.

Clinical Features Types

It occurs most frequently in boys than girls. Physiological attrition—Attrition which occur due
Eruption of permanent 1st molar into roots of to normal aging process, due to mastication.
primary 2nd molar may cause destruction of
distal root of maxillary 2nd molar. It may become Pathological attrition—It occurs due to certain
hopelessly locked and produce premature abnormalities in occlusion, chewing pattern or
exfoliation. due to some structural defects in teeth.

Management Etiology Factors for Pathological Attrition

You should brass looped wires in contact area. Development—Malocclusion and crowing of
teeth, may lead to traumatic contact during
Premature Exfoliation chewing, which may lead to more tooth wear.

Etiology Acquired—Due to extraction of teeth. Extraction

causes increased occlusal load on the remaining
• Syndrome—It is seen in Papillon lefevre teeth, as the chewing force for the individual
syndrome. remains constant.
• Hereditary—It occurs in familial juvenile
periodontitis and familial fibrous dysplasia. Abnormal chewing habits—Parafunctional
chewing habit like bruxism and chronic
• Nutritional disorders—Like hypophosphatasia
persistent chewing of coarse and abrasive food
and cyclic neutropenia
or other substances like tobacco.
• Others—Histiocytosis and acrodynia.
Occupation—In certain occupations, workers are
Clinical Features exposed to an atmosphere of abrasive dust and
can-not avoid it getting into mouth.
There is widespread loss of supporting alveolar
bone. There is also loosening, migration of the Salivary factor—Can also play role in attrition.
teeth. In some cases there is also spontaneous Structural defect—In defects like amelogenesis
loss of teeth. imperfecta and dentinogenesis imperfecta,
 Teeth Anomalies 115

hardness of enamel and dentin is reduced and further complicated by forward posturing of
such teeth become more prone to attrition. mandible. It is often observed, however, that
despite overall tooth surface loss, the freeway
Clinical Features space and the resting facial height appear to
remain unaltered primarily because of
Men usually exhibit more severe attrition than
dentoalveolar compensation. This is important
women due to greater masticatory force. It may
with respect to patient assessment. If restoration
be seen in deciduous as well as permanent
of worn teeth is being planned then the extent
dentition. It occurs only on occlusal, incisal and
of dentoalveolar compensation would appear to
proximal surfaces of teeth. Severe attrition is
determine the dentist’s strategy; defining the
seldom seen in primary teeth, as they are not
need to carry out measures such as crown
retained for any great period. Lingual cusps of
lengthening, to ensure the same vertical
maxillary teeth and buccal cusps of lower
dimension of occlusion and freeway space.
posterior teeth show most wear. The first clinical
Smooth wearing of incisal and occlusal surfaces
manifestation of attrition is the appearance of
of involved teeth is evident by shortened crown
small polished facet on a cusp tip or ridge and
image. Sclerosis of pulp chamber and canals is seen
slight flattening of an incisal edge.
due to deposition of secondary dentin which
Physiological attrition beings with wearing of
narrows the pulp canals. Widening of periodontal
the incisal edge of an incisor, which is followed
ligament space and hyper-cementosis. In some
by the palatal cusp of maxillary molars and
cases there is loss of alveolar bone.
buccal cusp of mandibular molars. It commences
at the time of contact or occlusion. Physiological
Management
tooth surface loss results in a reduction, in both
vertical tooth height and horizontal tooth width. Treatment of patient depends upon degree of
Due to slight mobility of teeth in their socket wear relative to the age of patient, etiology,
(which is a manifestation of resiliency of symptoms and patient’s desire. Patient should
periodontal ligament) similar facets occur at be advised of any possible bruxist habits. The
contact points. When the dentin gets exposed it provision of one of three different sorts of splints
generally becomes discolored i.e. brown in color. could be considered. A soft bite guard can help
in breaking a bruxist habit or simply will protect
There is gradual reduction in cusp height and
the teeth during the bruxist habit. A localized
consequent flattening of occlusal inclined plane.
occlusal interference splint is designed to break
There is shortening of the length of dental arch,
the bruxist habit and can be worn easily during
due to reduction in the mesiodistal diameter of
the day. A stabilization splint reduces bruxism
teeth. Secondary dentin deposition occurs.
by providing an ideal occlusion. It also enables
Pathological attrition: If pathologically vertical the clinician to locate and record centric relation.
dimension of tooth has reduced then, there is the In case of bruxism, use of night guards may be
possibility of compensatory growth effective in reducing attrition. Correction of
(dentoalveolar compensation) to some degree. malocclusion, stoppage of tobacco chewing habit
Dentoalveolar compensation: If attrition affecting and restriction of diet to noncoarse food are
the occlusal surfaces of teeth has occurred, then useful in avoiding attrition. Noncarious loss of
reduction in occlusal face height (vertical tooth tissue may require treatment for sensitivity,
dimension of occlusion) and increase in the esthetics, function and space loss in the vertical
freeway space could be anticipated. This may be dimension.
116 Textbook of Oral Pathology

Abrasion present. Exposed dentin appears highly

polished. Exposure of dentinal tubules and
It is the pathological wearing away of tooth
consequent irritation of the odontoblastic
substance through abnormal mechanical
processes stimulates secondary dentin formation
process.
which is sufficient to protect the pulp from
clinical exposure.
Etiology
Dental Floss or Tooth Pick Injury: Cervical portion
It is caused by use of abrasive dentifrices,
of proximal surfaces, just above the gingival
horizontal tooth brushing, and habitual opening
margin, is affected. Grooves on distal surface are
of bobby pins. It may also occur due to holding
deeper than on mesial surface.
nails or pins between teeth e.g. in carpenters,
shoemakers or tailors. Improper use of dental
Management
floss and tooth picks.
Examination and modification of teeth cleaning
Clinical Types habits will be indicated. Elimination of causative
agent should be carried out. Restoration, for
Habitual Abrasion: Habitual pipe smoker may
esthetics and to prevent further tooth wear.
develop abrasion on the incisal edges of lower
and upper anterior teeth. Improper and habitual
use of tooth prick or dental floss can cause Erosion
abrasion on the proximal surface of teeth. It is the loss of tooth substance by chemical
Occupational abrasion—It occurs when objects and process that does not involve known bacterial
instrument are habitually held between the teeth action. Dissolution of mineralized tooth structure
by people during working. occurs upon contact with acids. Erosion is a
chemical process in which the tooth surface is
Ritual abrasion—It is mainly seen in Africa. removed in the absence of plaque.
Tooth Brush Injury: It usually occurs on exposed
surfaces of roots of teeth. It occurs due to back Types (depending upon etiology)
and forth movement of brush with heavy • Intrinsic—Erosion that occur due to intrinsic
pressure causing bristles to assume wedge
cause, e.g. gastroesophageal reflux, vomiting.
shaped arrangement between crown and
• Extrinsic—Erosion occurring from extrinsic
gingiva. In horizontal brushing there is usually
sources, e.g. acidic beverages, citrus fruits.
a ‘V’ shaped or ‘wedge’ shaped ditch on the root
at cementoenamel junction. It is limited coronally
by enamel. It is more commonly seen on left side Etiology
of right handed persons and vice versa. Patient Local acidosis—In periodontal tissue from damage
develops sensitivity as dentin becomes exposed. due to traumatogenic occlusion.
The angle formed in the depth of the lesion
as well as that of enamel edge is a sharp one. Chronic vomiting—Complete loss of enamel on
Cervical lesions caused purely by abrasion have lingual surfaces of teeth through dissolution by
sharply defined margins and a smooth, hard gastric hydrochloric acid. Vomiting can also
surface. The lesion may become more rounded occur in alcoholics, peptic ulcer, gastritis,
and shallow if there is an element of erosion pregnancy and drug side effect.
 Teeth Anomalies 117

Acidic foods and beverages—Large quantities of ‘cupping’ in dentin. When erosion affects the
highly acidic carbonated beverages or lemon juice palatal surfaces of upper maxillary teeth, there
can produce erosion. Most of the fruits and fruits is often a central area of exposed dentin
juices have a low pH and can cause erosion. surrounded by a border of unaffected enamel.
Frequent consumption of carbonated drinks, In most cases, it results in little more than a loss
which are acidic in nature, may result in erosion of normal enamel contour, but in severe cases,
of teeth. dentine or pulp may be damaged.

Anorexia nervosa—It induces chronic vomiting Management

often after bouts of uncontrolled eating that is
interspersed between periods of starvation, In a patient where loss of tooth surface is
essentially caused by erosive fluids, advise
because of inner rejection of food.
regarding diet and use of sugar free chewing
Occupational—Workers involving in gum. Prescription of a fluoride mouthwash is
manufacturing of lead batteries, sanitary cleaners certainly indicated here. Modifying brushing
or soft drinks can develop erosion. habits and restoration of the defect, usually by
glass inomer cement should be done. For
Risk factors—Following factors are assume as risk
systemic management of vomiting, patient
factor for erosion. They are citrus fruits intake,
should be referred to the physician.
soft drink more 6 per week, bruxism habits,
vomiting and symptoms of gastroesophageal Abfraction
reflux disease.
It is also called as ‘stress lesion’.
Clinical Features
Causes and Mechanism
It occurs most frequently on labial and buccal
surfaces of teeth; sometimes may occur on It has been suggested that the stress lesion or
proximal surfaces of teeth. Usually it is confined abfraction is a consequence of eccentric forces
to gingival thirds of labial surface of anterior on the natural dentition. The theory propounds
teeth. Erosion may involve several teeth of tooth fatigue, flexure and deformation via
biomechanical loading of the tooth structure,
dentition. From extrinsic source, it causes erosion
primarily at the cervical region. Cusp flexure
on labial and buccal surface and from intrinsic
causes stress at the cervical fulcrum and results
source it causes erosion on lingual or palatal
in loss of the overlying tooth structure.
source. It is usually a smooth lesion which
exhibits no chalkiness. Loss of enamel often
Clinical Features
causes hypersensitivity in teeth and may also
trigger secondary dentin formation. It usually affects buccal/labial cervical areas of
Loss of tooth substance is manifested by teeth. Commonly affects single teeth with
shallow, broad, smooth, highly polished and excursive interferences or eccentric occlusal
scooped out depression on enamel surface loads. It appears as deep, narrow V-shaped
adjacent to cementoenamel junction. There may notch. The lesion is typically wedge shaped with
be pink spot on tooth which is attributable to the sharp line angles, but occlusal abfraction may
reduced thickness of enamel and dentin making present as circular invaginations. The magnitude
the pink hue of pulp visible. Erosive lesions cause of tooth tissue loss depends on the size, duration,
118 Textbook of Oral Pathology

direction, frequency and location of the forces. Etiology

Other factors, such as erosion and abrasion may
Physiological—Normal aging process.
play a significant role in tooth tissue loss, but
the initial force is of biomechanical loading. Reparative—Dental caries, abrasion, attrition,
erosion, tooth fracture, cavity preparation,
Dentinal Sclerosis chemical, thermal or mechanical insult.
It is also called as ‘transparent dentin’. It is a
Clinical Features
regressive alternation in tooth substance that is
characterized by calcification of the dentinal Anterior teeth exhibit higher incidence of
tubules. secondary dentin formation than molar teeth.
Decrease in tooth sensitivity occurs when
Etiology secondary dentin formation is extensive. It forms
an additional insulating layer of calcified tissue
It is caused by injury to dentin by caries or
between the pulp and the particular pathological
abrasion, normal aging process, abrasion or
process that initiate the dentinal response.
erosion of tooth.
Histopathological Features (Fig. 9.19)
Mechanism
It is well demarcated than primary dentin by
The mechanism of dentinal sclerosis or
deeply staining resting lines. It exhibits fewer
deposition of calcium salts is not understood,
tubules. Adventitious secondary dentin is
although most likely source of calcium salt is the
composed of few tubules that may be more
fluid or dental lymph within tubules. Increased
tortuous in course.
mineralization of the tooth decreases the
Sometimes, secondary dentin is formed at a
conduction of odontoblastic process.
rapid rate and odontoblasts may get entrapped
producing structure resembling bone. Such
Clinical Features
calcified tissue is called as osteodentin.
If it is examined under transmitted light, a
translucent zone is observed which is due to the
differences between the refractive indices of the
sclerotic or calcified dentinal tubules and the
adjacent normal tubules. It shows advancing
carious process. Sclerotic dentin is harder than
adjacent normal dentin. It is more highly
calcified than normal dentin.

Secondary and Tertiary Dentin

It is also called as ‘irregular dentin’. It is the dentin
which is formed after the deposition of primary
dentin. It serves to prevent involvement or
exposure of the pulp cavity. It can be
Figure 9.19: Reparative dentin showing well demarcated
physiological (occur due to age) and reparative resting line with few tubular structure (Courtesy:
(occur due to injury). Dr Sangamesh Halawar)
 Teeth Anomalies 119

Management between the tumor and the tooth and it is from

this tissue that cells, chiefly osteoclasts arise and
Root canal treatment should be done.
initiated root resorption. Cysts like apical
periodontal cyst may exert such pressure on the
Tertiary Dentin
apex of tooth the intervening connective tissue
There is no difference between secondary and may in turn get stimulated for osteoclasts
tertiary dentin. It is formed when pulp infection formation and thus, resorption begins. The apex
is very severe. It is localized exclusively adjacent may be lost leaving a flat or scalloped surface at
to irritated zone. The tubules are very irregular, the distal end of the root.
tortuous and reduced in number.
Excessive mechanical and occlusal forces—Usually,
Resorption of Teeth due to the force that is applied during orthodontic
treatment, the patient exhibits multiple areas of
It is chronic progressive damage or loss of tooth root resorption, irrespective of the manner of
structure due to the action of cells called treatment. Pressure from occlusal forces results
odontoclasts. It can be physiological as in case of in destruction, primarily of bone, then small
root resorption of deciduous teeth or pathological, lacunae often appear on the surface of cementum
which occurs in permanent teeth. Pathological and ultimately, it extends into the dentin.
resorption may be external or internal.
Impacted teeth—Teeth which are completely
External Resorption impacted or embedded in the bone, occasionally,
will undergo resorption of the crown and root.
It is lytic process occurring in the cementum or Impacted tooth also may cause resorption of
cementum and dentin of the roots of teeth. It can roots of adjacent teeth without itself getting
be resorption occurring at the apex or along the resorbed. This is commonly seen in case of
lateral surface of the root or it can be external horizontally or mesioangularly impacted
internal resorption. mandibular 3rd molars impinging on roots of
2nd molar.
Etiology
Overhanged root canal filling material—In such cases
Resorption associated with periapical infection— not only the apex get resorbed but more or less,
Periapical granuloma (arising due to pulpal sides of the root are affected. In some cases, all or
infection or trauma), causes subsequent most of the root disappears and the regenerating
resorption of root apex. On the radiograph, it bone eventually embraces the root canal filling
appears as slight raggedness or blunting of the intimately so that it appears as if the crown of the
root apex in the early stages. tooth is held in position by the root filling.
Reimplanted teeth—It may result in severe Idiopathic—In this case, burrowing type of external
resorption of root. Tooth root is resorbed and resorption can occur which is usually seen in
replaced by bone which produces ankylosis. relation with single or multiple erupted teeth.
Many implanted teeth exhibit complex
resorption of root and are gradually exfoliated. Clinical Features
Tumors and cysts—Resorption due to tumors and The affected tooth is usually asymptomatic. The
cysts appears to be essentially by pressure most frequent site for external resorption is
phenomenon. In most cases, tissue is present upper incisors, upper and lower bicuspids.
120 Textbook of Oral Pathology

When the root is completely resorbed, the multinucleated cells → resorption of internal
tooth may become mobile. If root resorption is wall of pulp.
followed by ankylosis then the tooth is immobile,
in infraocclusion and with high percussion Types
sound. • Internal inflammatory resorption—it occurs due
to intense inflammatory reaction within the
Histopathological Features
pulp tissue.
It is the result of osteoclastic activity on the root • Internal replacement resorption—it occurs due
surface of the involved tooth. Microscopically it to absence of any inflammatory reaction
varies from small area of cementum resorption within the pulp.
replaced by connective tissue or repaired by new
cementum; too large areas of resorption are Clinical Features (Fig. 9.20)
replaced by osseous tissues and ‘scooped out’ Appearance of pink hued area on the crown of
areas of are resorption replaced by inflammatory tooth, which represents hyperplastic pulp tissue
or neoplastic tissue. fitting the resorbed area and showing through
the remaining overlying tooth substance.
Management
It may affect any tooth in primary and
Apicectomy is treatment of choice. secondary dentitions, with prevalence in
If the area is broad and on lateral surface, permanent dentition. It is more common in central
curettage and filling of resorbed area should be incisor, lateral incisors, premolar and canine and
carried out. 3rd molar according decreasing frequency.
Multiple tooth involvement may be there.
Internal Resorption
It occurs during 4th and 5th decades of life
It begins certainly in the tooth. It is a condition and is more common in males. It is asymptomatic.
starting in the pulp, in which the pulp chamber The roots of teeth with internal resorption may
or the root canals or both, of the tooth expand manifest a reddish area, called the ‘pink spot’. This
by resorption of the surrounding dentin. reddish area represents granulation tissue
Definition showing through the resorbed area of crown.
When the lesion is located in the crown of
It is an idiopathic, slow or fast progressive teeth, it may expand to such an extent that the
resorptive process occurring in the dentin or
pulp chamber or root canals.

Etiology
It can be caused by inflammatory hyperplasia of
the pulp, direct and indirect pulp capping,
pulpotomy, enamel invagination, acute trauma
to teeth and pulp polyp.

Mechanism
Precipitating factor → vascular changes in pulp
→ inflammation and production of granulation
tissue → metaplasia of embryonic connective
tissue and macrophages → odontoclast like giant Figure 9.20: Internal resorption appearing as pink tooth
 Teeth Anomalies 121

crown shows dark shadow due to necrosis of the increasing age, i.e. as an age dependent factor.
pulp tissue. If the resorption is in the root, it may It is also be seen as a sign accompanying
weaken the tooth and result in fracture of the specific diseases, as for instance, Paget’s
tooth. It may perforate the crown with disease of bone.
hemorrhagic tissue projecting from the
perforation and result in infectious pulpitis. Etiology

Histopathological Features Accelerated elongation of a tooth—It occurs due to

loss of antagonist. It occurs due to inherent
Resorption of the inner or pulpal surface of tendency of the periodontium to maintain
dentin and proliferation of the pulp tissue, filling
normal width of the periodontal ligament.
the defect. It may show occasional osteoclasts or
odontoclasts hence it is called as odontoclastoma. Inflammation of the root—It does not occur at the
Pulp tissue usually exhibits chronic inflammatory apex of the root directly adjacent to the area of
reaction. Alternating periods of resorption and inflammation, as cementoblasts and their direct
repair are manifested as irregular lacunae like precursors in this area are lost. Instead, it occurs
areas in dentin that are partially or completely at some distance above the apex as the
filled with irregular dentin or osteodentin. inflammatory reaction acts as stimulation for the
When the root surface is perforated, it is very cementoblasts.
difficult to determine whether the lesion began Tooth repair—Occlusal trauma results in mild root
externally or internally. Multinucleated giant resorption and it is then repaired by cementum
cells and odontoclast are also present. formation. Root fracture is also repaired on
occasion by deposition of cementum between the
Management
root fragments as well as in their periphery.
Extirpation of pulp with routine endodontic
Osteitis deformans or Paget disease of bone—It is a
treatment or retrograde filling stops the internal
generalized skeletal disease characterized by
resorption process. Extraction of the tooth, if
excessive amount of cementum formation on
perforation occurs.
roots of teeth and by apparent disappearance of
Hypercementosis lamina dura of teeth.
It is also called as ‘cementum hyperplasia’ or Others—Hyperpituitarism, cleidocranial dysos-
‘exostosis of root’. It is characterized by deposition tosis can also cause hypercementosis.
of excessive amount of cementum on the root
surface. New tissue formation is in direct contact Clinical Features
with the cementum of roots of teeth. It is predominately seen in adults. Premolar teeth
are often affected and are often teeth are bilaterally
Types affected and symmetrical in distribution. The
• Localized—Hypercementosis of single tooth. It permanent teeth are affected more commonly
is usually a reactive, inflammation dependent than deciduous teeth. In multirooted teeth, one
phenomenon seen on singular teeth and or more roots are involved.
usually in relation to periapical osteitis or due There is no increase or decrease in tooth
to loss of occluding antagonistic teeth. sensitivity, unless periapical infection is present.
• Generalized—Generalized hypercementosis Teeth are vital and not sensitive to percussion.
affecting many (all) teeth, but which is There may be difficulty in extraction of teeth. In
seldom recognized as such, occurs with some cases hypercementosis is so extensive that
122 Textbook of Oral Pathology

it causes fusion of two or more adjacent teeth.

Roots appear larger in diameter than normal and
present rounded apices.
Spike formation of cementum—it is characterized
by occurrence of small spikes or outgrowth of
cementum on root surface. It occurs in cases of
excessive occlusal trauma probably due to
deposition of irregular cementum in focal group
of periodontal ligament fibers.

Histopathological Features
Excessive amount of secondary or cellular Figure 9.21: Cementicles lying free in the periodontal
cementum is found to be deposited directly, over ligament space (Courtesy: Dr Sangamesh Halawar)
typically thin layer of primary acellular
cementum. Secondary cementum is called as
‘osteocementum’ due to its cellular nature and its Etiology and Formation
resemblance to bone. Cementum typically They represent the areas of dystrophic calcification.
arranges in concentric layers around the root and Calcification of cell rest of Malassez occurs as a
frequently shows numerous resting lines result of degenerative changes. These bodies
indicated by deeply staining hematoxyphilic enlarge by further deposition of calcium salts in
lines parallel to the root surface. the adjacent surrounding connective tissue.
The continued proliferation of connective
Management tissue may lead to eventual union of cementicles.
Treatment of the primary cause should be done. It may result from focal calcification of connective
tissue between Sharpey’s bundles with no
Cementicles apparent central nidus. This occurs as small round
or ovoid globules of calcium salt. Small cementicle
Cementicles are small, spherical particles of
tears or fragments of bone detached from alveolar
cementum that may lie free in the periodontal
plate, if lying free in the periodontal ligament may
ligament adjacent to the cementum surface.
resemble cementicles. Cementicles appear to arise
Cementicles may be composed of fibrillar or
through calcification of thrombosed capillaries in
afibrillar cementum, or a mixture of the two.
periodontal ligament.
They are usually acellular.
Clinical Features
Types
It may impart rough globular outline to the root
Free cementicles—They are not attached to
surface; size is small, ranging from 0.2 to 0.3 in
cementum.
diameter.
Attached or sessile cementicles—They are attached
to the cementum surface. Histopathological Features (Fig. 9.21)
Embedded cementicles—They are incorporated Cementicles appears in the periodontal ligament
into the cementum layer. space. Size of the cementicle is usually varies.
 CHAPTER
Craniofacial
10 Anomalies

Congenital—Present at or before birth but not likely. There is no skipping of generation.

necessarily inherited, i.e. transmitted through the Affected person typically transmit trait to their
genes. offsprings.
Hereditary—They are apparent at birth but some
Autosomal Recessive Inheritance
may not become evident for years.
The cell consists of cytoplasm and nucleus. Appear only in their siblings, not on their
Nucleus of each somatic cell contains 23 pairs of offspring parent or other relatives. One fourth
chromosome out of which one is the pair of sex siblings are affected. Males and females are
chromosome; rest 22 pairs of chromosomes are equally likely to be affected.
called as autosomes. Chromosome is a nuclear
structure composed of DNA, which contains X-linked Dominant
units of hereditary gene. Gene is a portion of Since females have twice as many X
DNA coded for the synthesis of specific protein chromosomes as that of males, they exhibit a
or polypeptide chain. Genes are determinants of higher frequency of trait. Affected female will
hereditary characteristics. Locus is the site transmitted the gene to ½ of her offsprings.
occupied by the gene on chromosome. An allele
is number of alternative form that gene may take. X-linked Recessive
If both the allele at a given locus are identical Incidence of the trait is much higher in males than
to pair, it is called as homozygous and if the allele in females. Affected man can transmit it to his
are different it is called as heterozygous. A gene daughters who are carriers. Trait cannot be
showing trait in heterozygous is considered as transmitted from father to son.
dominant. Genes are transmitted from one
generation to other in a generally predictive way. DEVELOPMENTAL ANOMALIES OF JAWS

Autosomal Dominant Inheritance Agnathia

Every affected person has at least one affected It is also called as ‘hypognathous’. It is a extremely
parent. Males and females are both affected rare congenital defect characterized by absence
124 Textbook of Oral Pathology

of maxilla or mandible. If mandible is absent, the • True micrognathia—It is due to small jaw. It is
upper part of the face may be normal and the again classified as:
skin of the lower part will be continuous with – Congenital—it is present since birth
the suprasternal integument. – Acquired—it is acquired later in the life.
The hyoid bone is sometimes absent, despite
the presence of a rudimentary tongue. In the place Etiology
of buccal orifice, there may be a vertical slit. In
Congenital
case of unilateral absence of mandibular ramus,
it is not unusual for ear to be deformed or absent. In congenital type, etiology is usually unknown.
There may be absence of ears, absent or In many instances it is associated with other
hypoplastic tongue, cleft palate, dysplastic ears, congenital abnormalities, particularly congenital
hypertelorism, microstomia, narrow auditory heart disease and Pierre Robin syndrome (cleft
canal with palpebral fissures slanting down. palate, micrognathia, glossoptosis).

Agenesis Acquired

The failure of development of some part of Acquired type is post-natal type and result from
mandible or maxilla is termed as agenesis. disturbances in the area of TM joint (ankylosis).
Mandible is most commonly affected than Mouth breathing can be a predisposing factor for
maxilla and the condyle, entire mandible on one maxillary micrognathia. Agenesis of condyle also
side and ramus reveal the abnormality. In results in true mandibular micrognathia which
maxilla, one maxillary process or even may occur due to ankylosis of TMJ. Posterior
premaxilla are usually imperfect. positioning of mandible with regard to skull or
The commonest developmental defect in to a steep mandibular angle result in an apparent
mandible is the absence of condyle, in which there retrusion of the jaw.
is no articular fossa and the eminentia articularis
is either absent or rudimentary. Majority of the Clinical Features
patients have their chin deviating towards the
Micrognathia of maxilla is due to deficiency of
affected side. The ramus is small and usually
premaxillary area and patient with this
coronoid process is absent, with the result that
deformity appears to have the middle third of
the anteroposterior diameter of ramus is reduced.
face retracted.
True mandibular micrognathia is uncommon
Micrognathia
and patient appears clinically to have severe
It means small jaws as compared to normal size. retrusion of chin, steep mandibular angle and
In this case either maxilla or mandible may be deficient chin button.
affected. There are two types of micrognathia: Micrognathia is one cause of abnormal
alignment of teeth. This can be seen by observing
Types the occlusion of teeth. Often, there will not be
• Apparent micrognathia—This is not due to enough room for the teeth to grow. If the upper
abnormality of small jaw, in terms of size but jaw is short, then, occlusion may also be
rather to an abnormal positioning or abnormal.
abnormal relation of one jaw to another, In true micrognathia, the jaw is small enough
which produces illusion of micrognathia. to interfere with feeding of the infant and may
 Craniofacial Anomalies 125

require special nipples in order to feed Gummy smile—In certain patients with congenital
adequately. There may be difficulty in abnormalities, there may be elongation of
respiration. Due to the small size of the arch, the maxilla. There is much “show” when the patient
jaw is not able to accommodate the tongue, smiles, so that there is a so-called “gummy”
which is forced back into the oropharynx, smile. This is due to the upper jaw being too long.
blocking the air passage. Size of ramus is large, which forms less steep
angle with body of mandible. There is excessive
Syndromes associated with micrognathia—Pierre
condylar growth and anterior positioning of the
Robin syndrome, Hallerman-Streiff syndrome,
glenoid fossa, which may also result in
trisomy 13, trisomy 18, Turner’s syndrome,
mandibular prognathism. There is prominent
Treacher Collins syndrome and Marfan’s
chin button.
syndrome.
Management
Management
Resection of portion of mandible should be done
Surgery or orthodontic appliances may be
to decrease the length, followed by orthodontic
recommended. If upper jaw is short, then it can
treatment.
be corrected with surgical orthodontic treatment
by properly aligning the teeth and then moving
Facial Hemihypertrophy
surgically and elongating the short maxilla, in
order for three to four millimeters of the upper It is also called as ‘Friedreich’s disease’ It may
central incisors to show when an individual is involve entire half of the body, one or both limbs,
smiling. Small mandible can be corrected with, face, head and associated structures.
depending on the degree of deformity and
problem, advancement of mandible and chin Etiology
surgically.
Hormonal imbalance, incomplete twinning,
chromosomal abnormalities, localized alteration
Macrognathia
of intrauterine development, lymphatic
It refers to the condition in which jaw size is abnormalities, vascular abnormalities and
larger than the normal. It is also called as neurogenic abnormalities can leads to facial
‘megagnathia’. hemihypertrophy.

Etiology Clinical Features

It is cause by Pituitary gigantism, Paget’s disease of Females are affected more than males. It has
bone, acromegaly and in some form of fibrous dysplasia. vague onset, usually in childhood, adolescence
or early adult life. It may occur alone or in
Clinical Features generalized hemihypertrophy. It is prevalent in
Mandibular protrusion or prognathism is males. It involves the eyelids, cheeks, lips, facial
common occurrence, which is due to disparity bones, tongue, ears and tonsils. Occasionally,
in the size of maxilla to mandible and posterior poorly localized, vague, painful sensation in
positioning of maxilla in relation to the cranium. muscles affected. Enlargement of one half of the
Mandible is larger than normal which results in head present since birth. Enlarged side grows at
increased mandibular body length. a rate proportional to uninvolved side.
126 Textbook of Oral Pathology

Associated with other abnormalities like Etiology

mental deficiency, skin abnormalities and
Atrophic malfunction of cervical sympathetic
compensatory scoliosis. Hemifacial hypertrophy
nervous system. Trauma, infection and
may be accompanied by hemimegalencephaly,
hereditary factors can also cause Parry-Romberg
which is characterized by hypertrophy of one
syndrome. Peripheral trigeminal neuralgia.
cerebral hemisphere with ipsilateral ventricular
Localized scleroderma.
dilatation.
In most cases, Parry-Romberg syndrome
Pigmentation and hemangioma may occur
appears to occur randomly for unknown reasons
on skin. There is progressive asymmetry due to
(sporadically).
enlargement of soft tissues, including the lips,
ear and tongue.
Clinical Features
Syndrome associated—Syndromes associated with
Onset noted in 1st or 2nd decades of life as a
facial hemihypertrophy are Proteus syndrome,
white line furrow or mark on one side of face or
Maffucci’s syndrome, Ollier syndrome and
brow near midline. In rare cases, the disorder is
Klippel-Trenaunay-Weber syndrome.
apparent at birth. In most cases, progressive
Oral Manifestations tissue wasting is limited to one half of the face,
usually the left side.
Dentition is abnormal in three respects: In most affected individuals, hemifacial
• Crown size may be large. atrophy typically progresses over approximately
• Root size may enlarge. three to five years and then ceases. Affected areas
• Rate of development of permanent teeth on the may demonstrate shrinkage and atrophy of
affected side is more rapid and erupt before tissues beneath the skin (subcutaneous tissue),
their counterparts on the uninvolved side. in the layer of fat under the skin (subcutaneous
• Primary teeth shed early. fat) and in underlying cartilage, muscle and
Bone of maxilla or mandible is also enlarged. bone.
Tongue is commonly involved and bizarre
patterns of enlargement of papilla, in addition Skin—In addition, the skin overlying the affected
to general unilateral enlargement of tongue. areas may become darkly pigmented
Buccal mucosa frequently appears velvety and (hyperpigmentation) with, in some cases, certain
may hang in soft pendulous folds on affected side. areas of white (depigmented) patches
(vitiligo).There may be hollowing of cheek and
Management eyes may appear depressed in the orbits.
Cosmetic repair. Neurological manifestation—This may include
severe headache that lasts for extended periods
Facial Hemiatrophy
of time and may be accompanied by visual
It is also called as ‘Parry-Romberg syndrome’, abnormalities, nausea and vomiting (migraine).
‘Romberg hemifacial atrophy’, ‘hemifacial There is also facial pain due to trigeminal
microstomia’ and ‘progressive facial hemiatrophy’. neuralgia. There are periods of uncontrolled
Parry-Romberg syndrome is a rare disorder electrical disturbances in brain (seizures) that
characterized by slowly progressive wasting usually are characterized by rapid spasms of a
(atrophy) of the soft tissues of half of the face muscle group that spread to adjacent muscles
(hemifacial atrophy). (contralateral Jacksonian epilepsy).
 Craniofacial Anomalies 127

Hair—There is graying (blanching) of hair as well Segmental Odontomaxillary Dysplasia

as abnormal bald patches on the scalp with loss
It is also called as hemimaxillofacial dysplasia. It
of eyelashes and the middle (median) portion of
can be confused with facial hemihypertrophy.
eyebrows (alopecia).
Base of skull—There is underdevelopment of the Clinical Features
base of skull in some cases whose the face is
It is discovered in childhood. There is painless
affected.
unilateral enlargement of the maxillary bone.
Malar bones—When the malar bone is small, the There may be fibrous hyperplasia of overlying
side of the face is flat but an absent malar bone gingival soft tissue. Primary teeth in the affected
produces a depression inferior to the orbit. area may be hypoplastic showing enamel defect.
Face—The soft tissue of face are small and thinner
Histopathological Features
than normal.
Ear—Aplasia or hypoplasia of external ear. The Gingival soft tissue may show fibrosis. Affected
ear canal is missing. bone consists of irregular trabeculae with woven
appearance. There are also presence of resting
Oral Manifestations and reversal lines.

Lip and Tongue—Many individuals also experience Cleidocranial Dysplasia

atrophy of half of the upper lip and tongue.
It is also called as ‘cleidocranial dysostosis’, ‘Marie
Teeth—Delayed eruption or wasting of the roots and sainton disease’, ‘craniocleidodysostosis’.
of certain teeth on the affected side.
Jaws—In individuals with the disorder, initial Etiology
facial changes usually involve the tissues above Hereditary and when inherited, it appear as a
the upper jaw (maxilla) or between the nose and true dominant Mendelian characteristic.
the upper corner of the lip (nasolabial fold) and Incomplete penetration of genetic trait may be
progress to involve the angle of mouth, the areas observed.
around the eye, brow, ear and/or the neck.
Malocclusion—There is reduced growth of jaws Clinical Features
and eruption of teeth is retarded. There is also The disease affects men and women with equal
malocclusion on the affected side. frequency.
It primarily affects skull, clavicle and
Histopathological Features dentition. There may be complete absence of
clavicle and patients have unusual mobility being
There is atrophy of epidermis with perivascular
infiltrate of lymphocytes and monocytes. able to bring their shoulders forward until they
meet in midline. The head is brachycephalic (reduced
Degenerative changes in vascular endothelium.
anterior-posterior dimension but increased
skull width) or wide and short (Fig. 10.1).
Management
Nasal bridge is depressed with a broad base.
Orthodontic treatment, plastic surgery and In skull the fontanels often reaming open or at
hearing aids are recommended. least exhibit delayed closing and for this reason,
128 Textbook of Oral Pathology

Figure 10.2: Patient showing unerupted teeth

cementum is seen. Crowding and disorganiza-

tion of developing permanent dentition is also
observed. Presence of supernumerary teeth is
usually noted in anterior region. (Fig. 10.2).
High narrow arched palate and cleft palate
may be common. Roots of teeth are often some
what short and thinner than the normal. Maxilla
is small and mandible is usually normal in size,
which gives the appearance of prognathism. The
crown may be pitted as a result of enamel
hypoplasia.
Figure 10.1: Patient showing unusual mobility of
shoulder in cleidocranial dysplasia
Management
tend to be rather large. Open skull suture and
Treatment is not specific. Prophylactic dental
multiple wormian bones are present and there
care should be taken.
is occasional stunting of long bone.
Lacrimal and zygomatic bone are also under-
Craniofacial Dysostosis
developed. Sagittal suture is characteristically
sunken, giving the skull flat appearance. It is also called as ‘Crouzon’s disease or syndrome’.
In some instances, ‘Crouzon syndrome’ is
Oral Manifestations inherited as an autosomal dominant trait. In
other cases, affected individuals have no family
Maxilla and paranasal sinus are underdevel-
history of disease. The disorder is characterized
oped, resulting in maxillary micrognathia.
by distinctive malformations of the skull and
Maxilla is underdeveloped and smaller than
facial (craniofacial) region.
normal, in relation to mandible.
Prolonged retention of primary dentition and
Clinical Features
delayed eruption of permanent dentition.
Numerous unerupted teeth are found which are Crouzon’s syndrome is a rare genetic disorder
most prevalent in the mandibular premolar and that may be evident at birth (congenital) or
incisor area. Paucity and complete absence of during infancy.
 Craniofacial Anomalies 129

In most infants with Crouzon syndrome, the

fibrous joints between certain bones of the skull
(cranial sutures) close prematurely (craniosynos-
tosis). In addition, facial abnormalities typically
include unusual bulging or protrusion of the
eyeballs (proptosis) due to shallow eye cavities.
Outward deviation of one of the eyes (diver-
gent strabismus or exotropia); widely spaced
eyes (ocular hypertelorism). Exophthalmos (pro-
trusion of eyes) with divergent strabismus and
optic neuritis and choked disks, resulting fre-
quently in blindness.
Bulging of frontal bone in midline, over the Figure 10.3: Patient is having Crouzon’s syndrome. Note
nose and downward sloping of back of head parrot beak and bulging of frontal bone in midline
(Fig. 10.3). There is protuberant frontal region
with an anterior-posterior ridge overhanging the
frontal eminence and often passing to the root
of nose (triangular frontal defect).

Oral Manifestations
Maxillary hypoplasia with shortened
anteroposterior dimension of maxillary arch is
present. Dental arch width is reduced and this
gives an appearance of highly arch palate.
In some cases, facial angle is exaggerated and
the patient nose is prominent and pointed,
resembling ‘parrot beak’. Figure 10.4: High arch with cross bite in patient with
Crouzon’s syndrome
Unilateral or bilateral cross-bite is evident
with open bite and crowding in mandibular teeth autosomal dominant trait. It results from
(Fig. 10.4). Shovel shaped maxillary incisors cleft retardation or failure of differentiation of
lip and palate are also evident. The antrum are maxillary mesoderm at and after the 50 mm stage
small and underdeveloped. The mandible is of the embryo.
large as compared to maxilla, so there is
prognathism. Clinical Features
Underdevelopment of zygomatic bone, resulting
Management in midfacial deformities is usually observed.
Maxillofacial surgery for correction of facial Craniofacial malformations associated with
deformities. Treacher-Collins syndrome include under-
developed (hypo-plastic) or absent cheek (malar)
Mandibulofacial Dysostosis bone.
It is also called as ‘Treacher Collins syndrome’ and Downward inclination of palpebral fissure.
‘Franceschetti syndrome’. It is often inherited as There is deficiency of eyelashes. In some cases,
130 Textbook of Oral Pathology

eyes assume corresponding slant. A notching Oral Manifestations

(colobomas) from the outer third of lower Underdevelopment of mandible with steep man-
eyelids. There is varying degree of visual dibular angle gives characteristic appearance to
impairment in some cases. the face. Facial appearance resembles fish or bird.
Affected infants may also have underdevel- An incompletely developed, abnormally small
oped (hypoplastic) and/or malformed (dysplas- lower jaw. Macrostomia (an unusually large
tic) ears (pinnae) (Figs 10.5 and 10.6) with blind mouth) can also occur. There is presence of high
ending or absent external ear canals (microtia), arch palate with cleft palate. Abnormal position
resulting in hearing impairment (conductive and malocclusion of teeth with anterior open
hearing loss). Absence of external auditory ca- bite.
nal resulting in partial or complete deafness.
The normal prominence of cheek is either Management
missing or reduced depending upon the
presence or absence of malar bone. There is Cosmetic improvement and surgical interven-
usually hypoplasia of malar bone. tions to improve osseous and ear defect.
Secondary mental deficiency is observed.
Blind fistulae may be seen between the angle of Focal Osteoporosis Bone Marrow Defect
ears and the angle of mouth. They are derived from bone marrow hyperplasia
Associated features anal atresia/stenosis, of persisting embryonic marrow remnants and
congenital cardiac anomaly, rectovaginal fistula site of abnormal healing following extraction
and tracheo-esophageal fistula. trauma and local inflammation.

Figure 10.5: Treacher collin’ s syndrome: Bird or fish like Figure 10.6: Treacher collin’ s syndrome: Underdeveloped
appearance with ear deformities mandible with steep mandibular angle
 Craniofacial Anomalies 131

Clinical Features Oral Manifestations

• Asymptomatic with history of pain in that • Teeth are deficient in number; those which
region. do develop are small, rudimentary, conical,
• It is more common in females than males. spaced and irregular in position. The teeth
• It is common in molar, premolar region in are affected, with eruption occurring at birth
mandible. or shortly thereafter. The deciduous molars
• Usually it is more evident on the radiograph. present a crenate occlusal surface. The
permanent dentition is more likely to be
Histopathological Features defective than the deciduous one. Eruption
Bone marrow contain cellular hemopoietic is delayed.
component which may show variable fatty • The lip deformity often referred to as a
components. In some cases lymphoid aggregates “partial hairlip” results from an abnormally
may be present. short upper lip, which may also be sunken
secondary to hypoplasia of maxilla.
Management
There is no specific treatment for it. Arhinencephaly
It is a developmental abnormality of the skull
Chondroectodermal Dysplasia and face in which there is absence or deficiency
It is also called as ‘Ellis-van Crevald disease’. of the olfactory portion of brain. Absence of the
vertical and cribriform plates of ethmoid and of
Clinical Features crista galli result in the orbits being more closely
set, a condition known as hypotelorism. There is
• It is congenital and the patient present
depression of nose and absence of the bridge
evidence of chondrodysplasia, ectodermal
because of lack of nasal bones and nasal septum.
dysplasia, polydactylism and congenital
There is wide cleft in the central portion of the
morbus cordis. Post-axial polydactyly in the
lip, where the philtrum is absent and with failure
hands, i.e. an extrafinger lateral to the normal
of development of any part of the premaxilla,
fifth finger, is a consistent finding.
the cleft is continuous through the palate. In
Polydactyly in the feet is a rare finding.
some cases, an associated deformity of the frontal
• Bone dysplasia is characterized by
portion of skull presents an angular appearance
acromesomelia, i.e. relative shortening of the
resembling ‘the prow of a boat’ presumably due
distal (acromelic) and middle (mesomelic)
to premature fusion of metopic sutures.
segments. It may interfere with the ability to
make a tight fist. There may be a deformity DEVELOPMENTAL DISORDERS OF ORAL
of the knees that, frequently progress and MUCOSA
causes a significant malalignment. The nails
of the fingers and toes are dysplastic. The hair Labial and Oral Melanotic Macule
tend to be fine and sparse. It is also called as ‘focal melanosis’ or ‘solitary labial
• Congenital heart defects may include lentigo’. It represents an increase in synthesis of
hypoplasia of aorta, atrial and ventricular melanin pigments by basal cell layer melanocytes
septal defects and a single atrium. There is without increase in the number of melanocytes.
dwarfism, i.e. the long bones being short and It is the most common pigmentation to occur in
the trunk of normal length. oral cavity of light skinned individuals.
132 Textbook of Oral Pathology

Clinical Features most commonly found bilaterally in symmetrical

• Mean age 41 to 45 years pattern on mucosa of the cheek, opposite to the
• Equal occurrence in both sexes. molar teeth. It is also found on inner surface of
• It is attributed to actinic exposure and therefore lips, in retromolar area lateral to anterior faucial
occurs on vermilion border of the lower lip. pillar and occasionally on the tongue, gingiva,
• Sometimes it can also occur on gingiva, palate frenum and palate.
and buccal mucosa. They appear as small yellow spots, either
• It present as small, flat macule which may discretely separated or forming relatively large
be single or multiple. Color of pigmentation plaques often projecting slightly above the
is brown or brown black pigmentation. surface of tissue. The granules may be isolated
Solitary lesion is less than 1 cm in diameter or they may occur in confluent sheets. Sometime
and constant in size. they may occur in clusters and may form plaque
• Lesions are oval or irregular in outline. It is like lesions.
an asymptomatic condition. On the tongue it appears as dome-shaped
nodules from a few millimeters to 2 cm in
Histopathological Features diameter on the midline dorsum of the tongue.
Increase amount of melanin in basal cell layer. They are more yellow than white. It increases
Also there is melanin in lamina propria. Increase rapidly in number at puberty and continues to
number of clear cells and dendritic cells are also increase through adult life. They are neither
found. Melanophagocytosis can also be seen. ectopic nor adenomas and usually are
Normal stratified squamous epithelium is seen submucosal. They are sharply delineated and
in this case. There is presence of melanin with smooth surface which is not ulcerated. They
incontinence (deposits in subepithelial stroma) in have got slightly cheesy consistency.
macrophages or melanophages. The melanin can
be distinguished from iron deposits by the loss of Histopathological Features
brown color after bleaching.
It consists of submucosal cluster of sebaceous
Fordyce’s Granule acini and communicated with the oral
epithelium by the way of duct. In some cases all
A Fordyce’s granule is a developmental anomaly
are grouped around one or more duct which
characterized by heterotrophic collection of
opens on the surface of mucosa. These ducts may
sebaceous glands at various sites in oral cavity
show keratin plugging. They are identical with
which is covered with intact mucosa.
those seen with normal sebaceous gland on the
Pathogenesis skin except for the absence of hair follicles
(Figs 10.7 and 10.8).
It has been postulated that the occurrence of
sebaceous glands in the mouth may result from Management
the inclusion in the oral cavity of the ectoderm
having some of the potentialities of the skin in If it causes disfigurement then surgical removal
the course of development of the maxillary and can be done.
mandibular processes during embryonic life.
Focal Epithelial Hyperplasia
Clinical Features It also called as ‘Heck disease’. It is viral induced
It is seen in any age group with somewhat more oral mucosal hyperplastic response characterized
prevalent in males as compared to females. It is by multiple, more or less papillomatous like lesion.
 Craniofacial Anomalies 133

• They become less conspicuous when the

mucosa is stretched. It is nontender. After
drying, the lesion it reveals finely granular
surface texture.
• These lesions are soft having size of 1 to 5
mm in diameter with same color as adjacent
mucosa. Sometimes cluster so closely
together that they appear as Cobblestone or
fissured appearance.
• They are pale to normal in color. They often
appear to undergo spontaneous regression
after 4 to 6 months.
Figure 10.7: Sebaceous gland showing
pilosebaceous tract Histopathological Features
There is thickening of spinous layer with
presence of mitosoid cells in the upper layer. It
compromises hyperplastic epithelium without
keratosis and thickening or elongation of rete
pegs. The lamina propria of the underlying
connective tissue may occasionally show some
signs of inflammation. There is acanthosis with
mild hyperparakeratosis usually present over the
surface. Lymphocyte infiltration with occasional
collection of polymorphonuclear leukocytes.
Some focal areas of liquefaction degeneration of
the basal layer may be found.
Figure 10.8: Fordyce’s granule
Differential Diagnosis (Pathological)
Etiology
Squamous papilloma, verruca vulgaris—In this
It is possibly cause by virus (may be papovavirus
condition there is no presence of mitosoid cells
group). Some lesion may be seen in HIV
and lack of connective tissue cores in the surface
seropositive patient.
projection.
Clinical Features Keratoacanthoma—In this condition there is
• It occurs predominately in children between elongation of thin rete pegs which is absent in
ages of 3 to 18 years. Common sites are lip, Heck disease.
buccal mucosa, commissures, tongue and less Verruciform Xanthoma—In Heck disease you will
commonly on the gingiva, and anterior not find subepithelial foamy or granular
fascial pillar. histiocytes like cells which are present in
• It present as multiple nodular lesions with verruciform xanthoma.
sessile base. It can occur in cluster or in a
isolated crops. Sometime it is present as flat, Management
slightly raised whitish plaque on roughened No treatment is necessary as these are harmless
surface. lesions and it will regress spontaneously.
134 Textbook of Oral Pathology

CHAPTER

11 Benign Tumors

Tumor is a new growth representing the tissue • Neoplasm — Tumors that continue to grow
of origin. The study of the tumors of the oral indefinitely are called as neoplasm.
cavity and adjacent tissues constitute an • Hypertrophy — Enlargement caused by an
important phase of dentistry or because the role increase in the size of cells.
of a dentist in the diagnosis of the lesion. • Hyperplasia — Enlargement caused by an
increase in the number of the cells.
Characteristics of Benign Tumor
Classification
• Benign tumor shows insidious onset and the
rate of growth is generally slow and is for a A. Epithelial tissue
• Papilloma
longer period of time. • Keratoacanthoma
• It has got well defined mass of regular • Squamous acanthoma
smooth outline and it possesses a fibrous • Nevus
B. Fibrous connective tissue
capsule. • Fibroma
• Benign tumors usually produce symptoms • Fibrous hyperplasia
due to the swelling and pressure effect on the • Fibrous epulis
• Giant cell fibroma
surrounding structures. • Fibrous histiocytomas
• Benign tumors are smaller, as compared to • Desmoplastic fibroma
malignant tumors. • Myxoma
• Myxofibroma
• There is displacement of adjacent normal
C. Cartilage tissue
tissues. • Chondroma
• It is usually painless and they never metas- • Chondroblastoma
• Chondromyxoid fibroma
tasize.
D. Adipose tissue
• Lipoma
Terminology • Angiolipoma
E. Bone
• Hamartomas — It is an abnormal proliferation • Osteoma
of normal tissue at its usual location. For • Osteoid osteoma
• Osteoblastoma
example, hemangioma
 Benign Tumors 135

• Exostosis tori torus palatinustorus mandibularis Clinical Features

• Osteomatosis
F. Vascular tissue • This is more commonly seen during 3rd and
• Hemangioma 4th decades of life, only 20% of cases are
• Hereditary hemorrhagic telangiectasia
• Lymphangioma found below 20 years of age.
• Arteriovenous fistula • It is most commonly seen on tongue, palate,
• Glomus tumor buccal mucosa, gingiva, lip, mandibular
G. Neural tissue
• Neurofibroma ridge and floor of mouth.
• Neurolemmoma • It is a typically an exophytic lesion with a
• Schwanoma cauliflower-like surface or with finger-like
• Ganglioneuroma
• Traumatic neuroma projection. This appearance is caused by
• Melanotic neuro ectodermal tumor of infancy presence of deep clefts that extend well into
H. Muscles
lesion from the surface.
• Leiomyoma
• Rhabdomyoma • It is generally arising from a pedunculated
• Granular cell myoblastoma base. Sometimes base may be broad rather
I. Teratoma
J. Salivary gland tumor
than pedunculated. The size of tumor may
• Adenoma vary from 2 millimeters but it is seldom larger
• Oxycytoma than 2 centimeters.
• Oxyphilic adenoma
• Warthin’s tumor
• Tumor in which there is much keratinization
• Pleomorphic adenoma is white and lesion with less keratinization are
grayish pink in color. Tumor is firm, when
EPITHELIAL ORIGIN keratinized and soft when it is non-
keratinized.
Papilloma • The term focal dermal hypoplasia syndrome
is used when there are multiple oral
It is a relatively common, benign neoplasm of
papillomas, dermal hypoplasia and
unknown origin, which arises from the surface syndactyly with fatty herniation, coloboma
of epithelium. It may be caused by papilloma
and strabismus. The other syndromes which
virus.
may be associated with multiple papillomas
are Cowden's syndrome, Down's syndrome
Types and nevus unius lateris.
• Squamous cell papilloma—It may arise in the
Histopathological Features
tongue, cheek, lip and esophagus
– Congenital — It is usually present since It consists of many long, thin, finger like
birth. projections extending from the epithelium and
– Infective — It arises from viral infection. containing a thin, central connective tissue core,
– Soft papilloma — It is often seen in eyelids which supports the nutrient blood vessels.
of elderly people. Keratin covering the epithelium follows.
– Keratin horns — It is due to excess keratin Superficial vacuolated cells are present in
formation and seen in older people. papilloma. The supporting fibrous connective
• Basal cell papilloma — It is also called as tissue stroma often contains prominent number
'seborrhoeic or senile wart'. It occurs on the of small blood vessels and inflammatory cell
trunk, face, arms and arm pits. infiltrate.
136 Textbook of Oral Pathology

color, but sometimes pale or hyperkeratotic

lesion with a rough pebbly surface may be
seen.
• It is either sessile or has a pedunculated base
with size as small as 2 mm to as large as 1.5 cm.

Histopathological Features
The lesion has a verrucous, hyperkeratotic surface
with severe parakeratin plugging. Surface
parakeratin is often shaggy with superimposed
bacterial colonies. The rete pegs are extremely
elongated but in a uniform fashion (Fig. 11.2).
Figure 11.1: Papilloma shows many fingers like projections No increase in mitosis or pseudo-
(P) of epithelial cells (E). Each projection is supported by
connective tissue papilla (CT). Keratin (K) is seen at the surface epitheliomatous hyperplasia is found. There is
presence of large swollen foam cells or xanthoma
Management cells, which are presumably histiocytes, which
fill the connective tissue papillae between the
Elliptic incision on the tissue underlying the epithelial rete pegs (Fig. 11.3).
lesion should be done. Incision should be from
the base of mucosa, into which the pedicle or Management
stalk is inserted. Application of formaldehyde Simple surgical excision.
at night on the wart may cure the condition.
Sometimes, silver nitrate application also cures
the condition. If the tumor is properly excised,
recurrence is rare.

Verruciform Xanthoma
It is also called as Histiocytosis Y. It is a papilloma-
tous lesion of oral cavity in which the foam cells
fill the connective tissue papillae between the
epithelial pegs. Etiology is unknown.

Clinical Features
• There is slight female predilection and is
usually seen in middle age.
• It can occur at any site and is most frequently
found on the gingiva or alveolar ridge,
followed by the buccal mucosa, palate, floor
of the mouth, lip and lower mucobuccal fold. Figure 11.2: Papillary projections (P) of verrucous xanthoma.
• It occurs as a solitary lesion. Connective tissue cores below epithelium (E) show presence
of foam cells (F). Inflammatory cells (IC) are seen (Courtesy:
• In some cases crateriform surface has also been Dr Alka Kale, Prof and Head, Oral Pathology, KLES’s Institute
reported. It has got either normal or red in of Dental Sciences, Belgaum)
 Benign Tumors 137

lip the lesion shows smooth, raised, rolled

borders with a central plug of hard keratin. The
lesion is round with rolled margins. Margins are
sharply delineated. There may be elevation of
the rolled margins. Keratin pit is frequently
discolored, being yellowish brown in color. It
grows to maximum size of 1 to 2 cm in diameter.
The lesion is often painful and regional
lymphadenopathy may be present. Lesion
appears fixed to the surrounding tissues. It
begins as small, firm nodule that develops to full
size over a period of four to eight weeks and
persists as static lesion for another 4 to 8 weeks.
After that it undergoes spontaneous regression
over the next six to eight weeks period by
expulsion of the keratin core with resorption of
Figure 11.3: Foam cells (F) are seen in the connective tissue
papilla in verruciform xanthoma (Courtesy: Dr Alka Kale, Prof the mass. There may be unsightly scar formation.
and Head, Oral Pathology, KLES’s Institute of Dental
Sciences, Belgaum) Histopathological Features

Keratoacanthoma • The lesion consists of hyperplastic squamous

epithelium growing into the underlying
It is also called as 'self-healing carcinoma', 'mollus-
connective tissue.
cum sebaceum'. It, clinically and histologically,
• The surface is covered by a thickened layer
resembles epidermoid carcinoma and it is fre-
of parakeratin or orthokeratin with central
quently mistaken as cancer. It is believed to arise
plugging.
from hair follicles.
• At the deep margins of the tumor, islands of
Etiology epithelium often appear to be invading.
• Epithelial tissue adjacent to the lesion is
Both, genetic and viral etiological factors have
sharply demarcated from that of the lesion,
been demonstrated. Exposure to sun is also a
which appears to lie in a cup-shaped
etiological factor. In some cases trauma and
depression.
chemicals, such as coal tar and mineral oil can
• The connective tissue in the area shows
be responsible for keratoacanthoma.
chronic inflammatory cell infiltration.
Clinical Features • The most characteristic feature of the lesion
is found at the margins, where the normal
It is more common in males as compared to
adjacent epithelium is elevated towards the
females (2:1). Majority of cases occur between
center. An abrupt change in the normal
the ages of 50 to 70 years. Exposed skin including
epithelium occurs as the hyperplastic
cheeks, nose and dorsum of the hands are the
acanthotic epithelium is reached.
most common site of occurrence. Intraoral lesion
is uncommon; if found, is more common on lips.
Management
The lesion appears as an elevated umblicated or
crateriform with depressed central core. It It often resolves spontaneously without
appears as dome shaped swelling. On the lower treatment. The lesion may be treated by surgical
138 Textbook of Oral Pathology

excision as the scar remaining from excision will Clinical Features

be more cosmetic than that resulting from
• It is seen occasionally in the oral cavity, but
spontaneous regression.
frequently on the skin.
Squamous Acanthoma • Congenital nevi — The congenital nevi, with
passage of time, may change from flat, pale
It is an uncommon lesion which probably
tanned macules to elevated, verrucous, hairy
represents a reactive phenomenon, rather than
lesions.
a true neoplasm. It is caused by trauma.
• Acquired nevi — Acquired nevi are extremely
Clinical Features common and appear at 8th month of life and
increase in number with age, apparently
It is generally described as a small, flat or elevated,
reaching their peak numerically in the late
white, sessile or pedunculated lesion on the
mucosa. No site and age predilection is observed. third decade of life.
• Intradermal nevus — It is most common and
Histopathological Features affected persons exhibit several nevi
It consists of well demarcated elevated and/or scattered over the body. It is more common
umblicated epithelial proliferation with a in children and it is referred as 'common
markedly thickened layer of orthokeratin and mole'. It may be smooth flat lesion or may be
underlying spinous layer. There are epithelial elevated above the surface. It may or may not
alterations beginning with a localized exhibit brown pigmentation and it often
pseudoepitheliomatous hyperplasia. shows strands of hair growing from the
surface. It is firm on palpation and rise above
Pigmented Cellular Nevus the surface.
Nevus is defined as a congenital, developmental • Junctional nevus — It may appear clinically
tumor like malformation of the skin or mucous similar to intra-dermal nevus with distinction
membrane. Pigmented cellular nevus is also chiefly being histological. Early in the life, it
called as 'pigmented mole' or 'benign melanocytic arises from the basal cell layer of melanocytes.
nevus'. It is a superficial lesion composed of so Junctional type derived name from it
called nevus cells; hence the term 'cellular nevus'. location. They are situated in basal layer just
above junction of epidermis and dermis. It is
Types less common as compared to intradermal
• Congenital nevi. They are flat and brown and have
– Small — They are greater than 1 cm in regular, oval and round outline. It appears
diameter and are usually 3 to 5 cm. as brown black macules affecting, both skin,
– Garment — They are greater than 10 cm in as well as the oral mucosal surface.
diameter and can cover larger areas of the • Compound nevus — It is more common on
skin. skin, as compared to oral mucosa. It is firm,
• Acquired raised nodule or polypoid mass. The lesion
– Intradermal nevus (common mole) is composed of two parts, intradermal and
– Junctional nevus junctional nevus.
– Compound nevus • Spindle cell or epitheloid cell nevus — It occurs
– Spindle cell or epitheloid cell nevus in children and may appear histologically
– Blue (Jadassohn-Tieche) nevus. similar to malignant melanoma in adults.
 Benign Tumors 139

• Blue nevus — It occurs chiefly on buttock, tissue stroma. The nevus cells are situated within
dorsum of the feet and hands, face and the connective tissue and are separated from the
occasionally on other areas. The majority of overlying epithelium by a well defined band of
blue nevi are present at birth and early connective tissue. Thus in it, nevus cells are not
childhood. They persist unchanged in contact with the surface epithelium. Multiple
throughout the life. The lesions are smooth multinucleated giant cells may be seen. Few cells
and exhibit hair growing from the surface. are spindle shaped.
The color of blue nevi occurs as melanocytes
Junctional nevus — Zone of demarcation is absent
reside deep in the connective tissue and the
and the nevus cells contact and seem to blend
overlying vessels dampen the brown
into the surface epithelium. This overlying
coloration of melanin and thus yield a blue
epithelium is usually thin and irregular and
tint. It is composed of dermal melanocytes,
shows cells apparently crossing the junction and
which only rarely undergo malignant
growing down into the connective tissue—the
transformation.
so called "abtropfung" or "dropping off" effect.
• Spitz nevus — It is also called as juvenile
This junctional activity has serious implication
melanoma and it shares many histo-
as it has been known to undergo malignant
pathological features with melanoma. It
transformation (Fig. 11.4).
occurs during childhood on face or
extremities. It appears solitary, dome shaped, Compound nevus — It shows features of both, the
pink to reddish brown papule. Size is smaller junctional nevus and intradermal nevus. Nests
than 6 mm. of nevus cells are seen dropping off from the
epidermis while large nests of nevus cells are also
Oral Manifestations present in the dermis.
Intraorally nevus can occur at any site but most
commonly occur on hard palate, buccal mucosa,
and lips and in gingiva. Most nevi present as
raised, macular lesions, but some are flat and
macular. They are slow growing and their size
is usually less than 1 cm in diameter. Compound
nevi appear as pigmented papules or macules
over the hard palate. Blue nevi may be macular
or nodular in appearance.

Histopathological Features
The nevus cells are large discrete cells with an
ovoid, vesicular nucleus and pale cytoplasm.
They tend to group in sheets or cords and may
contain granules of melanin pigment in their
cytoplasm. The arrangement of these cells in an
alveolar pattern is referred to as 'theques'.
Intradermal nevi — It is composed of bulk of cells Figure 11.4: Junctional nevus showing nevus cells (NC) at
packed within dense collagenous connective the junction of epithelium (E) and connective tissue (CT)
140 Textbook of Oral Pathology

Spindle cell and epithelioid nevus — It is commonly

composed of pleomorphic cells of three basic
types; spindle cell, oval or epithelioid cells and
both, mononuclear and multinuclear giant cells.
These are arranged in well circumscribed sheets
and there is generally, considerable junctional
activity.
Blue nevus — It is of two types, i.e. the common
blue nevus and cellular blue nevus. In the
common blue nevus, elongated melanocytes
with long branching dendritic processes lie in
bundles, usually oriented parallel to the
epidermis, in the middle and lower thirds of Figure 11.6: Cellular blue nevus

dermis (Fig. 11.5). There is no junctional activity.

The melanocytes are typically packed with
melanin granules, sometimes obscuring the
nucleus. In cellular blue nevus an additional cell
type is present which is a large, round or spindle
cell with pale vacuolated cytoplasm ( Fig. 11.6).
These cells are commonly arranged in alveolar
pattern. Blue type has stellate and fusiform cells
that contain melanin and are located deep in
lamina propria. Few pigmented macrophages
may be present among the dendritic nevus cells.
Spitz nevus—Cells are spindle shaped
(epithelioid) or plump. Epithelioid cells are Figure 11.7: Spitz nevus showing multinucleated
epithelioid cells (Courtesy: Dr Sangamesh Halawar)
multinucleated arranged in bizarre pattern.
There is lack of cell cohesiveness (Fig. 11.7).
Management
It has been customary to recommend the
removal of pigmented mole if it occurs in areas
where they are irritated by clothing, such as belt
of collar line or if they suddenly begin to increase
in size, deepen in color or become ulcerated.

FIBROUS CONNECTIVE TISSUE

Fibroma
It is a benign soft tissue tumor found in the oral
cavity. True benign neoplasm of the fibrous
tissue is relatively an infrequent lesion. Most of
Figure 11.5: Dendritic melanocytes of cellular blue nevus these lesions are infact hyperplasia or reactive
seen below epithelium. NC—nevus cells, E—epithelium proliferation of fibrous tissue.
 Benign Tumors 141

Clinical Features
• It can occur at any age but is common in 3rd,
4th and 5th decades.
• There is no sex predilection.
• It occurs on the gingiva, tongue, buccal
mucosa and palate (Fig. 11.8).
• They are usually painless, but if they are in a
position where they can be bitten or injured,
there may be pain and discomfort.
• It is most often sessile, dome shaped or
slightly pedunculated with smooth contour.
Figure 11.8: Fibroma occurring on gingiva which is
• The lesions on lips and tongue present as dome shaped
circumscribed nodules.
• Tumor sometimes becomes irritated and
inflamed and may show superficial
ulceration.
• Tumor may be very small or in rare instances
may range up to several centimeters in
diameter.
• The consistency can range from soft and
myxomatous to firm and elastic. According
to the consistency the tumor is termed as
'hard fibroma' and 'soft fibroma' (Fig. 11.9).
Figure 11.9: Fibroma on buccal mucosa
Histopathological Features

Fibroma consists of dense bundles of collagen

fibers which interlace with each other. These
bundles are associated with fibroblasts are
plump cells actively secreting collagen fibers. In
addition to these inactive fibrocytes are also seen.
As collagen accumulation increase it creates
tension on the surrounding epithelium. This
causes epithelium to stretch resulting in
epithelial atrophy (Fig. 11.10).
As fibroma is growth seen on the mucosa it
is frequently subjected to trauma. If trauma to
tissue occurs, vasodilation, edema and Figure 11.10: Fibroma showing fibrous tissue (FT) covered
inflammatory cell infiltration will be seen. by an atrophic epithelium (E) devoid of rete pegs. BV—blood
vessels
Sometimes diagnosing fibroma from
inflammatory fibrous hyperplasia becomes fibrous tissue. When healed completely these
difficult. It is inflammatory lesion caused by resemble fibroma. But still there are some
trauma to the tissue that heals by formation of differences between both the lesions (Table 11.1).
142 Textbook of Oral Pathology

Table 11.1: Differences between fibroma and Fibrous Histiocytoma

inflammatory fibrous hyperplasia
It is also called as 'fibroxanthoma'. It may occur
Characters Fibroma Inflammatory fibrous
hyperplasia
in dermis and is rare in oral cavity.
Nature Neoplasia of Inflammatory process Clinical Features
connective tissue
origin • It is common in young adults, with male
Etiology Constant irritation Trauma predominance.
Reversibility Does not regress even Promptly resolves • It is common on lips, tongue, buccal mucosa
after removal of cause once irritant is and palate.
removed
• There is soft, nontender, firm swelling of
Epithelium Stretched and atrophicProliferative with
pseudo- varying size.
epitheliomatous • There is displacement of regional teeth.
hyperplasia • It is a locally aggressive tumor.
Inflamma- It is seen only if lesion Inflammation is
tion is traumatized integral part of the
lesion. Histopathological Features
A benign richly vascular growth, made up of
Management
histiocytes and collagen producing fibroblast like
It is treated by conservative surgical excision. cells, which are arranged in a whorl or cartwheel
pattern. Fibroblasts are elongated spindle shaped
Fibrous Epulis cells, which synthesize collagen. Histiocytes are
Epulis is a term used to denote any growth on large cells with oval nuclei and very thin
the gingica. Fibrous epulis is a term used when cytoplasm.
fibrous growth occurs in the gingiva. The
possible cause of it is irritation from subgingival Management
calculus or adjacent carious tooth. Histo- Surgical excision can be done and recurrence is
pathologically it similar to fibroma. uncommon.
Clinical Features
Desmoplastic Fibroma
• The interdental papilla of the gingiva is the
commonest site. It is intraosseus counterpart of fibroma. It arises
from the mesenchyme of bone. It is also called
• It is more common in females in the ratio of 4:1.
as 'aggressive fibromatosis'. It produces
• The lesion forms a sessile or pedunculated
mass covered by the mucous membrane. abundant number of collagen fibers.
• Color varies from normal tint to deep red,
Clinical Features
depending upon the vascularity and
inflammatory changes. • It most commonly occurs in 2nd decade of life.
• Occasionally superficial ulceration can be • There is no sex predilection.
seen. • In oral cavity it involves mandible more
commonly than maxilla, usually at molar-
Management ramus-angle area.
Lesions are excised with small amount of • Swelling of the jaw and sometimes, pain and
adjacent normal tissue. tenderness may be present.
 Benign Tumors 143

• Buccal cortex is enlarged and more advanced Table 11.2: Differences between fibroma and giant cell
lesions exhibit facial asymmetry. fibroma
• Lesions are aggressive and proliferate Characters Fibroma Giant cell fibroma
rapidly. Etiology Chronic irritation Unknown
• It may be associated with Gardener's Age Seen more commonly Seen more frequently
syndrome. after age of 30 years during third decade.
Size Larger (1to 2 cm) Smaller than fibroma
Histopathological Features (5 mm)
Site It is seen more Seen more commonly
It is composed of cells that may be either small
commonly on cheek on gingival
or uniform, plump, or both. The fibrous product and lips
is generally mature with thick, wavy collagen Surface Smooth Lobulated or papillary
fibers arranged in fascicle. The collagen fibers
are usually thin and delicate with fasciculations
producing a 'herring bone' or 'storiform' pattern.

Management
Wide local excision is the treatment of choice in
these cases. Involved teeth should be extracted.

Giant Cell Fibroma

It is a well circumscribed, benign hyperplastic
lesion of oral mucosa. Although name seems to
indicates presence of giant cells in this lesion,
multinucleated giant cells are not found. Instead Figure 11.11: Giant cell fibroma showing many stellate
fibroblasts with single nucleus which are very shaped fibroblasts
big are seen.
vesicular. Stellate cells have large well
Clinical Features demarcated cytoplasm and dendritic-like
processes which give star shape to the cells.
It has no sex predilection and occurs at any age. These giant cells are present at the surface in
It is slightly more prevalent in women. It is more numbers. Sometimes multinucleated giant
commonly seen in gingiva followed by tongue, cells may be found. Some cells may occasionally
palate, buccal mucosa and lips. It is usually small, contain melanin pigment (Fig. 11.12).
raised, pedunculated, papillary lesion, less than The surface of the lesion is covered by
1 cm in diameter. It is asymptomatic and may mucosal squamous epithelium, which may
be present for several years. exhibit areas of thickening and acanthosis in the
form of deeply extending but narrow and
Histopathological Features
tapering rete pegs.
This tumor is similar to fibroma (Table 11.2). But There are few lesions which are similar to
there is presence of characteristic giant cells giant cell fibroma. These are retrocuspid papilla,
(Fig. 11.11). These are large stellate giant fibrous papule of nose, acral fibrokeratoma,
fibroblasts in the connective tissue. The nuclei fibroblastoma, pearly penile papule of the glans
with giant cells are large, hyperchromatic and penis.
144 Textbook of Oral Pathology

Figure 11.12: High power view of giant cell fibroma Figure 11.13: Myxoma showing reticulin fiber and loose
showing stellate fibroblasts textured tissue (Courtesy: Dr Sangamesh Halawar)

Management is not encapsulated and may invade the

surrounding tissues (Fig. 11.13).
Surgical excision is done and recurrence is rare.
Management
Myxoma It is essentially surgical, recurrence is common.
It is composed of stellate cells arranged in a loose Myxofibroma
mucoid stroma, which also contains delicate
Some areas of fibroma undergo myxomatous
reticulin fibers. Soft tissue myxoma is very rare
degeneration.
in the oral cavity.
Clinical Features
Clinical Features
It occurs anywhere in the oral cavity, most
It can occur at any age and there is no definite commonly on palate, lip and gingiva. It feels
sex predilection. They are deeply situated softer than fibroma and is pale.
lesions, occurring in the skin of the subcutaneous
tissues, genitourinary tract and gastrointestinal Histopathological Features
tract or in organs such as liver, spleen, or even There are regions of dense fibroblastic tissue
in parotid gland. Intraorally, it occurs on the which are interspersed with pale myxomatous
tongue, buccal mucosa and retromolar area. appearing tissue.
The nerve sheath myxoma is a benign tumor
thought to arise from perineural cells of peripheral Management
nerves and is characterized by occurrence of Complete excision.
stellate cells in prominent mucoid matrix.
CARTILAGE
Histopathological Features Chondroma
The soft tissue myxoma is characteristically a It is a benign cartilaginous tumor. In spite of the
loose textured tissue containing moderate fact that mandible and maxilla are membranous
number of delicate reticulin fibers and mucoid bones, they sometimes contain vestigial rests of
material, probably hyaluronic acid. The tumor cartilage.
 Benign Tumors 145

Types
• Enchondroma or central — It develops deep
into the bone. It is more commonly seen.
• Ecchondroma — It develops on the surface.

Origin
Meckel's cartilage is present in the mandibular
arch, prior to the appearance of the bone. It
usually disappears with the beginning of
ossification in the mandibular arch, but it is
possible that the remnants to persist. In some
cases secondary cartilage like fibrocartilage of
mandibular symphysis may persist in the jaw Figure 11.14: Chondroma showing small chondroblasts (CB)
enclosed in lacunae and producing large amount of ground
bone and thus can will give rise to chondroma. substance hyaline cartilage (HC)
The maxilla develops in close association
with chondrocranium. The maxillary sinus The cartilage cells or chondrocytes appear
develops as an outgrowth from the lateral walls small, contain only single nuclei and do not
of the nasal capsule. As it grows into the maxilla, exhibit great variation in size, shape or staining
it may take with it remnants of the cartilage from reaction. These are present in sharp edged
the capsule. In some cases, remnants of the lacunae. These are surrounded by large amount
paraseptal cartilage might persist within the of ground substance (Fig. 11.14). Ground
maxilla. substance stains with blue color. The cytoplasm
of the cells is slightly eosinophilic, granular and
Clinical Features often shows presence of vacuoles (Fig. 11.14).
It occurs in the 5th and 6th decades and males
are slightly favored. It usually occurs in the Management
phalanges and metacarpals. Intraorally, maxilla It should be excised along with the lining
is slightly favored and it occurs in the anterior capsule. It should be covered by chip graft.
region while in mandible, it occurs in premolar- Recurrence is common.
molar region and at symphysis; it may also be
found in condyle and coronoid process. Chondroblastoma
It is painless, slowly growing and is locally It is also called as 'Codman's' tumor. It usually
invasive. Teeth become loose and may be ex- involves long bone.
foliated. The overlying mucosa is seldom ul-
cerated. It is associated with Ollier's syndrome, Clinical Features
in which there are multiple enchondromatosis. It occurs in young persons, under the age of
25 years, with male predominance over the
Histopathological Features
female, usually in the ratio of 2:1. It occurs
Chondroma is cartilage producing tumor. It is usually in epiphyseal region of long bones. The
made of mass of hyaline cartilage, which may usual site is femur and tibia. There are reports
exhibit areas of calcification or necrosis. Tumor that it can occur in condyle of mandible. It is slow
shows hypocellularity and it is avascular. growing, painless mass.
146 Textbook of Oral Pathology

Histopathological Features
It exhibits lobulated myxomatous and fibrous
areas and has a chondroid appearance, i.e. cells
resembling chodroblasts and chondrocytes lie in
lacunae in a chondroid matrix. Foci of
calcification are sometimes found.

Management
Conservative surgical excision should be done.

ADIPOSE TISSUE
Lipoma: It seldom occurs in oral cavity. It is a
Figure 11.15: Chondroma showing chondroblast under
benign, slow growing, tumor composed of
high power
mature fat cells.
Histopathological Features
Types
The tumor is composed of relatively uniform,
• Encapsulated lipoma — It is the commonest
closely packed, polyhedral cells, with occasional
tumor.
foci of chondroid matrix. A scattering of
• Diffuse lipoma — It does not posses typical
multinucleated giant cells may be found, usually
features of lipoma. It is also called as
associated with areas of hemorrhage, necrosis or
'pseudolipoma'.
calcification of the chondroid material. Such
• Lipomatosis — It has multiple lipomas. It
giant cells resemble osteoclasts. Formation of
refers to the symmetrical masses of fat, which
bone and osteoid also occurs. The calcification
sometimes occur around the neck in middle
is present around the cells and follows chicken
age aged man and occurs as painful deposits
wire arrangement.
of fat in women in Dercum's disease.
Management
Clinical Features
Conservative surgical excision is carried out.
It occurs after 40 years of age with peak at
Recurrence is possible after excision.
50 years, male to female ratio is 1:1. It usually
Chondromyxoid Fibroma occurs in upper parts of the trunk, neck and
arms. In oral cavity, it occurs on buccal mucosa
It is an uncommon benign tumor of cartilaginous and mucobuccal fold followed by tongue, floor
derivation. of mouth and lip. It appears as a solitary lesion
with sessile, pedunculated or submerged base.
Clinical Features
Size of lesion is approximately of 1 cm in
It occurs in young persons under the age of diameter. Margins are well contoured and well
25 years, with no definite sex predilection. It is defined. It grows as round or ovoid mass in oral
extremely rare in jaws, but there are some cases cavity. It may be lobulated or may be broadly
reported in mandible. Pain is outstanding feature based or have narrow pedicle. Due to thinness
of this disease and sometimes, swelling can be of the overlying epithelium, yellow coloration
seen. of the fat can be seen.
 Benign Tumors 147

by the occurrence, in infants with solitary or

multiple soft tissue masses, developing at
various sites such as the buttocks, chest, axilla
or neck.
Hibernoma is developed as multivacuolated
fat that is analogous to the brown fat of
hibernating animals; however lesions in oral
cavity are not reported.

Histopathological Features

Figure 11.16: Lipoma presented as round and It is made of circumscribed mass of mature fat
lobulated mass cells. These cells are arranged in lobular pattern
which is formed by connective tissue septae
Most of the lesions are fluctuant and are not coursing through the tumor. These septae carry
freely movable. Surface is smooth, non tender, blood vessels and nerve fibers (Fig. 11.18).
soft and cheesy in consistency. The epithelium Fat cells are large polygonal cells. They
is usually thin and the superficial blood vessels contain large amount of fat. This fat pushes the
are readily visible over the surface. Some lesions cytoplasm and nucleus to the periphery. Nucleus
of lipoma are deep and feel fluctuant on is flattened against the cell wall (Fig. 11.19).
palpation; may be mistaken as cysts.
Management

Surgical excision and recurrence is uncommon.

Figure 11.17: Lipoma showing translucent swelling

on the cheek mucosa

Slip sign, i.e. the edge of lipoma is soft,

compressible and often slips away from the
examining fingers, is positive in this case.
Transillumination test is also positive.
Lipoblastomatosis is a variant of lipoma, but is
not a true neoplasm. It is the continuation of the
normal process of fetal fat development carried Figure 11.18: Lipoma showing collection of adipocytes (A)
into the postnatal life. It is characterized clinically laden with fat. Connective tissue septa (CS) is seen
148 Textbook of Oral Pathology

Figure 11.19: Lipoma (high magnification) Figure 11.20: Cancellous osteoma showing bony trabeculae
surrounded by oral epithelium and containing fibro-fatty
marrow (Courtesy: Dr Alka Kale, Prof and Head, Oral
Pathology, KLES’s Institute of Dental Sciences, Belgaum)
BONE
• It occurs exclusively on skull and facial bone.
Osteoma • It may occur in more than one bone. Mandible
It is a benign neoplasm characterized by is more affected on the lingual side of ramus
and inferior border, below the molars.
proliferation of either compact or cancellous
• There is asymmetry caused by bony hard
bone, usually in an endosteal or periosteal
location. swelling of jaw.
• It is usually painless.
• Mucosa is normal in color and freely movable.
Origin
• Mandibular lesion may be exophytic
It may arise from cartilage or embryonic extending outwards in soft tissues.
periosteum. Periosteal types arise on the surface • Ivory osteoma of the jaw is sometimes
of bone as a pedunculated mass. Endosteal is pedunculated.
located in the medullary bone. Cancellous osteoma:
• It more commonly occurs in females with age
Types same as for ivory osteoma.
• There is predilection to occur in the alveolar
• Compact osteoma (Ivory Osteoma) — It consists process.
of compact bone, which has dense lamellae • It is usually pedunculated, although it might
of bone. have a broad base.
• Cancellous osteoma — Consisting of trabeculae • The surface may be smooth or slightly
of bone. irregular.
Osteomatosis: Multiple osteomas may occur as a
Clinical Features
feature of Gardner's syndrome. Multiple
Compact osteoma: osteomas can also occur in absence of other
• It occurs in individuals older than 40 years. abnormalities. They have been reported in the
It is more common in males, as compared to mandible, frontal and maxillary sinus. Multiple
females. osteomas rarely occur in soft tissue.
 Benign Tumors 149

occurs in vertebral column and sacrum. It is rare

in jaws and if occurs, is found more commonly
in tooth bearing areas of mandible. It is
characterized by pain and swelling of the affected
region, which may be of few weeks to a year
duration. There is local expansion of the bone.

Histopathological Features
Histopathologically it resembles osteoid
osteoma. It is made up of spicules of owen bone.
These spicules are arranged hapazhardly. Many
giant cells are seen in the tumor. Numerous
osteoblasts line these bony trabeculae. These
tumors are quite vascular. Many dilated
capillaries are seen scattered throughout the
tissue. The actively proliferating osteoblasts pave
Figure 11.21: Cancellous osteoma showing bony way for the irregular trabeculae of new bone
trabeculae (T) enclosing marrow spaces (MS)
(Fig. 11.22).
Histopathological Features Management
It is composed of either extremely dense compact Curettage and conservative surgical excision.
bone or of coarse cancellous bone. Cancellous Recurrence may occur.
osteomas are composed of trabeculae of
cancellous bone and the marrow tissue is fatty. Osteoid Osteoma
Prominent osteoblastic activity can be seen It is a variant of osteoblastoma. It is a true
(Fig. 11.21). neoplasm of osteoblastic derivative. It has small
Compact osteoma consists of a dense mass
of lamellar bone with very few marrow spaces.
It is most often well circumscribed and in some
cases, foci of cartilage can be seen.

Management
Resection of osteoma is generally successful.

Osteoblastoma
It is also called as 'Giant osteoid osteoma'.
Osteoblastoma is a rare benign bone forming
neoplasm which produces woven bone spicules,
which are bordered by prominent osteoblasts.

Clinical Features
Male to female ratio is 2:1. Most lesions occur in Figure 11.22: Benign osteoblastoma showing irregular
2nd and 3rd decades of life. It more commonly bony trabeculae
150 Textbook of Oral Pathology

oval or rounded tumor-like nidus which is

composed of osteoid and trabeculae of newly
formed bone deposited within the substratum
of highly vascularized osteogenic connective
tissue tumor.

Clinical Features
It occurs in males between the age of 10 to 15
years. Any part of the skeleton maybe involved,
including the small bone of the hands, feet and
vertebrae. The skull and jaws are rarely involved,
with slight predilection for the mandible. The
main feature of this neoplasm is severe pain, Figure 11.23: Osteoid osteoma showing central nidus (CN)
inspite of the small size of the lesion. Pain around which bony trabeculae (T) enclosing marrow spaces
(MS) are formed
usually occurs at night.
Soft tissues over the involved bone area may
be swollen and tender. It is an oval or round Clinical Features
tumor like lesion. It is has a core of about 1 cm in Central: Age, sex and site — It has got no
diameter. There tends to be a marked reaction, predilection for any sex, age and any particular
which may extend for a considerable distance site. Involvement of jaws is very rare.
from the tumor itself.
Peripheral: It occurs most often in women,
Histopathological Features between the ages of 20 to 39 years. Tongue is the
most common site of involvement in the oral
Osteoid osteoma consist of bony trabeculae that
cavity. The coronoid is also affected. On the
are formed around a central core (Fig. 11.23). The
core is composed of osteoid and newly formed tongue, it appears as a pedunculated swelling
of about 1 to 2 cm in the posterior part of the
trabeculae within highly vascularized osteogenic
dorsum of tongue, near the foramen cecum. It
connective tissue. From this core osteoblasts
differentiate towards surface. Formation of has broad base.
Dysphagia may be the only symptom in this
definite trabeculae, with rimming of active
patient. If there is involvement of the condyle,
osteoblasts occurs. Osteoclasts and foci of bone
resorption are also usually evident. there is difficulty in movements of mandible.
Pain is experienced, either in opening and
Management closing the mouth or in deviating the mandible
Severe pain may be relieved by mild obtundants to one side.
such as aspirin. Complete excision.
Histopathological Features
Osteochondroma This tumors consists of three layers (Fig. 11.24).
It is most likely to represent a choristoma, rather Outer periochondrium, cartilage and innermost
than a neoplasm. It is developmental in origin. layer of bone. Perichondrium is continuous with
There is intermingling of two lesions resulting the periosteal layer of bone in which this tumor
in the term osteochondroma. It may be of central develops. Beneath this layer chondrocytes are
and peripheral types. clustered. This arrangement gives appearance of
 Benign Tumors 151

The growth may be slow or there may be a

period of rather rapid growth which ceases
altogether before the patient has advanced into
adult life. It may be variable in size and shape
and be described as flat, spindle shaped, nodular
or mushroom like. It is covered with normal
mucosa, which appears pale and occasionally
ulcerated, when traumatized. There is a nodular
irregularity around some of the masses of new
bone, formed on one side of the midline.

Histopathological features
It is usually composed of compact bone or
cancellous bone surrounded by a capsule of
compact bone. An inner core of cancellous bone
may be present in the larger specimens.
Figure 11.24: Osteochondroma showing outer peri- Management
chondrium, middle cartilage layer and innermost bone layer
As it is benign, it is usually not treated, except in
some cases where patient requires a complete
a growth plate. Chondrocytes form cartilage that
undergoes ossification. Bone may contain denture and tori is causing more undercuts and
problems for fitting of complete denture. In these
normal bone marrow.
cases surgical removal of tori can be done.
Management Torus Mandibularis
It consists of surgical removal of tumor mass. It is also called as 'mandibular torus'. It is an
exostosis or outgrowth of bone found on the
Torus Palatinus lingual surface of the mandible. They consist
primarily of compact bone.
It is also called as 'palatine torus'. It is a slowly
growing flat based bony protuberance or Causes
excrescence which occurs in the midline of the
Genetic and environmental factors are
hard palate. It has been stated that functional
responsible for its formation. Masticatory stress
stress and genetic factors are important for its
is reported as an essential factor underlying the
origin.
formation.

Clinical Features Clinical Features

It occurs in about 20% of the population. Females It occurs in 8% of the population. It is usually
are affected twice more commonly than males. discovered in middle aged adults. It may occur
Development is initiated in young adults, before singly, multiply; unilaterally, but is usually
30 years. It occurs in the midline of the palate bilateral in premolar region. There is growth on
and may extend to involve the palatal process the lingual surface of the mandible, above the
of the palatine bone. mylohyoid line, usually opposite to the bicuspid
152 Textbook of Oral Pathology

teeth. Their size is variable ranging from an Management

outgrowth that is just palpable to one that
Most cases are unrecognized and are not
contacts a torus on the opposite side.
clinically important.
Management
VASCULAR TISSUE
It does not require any treatment. Removal may
be necessary, if a mandibular denture is planned. Hemangioma

Exostosis It is also called as 'vascular nevus'. It is a benign

tumor which occurs most commonly in vertebrae
It is also called as 'hyperostoses'. They are small
and skull. It is characterized by proliferation of
regions of osseous hyperplasia of cortical bone
blood vessels. It is often congenital in origin. It
and occasionally, cancellous bone, on the surface
is composed of seemingly disorganized vessels
of the alveolar process.
that are filled with blood and is connected to the
Clinical Features main vascular system.
They are less common than mandibular and Types
palatal tori. It may develop on the palatal surface
of maxillary alveolar process, at the border • Central — It occurs in the bone.
between the attached gingiva and vestibular • Capillary hemangioma — It is a mass of
mucosa, in canine or molar area. They seldom intercommunicating capillary vessels of more
attain large size and may be solitary or multiple. or less normal size and structure.
Their shape may be nodular, pedunculated or – Strawberry angioma
flat protuberance on the surface of bone. They – Port wine stain
are bony hard on palpation. – Salmon's patch
• Cavernous hemangioma — It consists of dilated
Management blood containing spaces, lined by
endothelium.
They do not required any treatment. • Arterial or plexiform hemangioma — It arises
from arteries.
Enostosis
It is also called as 'dense bone island'. They are Clinical Features
internal counterparts of exostosis. They are
Central: It is rarely discovered in the jaws. Most
localized growth of compact bone that extends
cases are found at birth or arise at early age.
from the inner surface of cortical bone into the
Female to male ratio is 2:1. The lesion originates
cancellous bone. It is also called as 'whorl'. A rare
either from the periosteum and resorbs the
condition in which there are thousand of dense
underlying bone or it occurs within the bone as
islands of bone scattered through the skeleton is
an anomaly of blood vessels in the marrow
known as 'osteopoikile' or 'osteopoikilosis'
spaces. Mandible to maxilla ratio is 2:1. 50% cases
are found in mandible, mostly in the body and
Clinical Features
ramus area. There is nontender expansion of jaws
It is more common in mandible than maxilla and and the swelling is bony hard in consistency.
commonly found in premolar-molar area. It is Pain is present in many cases and is probably
asymptomatic. throbbing in nature.
 Benign Tumors 153

Compressible swelling, which may pulsate

and bruit may be detected on auscultation. There
is anesthesia of skin supplied by mental nerve.
Bleeding from gingiva around the neck of
affected teeth can occur. Pumping tooth syndrome
is present, i.e. it demonstrates pumping action,
i.e. if the tooth is depressed into the socket, it
will rebound into its original position within few
minutes. Aspiration of the lesion produces blood.
Teeth in affected area may be loosened and may
migrate.

Cavernous Hemangioma
It is common in 1st and 3rd decades and female
to male ratio is 2:1. It may fluctuate in size in
pregnancy and menarche. The oral or
pharyngeal hemangiomas are diagnosed at an
older age, than the lesions from other sites. The
most common site of occurrence are the lips, Figure 11.25: Cavernous hemangioma of lip showing well
tongue, buccal mucosa and palate (Fig. 11.25). circumscribed elevated lesion
It appears as a flat or raised lesion of mucosa.
It is usually deep red or bluish red and seldom mucosal hemangioma is typically a soft,
is well circumscribed. Mass may vary in size and moderately well circumscribed lesion. The large
may become larger on physical activity or lesions are warm and may even be pulsatile if
standing, but may reduce in size once the patient associated with large vessels. The tumor more
is flat on the examining table. Size will not reduce often is traumatized and bleeds profusely. It
with simple elevation. Some lesions are also undergoes ulceration with secondary
pedunculated and globular and some are broad infection. The more superficial ones are often
based and flat or slightly raised. lobulated and will blanch under finger
Compressibility test is positive continued pressure. Deeper lesions tend to be dome-
pressure and squeezing will drive the blood out shaped with normal or blue surface coloration,
of the lesion and the swelling crumbles. As soon they seldom blanch.
as the pressure removed, the swelling reappears
with refilling. In cases of hemangioma affecting Strawberry Angioma
the tongue, there may be loss of mobility of the The typical history of red mark is noticed after
tongue. It may affect a part of tongue or the entire 1-3 weeks of birth. After 1st birthday it regresses
tongue. Some times lesion may increase in size, in size and involution is completed by 7 to 8
which can burry the teeth and cause serious years. Red mark increases in size for a few
deformity and disfigurement. The texture of months, till it takes a typical strawberry or
mucosa may be more or less unchanged, raspberry like swelling. Subcutaneous tissues as
showing an increased vascularity on the surface; well as skin are often involved. Swelling is
but in some cases, appearance is pebbly. The compressible. It is dark red in color. It slightly
154 Textbook of Oral Pathology

protrudes from the skin surface as a sessile

hemisphere. It is irregular and there may be
small areas of ulceration with scabs (Fig. 11.26).

Port Wine Stain

Red hemangiomas are usually capillary, rather
than cavernous variety which are usually more
bluish. These reddish macular hemangiomas are
called as port wine stain (Fig. 11.27). It generally
starts at birth and darkens as the child grows,
but it does not really grow. It is common on the
face and at the shoulders, neck and buttock. The Figure 11.26: Strawberry angioma of lip showing irregular
margin and ulceration
port wine stain is generally smooth, but could
be slightly raised. It is seldom over 5 mm in
diameter. It is deep purple-red in color, which
may become paler in later life. Color blanches
readily on pressure.

Salmon Patch
It is present since birth and usually disappears
before the first birthday. It is seen over the
forehead, occiput or anywhere in the midline of
the body.

Arterial or Plexiform Hemangioma

It is a type of congenital arteriovenous fistula.
There is a pulsatile swelling of the arteries, veins
become tortuous and thick walled; pulsatile
feeling like a bag of pulsating earthworms, is
elicited.

Histopathological Features Figure 11.27: Port wine stain is deep red in color
Capillary: In it, many small capillaries lined by a
single layer of endothelial cells supported by While lacking a capsule, the capillary
connective tissue stroma, are seen (Figs 11.28 and hemangioma is often well circumscribed and
11.29). It is comprised of numerous intertwining there is typically a central feeder vessel with
capillary-sized vessels, lined by endothelium radiating, lobular extensions or vascular
with relatively flat or plump nuclei; depending proliferations, leading some to prefer the
on the duration of the lesion. Those with plump diagnosis of lobular hemangioma. The lumina
endothelial nuclei are younger and often in capillary hemangioma are typically small,
demonstrate mitotic activity, a feature not perhaps to the point of masking the vascular
present in older lesions. nature of the lesion.
 Benign Tumors 155

Figure 11.30: Large dilated cavernous blood spaces

containing clotted blood

Figure 11.28: Blood capillaries (BV) of capillary hemangioma deeper in submucosal tissues. Sluggish blood
tend to be small and of irregular shape. They are lined by flow may result in organized or dystrophically
plump endothelial cells (EC)
calcified thrombi within the dilated vessels. Such
hemangiomas are called as sclerosing
hemangiomas. The vessels may be arranged in
a haphazard or a somewhat lobular pattern and
there may be areas with fibrosis of the
background stroma.
Occasional vascular lesions, in fact, are
dominated so much by dense fibrous stroma that
they are called sclerosing hemangiomas. Chronic
inflammatory cells may be scattered in multiple
foci. Walls are occasionally thickened as a result
of adenventitial fibrosis and inflammatory cells
may be scattered throughout the stroma. In long
Figure 11.29: Capillary hemangioma
standing cases thrombus formation may take
(Courtery: Dr Sangamesh Halawar) place, followed by calcification. Such calcifica-
tions are called as phleboliths.
Cavernous (Fig. 11.30): In cavernous type there
are large dilated blood sinuses which have Management
thin walls each showing endothelial lining.
The sinusoidal spaces are usually filled with It usually regresses by itself during adolescent
blood. period. Laser surgery, cryosurgery by dry ice can
When lesional vascular channels are also be effective.
considerably enlarged, the term cavernous Sclerosing technique — Intralesional injections of
hemangioma has traditionally been applied. This sclerosing chemicals, such as 3% sodium
differs from capillary hemangioma in that it is morrhuate are effective. Injection of boiling water
less well circumscribed, is larger and is usually or hypertonic saline may also be given.
156 Textbook of Oral Pathology

Hereditary Hemorrhagic Telangiectasia

It is also called as 'Rendu-Osler-Weber disease'. It
is a form of hemangioma and is characterized
by numerous telangiectasia or angiomatous
areas which are widely distributed on the skin
or mucosa of the oral cavity. Telangiectasia
represents permanently enlarged capillaries that
are localized superficially, just under the skin
and mucosa.

Clinical Features
They are occasionally present at or shortly after Figure 11.31: Telangiectasia showing defect in vascular
tissue bed
birth, although majority of the cases will not
become conspicuous until puberty. As the Types
patient ages, they appear to increase in number • Superficial—It presents as a circumscribed
and prominence. It occurs on the skin, mucous lesion, which appears as small blisters and
membrane, including oral mucosa and in slightly elevated skin patches.
internal organs. The skin lesions are more • Deep—They are large, cystic, and translucent
common on the face, neck and chest. In oral and may be seen in the neck, mediastinum
cavity, most commonly affected areas are lips, or axilla. These are called as cystic hygroma.
gingiva, buccal mucosa and palate as well as the
floor of the mouth and tongue. There may be Clinical Features
epistaxis and bleeding from oral cavity. The skin
lesions appear as flat macule, raised nodules or They may be present at birth with majority
capillary network. becoming clinically evident early in life, but with a
small number not being manifested for a number
Histopathological Features of years. It may occur alone or in association with
Intrinsic defect in the endothelial cells permitting hemangioma or other anomalous blood vessels. It
their detachment or defect in the perivascular occurs in dorsal and lateral borders of tongue, lips,
supportive tissue bed, which weakens the vessels gingiva and buccal mucosa.
(Fig. 11.31). Usually the disfigurement is noticed by the
parents. Occasionally, the vesicles may be
Management rubbed with clothes, get infected and become
painful. They are soft masses that dissect along
Spontaneous hemorrhages may be controlled by the tissue planes and turn out to be more
pressure packs, particularly nasal bleeding. extensive than anticipated. The surface of the
lesion may be smooth or nodular. Color ranging
Lymphangioma
from normal mucosal pink to bluish and may be
It is a benign hamartomatous proliferation of quite translucent. They are liable to trauma. Due
lymphatic vascular tissue. It is a hamartoma, to this, lesions are subjected to periodic attacks
rather than a neoplasm. In it abnormal vessels of inflammation which cause the swelling to
are filled with clear protein rich fluid containing become larger and tender for the time being.
lymph rather than blood. Aspiration yields lymph that is high in lipids.
 Benign Tumors 157

If the tongue is affected, enlargement may primarily devoid of erythrocytes. Cavernous

occur and the term 'macroglossia' is applied. On type is characterized by presence of dilated
the tongue, it is characterized by irregular sinusoidal endothelium lined vascular
nodularity of the surface of the tongue with gray channels, devoid of erythrocytes. Occasional
and pink, grapelike projection. They are often channels may be filled with blood and it is
elevated and nodular in appearance and may called as hemangio-lymphangioma.
have the same color as the surrounding mucosa.
Lip involvement and its deformity is called as
macrocheilia.
Cystic hygroma is the term used for large
lymphangiomas spreading into and distending
the neck.

Figure 11.33: Lymphangiomas high power

Management
Treatment is generally not indicated for small
lesions. Surgical removal of the bulk of the lesion
can be done. Partial or complete spontaneous
involution is occasionally noted.

Arteriovenous Fistula
It also called as 'arteriovenous shunt' or
'arteriovenous malformation'. It is a direct
communication between an artery and vein that
Figure 11.32: Lymphangioma showing lymph channels with bypasses the intervening capillary bed. It may
this wall consisting of thin endothelial lining. Channels contain
lymph
be congenital or acquired. A lesion with a thrill
or bruit, or with an obviously warmer surface, is
most likely a special vascular malformation,
Histopathological Features
called arteriovenous malformation, with direct
Capillary types are composed of proliferation flow of blood from the venous to the arterial
of thin walled endothelium-lined channels, system, bypassing the capillary beds.
158 Textbook of Oral Pathology

Classification in nature. The color varies from deep red to

• Cirsoid aneurysm—It is a tortuous mass of purple or blue.
small arteries and veins linking a larger artery
and vein. Histopathological Features
• Varicose aneurysm—It consists of endothelium It consists of glomus cells and these may
lined sac connecting an artery and a vein. reproduce to some extent, the structure of the
• Aneurysm varix—It is a direct connection normal glomus (Fig. 11.34). In some cases,
between artery and vein. glomus cell may be arranged around the blood
vessels in a manner suggesting hemangioperi-
Clinical Features
cytoma or the blood vessels may be so prominent
It can occur in alveolar ridge, palate and soft as to resemble cavernous hemangioma.
tissues. There may be extraosseous soft tissue
swelling. It shows purplish discoloration. Bone Management
may be expanded. Pulse may be detected on
auscultation and aspiration produces blood. It is a benign tumor and removal effects cure.

Management Hemangiopericytoma
It should be treated by surgical excision.
It is rare tumor derived from pericytes. It is most
Glomus Tumor common seen in lower extremities with some
cases also occurs in head and neck region.
It is also called as 'glomangioma'. It is a rare
neoplasm derived from glomus cells. They are
thought to be closely related to hemangi- Clinical Features
opericytoma. The glomus is arteriovenous Adults are more commonly affected. It is slow
anastamosis that controls the blood supply and growing, painless mass. In some cases of
temperature of the skin and certain deeper superficial region there may be vascular
tissues. These functions appear to be mediated
pigmentation. In nasal cavity it may result in
in some way by the rich nerve supply and by
symptoms of nasal obstruction and epistaxis.
certain epithelioid cells that ensheath the
arteriole of the glomus. The epithelial cells are
though to be comparable to pericytes.

Clinical Features
The tumor probably arises from these specialized
glomus cells and occur most frequently under
the nails and on the body surface especially in
head and neck area. In the oral cavity the lesion
is usually located on the dorsum of the tongue,
lip, palate, buccal mucosa and tongue. The
tumor usually occurs in the 5th decade. They are
small lesions, rarely exceeding 1 cm in diameter.
They often give rise to attacks of very severe Figure 11.34: Glomus cells (G) of glomus tumor
pain and are exquisitely tender. Pain is stabbing (Courtesy: Dr Sangamesh Halawar)
 Benign Tumors 159

Histopathological Features Clinical Features

It shows tightly packed cells which surrounds It typically occurs near the mental foramen, on
endothelial lined vascular channels. Arrangement the alveolar ridge in edentulous areas or on the
of cells is haphazard with round to ovoid nuclei. lips and tongue. It appears as a small nodule or
The blood vessels (Fig. 11.35) show irregular swelling of the mucosa. Due to the pressure
branching which results in 'staghorn' and 'antler applied by enlargement of the tangled mass in
like' appearance. Dense reticulin network also its bony cavity, severe pain may be experience.
surrounds the vessels. It may have reflex neuralgia with pain referred
to the eye, face and head. It is a slow growing
NEURAL TISSUE reactive hyperplasia that seldom becomes large,
rarely in excess of 1 cm in diameter.
Neuroma
Histopathological Features
It is also called as 'amputation neuroma' or
'traumatic neuroma'. It is not a true neoplasm, but It shows mass of irregular and interlacing
an exuberant attempt at repair of a damaged neurofibrils and Schwann cells, situated in
nerve trunk. connective tissue stroma of either scanty or
plentiful proportion (Fig. 11.36).
Pathogenesis The proliferating nerve fibers themselves
may occur either in small discrete bundles or
Nerve damage may result from fracture,
spread diffusely throughout the tissue.
dissection, removal of cyst, nerve avulsion for
neuralgia or even extraction of teeth. It is an Management
overgrowth of severed nerve, attempting to
regenerate when the scar tissue or malalignment Simple excision of nodule along with proximal
portion of the involved nerve.
of a fractured nutrient canal blocks the distal end.
Proliferating nerve forms unorganized collection
Neurilemmoma
of nerve fibers, composed of varying proportion
of axons, perineural connective tissue and It is also called as 'schwannoma', 'perineural
Schwann cells. fibroblastoma', 'neurinoma' and 'lemmoma'. It is of

Figure 11.35: Hemangiopericytoma showing stag Figure 11.36: Traumatic neuroma showing neurofibrils
horn pattern and Schwann cells (Courtesy: Dr Sangamesh Halawar)
160 Textbook of Oral Pathology

neuroectodermal origin, arising from Schwann

cells that make the inner layer covering the
peripheral nerves.

Clinical Features
It occurs at any age, from very young to very
old, with equal frequency in both the sexes. The
tumor usually occurs in the subcutaneous tissue,
but internal organs such as stomach may be
affected. Intraorally, mandible is the most
commonly affected site for central lesion. Other
sites which can be involved in these tumors are
palate, floor of mouth and buccal mucosa. It is a
slowly growing lesion and is usually of long
duration at the time of presentation.
Figure 11.37: Neurilemmoma showing antonym A types
Usual complaint is lump in jaw, in case of cells surrounding verocay body (arrow)
central tumor and single circumscribed nodule,
in case of soft tissue lesions. Paresthesia may be
Antoni type-B—It does not exhibit this
associated, which occurs anterior to the tumor. characteristic palisading, but rather a
Pain is localized to the tumor site. It usually
disorderedly arrangement of cells and fibers,
occurs singly and jaw expansion may lead to
with vacuolated areas.
perforation. The mass is firm on palpation. It is
non-productive to aspiration. Management

Histopathological Features Surgical excision is the treatment of choice.

Schwannoma is proliferation of schwann cells. Neurofibroma

The tumor is well encapsulated with epineurium
making the capsule. It is composed of two types It is also called as 'neurofibromatosis, 'von
of tissues: Antoni type A and Antoni type B. Recklinghausen's disease of skin' or 'fibroma
Molluscum'. It is inherited as a simple autosomal
Antoni type A — This pattern is distinctive and dominant trait with variable penetrance.
well organized. It is made up of cells with
elongated or spindle shaped nuclei, which are Origin
aligned to form a characteristic palisading
It arises from the connective tissue sheath of
pattern, while intercellular fibers are arranged
Schwann cells and axons. There are three sites of
in parallel fashion between rows of nuclei. When
such clusters of palisaded cells occur around origin: within the inferior dental canal, in the
substance of the bone and beneath the periosteum.
esinophillic substance they create verocay body
(arrow) (Fig. 11.37). The esinophillic material
Clinical Features
substance is made up of cytoplasmic processes
of Schwann cells and duplicated basement It occurs at any age but is found more in younger
membrane. group with no sex predilection. Most commonly
 Benign Tumors 161

affected sites are trunk, face and extremities. It

may appear as numerous sessile or
pedunculated, elevated smooth surfaced
nodules of variable size, which are scattered over
the skin surface.
In other forms, there are deeper, more diffuse
lesions which are often of greater proportion
than superficial nodules and are sometimes
referred to as 'elephantiasis neuromatosa'. In some
cases, loose overgrowth of thickened, pigmented
skin may hang in folds. Café-au-lait spots—in
addition majority of the patients exhibit
asymmetric areas of cutaneous pigmentation,
often described as "cafe-au-lait" spots.

Oral Manifestations
It may occur in mandibular canal, buccal mucosa, Figure 11.38: Herring bone pattern (H) seen in
neurofibroma
and alveolar ridge. The central lesion may have
multiple lesions, occurring in both jaws nerve fibers, the later running throughout the
simultaneously, expanding and filling the
lesion. Mast cells are typically found in this lesion.
maxillary sinus. Solitary central lesion may
Cellular and myxoid patterns predominate.
infrequently be associated with brown spots on Melanocytes are sometimes found in addition to
skin.
mast cells. Some of the lesions may consist of
There are discrete, nonulcerated nodules,
masses of convoluted nerves the individual
which tend to be of the same color as the normal axons of which are surrounded by thickened
mucosa. It may produce pain or paresthesia, if
perineurium. Lesion of this type is called as
associated with mandibular nerve. It may
'plexiform neuromas'.
expand and perforate the cortex, producing a
swelling that is either hard or firm on palpation. Management
Macroglossia may be there due to diffuse
Solitary lesion may be surgically excised.
involvement of the tongue. Prognosis - it has got high potential for malignant
change.
Histopathological Features
Neurofibroma is proliferation of fibroblasts Ganglioneuroma
surrounding the neurites as well as of neurites It is same like neuroblastoma, but in this lesion
themselves. It is composed of proliferation of differentiated cells are numerous. It grows less
delicate spindle cells with thin wavy or rapidly than the neuroblastoma and when fully
serpentine nuclei intermingled with neurites in differentiated, it is depicts benign characteristic.
an irregular pattern as well as delicate The fully differentiated ganglioneuroma is com-
intertwining connective tissue fibrils. Fibers give posed of cells that are very much like the normal
rise to herring bone or storiform pattern cells. Numerous nerve fibers and well differenti-
(Fig. 11.38). It consists of collagenous tissue and ated nerve bundles are present.
162 Textbook of Oral Pathology

Melanotic Neuro Ectodermal Tumor of Infancy Clinical Features

It is also called 'pigmented ameloblastoma', It occurs in middle decades of life. Males are
'melanoameloblastoma', 'melanotic ameloblastoma', affected more commonly than females. It usually
'retinal anlage tumor', and 'melanotic progonoma'. occurs in uterus. It is uncommon in oral cavity
The tumor is of neural crest origin. due to general absence of smooth muscles except
in blood vessel walls and circumvallate papillae
Clinical Features of tongue. The smooth muscle of the arrectores
It occurs in infants under the age of six months, pilorum may be a source of cheek tumor.
with equal sex distribution. Maxilla is more It is a slow growing, painless lesion, which is
commonly affected than mandible. The tumor superficial and often pedunculated. The patient
forms a mass that expands the bone without pain may complain of sore throat or tumor in the
and tenderness. It has rapidly growing, non- throat. In some cases, there may be pain. In most
ulcerated, darkly pigmented lesion. of the cases, the lesion is small. It does not
ulcerate and resembles the normal mucosa in
Histopathological Features color and texture.
It consists of, both pigmented and non
pigmented cells. Pigmented cells are cubical or Histopathological Features
rather flattened and have large, pale nuclei. The It is composed of interlacing bundles of smooth
cytoplasm contains melanin in the form of muscle fibers, interspersed by varying amounts
minute rod shaped particles, often aggregated of fibrous connective tissue. The muscle nuclei
into large masses that obscure all the internal are typically spindle shaped with blunt ends
cellular details. These pigmented cells are (Fig. 11.39).
arranged in solid groups or form a lining of the The bundles of fibers appear to form whorls
small cleft like spaces. Unpigmented cells are because of their fascicular arrangement in
small, round well stained nucleus that nearly fills varying planes. There may be palisading
the cell body. They occur in groups, often within arrangement of the nuclei.
the spaces lined by the pigmented cells. The In some cases, origin from the blood vessels
central portion of the alveolar spaces contain is obvious since the vessels are enlarged with
many neuroblast like cells which show little
cytoplasm and exhibit a round deeply staining
nucleus.

Management
Conservative surgical excision and recurrence is
extremely low.

MUSCLE

Leiomyoma
It is a benign tumor derived from smooth muscle
and is found in a variety of anatomic sites like Figure 11.39: Leiomyoma showing fascicular
skin and subcutaneous tissues. arrangement (Courtesy: Dr Sangamesh Halawar)
 Benign Tumors 163

Histopathological Features
The nucleus is vesicular and cells with several
nuclei are sometimes seen. Based on histological
features, there are two types seen, i.e. adults and
fetal.
In adult type, the tumor is composed of large,
round cells that have granular, eosinophilic
cytoplasm and show irregular cross striations.
The cytoplasm is rich in glycogen and
glycoprotein.
The fetal type is characterized by immature
skeletal muscles in varying stages of develop-
ment and undifferentiated mesenchymal cells.

Management
Figure 11.40: Leiomyoma showing fiber bundles with
blunt ended nucleus (Cigar shaped nucleus) It is excised conservatively usually enucleating
with ease.
thick muscular walls, around which the tumor
muscle fibers are dispersed in a circular manner. Granular Cell Myoblastoma
It is called as angioleiomyoma or angiomyoma.
It is also called 'myoblastic myoma', 'granular cell
Rarely, angiomyoma may contain adipose tissue:
tumor' and 'granular cell Schwannoma'.
then it is called as angiomyolipoma.
Origin
Management
It may be derived from striated muscles. But,
It is treated by conservative surgical excision of
these tumors may be found in areas like breast
the tumor.
and skin, where the striated muscles are absent.
In neural theory it is proposed that these tumors
Rhabdomyoma
are derived from the connective tissue of nerves
It is a benign tumor of striated muscle origin. It and hence was called as 'granular cell neural
is an exceedingly rare lesion. fibroma'. Some authors state that it is derived
from stem cells with a leiomyofibrillogenic
Clinical Features capacity, which may be some type of specialized
Most commonly occurs in 5th decade of life with smooth muscle cells peculiar to certain tissue,
male to female ratio of 2:1. It mostly occurs in that are found in characteristic sites of the tumor.
head and neck region. The most common sites
Clinical Features
of occurrence, intraorally, are the floor of the
mouth, tongue, soft palate, buccal mucosa and Most common site of occurrence is dorsum of
lower lip. It is painless and slowly growing. It tongue followed by skin, lips, breast,
presents as a well circumscribed tumor mass subcutaneous tissue, vocal cord and floor of
which may have a known duration of months mouth. It can occur at any age and females are
or even several years. affected more commonly than males.
164 Textbook of Oral Pathology

Lesion which is found on tongue is usually

single, firm, submucosal nodule within the
substance of the tongue itself. The size of the
tumor varies from a few millimeters to few
centimeters. Lesion is not ulcerated and may
have normal covering or may exhibit some
clinical leukoplakia.

Histopathological Features
The lesion is composed of two types of cells
granular cells and satellite cells. It is made up of
strands and fascicles of cells which are large, 20
to 40 microns in diameter and show an extremely Figure 11.41: Granular cell myoblastoma showing
granular eosinophilic cytoplasm which is abundant cytoplasm (Courtesy: Sangamesh Halawar)
interspersed with a collagenous stroma and
Giant cells might be derived from the
covered with hyperplastic lingual mucosa
proliferating giant cells associated with the
(Fig. 11.41).
resorption of deciduous tooth roots. But for this
The large granular cells are called as
association of the lesion in transition period, i.e.
Abrikossoff myocytes. These granules may be
from deciduous to permanent dentition should
fine or in some instance may be very coarse. In
be there, but such association is found in only
some cases the tumor cells have been found to
few cases.
be arranged in concentric whorls around
Another theory states that it originated from
myelinated nerve fibers.
the endothelial cells of capillaries. To support this
Management theory: there is a common occurrence of giant
cells in vascular channels, suggesting that they
Surgical excision is recommended.
arise from endothelial cells.
Sapp found that giant cells, ultrastructurally,
GIANT CELL NEOPLASM
contain a sufficient number of features in
Giant Cell Tumor common with osteoclasts. This concludes that
they represent a slightly modified form of the
It is applied for the lesions, which contain giant cells.
cells. It is chiefly a tumor of long bones, occurring
at the epiphyseal end involving the adjacent Clinical Features
metaphysis. It is an extremely uncommon tumor It is most frequently seen in 3rd and 4th decades,
of head and neck and creates a lot of confusion
and unusual in patients less than 20 years. The
with respect to diagnosis. It exhibits variable
commonest sites for giant cell tumor are the
clinical and histopathological features ranging lower end of the femur, upper end of the tibia
from benign to malignant.
and lower end of the radius. In the oral cavity it
is rare. The principal symptoms are swelling of
Origin of Giant Cells
the bone accompanied in some cases by pain.
Different theories are given to describe the origin The swelling may be tender and egg shell
of giant cells in these lesions: crackling can be elicited in large tumors.
 Benign Tumors 165

Histopathological Features Peripheral Giant Cell Granuloma

The tumor forms a maroon or reddish brown It is also called as 'peripheral giant cell reparative
fleshy mass that replaces the spongiosa of the granuloma', 'giant cell epulis', 'osteoclastoma' and
bone. It consists of numerous giant cells lying in 'peripheral giant cell tumor'. It is five times more
cellular matrix composed of spindle shaped cells common as compared to central giant cell
and scanty collagen. The giant cells are large with granuloma. It seems to originate from either
numerous vesicular nuclei situated towards the periodontal ligament or mucoperiosteum.
centre of the cells leaving a clear area of
Etiology
cytoplasm around the periphery. The cytoplasm
is granular and vacuoles are often present. There It is an unusual response of tissue to injury. The
is even distribution of giant cells throughout the trauma may be caused by tooth extraction and
lesion (Fig. 11.42). dental irritation. It can occur in chronic
infections. It may appear under the stimulus of
Management increased circulating parathormone, i.e. primary
and secondary hyperparathyroidism.
The usual method of treatment is curettage, but
this is followed by a high recurrence rate. Clinical Features
It is most oftenly seen over 20 years of age, with
Giant Cell Granuloma
an average of 45 years. Females are affected twice
Types as common as males. It is predominant in white
persons. It occurs on gingiva and alveolar
• Peripheral giant cell granuloma—It involves
mucosa, most frequently anterior to molars. It is
gingiva and alveolar mucosa.
• Central giant cell granuloma—Occurs as an common in mandible than maxilla.
In early stage it appears as discoloration and
endosteal lesion in the jaw bones.
slight swelling of the buccal aspect of the gingiva.
Later the lesion increase in size and becomes
rounded and very often pedunculated.
Sometimes, it grows in an hour-glass manner
(Fig. 11.43), with the waist of the lesion between

Figure 11.42: Giant cell tumor showing many giant cells Figure 11.43: Peripheral giant cell granuloma showing
dispersed in cellular background growth in hour glass manner
166 Textbook of Oral Pathology

two teeth and the globular extremities presenting

buccally and lingually.
The color of the lesion is usually dark red or
maroon color. If sufficient amount of
hemosiderin exists near the periphery, the lesion
is bluish, other wise it is red or pink. Lesions with
much fibrous tissue are paler. It may feel soft to
hard. The lesions vary in size from 0.5 to 1.5 cm
in diameter. It is vascular or hemorrhagic and
sometimes ulceration is also present (Fig. 11.44).
There may be tenderness on palpation. In
edentulous patients, it may present as a vascular, Figure 11.44: Peripheral giant cell granuloma showing
ulcerated growth
ovoid or fusiform swelling of the crest of the
ridge, seldom over 1-2 cm in diameter or there
may be granular mass of tissue which seems to
be growing from the tissue covering the slope of
the ridge.

Histopathological Features

Multinucleated giant cells and young spindle

shaped cells are scattered throughout the
granulation tissue in delicate reticular and
fibrillar connective tissue stroma. Giant cells are Figure 11.45: Giant cell granuloma showing epithelium
usually more numerous and may be large.
The matrix consists of spindle shaped cells
with oval nuclei, similar to those of the giant cells. Teratoma
Capillaries are numerous around the periphery It is a neoplasm composed of a mixture of tissues
of the lesions and giant cells, sometimes, may be more than one of which exhibit neoplastic
found in it. proliferation. It is congenitally acquired and
Extravasated erythrocytes and varying usually found in the ovary. The finding of
amounts of hemosiderin are also present various organs like structure, i.e. teeth, tissue,
(Fig. 11.45). The overlying epithelium is normal, hair and skin are seen in this tumor. Lesions that
but sometimes it may shows acanthosis and arise in the base of the skull often extend into
ulceration. the cranial and oral cavity; newborn infants with
such lesions rarely survive. True teratoma occurs
Management sometimes and it is called as 'epignathus'. It
arises from the hard and soft palate which may
Excision with borders of normal tissue with be perforated.
entire base of the lesion should be done to avoid The tumor tends to fill the buccal cavity and
recurrence. Elimination of chronic irritant should may extend into the mouth. The tumor is made
be done. up of three germinal layers and it is usually in
 Benign Tumors 167

the midline region. Epignathi may develop in the

region of Rathke's pouch and may be found
entirely above the palate or even within the
cranium. Growths consist mainly of adipose
tissue, often with a central area of cartilage or
bone and are covered by stratified squamous
epithelium, with pilosebaceous follicles. In some
growths, nerve tissue is present. In rare cases,
grapefruit size of tumor protruding from the
mouth arise from the palate and be a well formed
finger with a finger nail on its upper part.
Microscopically, it consists of connective tissue,
smooth muscles, skin and dermal appendages
(Fig. 11.46).

Nasopharyngeal Angiofibroma
It is rare vascular and fibrous tumor like lesion
that occurs only in nasopharynx. It is locally
destructive behavior.

Clinical Features
It occurs exclusively in males in age group of
second decade.
Symptoms — Nasal obstruction and epistaxis are
common symptoms.
This tumor expands medialy via sphenopa-
latine foramen.
Figure 11.46: Teratoma showing dermal appendages

Histopathological Features
tissue that consists of proliferative endothelial
It consist of dense fibrous connective tissue that tissue with rich patent capillary bed, chronic
contain numerous dilated, thin walled blood ves-
inflammatory cells and few fibroblasts. They are
sels. Vascular components are more prominent
smoothly contoured or lobulated with very red
at periphery than at the center (Fig. 11.47). appearance due to rich vascularization and
transparency of nonkeratinized epithelial
Management
covering. It has got soft, spongy and bland sessile
Surgical excision should be done. base (Fig. 11.48).

Inflammatory Hyperplasia Pyogenic Granuloma

Chronic injury produces inflammation, which in It is also called as 'granuloma pyogenicum'. It is a
turn stimulates the formation of granulation response of tissues to nonspecific infection of
168 Textbook of Oral Pathology

by nonspecific of microorganisms. It is an
inflammatory response to local irritation such as
calculus. It is contributing factors to the
development of some pyogenic granulomas.

Clinical Features
Females are affected more than males with
common age of occurrence are 11 to 40 years.
Common sites are gingiva, lip, tongue, buccal
mucosa, palate, vestibule and alveolar mucosa. It
is an asymptomatic papular, nodular polypoid
mass.
Lesions are elevated, pedunculated or sessile
Figure 11.47: Dilated blood vessels present in fibrous masses with smooth, lobulated or even warty
stroma in case of nasopharyngeal angiofibroma
surface, which commonly ulcerates and shows
tendency to hemorrhage upon slightest pressure
or trauma. It has variegated red and white
pattern. Sometimes, there is exudation of
purulent material.
It is deep red to reddish purple depending
on the vascularity; painless and rather soft in
consistency, with some lesions having brown
cast, if hemorrhage has occurred into the tissues.
It feels moderately soft and bleeds readily. If
lesion is of mixed variety it appears red with pink
areas and if it is completely fibrous it is slightly
pink and firm to palpation. It may develop
Figure 11.48: Inflammatory hyperplasia present on lower
rapidly, reaching full size and remain static for
gingiva
an indefinite period with size ranging from few
Staphylococcus or streptococci. It is relatively a millimeters to centimeters. Average size of lesion
common soft tissue tumor of the skin and mucus being 0.9 to 1.2 cm. It may become mature and
membrane. It is known to be a reactive becomes less vascular and more collagenous
inflammatory process in which there is an gradually converting into a fibrous epulis.
exuberant fibro- vascular proliferation of the Average size of lesion being 0.9 to 1.2 cm.
connective tissue, secondary to some low grade
chronic irritant. Histopathological Features

Granulation tissue is exuberant and is usually

Etiology
localized. The overlying epithelium, if present,
It is usually due to botryomycotic infection, is generally thin and atrophic, but may be
staphylococci and streptococci. It may occur as hyperplastic. If the lesion is ulcerated, it shows
a result of minor trauma to the tissue, which a fibrinous exudate of varying thickness over the
provides a pathway for the invasion of tissues surface. There is occurrence of a vast number of
 Benign Tumors 169

endothelium-lined vascular spaces with extreme Pregnancy Tumor

proliferation of fibroblasts and budding
Clinical Features
endothelial cells (Fig. 11.49).
There is moderately intense infiltration of It occurs during pregnancy, often in about 3rd
polymorphonuclear leukocytes, lymphocytes month of pregnancy and sometimes later. It
and plasma cells. The connective tissue stroma occurs as a result of local minor trauma and in
is delicate, although fasciculi of collagen fibers cases where tissue reaction is intensified by
are noted coursing through the tissue mass. If endocrine alteration occurring during
the lesion is not excised, then there is gradual pregnancy. It is a well defined lesion, which
obliteration of many capillaries, as it assumes gradually increases in size and may or may not
fibrous appearances. regress after delivery. If surgically removed
during pregnancy, it usually recurs
Management
Surgical excision and care is taken to remove the Histopathological Features
calculus of adjacent teeth as it may act as a local It is histologically identical to pyogenic
irritant and cause recurrence. Elimination of the granuloma of gingiva.
causative agent.
Hormonal Tumor
It is an inflammatory hyperplasia type of lesion
seen at the border of ill fitting dentures. In most
of the cases, dental flanges are over extended due
to alveolar bone resorption.

Clinical Features
It occurs in old age with female predominance.
It is more common in maxilla than mandible;
anterior region is more affected than posterior
region. It occurs under the buccal and labial
flanges. The exophytic elongated epulis has at
least one cleft, with proliferation of tissues on
each side. It is asymptomatic.

Management

Figure 11.49: Pyogenic granuloma showing Reduce the denture flanges if the lesion is small.
inflammatory infiltrate Excision with sulcus deepening for larger lesions.
170 Textbook of Oral Pathology

CHAPTER
Premalignant Lesions
12 and Conditions

CONCEPT OF PRECANCER normal counter parts. For example leukoplakia,

erythroplakia, mucosal changes associated with
It is applied to that altered state of tissue which smoking habits, carcinoma in situ, Bowen
often, but not always, has high potential to disease, and actinic keratosis, cheilitis and
undergo malignant transformation. Some benign elastosis.
lesions or conditions, for varying length of time,
Precancerous condition — It is defined as a
also generally precede oral cancer. Interestingly,
generalized state or condition associated with
these lesions or conditions share the same
significantly increased risk for cancer
etiological factors with oral cancer, particularly
development. For example, oral submucus
the use of tobacco and exhibit same site and habit
fibrosis (OSMF), syphilis, sideropenic dysphagia,
relationship. Many of them show high potential
oral lichen planus, dyskeratosis congenita and
to become cancer and are therefore termed as
lupus erythematosus.
precancerous.
It is especially important to remember that a
Leukoedema
premalignancy is not guaranteed to eventually
transform into cancer, as is often but erroneously It is an abnormality of the buccal mucosa, which
believed. Many precancers, in fact, do so only in clinically resembles early leukoplakia.
a small proportion of cases, forcing the clinician
to make some very real choices relative to the Etiology
management of such lesions. Individuals with Use of tobacco, racial and related to poor oral
oral precancer run 69 times greater risk of hygiene.
developing oral cancer as compared to tobacco
users who do not have precancer. Clinical Features
Oral precancer is distinguished into: It is common in age group of 15 to 35 years with
Precancerous lesion—It is defined as a prevalence in black. Male predilection in the ratio
morphologically altered tissue in which cancer of 2:1. The most common sites of involvement
is more likely to occur, than its apparently are buccal mucosa and lip. The lesion is bilateral.
 Premalignant Lesions and Conditions 171

Buccal mucosa retains the normal softness not associated with any other physical or chemical
and flexibility but exhibits grayish white, slightly causative agent except the use of tobacco.
folded opalescent appearance that is described
as epithelium covered with diffuse edematous Classification
film or velvetlike veil. According to clinical description
Mother of pearl appearance—In some cases, • Homogenous — It is completely whitish lesion
lesion is diffuse and shows a filmy, mother of – Flat — It has smooth surface
pearl appearance, often with delicate over- – Corrugated — Like a beach at ebbing tide
lapping curtain like mucosal folds. – Pumice like — With a pattern of fine lines
In this disease desquamation may occur (cristae)
which may leave surface of the lesion eroded. It – Wrinkled — Like dry, cracked mud surface.
can be eliminated by the stretching and scraping • Nonhomogenous
of mucosa but re-establishes itself almost – Nodular or speckled — Characterized by
immediately. white specks or nodules on erythematous
base
Histopathological Features – Verrucous — Slow growing, papillary
Increased thickness of epithelium, broad rete pegs proliferations above the mucosal surface
and intracellular edema of spinous layer. Cells at that may be heavily keratinized. Extensive
the surface are flattened and may retain pyknotic lesion of this type is called as ‘oral florid
nuclei that contain glycogen. The characteristic papillomatosis’.
edematous cells appear extremely large and pale, – Ulcerated — Lesion exhibits red area at the
and they present a reticular pattern. periphery of which white patches are
present
Management – Erythroleukoplakia—Leukoplakia is
present in association with erythroplakia.
No treatment is necessary as it has not got any
pre-malignant potential. According to risk of future development of oral
cancer
LEUKOPLAKIA • High-risk sites
– Floor of mouth
Definitions – Lateral or ventral surface of tongue
– Soft palate
The term leukoplakia originates from two Greek
• Low-risk sites
words - leuko, i.e. white and plakia, i.e. patch. It
– Dorsum of tongue
is defined as any white patch on mucosa, which
– Hard palate
can not be rubbed or scraped off and which can
• Intermediate group
not be attributed to any other diagnosable
– All other sites or oral mucosa.
disease. It may also be defined as a keratotic
white lesion of oral mucosa that can not be According to histology
characterized clinically or histopathologically as • Dysplastic
any other disease entity. • Nondysplastic.
By WHO — It is a whitish patch or plaque According to extent
that can not be characterized, clinically or • Localized
pathologically, as any other disease and which is • Diffuse.
172 Textbook of Oral Pathology

According to Banoczy are the extracts of tobacco, containing various

• Leukoplakia simplex—A uniform raised plaque chemical constituents such as nitrosonornicotine,
formation, varying in size, with regular nicotine, pyridine, picoline and collidin. All these
edges. It corresponds to homogenous type of chemical constituents as well as the alkaline pH
leukoplakia. of snuff (8.2 to 9.3) act as local irritants and are
• Leukoplakia erosiva—A lesion with slightly related to the alterations of mucosa. Smokeless
raised, rounded, red and/or whitish tobacco is believed to result in chemical damage
excrescence, that may be described as that produces sublethal cell injury within the
granules or nodules. deeper layers of oral epithelium. This in turn,
• Leukoplakia verrucosa — It is characterized by induces concomitant epithelial hyperplasia.
verrucous proliferation raised above the Smoking tobacco is harmful as this smoke
mucosal surface. contains polycyclic hydrocarbons, beta-
naphthylamine, nitrosamines, carbon monoxide,
Staging of Leukoplakia nicotine, etc. which act as source of irritation. The
In this staging three parameters are used: heat produced by smoking tobacco also plays a
• Size — It is denoted by L major role. Exposure to heat results in alteration
– L1 – Size is less than 2 cm of tissue. The initial signs of heat induced
– L2 – Size is in the range of 2-4 cm alteration of tissue are increased reddening and
– L3 – Size is more than 4 cm stippling of mucosal surface. As the use of irritant
– Lx – Size is not specified continues with the exposure to heat, minute
• Clinical aspect — It is denoted by C white and red striations are formed and the
– C1 – Homogenous tissue surface may appear slightly swollen. The
– C2 – Nonhomogenous striations may be caused by increased capillary
– Cx – Not specified proliferation and keratin formation. With
• Pathological features — It is denoted by P continued irritation, the lesion precipitates with
– P1 – No dysplasia circumscribed configuration.
– P2 – Mild dysplasia
– P3 – Moderate dysplasia Alcohol
– P4 – Severe dysplasia The prevalence of leukoplakia is higher among
– Px – Not specified. the regular and occasional drinkers than the
nondrinkers. It causes irritation and burning
Etiopathogenesis sensation of oral mucosa, when applied locally.
Local Factors Alcohol facilitates the entry of carcinogen into
exposed cells and thus alters the oral epithelium
Tobacco and its metabolism. But most alcohol consumers
use tobacco in some form or the other.
It refers to dried leaves of nicotina tobaccum.
Tobacco is used widely in two forms. It can be
Chronic Irritation
used in smokeless tobacco like chewable tobacco
and oral use of snuff or it can be used as smoking Continuous trauma or local irritation in the oral
tobacco such as cigar, cigarette, bidi and pipe. cavity is suspected as a causative agent for
When tobacco is chewed, various materials leach leukoplakia. The source of irritation or trauma
out of it, such as tobacco tars and resins. These may be any viz. malocclusion, ill fitting dentures,
 Premalignant Lesions and Conditions 173

sharp broken teeth, hot spicy food, root piece etc.

The usual site to such irritation is the buccal
mucosa and less often the alveolar ridge. Chronic
irritation must be intense enough to induce
surface epithelium to produce and retain keratin.
Leukoplakia is a protective reaction of the
traumatized mucosa against chronic irritation.
Mechanical factor in combination with other
thermal and chemical factors, account for the
etiology of more than 40% cases of leukoplakia.

Candidiasis
The presence of Candida albicans has been
reported very frequently in association with
leukoplakia, more commonly with nodular type.
Candidial leukoplakia may be associated with
other local factors, such as tobacco smoking,
denture wearing or occlusal friction. Tobacco
smoking may result in candidal colonization Figure 12.1: Etiopathogenesis of leukoplakia
because of increased keratinization, reduced
salivary immunoglobulin. A concentration or predilection for the actively mobile tissues of
depressed polymorphonuclear leukocyte tongue. These tissues are heavily involved
function (Fig. 12.1). during secondary stage of syphilis which leads
Electromagnetic reaction or galvanism: Galvanism to diffuse vasculitis and progresses to obliterative
is the generation of current due to difference in endarteritis, eventually resulting in a circulatory
the electrical potential of two dissimilar metals. deficiency to the lingual papillae. This causes
Galvanic current may arise in mouth between atrophy of filiform and fungiform papillae and
dissimilar, opposing or adjacent metallic results in bald, smooth lingual surface. Shrinkage
restorations. Patient’s complaint may range from of the lingual musculature may also occur
a mere metallic taste to persistent pain, due to resulting in a wrinkled surface. With the
chronic inflammation of adjacent oral mucosa protective papillae missing, the dorsum of
to even neuralgic pain. These mucosal changes tongue is left extremely susceptible to oral
may promote malignant transformation of irritation and leukoplakia frequently develops
leukoplakia. on it. Leukoplakic involvement may be minor
or severe and it may be diffuse or localized. In
Regional and Systemic Factors many cases, leukoplakia is of dysplastic variety.
Carcinoma of tongue frequently develops in such
Syphilis
cases of leutic glossitis.
It is regarded as a predisposing factor for the
development of leukoplakia. White patches are Nutritional Deficiency
often seen on tongue in tertiary syphilis. Deficiency of vitamin A is known to produce
Spirochete, the causative agent for syphilis has metaplasia and keratinization of certain
174 Textbook of Oral Pathology

epithelial structures. Hence, it may be causative It can occur anywhere on the oral mucosa.
factor for leukoplakia. Patients with leukoplakia Buccal mucosa and commissures are more
show lower serum levels of vitamin A. Vitamin commonly involved. The lesion is regarded as
B complex deficiency has also been suggested ‘commissural’, if it extends posteriorly from labial
as a predisposing factor. It might be related to commissure over a distance of about 2 cm, in
alteration in the oxidation pattern of the triangular shape and as buccal, if it involves the
epithelium, making it more susceptible to central zone of buccal mucosa in the molar region
irritation. In some cases of sideropenic anemia and along the occlusal line. In males commissural
leukoplakia can occur. involvement occurs more frequently than buccal
Condition causing xerostomia: Salivary gland one with the latter being the commoner site in
diseases, anticholinergic drugs and radiation can females. Lip lesions are more common in men
cause some cases of leukoplakia. and tongue lesions are more common in women.
The involvement of various sites depends upon
Hormones: It is difficult to demonstrate signi- the type of tobacco habit. Sublingual keratosis refers
ficance of male and female sex hormone
to leukoplakia occurring in floor of mouth and
deficiency and endocrine dysfunction in the
ventral surface of tongue.
etiology of leukoplakia. The extent of involvement may vary from
Drugs: Some drugs like anticholinergic, small, well-localized, irregular patches to
antimetabolic and systemically administered diffused lesions involving considerable portion
alcohol may predispose for the leukoplakia. of oral mucosa. Multiple are Patch of leukoplakia
Virus: Two types of viruses have been linked with may vary from nonpalpable, faintly translucent,
leukoplakia viz. herpes simplex and human white area to thick fissured papillomatous
papilloma virus HPV. A specific increase in cell- indurated lesion areas of involvement are not
mediated immunity to HSV was observed in uncommon. The surface of the lesion is often
dysplastic leukoplakia. HPV associated antigen finely wrinkled or shriveled in appearance and
has also been demonstrated in cases of may feel rough on palpation. Lesion may be
leukoplakia. These viruses are believed to induce white or yellowish white, but with heavy use of
mucosal changes by altering the DNA and tobacco may assume brownish color.
chromosomal structure of the cells and by Some leukoplakias that occur in the floor of
inducing proliferation of such altered cells. the mouth are referred to as ‘ebbing tide’ type
since they appear similar to the undulations left
Idiopathic or cryptogenic leukoplakia — In a small
on the sand by the ebbing tide. It occurs due to
proportion of cases, no underlying cause has
loose binding and consequent movement of the
been found. Such lesions are termed as idiopathic
mucosa in the floor of mouth.
(cryptogenic) leukoplakia. These lesions have
Some patients may report a feeling of
higher potential for malignant transformation.
increased thickness of mucosa. Those with
ulcerated and nodular type may complain of
Clinical Features
burning sensation. Enlarged cervical lymph
Sex and age distribution: It occurs more commonly nodes may signal occurrence of metastasis. Some
in older age group, i.e. 35 to 45 years and above. times leukoplakic changes may be reversed by
Males are affected more frequently than females merely removing the source of irritation.
due to direct consequence of tobacco habit. Preleukoplakia: A different entity termed as ‘pre-
Prevalence in India is 0.2 to 4.9%. leukoplakia’ has been distinguished. It is a low
 Premalignant Lesions and Conditions 175

grade or very mild reaction of the mucosa

appearing as grayish white but not completely
white lesion with slight lobular pattern and
indistinct borders blending with the adjacent
normal mucosa.

Staging (Sharp)
• Stage I—Earliest lesion—nonpalpable, faintly
translucent, white discoloration.
• Stage II — Localized or diffuse, slightly
elevated plaque of irregular outline. It is
opaque white and may have fine granular Figure 12.2: Leukoplakia on right side of buccal mucosa
showing homogenous pattern
texture.
• Stage III — Thickened white lesion showing
induration and fissuring.

Clinical Types
Homogenous
It is also called as leukoplakia simplex. It accounts
for 84% of cases. It is usually, localized lesions
of extensive white patches present a relatively
consistent pattern throughout. However
sometimes the surface lesion may be described
as corrugated, with pattern of fine lines, or
wrinkled or papillomatous surface (Fig. 12.2). It Figure 12.3: Homogenous leukoplakia seen on right
is characterized by raised plaque formation buccal mucosa
consisting of single or group of plaques varying
in size with irregular edges. They are usually pigmentation of varying intensity, usually on the
white in color but may be yellowish white or periphery of the lesion. Heat produced during
yellow (Fig. 12.3). Leukoplakia seen amongst smoking also contributes to the occurrence of
clay pipe smokers and betel quid chewers are pigmentation.
generally of homogenous type. They have no red
component. Nodular Leukoplakia
It is also called as ‘leukoplakia erosiva’ or ‘speckled
Ulcerated Leukoplakia leukoplakia’. A mixed red white lesion is seen in
It occurs in 13% of cases. It is characterized by which small keratotic nodules are scattered over
red area, which at times exhibit yellowish areas an atrophic patch of oral mucosa. Nodules may
of fibrin, giving the appearance of ulceration. be pinhead sized or even larger. It has got a high
White patches are present at the periphery of the malignant potential.
lesion. It is seen exclusively at commissures and
anterior part of buccal mucosa. Sometimes, it Verrucous Leukoplakia
manifests as an ulceration with minimum It is also called as ‘leukoplakia verrucosa’. It is
keratinization. Sometimes, it is associated with characterized by verrucous proliferation above
176 Textbook of Oral Pathology

the mucosal surface. The most common sites are

buccal mucosa, alveolar mucosa, and tongue,
floor of mouth, gingiva and lips. The disease is
most commonly seen in elderly women and is
usually detected several years after its occurrence
due to its slow growing nature. It is seen in 6th
and 7th decades of life. It is seen on alveolar
mucosa and cheek. It is a white lesion with a
broken up surface due to multiple papillary
projections that may be heavily keratinized. They
may be accompanied by homogenous leukoplakia
on other oral mucosal surfaces. It begins as a
simple solitary hyperkeratosis, but tends to Figure 12.4: Frictional keratosis
become multifocal over varying period of time. It (Courtesy: Dr Sangamesh Halawar)

is slow growing, persistent and irreversible. In the

course of time erythematous component may
develop in the lesion. Later, it becomes exophytic
Epithelial Changes
and wartlike and transforms into a lesion that is
clinically and microscopically identical to Vacuolar degeneration: Cytoplasmic structure is
verrucous carcinoma or squamous cell carcinoma. indistinct and the nuclei are pushed to the cell
Oral florid papillomatosis: Extensive lesions of periphery. Vacuolar degeneration may occur in
verrucous leukoplakia are called ‘oral florid the stratum spinosum or stratum germinatum.
papillomatosis’. It may be called as ‘liquefaction degeneration’.
The changes may cause separation of the
Histopathological Features
epithelium from the subepithelial connective
Surface changes: There occurs keratinization of tissue, by deposition of fluid.
mucosal epithelium that is normally non-
keratinized or there is an increase in the keratin Acanthosis: An increase in the number of cells of
layer in the areas that are normally keratinized. stratum spinosum (spinous layer or prickle cell
layer) leading to the abnormal thickening of the
Hyperorthokeratosis: In normal state, cells of the layer (Fig. 12.5).
superficial layer of epithelium are nearly homog-
enous, eosinophilic and anuclear with stratum Basal cell hyperplasia: An increase in the number
granulosum always present (Orthokeratosis). In of cells in the basal layer of epithelium (Fig. 12.6).
hyperorthokeratosis there is abnormal increase in Intra-epithelial edema: There can be intracellular
thickness of orthokeratin layer or stratum cor- edema (hydropic degeneration) which consists
neum in a particular location. of swollen, rounded epithelial cells with a
Hyperparakeratosis: Superficial epithelial cells are honeycomb like cytoplasm, but with the same
flat and acidophilic with pyknotic nuclei; staining affinity for the nucleus or intercellular
stratum granulosum may or may not present edema (spongiosis) which consists of dilation of
(Parakeratosis). In hyperparakeratosis there is intercellular spaces and disruption of
increased thickness of parakeratotic layer, intercellular junctions due to accumulation of
exceeding the normal thickness (Fig. 12.4). fluid between the epithelial cells.
 Premalignant Lesions and Conditions 177

is replacement of degenerated elastic fibers by a

hyaline fibrous tissue. This is known as hyaline
degeneration. It is seen in 10% of cases.

Dysplastic Changes

All leukoplakias do not lead to malignancy. Only

a few of them do so. There are various features
which indicate probability of leukoplakia
progressing to malignancy which are called as
dysplastic features and lesions which show such
features are said to be having epithelial
dysplasia. Epithelial dysplasia is reported in 3%
of snuff induced leukoplakias and 16% of
smoking habit-related to leukoplakia. Nodular
leukoplakia shows higher frequency of epithelial
Figure 12.5: Leukoplakia showing acanthosis of stratum dysplasia as compared to others. It is usually
spinosum graded into mild, moderate and severe
categories, depending upon the degree and
extent of involvement of the epithelium.
Histological diagnosis is established if two or
more of the following signs are present:
Microscopic features associated with oral
epithelial dysplasia.

Architectural (tissue) Changes

• Loss of polarity
• Abnormal orientation of epithelial cells
• Basal cell hyperplasia
• Increased cellular density
• Bulbous drop-shaped rete pegs
• Abnormal stratification of the epithelium
Figure 12.6: Leukoplakia showing 1- hyperorthokeratosis, • Disordered maturation from basal to
2-acanthosis, 3-juxtaepithelial inflammatory cells, 4- broad squamous cells
blunt retepegs, 5-basal cell hyperplasia (Courtesy:
Dr Sangamesh Halawar)
• Dyskeratosis.

Cellular Changes
Connective Tissue Changes
• Abnormal variation in nuclear size
Chronic inflammatory cell infiltration is seen in (anisonucleosis)
connective tissue of leukoplakia in 50% cases. • Abnormal variation in cell size (anisocytosis)
Submucosal, homogenous, eosinophilic material • Increased nuclear/cytoplasmic ratio
is usually seen in connective tissue. Also, there • Enlarged nuclei and cells
178 Textbook of Oral Pathology

• Hyperchromatic nuclei is abnormal and it may start from the lower level
• Increased mitotic figures itself. So, in such lesion keratinised epithelial cells
• Abnormal mitotic figures (abnormal in shape may be found in prickle cell layer where they
or location) are usually not present. This is called as
• Nuclear and cellular pleomorphism individual cell keratinisation. Sometimes a group
• Increased number and size of nucleoli. of flattened epithelial cells forming tight
concentric rings leading to formation of keratin
Cytological Features of Dysplasia pearl may be seen.
Loss of polarity and abnormal orientation of epithelial
cells: Basal epithelial cells are usually cuboidal Cellular Changes
or short columnar. They are arranged
Abnormal variation in nuclear size (anisonucleosis):
perpendicular to basement membrane. In
In dysplasias there are profound changes in the
dysplastic lesions this arrangement is changed.
nucleus. These include variation in nuclear size
Cells become hapazhardly arranged on the
and shape. Nucleus becomes enlarged with
basement membrane.
prominent nucleoli. All these changes indicate
Basal cell hyperplasia: Basal cells are most active that nucleus is very active.
cells that have capacity to divide. As in dysplasia
Abnormal variation in cell size (anisocytosis):
there is increased mitosis the basal cells devide
Cytoplasm also shows great variation is size and
to form a large number of basaloid cells.
shape. Few cells become very big gaining giant
Increased cellular density: Because of increased proportions.
mitosis there is increase in the cell density in the
Increased nuclear/cytoplasmic ratio: Normal
epithelium.
nuclear/cytoplasmic ratio is in the range of 1:4
Bulbous drop-shaped rete pegs: As basal cell divide to 1:6. In malignancy and premalignant lesions
they make the basal part of rete peg very broad. this ratio is altered as there is increase in size of
This gives rete peg bulbous shape of drop shape. nucleus and cytoplasm. Nucleus and cytoplasm
Disordered maturation from basal to squamous cells: become almost equal in volume.
Epithelial cells gradually mature as they move Abnormal mitotic figures (abnormal in shape or
towards the surface. As they move they start location): In addition to increased mitosis there
forming keratin and accumulating it. This is formation of abnormal mitosis leading to
process is called as maturation. In dysplasia this formation enlarged tripolar or tetrapolar mitotic
process is hampered. Cells fail to mature and figures. Sometimes nucleus divides without
retain their basal cell appearance. division of cytoplasm (poikilokaryosis). Normally
Abnormal stratification of the epithelium: As mitosis is limited to basal cells. But in dysplasia
maturation is affected, different layers of the even upper layers show mitotic figures.
epithelium are not clearly seen. All cells appear
similar to each other. Grading of Dysplasia
Dyskeratosis: Keratinisation is a process where There are various schemes for grading
epithelial cells form accumulate keratin proteins. dysplasia.These are WHO system, Ljubljana
This process is more prominent in the upper system Smith and Pindborg system of
parts of the epithelium. In dysplasia this process photographic standards, etc.
 Premalignant Lesions and Conditions 179

WHO Dysplasia System which constitutes up to one-half of the total

epithelial thickness. It is important that
A. Hyperplasia with increased number of cells: This
stratification is fully retained. Occasionally,
may be in the spinous layer (acanthosis) or
more than this proportion of the epithelium
in the basal and parabasal cell layer (basal
may be involved by the hyperplastic cells
cell hyperplasia). The architecture of the
without significant atypical nuclear changes.
epithelium is preserved, and there is no
Nuclei in the cells of the augmented basal and
cellular atypia.
parabasal layers may be moderately enlarged
B. Dysplasia with three grades
but still maintain a uniform distribution of
1. Mild dysplasia: Architectural disturbance
nuclear chromatin. Occasional typical
is limited to the lower third of the
mitoses may be found in or near the basal
epithelium, accompanied by cytological
layer. Small numbers of epithelial cells, less
atypia.
than 5%, are dyskeratotic.
2. Moderate dysplasia: Architectural distur-
• Atypical hyperplasia: (“risky” epithelium)
bance extends into the middle third of the
Epithelium demonstrates a recognizable
epithelium, accompanied by an upgraded
alteration of epithelial cells towards
degree of cytological atypia.
malignancy, but not to such a degree as is
3. Severe dysplasia: Architectural distur-
seen in carcinomatous cells. Stratification is
bance is greater than two-thirds of the
still preserved in the general epithelial
epithelium with associated cytological
structure. The nuclei are enlarged and the
atypia or architectural disturbance in the
nuclear contour may be irregular with
middle third of the epithelium with
marked variations in staining intensity. The
sufficient cytological atypia to upgrade
nuclear/cytoplasmic ratio is increased.
from moderate to severe dysplasia.
Mitotic figures are increased but not
C. Carcinoma in situ: Full or almost full thickness
numerous, and they are found within the two-
of the epithelium shows architectural
thirds of the epithelium above the basement
disturbance, accompanied by pronounced
membrane. They are rarely, if ever, abnormal.
cytological atypia. Atypical mitotic figures
Dyskeratotic cells are frequent. Civatte bodies
and abnormal superficial mitoses are present.
(apoptotic cells) may be present.
• Carcinoma in situ: Shows the features of
Ljubljana Classification System
carcinoma without invasion. Stratification of
Simple Hyperplasia the epithelium as a whole is lost. Marked
A benign hyperplastic process with retention of cellular alterations of the type found in
atypical hyperplasia are present to a
the normal pattern of the epithelium which is
considerably greater degree. Many mitotic
thickened because of an increased prickle cell
layer. The cellular components of the basal and figures present throughout the epithelium,
including its upper one-third and abnormal
parabasal region of the epithelium (one to three
mitoses are frequently found.
layers) remain unchanged. There is no cellular
atypia.
Malignant Potential
• Abnormal hyperplasia: A benign augmentation
of basal and parabasal layers. Basal and The term malignant transformation is used to
parabasal cells are augmented to a degree denote development of oral cancer from pre-
180 Textbook of Oral Pathology

existing leukoplakia. Malignant transformation Conservative Treatment

occurs in 0.3% to 10% of cases. It is higher in
• Vitamin therapy: It has a protective effect on
women (6%) than men (3.9%), due to
the epithelium. Daily requirement is 4000 IU.
involvement of endogenous factors. Leukoplakia
It is given orally, parentally or topically.
associated with chewing habit of tobacco shows
Therapeutic dose - 75000-300000 IU for 3
higher rate of transformation as compared to
months. Vitamin A may be used topically
others. In buccal mucosa and commissure region
after painting the lesion with podophyllin
1.8% malignant transformation occurs. In lip and
solution (it inhibit mitosis).
tongue region 16% to 38.8% malignant
• Vitamin A + vitamin E: This therapy is given
transformation occurs. Nodular dysplasia has
to inhibit metabolic degradation.
got higher risk of malignant transformation than
• 13-cis-retinoic acid: It is a synthetic analogue
other clinical types. Idiopathic leukoplakia and
of vitamin A; usually given in high doses of
candida associated leukoplakia also come under
1.5 to 2 mg/kg body weight for 3 months. If
high risk (Table 12.1).
relapse occurs then low doses of 13-cis-
If following features are present, there is a
retinoic acid 0.5 mg/kg body weight are
high risk of malignant transformation:
given for 9 months. It may be given with b
• Persistence of lesion for several years
carotene (this drug may produce a variety of
• Female patient
toxic effects which include facial erythema,
• Lesion situated on the margins, base of
dryness, peeling of skin, conjunctivitis and
tongue and floor of mouth
hypertriglyceridemia).
• Erosive lesions
• Antioxidant therapy: Considerable data shows
• Combination of above factors.
that β-carotene supplementation can be
Table 12.1: Malignant transformation potential
beneficial for treatment of oral leukoplakia.
of various lesions • Vitamin A palmitate: Short term treatment
Disease Malignant with vitamin A palmitate along with
potential aromatic retinoid of all trans -B-A vitamin
Proliferative verrucous leukoplakia ****** acid plus B-cis-B-A vitamin acid may show
Erythroplakia ******
Nicotine palatinus in reverse smokers *****
healing and improvement.
Oral submucous fibrosis **** • Nystatin therapy: It is given in candidal
Speckled, granular (non-homogeneous) leukoplakia. 500,000 IU twice daily plus 20%
leukoplakia ****
Actinic cheilitis *** borax glycerol or 1% gentian violet or mouth
Smooth, thick (homogeneous) rinses with chlorogen solution
leukoplakia ** • Vitamin B complex: It is given as supplement
Smokeless tobacco keratosis *
Lichen planus * in cases of commissural and lingual lesion.
• Antimycotic preparation: The antimycotic
preparation canesten and pimafucin have
Management
also been effective.
Elimination of etiological factor—Prohibition of • Panthenol: Panthenol lingual tablet and oral
smoking. To remove sharp, broken down teeth. spray may be used against glossitis and
To exchange faulty metal restorations and metal glossodynia, in case of tongue lesion.
bridge. Elimination of other etiological factors • Estrogen: In some cases, administration of
like syphilis, alcohol etc. estrogen can be helpful.
 Premalignant Lesions and Conditions 181

Surgical Management Classification

To give proper treatment microscopic • Homogenous

examination is necessary for which biopsy is • Erythroplakia interspersed with patches of
taken. The most meaningful sites for taking leukoplakia
biopsy specimen are the areas that display • Speckled or granular.
greater surface irregularities such as cracks and
Etiology
fissures and those associated with erythematous
areas. We can go for conventional surgery or It can be idiopathic. Alcohol and smoking can be
cryosurgery or fulguration or LASER. causative factor for erythroplakia. A secondary
infection or super infection with candidiasis may
Erythroplakia be associated with dysplastic oral mucosal cells.
Candida albicans has often been demonstrated in
It is also called as erythroplasia of Queyrat. erythroleukoplakia lesions and the red
Erythroplakia is a persistent velvety red patch. component of these lesions (often the white
Reddish color results from absence of surface component as well) diminishes or disappears after
keratin layer and due to presence of connective antifungal therapy, in at least some cases.
tissue papillae containing enlarged capillaries
projected close to the surface. The first mention Clinical Features
of a non-hemorrhagic red patch of an upper
aerodigestive tract site was in a vocal cord lesion It has got male predilection and most common
reported in 1852. The first truly descriptive paper in 6th and 7th decade of life. Erythroplakia
of erythroplakia, however, was a 1911 report by occurs on all mucosal surfaces of the head and
Queyrat, of a red macule on the glans penis of a neck area. Half of all cases, however, are found
syphilitic patient. It was Queyrat who coined the on the vermilion or intraoral surfaces, with the
term "erythroplasia." About the same time, rest being evenly divided between the larynx and
Rubin used the term "incipient carcinoma" to the pharynx. Vermilion lesions are relatively
describe the microscopic features of common and are most often seen on the lower
erythroplakia, most common histopathologic lip. Intraorally, the lateral and ventral tongue,
diagnosis as carcinoma in situ, based on his oral floor and soft palate are most frequently
experience with lesions of uterine cervix. involved (Fig. 12.7). Erythroplakia, as the name
obviously implies, is asymptomatic.
It is nonelevated, red macule or patch on an
Definition
epithelial surface. The exact cause of the red
It is applied to any area of reddened, velvety- appearance is unknown, but may be related to an
textured mucosa that can not be identified on increase in the number of underlying blood
the basis of clinical and histopathologic vessels through which the blood flows, which in
examination as being cause by inflammation or turn may be secondary to localized inflammatory
any other disease process. A chronic red mucosal or immunological responses caused by the
macule which cannot be given any other specific dysplastic, i.e. 'foreign,' epithelial cells. In some
diagnostic name and cannot be attributed to cases, the color may result from a lack of surface
traumatic, vascular or inflammatory causes. keratin or extreme thinness of the epithelium.
182 Textbook of Oral Pathology

examined clinically. Erythroplakia interspersed

with patches of leukoplakia in which
erythematous areas are irregular and often not
as bright as homogenous form, are most
frequently seen on tongue and floor of mouth.
The borders may be well circumscribed or blend
impercibly with surrounding oral mucosa.
Unlike leukoplakia, erythroplakia is seldom
multiple and seldom covers extensive areas of
mouth. Also unlike leukoplakia, erythroplakia
seems seldom to expand laterally after initial
Figure 12.7: Erythroplakia on right side showing red lesion diagnosis, although this may be an artifactual
feature because most lesions are completely
Homogenous form: Commonly found on buccal removed or destroyed immediately after formal
mucosa and soft palate and rarely on tongue and diagnosis.
floor of mouth. Homogenous form appears as a
bright red, soft, velvety lesion with straight or Histopathological Features
scalloped, well-demarcated margins. It is often It exhibits epithelial changes ranging from mild
quite extensive in size. Regardless of the cause dysplasia to carcinoma in situ and even invasive
of the color change, the typical lesion is less than carcinoma. Epidermoid carcinoma may show
1.5 cm. in greatest diameter and half are less than any degree of differentiation from poorly to well
1.0 cm, but examples larger than 4 cm. have also differentiated. It appears multicentric in origin.
been seen. It usually quite sharply demarcated The carcinoma in situ shows epithelial
from the surrounding pink mucosa and its dysplasia throughout the entire thickness of the
surface is typically smooth and regular in epithelium, without invasion into the underlying
coloration. connective tissue. Connective tissue rete pegs
Granular or speckled form: These are soft, red extend very high into the epithelium and the
lesions that are slightly elevated with irregular epithelium over the tips of these rete pegs is often
outlines and granular or finely nodular surface very thin, capillaries in these superficial pegs are
speckled with tiny white plaques. frequently dilated and the absence of any
Smooth erythroplakia is soft to palpation and significant amount of surface orthokeratin or
has often been described as having a velvety feel. parakeratin or at the most only thin layer, also
The pebbled lesions tend to be somewhat firm, contributes to the red color of the lesion.
but erythroplakia never actually becomes hard The spinous layer contains cells displaying
or indurated, until an invasive carcinoma atypia, hyperchromatism, pleomorphism and
develops within it. increase in number of mitotic figures.

Erythroleukoplakia: It is quite common to see Diagnosis

erythroplakia admixed with or adjacent to
leukoplakia in mouth. In such lesions, the red Differentiation of erythroplakia with malignant
areas are the sites most likely to contain or to changes and early squamous cell carcinoma,
develop dysplastic cells and should therefore be from the benign inflammatory lesions of oral
the sites most readily biopsied and most carefully mucosa is enhanced by use of 1% toludine blue
 Premalignant Lesions and Conditions 183

test. The solution is applied locally by swab or mouth, tongue and lips. Appearance of the lesion
oral rinse. Malignant type retains it, owing to may be like leukoplakia, like erythroplakia. It
increased nuclear DNA content of tumor cells. may be a combination of leukoplakia and
erythroplakia, ulcerated lesion, ulcerated and
Management white lesion, red and ulcerated lesion or may be
Incisional biopsy is always the preferred method nonspecific.
for establishing a microscopic diagnosis of
suspicious intraoral lesions. Since erythroplakia Histopathological Features
is so closely correlated with severe dysplasia, Keratin may or may not be present in/on the
carcinoma in situ and invasive carcinoma, surface of lesion but if present is more apt to be
incisional biopsy is especially indicated. parakeratin, rather than orthokeratin. Loss of
Excisional biopsy of a potential malignancy may orientation of cells and their polarity. Sharp line
result in under treatment and violation of surgical of division between normal and altered
oncologic principles. The definitive treatment of epithelium extending from the surface, down to
erythroplastic lesions remains controversial. A the connective tissue rather than blending of the
conservative surgical procedure such as mucosal epithelia.
stripping is often performed, with minimal In some cases, there appears to be
damage to the deeper connective tissues. This has hyperplasia of the altered epithelium, while in
the distinct advantage of preserving tissues for others there may be atrophy. An increase in
microscopic evaluation of potential regions of nuclear/cytoplasmic ratio and nuclear
invasion. hyperchromatism are sometimes seen (Fig. 12.8).
Destructive techniques such as laser ablation, It is also unusual to find multiple areas of
electrocoagulation and cryotherapy have also carcinoma in situ interspersed by essentially
proved to be effective. The key to therapy in this normal appearing epithelium producing
disease is extended clinical follow-up. Patients multifocal carcinoma in situ.
should be examined every 3 months for the first
postoperative year and every 6 months for an Management
additional 4 years. After that, annual reevalua- No uniformly accepted treatment. Lesion may be
tion with a thorough head and neck examination surgical excise, cauterized and even exposed to
is advisable. Elimination of a suspected irritant solid carbon dioxide.
Carcinoma in Situ
It is also called as ‘intraepithelial carcinoma’.
Severe dysplastic changes in a white lesion
indicate considerable risk of development of
cancer. The more severe grade of dysplasia
merges with the condition known as carcinoma
in situ. It is more common on skin but can also
occur on mucous membrane

Clinical Features
Male predilection and occurs more commonly Figure 12.8: Carcinoma in situ showing nuclear
in elderly persons. It is common sites are floor of hyperchromatism (Courtesy : Dr Sangamesh Halawar)
184 Textbook of Oral Pathology

Bowen’s Disease cell infiltration is prominent (Fig. 12.9). The

It is localized ‘intraepidermoid carcinoma’ that epithelium around the duct shows excessive
may progress to invasive carcinoma over many thickening and keratinization. Fissures and crack
years, which is characterized by progressive scaly may appear, producing a wrinkled, irregular
or crusted plaque like lesion. It can be caused by surface.
sun exposure and arsenic ingestion. Clinical types — Clinical type of stomatitis
nicotine in reverse smoking are as follows:
Clinical Features • Mild — Consisting of red, dot like opening
It occurs on male and female genital mucosa and on blanched area.
in oral mucosa as erythroplakia, leukoplakia or • Moderate — Characterized by well define
erythematous lesion. It appears as slowing elevation with central umbilication.
enlarging erythematous patches with little to • Severe — Marked by papules of 5 mm or more
suggest the malignant process. There is a red and with umbilication of 2-3 mm.
slightly scaly area on the skin, which eventually Palatal changes consequent to reverse chutta
enlarges and turns into white or yellowish lesion. smoking are diverse, consisting of several
When these scales are removed it produced a components.
granular surface without bleeding. • Keratosis — Diffuse whitening of the entire
palatal mucosa.
Histopathological Features • Excrescences — 1 to 3 mm elevated nodules,
often with central red dots corresponding to
There are intraepithelial features of malignancy. the opening of palatal mucous glands.
The epithelial cells of the lesion lie in complete
• Patches — Well defined, elevated white
disarray with highly atypical hyperchromatic
plaques, which could qualify for the clinical
nuclei and multiple nuclear forms. term leukoplakia (Fig. 12.10).
• Red areas — Well defined reddening of the
Management
palatal mucosa.
Use of freezing technique, diathermy, cauteriza- • Ulcerated area — Crater like areas covered by
tion, radiotherapy or application of cytotoxic fibrin.
drugs.

Oral Lesion Associated with Use of Tobacco

Smoker’s palate or stomatitis nicotina— It is seen in
persons who are heavy pipe and cigar smokers
and it is most common in males. It is most
commonly seen in reverse smoking habit. There
is redness and inflammation of the palate.
The palate develops diffuse, grayish-white,
thickened, multinodular papular appearance.
There is small red spot in the center of each tiny
nodule, representing a dilated and sometimes
partially occluded orifice of an accessory palatal Figure 12.9: Stomatitis nicotina showing red spot
salivary gland duct around which inflammatory representing palatal salivary gland duct
 Premalignant Lesions and Conditions 185

LICHEN PLANUS

Erasmus Wilson described it in 1869. Various

mucosal surfaces may be involved, either
independently or concurrently, with cutaneous
involvement or serially. Oral mucosa is frequently
involved. It is a probable pre-cancerous condition.
Lichen planus is a common inflammatory
disease of the skin presenting with characteristic
violaceous, polygonal, pruritic papules. The
disease may also affect the mucosa, hair and nails.
Figure 12.10: White plaques seen on palate in patient Relatively common dermatological disorder
with stomatitis nicotina
occurring on skin and oral mucus membrane and
refers to lace-like pattern produced by symbolic
• Nonpigmented areas — Area of palatal mucosa
algae and fungal colonies on the surface of rocks
which is devoid of pigmentation.
in nature (lichens).
Snuff Dipper Lesion
Etiology
Four specific clinical lesions
• Hyperkeratosis or erythematous lesion of Immunology
oral mucosa
Cell-mediated immune response: It is a cell
• Gingiva and periodontal inflammation
mediated immune response associated with
• Combination of above
lymphocyte-epidermal interactions, resulting in
• Cervical erosion of teeth.
degeneration of basal cell layer. Possible
It occurs in mucosal surface, where snuff is
hypothesis are alteration of keratinocytes as a
habitually held. The compound N-nitro-
result of unknown events resulting in antigenic
sonornicotine (NNN), which is derived partly
alternation of these cells thereby stimulating
from bacterial action on nicotine during the
immunological reaction or a primary
curing process, is contributed by the action of
immunologic reaction causing alternation and
salivary nitrites, when tobacco is held in the
degeneration of keratinocytes.
mouth; occurs in greater concentration in snuff
Cell-mediated immune response may be
tobacco. Lesion has wrinkled appearance. If habit
caused by various mononuclear cells, i.e.
is eliminated, majority of the lesion disappear
Langerhans cell, macrophages predominantly T
in about 2 weeks. Long exposure to snuff results
lymphocytes, lymphoblast cells, B lymphocytes
in for malignant changes.
and mast cells. These cells infiltrate the upper
Cigarette smoker’s lip lesion: They are generally flat part of lamina propria of sub mucosa. In this cell-
or slightly elevated nodular white lesion on one mediated response Langerhans cells are potent
or both lips, corresponding to the site at which antigen presenting cells, lymphocytes are
the cigarette is held and apparently smoked effective cells and keratinocytes are target cells.
down to an extremely short length. There is The macrophages are mostly mature, which
increased redness and stippling of lip in localized probably have functional role with mononuclear
area. It has elliptical, circular or irregular borders. cells is suggesting of cell to cell cooperation.
Pale to white and were slightly elevated with Recent hypothesis of pathogenesis of lichen
nodular or papillary shape. planus. In a genetically predisposed individual,
186 Textbook of Oral Pathology

haptens (certain drug or dental material), • Ulcerative

conventional antigen or super-antigen of oral • Hypertrophic
microbial origin can induce cell mediated • Erosive
immune response resulting in subepithelial T cell • Bullous
infiltration of the site in oral mucosa with • Hypertrophied
cytokine generation HSP-60 and C 1/10 • Annular
expression by basal keratinocytes. If individual • Actinic
is not predisposed to react to HSP-60, then • Follicular
nonspecific mucositis occur. If the individual has • Linear.
genetic predisposition, it results in autoimmune
Immunodeficiency: There has been report of
reaction → activation of cytotoxic T cell →
decreased serum levels of IgG, IgA, or IgM in
destruction of basal keratinocytes → oral lichen lichen planus and the possibility of it as a
planus.
manifestation of immunodeficiency has been
Autoimmunity: The activated T lymphocytes also raised. But at the same time, reports of normal
secrete gamma interferons which induce concentrations of IgA and IgM are found;
keratinocytes to produce HLA-DR and increase therefore the role of immunodeficiency is
their rate of differentiation with formation of questionable.
thickened surface. Antigenic information is
Genetic factors: Cases of lichen planus are
transferred from Langerhans cells to lymphocytes, reported in families, twins and husband and wife
when there is mutual expression of HLA-DR.
clinically, familial lichen planus is somewhat
Lymphocytes normally are attracted towards
unusual as it appears to affect young patients, is
HLA-DR expressing keratinocytes and may severe, often extensive, involves skin nails and
contact the epithelial cell. During this contact,
mucous membrane and is persistent. It has also
inappropriate epithelial antigenic information
been suggested that familial cause might be
may be passed to lymphocytes due to HLA-DR environmental and related to infection, rather
linkage. With this mechanism, self-antigen may
than to genetics.
be recognized as foreign bodies, leading to
destruction of basal cells, resulting in an Infections: A bacterial etiology may be there but
autoimmune response. Autoimmune disorders results are not confirmed. Spirochetes and rod
classically have female predilection and are like bodies resembling bacteria have also been
associated with serum antibodies with detected.
hypergammaglobulinemia. There are numerous Drugs and chemicals: It is also called as lichenoid
studies which show immune deposits in lichen reaction. Although the clinical disease of lichen
planus affected tissues but it is not specific. planus has an immunological basis, some
persons with lichen planus have a diathesis for
Types
the disorder. The tissues of a person with
• Reticular diathesis react in a special way to certain extrinsic
• Papular stimuli, making it more susceptible to certain
• Plaque diseases. Drugs act to increase temporarily the
• Atrophic specific antigenic stimulus and hence increase
• Classical the reaction. If the drug is withdrawn at a later
• Erythematous time, the antigenic stimulus is reduced, followed
 Premalignant Lesions and Conditions 187

by reduction in clinical severity. It is suggested are known as vaginogingival syndrome. Other

that drugs that are known to induce lichenoid mucosal surfaces including larynx, glans penis,
response, act as agents which amplify the esophagus, stomach, nasal and vulva may get
disorder, rather than induce it. To implicate a involved. The chief complains is usually of
drug responsible for a lichenoid reaction can be intense pruritus. The itching associated with LP
difficult as there is no specific test for it. usually provokes rubbing of the lesions, rather
Association between dental filling material and than scratching. The lesions have a characteristic
lichen planus has also been suggested. violet hue. They are flat-topped, shiny, polygonal
Psychogenic factor: A relationship of lichen planus papules and plaques. The surface is dry with
thin, adherent scales. Six P of lichen planus—six
with stress is quoted and neurogenic basis is
'p's characterize the lesions LP: they are planar,
suggested. Observation mostly in nervous and
highly stressed persons is associated with polygonal, purple, pruritic, papules and plaques.
emotional upset, over work and some form of Cutaneous: Common sites are flexor surface of
mental strain. wrist and forearm, inner surface of knees and
thigh and the trunk, usually the lumbar and
Habit: Oral lichen planus have shown association
sacral areas. Skin lesions appear as small,
with tobacco habit. Chewers of tobacco and betel
angular, flat topped papules, only a few mm in
have increased prevalence of oral lichen planus.
diameter, which may be discrete or gradually
Smoking may play a role in initiating oral lichen
coalesce into larger plaque and is covered by fine
planus of plaque type.
glistening scales. Primary symptom is severe
Miscellaneous: Occurrence of lichen planus is also pruritis that may be intolerable. Papules are
suggested in association with deficiency of sharply demarcated from surrounding skin,
vitamin B1, B6 and C, electric potential difference, which appears red but soon takes reddish purple
anemia and patients with secondary syphilis. It or violaceous blue color. Later, dirty brown color
can also occur in some cases due to trauma and develops. Center of papule may be slightly
malnutrition. Exacerbation of lichen planus also umblicated.
correspond to periods of emotional upset,
Wickham’s Striae: These are very fine grayish
overwork, anxiety, hysteria attack, depression
lines which cover the papules (Fig. 12.11).
and some form of mental strain.
Graham little syndrome: Alopecia occurs in some
Clinical Features patients which may be termed lichen planus
planopilaris (Graham-Little syndrome). Nail
General: It occurs in adulthood with age range changes, particularly longitudinal ridging and
for males as 35-44 years and for females 45-54
grooving may be seen.
years. It has more predilections for females.
Prevalence of lichen planus in general Variant: Various variants of lichen planus like
population is about 0.9% to 1.2% and prevalence hypertrophied form, actinic form, ulcerative
of oral lichen planus is reported between 0.1% form, linear form, etc are present, but in dental
to 2.2%. It may involve skin, oral and other point of they are not found intraorally so they
mucous membranes. About 50% of the patients are not discuss here.
with skin lesions have oral lesions whereas 25% Koebner phenomenon: Fresh lesions may appear
of all lichen planus have only oral lesions. Oral on scratch marks or at sites of other nonspecific
lesions coexisting with genital mucosal lesions trauma.
188 Textbook of Oral Pathology

Figure 12.12: Reticular types of lichen planus showing

annular arrangement

Figure 12.11: Lichen planus showing Wickham’s Striae

Oral Lichen Planus

Common sites are buccal mucosa (84%) and to
lesser extent tongue, lips, gingiva, floor of mouth
and palate. Patient may report with burning
sensation of oral mucosa. Oral lesion is
characterized by radiating white and gray
velvety thread like papules in a linear, angular
or retiform arrangement forming typical lacy, Figure 12.13: Reticular types of lichen planus
reticular patterns, rings and streaks over the
buccal mucosa and to a lesser extent on the lip,
tongue and palate. Wickham’s striae — tiny white
elevated dots are present at the intersection of
white lines, called as Wickham’s striae.
Reticular type: Most common form and it is
mostly bilateral. Consists of slightly elevated fine
whitish lines that produce lace like pattern of fine
radiating lines, called as Wickham’s striae
(Figs 12.12 and 12.13). The lesion may present
radiating white thread like papules in a linear,
annular or retiform arrangement. A tiny white
dot is frequently present at the intersection of
white lines.
Papular: Whitish elevated lesions of 0.5 mm to 1
mm in size, well seen on keratinized areas of oral
mucosa (Fig. 12.14). Papules are spaced apart, Figure 12.14: Papular type of lichen planus showing
still close enough to give pebbled white or gray whitish elevated lesion
 Premalignant Lesions and Conditions 189

color. Sometimes they coalesce. Most oftenly,

papules are seen at the periphery of reticular
pattern.
Plaque: It is seen on dorsum of tongue and buccal
mucosa. In case of plaque of tongue, disappearance
of the tongue papillae is seen. It spreads in
concentric peripheral growth. It consists of either
pearly white or grayish white plaque. Such plaques
generally range from slightly elevated and smooth
to slightly irregular form.
Atrophic form: Appears as smooth, red, poorly Figure 12.15: Annular types of lichen planus of labial
defined area, often but not always, with mucosa showing round appearance
peripheral striae evident. The attached gingiva
is frequently involved in this form of lichen morphology and configuration or there may be
planus in a so called desquamative gingivitis modifications of clinical features by the site of
pattern. At the margins of atrophic zones, involvement.
whitish keratotic striae are usually evident,
radiating peripherally and blending into Variations in Morphology
surrounding mucosa. The gingiva tends to show
• Hypertrophic lichen planus: This may occur as
patchy distribution over all the four quadrants
a sole manifestation or as a part of a more
in a relatively symmetrical pattern. It is always
generalized subacute disease. The lesions are
symptomatic with complain of pain and burning
elevated, warty, pigmented plaques typically
in the areas of involvement.
occurring on the shin or around the ankle.
Bullous form: It consists of vesicles and bullae These lesions tend to be persistent.
which are short lived. These upon rupturing, • Atrophic lichen planus: The lesions are few in
leave an ulcerated extremely uncomfortable number. There are depressed, pigmented
surface. The most common site is buccal mucosa lesions.
especially into posterior and lateral margins of • Follicular lichen planus: It also known as lichen
tongue. It is often associated with striated or planopilaris, this may accompany typical
keratotic component. lesions or may be the sole morphologic type.
Hypertrophic form: It appears as well circums- There are small lesions centered around hair
cribed, elevated white lesion resembling follicles. Lesions on scalp may result in
leukoplakia. alopecia. The combination of follicular LP
with scarring alopecia of scalp and
Annular form: Appears as round or ovoid, white
nonscarring alopecia of axilla and pubis or
outline with either pink or reddish pink center
other areas is known as Graham-Little
(Fig. 12.15). In about 11% of cases, oral lichen
syndrome.
planus may be associated with pigmentation.
• Actinic lichen planus: Described mainly from
the Middle East and India, these lesions are
Clinical Variation of Lichen Planus
common on the face and present as
In many cases, the clinical picture may differ hyperpigmented patches with a surrounding
from the classical one, the variations being in zone of hypopigmentation.
190 Textbook of Oral Pathology

• Bullous lichen planus: Vesicles and bullae may Special Variant

occur on typical lesions of LP in this variant,
• Drug-induced lichenoid reactions: A number of
due to severe basal cell degeneration induced
drugs may induce development of lesions
by the inflammatory process.
that clinically and histologically resemble
• Ulcerative lichen planus: Chronic ulcers occur
idiopathic lichen planus. The principal
on the feet. It may accompany oral ulcer and
offenders are: gold, penicillamine, beta-
loss of nails.
blockers antimalarials, captopril, lithium,
• Lichen planus pigmentosus: Described mainly
carbamazepine, chlorpromazine, thiazides,
from India and Middle East, these lesions
methyldopa, INH, PAS and NSAIDs.
present with deeply pigmented macules on
• Lichen planus pemphigoides: Bullous lesions
the face and extremities.
occur on lesions as well as normal skin. This
appears to be a variant with combined
Variations in Shape
features of lichen planus and bullous
• Linear lichen planus: This may follow marks pemphigoid.
of injury by koebnerization, or may occur • Lichenoid contact dermatitis: Contact with color
spontaneously in long linear arrays or rarely film developer containing paraphenyl-
in a dermatome in a zoster-like fashion. diamine may give rise to lichen planus like
• Annular lichen planus: These lesions have lesions on hands. Amalgams in dental filling
central depressed areas with raised margins. material may also give rise to lichenoid oral
Typically occurs on penis. lesions.
• Guttate lichen planus: Large number of drop- • Lichen planus-lupus erythematosus overlap:
shaped lesions. Lesions with atrophic center violaceous
border occur mainly on hands and feet.
Variation by Site Immunohistologic evidence of both lichen
planus and lupus erythematous, are present.
• Oral: Oral lesions frequently occur in lichen
planus.
Histopathological Features
• Genital: Annular plaques often occur on glans
penis, vulval or vaginal lesions, often Hyperorthokeratosis and hyperparakeratosis,
presenting with chronic ulcers, may rarely acanthosis with intercellular edema of spinous
occur. cells occurs in some instances. Civatte bodies are
• Nails: Nail dystrophies may occur in lichen often seen in the epithelium mostly in the
planus. The various changes that may occur are: spinous and basal cell layers and lamina propria.
thinning of nail plate, longitudinal ridging, They appear as round or ovoid, well defined
onycholysis, subungual hyperkeratosis and eosinophilic globule with a maximum size of
pterygium formation (fusion of proximal nail about 20 μm, probably representing fibrillar
fold with the nail plate). transformation degeneration and premature cell
• Palms and soles: Lichen planus of these death of basal keratinocytes.
locations lack the typical color; they are Saw tooth appearance of the rete pegs is seen
yellowish rather than violaceous papules and (Fig. 12.16). Necrosis or liquefaction degenera-
plaques. tion of basal cell layer with appearance of a band
• Scalp: Follicular lesions with scarring of eosinophilic coagulum in place of the basal
alopecia are seen at this site. layer. Thickening of granular cell layer. Band like
 Premalignant Lesions and Conditions 191

Histopathological Features of Different

Types of Oral Lichen Planus
Reticular: It shows hyperparakeratosis or
hyperorthokeratosis. In some cases, both types
of keratinization may be seen. Granular cell layer
is also seen. It also features epithelial hyperplasia,
although atrophy is present in some cases.
Accentuation of rete pegs in the typical saw-
tooth configuration is uncommon. Basal cell
layer shows liquefaction degeneration. Cellular
Figure 12.16: Lichen planus showing subepithelial
inflammation and saw tooth appearance infiltrate is primarily of lymphocytes, plasma
cells may be seen (Fig. 12.17).
dense inflammatory cellular infiltrate, containing
Papular: Keratosis, usually hyperkeratosis or
lymphocytes and occasional plasma cells, in the
parakeratosis, may be extensive and stratum
upper lamina propria.
corneum may show considerable increase in
Sometimes necrotic material is exuded into
width. Parakeratosis is found more commonly
lamina propria (apoptosis). The material may be
than hyperkeratosis, although alternate areas of
taken up by phagocytes or provide a matrix for
both types may be present. Acanthosis is not
the deposition of serum protein including
usually seen. Epithelium shows moderate
immune component, resulting in colloid bodies
hyperplasia and saw tooth configuration of rete
formation. Cells from the lamina propria may pegs is rarely observed. Basal cell layer shows
also contribute to the formation of colloid bodies. liquefaction degeneration and it consists of inter-
Cellular infiltrate consist of lymphocytes but
plasma cell and histiocytes are also present
sometimes.
Mast cells are observed sometimes below the
sub-epithelial infiltrate. In deeper layers they
contain granules but undergo degranulation. It
is suggested that mast cells participate in
recruitment of lymphocytes to subepithelial
infiltrate. Ryan suggested that the pattern of
striae of Wickham’s is due to absence of
capillaries in the center with growing vessels at
the periphery.

Immunofluorescent study: Positive for direct

immunofluorescence reaction with IgA, IgM,
IgG antisera. Most constant feature is presence
of subepithelial deposits of fibrinogen and Figure 12.17: Lichen planus showing 1-saw tooth rete pegs
and hydropic degeneration of basal cells, 2-orthokeratosis,
antigenically related substance, which can be 3-juxtaepithelial band of inflammatory cells (Courtesy:
stain by antifibrinogen antisera. Dr Sangamesh Halawar)
192 Textbook of Oral Pathology

and intracellular vacuoles. Juxtaepithelial Management

connective tissue consists of broad band of
Removal of cause: The causative factor is removed
lymphocytic infiltration. Occasional plasma cell
and this may lead to resolution of lesion
and histiocytes may be seen. Inflammatory cells
subsequently. This can be particularly applicable
usually penetrate into lower layers of epithelium
to lichenoid drug eruption.
and are seen between degenerating epithelial
cells. Medicinal therapy: In most patients with erosive
and ulcerative lesion steroids are commonly
Plaque: Marked hyperorthokeratosis and used. The rationale behind their use is their
hyperparakeratosis is seen in connection with ability to modulate inflammation and immune
stratum granulosum. Epithelial hyperplasia as response. Small and moderately sized painful
well as atrophy is seen. A narrow zone, free of lesions can be treated with beclomethasone
inflammatory cells is seen in relation with dipropionate spray, triamcinolone acetonide in
basement membrane. Lymphocytic juxtaepi- gel or cream base. Topical and intralesional
thelial infiltration is seen in connective tissue routes are used when systemic steroids are
stroma. contraindicated. These routes are useful when
the patient refuses needle injection or when
Atrophic: The epithelium is thin. There is little
treating painful gingival sites, where injection
keratinization at the surface and stratum delivery is impossible. The topical, injectable and
corneum tends to blend into stratum spinosum. systemic routes are used when there is no
The basal cells present hydropic degeneration. systemic contraindication and a full steroid dose
Rete pegs are absent. Pattern is similar to is required. In case of some painful gingival
desquamative gingivitis. lesions topical steroids may be applied using soft
custom trays by coating steroids on the
Bullous: It shows hydropic degeneration of basal
undersurface of the tray. This tray anchors to the
cell layer. Due to this there is collection of edema
dental arch while covering the painful gingival
at the epithelial connective tissue interface,
lesion. Earlier regime consists of triamcinolone
resulting in the formation of subepithelial bulla.
acetonide topically, methyl-prednisolone 40 mg/
The bulla or vesicle contains clear fluid and
ml as injectable and prednisolone 5 mg tablet as
occasionally hemorrhage. A broad band of systemic steroid therapy. Injection triamcinolone
lymphocytic cells is seen in the upper corium, acetonide 10 mg/ml was also used in patients
prior to rupture of vesicle or bulla. with serious complain, in a dose of 0.1 ml/cm2.
This earlier regime called for topical delivery
Malignant Potential qID, for 3 or more weeks, once a week
intralesional injection, for 3 weeks (usually—0.5
The incidence of malignant transformation ml—1.5 ml) and systemic steroid (prednisolone
ranges form 0.4% to 12.3%. In India, the 5 mg tab) in tapering doses of 30 mg/day for the
incidence of malignant transformation is 0.4 %. first of 3 weeks, 15 mg/day for second week and
Carcinoma development is more common in 5 mg/day for third and final week. Topical
women than in men. Atrophic, erosive and application of fluocinolone acetonide for 4 weeks
ulcerative lesions showing erythroplakic is also effective in curing the disease. Another
component and tobacco chewers are indicated regime consisting of prednisolone and
to be more cancer prone. levamisole has also been tried successfully
 Premalignant Lesions and Conditions 193

recently. This systemic regime calls for and small solitary lesions. In some cases,
prednisolone 5 mg and levamisole 50 mg tablets cryosurgery and cauterization have also been
for first three days of rest and this schedule to be tried.
followed for two to three more weeks.
Psychotherapy: Emotional status of the patient is
Topical application of antifungal agent: Steroid important in the development of this disease. In
therapy is routinely accompanied by antifungal some cases, the lesion may regress when the
treatment as steroid therapy tends to generate patient is made aware of psychogenic
an oral fungal infection. The prophylactic implication of the condition and the nature of
antifungal therapy usually consists of emotional stress is understood. When the lesions
clotrimazole oral torches. Nystatin and are asymptomatic and there is no source of
ketoconazole can also be used. emotional distress it is often advisable to refrain
Vitamin A (Retinoid) analogue: Retinoids are from therapy as failure to eradicate the lesion
useful usually in conjunction with topical by medication may trigger the patient into
corticosteroids as adjunctive therapy either becoming fearful of cancer. Tranquilizers have
topically or systemically. This is because of their been also been tried to reduce anxiety.
antikeratinizing and immunomodulating effects.
Dapsone therapy: Dapsone diamino-
They are particularly effective against
diphenylsulphone is an antibacterial sulphone.
keratinized reticular and plaque variants.
It is postulated that this particular agent may
Topical vitamin A acid cream (0.1%) Tretinoin,
help to control the lymphocyte mediated
beta altransretinoic acid, systemic etretinate and
progress of lichen planus by modulating the
systemic and topical isotretinoin are also useful
release of inflammatory or chemotactic factor for
in resolution of the lesions, but withdrawal of
mast cells or neutrophils. It is used in severe form
the medication leads to rapid recurrence to the
lesion very oftenly. The side effects of retinoids of erosive lesions.
are more and it includes foci of erythema during PUVA therapy: In this form of therapy, psoralens
and after the topical treatment. For systemic and high intensity long wave ultraviolet (PUVA)
retinoids, it includes liver dysfunction, cheilitis light is used as a therapeutic agent. The lesion
and dryness of mucous membrane. A new shows improvement during and immediately
systemic retinoid; temarotene, has been reported after the treatment.
effective and free of side effects, other than a
slight increase in liver enzymes. Erosive Lichen Planus
Cyclosporin: It is a selective inhibitor of CD4 It presents as chronic multiple oral mucosal
helper T lymphocytes that is used systemically ulcers, which occur when there is extensive
to achieve immunosuppression. It can be used degeneration of basal cell layer of epithelium.
both, topically and systemically. The lesion
shows complete healing with no recurrence Etiology
following 8 weeks of systemic cyclosporin 8 mg/
kg/day. Oral lesions can be treated using Drug therapy like NSAIDs, hydrochlorothiazide,
cyclosporin as a rinse and expectorant. It is used penicillamine, angiotensin converting enzyme
as 5 ml rinse, tid, for 8 weeks. inhibitors. Chronic hepatitis: Underlying medical
disorders like chronic hepatitis.
Surgical therapy: It is indicated when
conventional methods fail in ulcerative lesion Dental restoration: Reaction to dental restorations.
194 Textbook of Oral Pathology

Graft versus host disease: Graft versus host disease seen. Erosive lesion appears as ulceration,
due to bone marrow transplantation. epithelial thinning and eventual destruction.
Stress: Emotional stress can lead to erosive lichen Hemorrhage may be noted. It shows classical
planus. feature of lichen planus, i.e. hydropic
degeneration of basal cell layer with
Clinical Features juxtaepithelial inflammatory cell infiltrate. The
Female to male ratio is 2:1. Average age is basal cells are gradually destroyed and overlying
50 years. It is primarily a disease of whites, but epithelium becomes thin and atrophic.
it may be seen in blacks. Common site is buccal Eventually, epithelium undergoes necrosis and
mucosa and lingual mucosa. There is complain an area of ulceration appears. Ulcerated area, if
of burning sensation and pain. After rupture of infected may show altered population of
vesicles, eroded or frankly ulcerated lesion are inflammatory infiltrate with polymorphonuclear
seen which appears as a raw painful areas. Lacy leukocytes predominating at the ulcerated
white pattern may be present. Eroded and surface, which is covered by fibrin.
frankly ulcerated lesions are irregular in size and
Oral Submucous Fibrosis
shape and appear as raw and painful areas
(Fig. 12.18). The surface is generally granular and It is a chronic and high risk precancerous
brightly erythematous and may bleed upon condition. The condition was prevalent in the
slight provocation or manipulation. A fibrinous days of Sushruta (600 BC), a great practitioner
plaque or pseudomembrane may be seen over of ancient medicine where he labeled this
erosion, while later is significant. condition as ‘Vidhari’. After lapse of many years,
Schwartz (1952) was the first person to bring this
Malignant potential: Malignant potential is 1 to condition again into limelight. He described the
25%. Present for a week to month and heal in
condition as ‘atrophica idiopathic mucosae oris’.
periods of 10 days to 2 weeks. They may change
After that the condition has also been described
the pattern of presentation and involvement as idiopathic scleroderma of mouth, idiopathic
from time to time.
palatal fibrosis and sclerosing stomatitis.
Histopathological Features
Definition
In the erosive form, the epithelium is completely
An insidious, chronic disease affecting any part
missing or only remnants of epithelial tissues are
of the oral cavity and sometimes pharynx.
Although occasionally preceded by and/or
associated with vesicle formation, it is always
associated with juxtaepithelial inflammatory
reaction followed by fibroelastic changes of
lamina propria, with epithelial atrophy leading
to stiffness of oral mucosa and causing trismus
and inability to eat.

Etiology
Chronic Irritation

Figure 12.18: Erosive lichen planus showing raw painful Chillies: The use of chillies (capsicum annum and
area capsicum frutescence) have been thought to play
 Premalignant Lesions and Conditions 195

an etiological role in oral submucus fibrosis. stimulates fibroblastic proliferation and collagen
Capsaicin is the active ingredient of chillies. It is synthesis. The flavanoid catachin and tannins are
the vanillylamide of 8-methyl-6-nonenic acid, also components of areca nut and they stabilize
which is the active irritant of the chillies. the collagen fibrils rendering them resistance to
degradation by collagenase. The attendant
Tobacco: It is a known irritant and causative
trismus is the result of juxtaepithelial hyaliniza-
factor in oral malignancy. It may act as a local
tion and secondary muscle involvement.
irritant.
Glycogen consumption is physiologically related
Lime: Lime is used with betel nut for chewing. It to cellular activity. Over activity of muscle results
causes local irritation and damage to the mucosa in excessive glycogen consumption, leading to
with vesicle and ulcer formation in susceptible glycogen depletion. The increased muscle activity
individuals. It acts as a local irritant. and diminished blood supply, following
connective tissue changes; owing to extensive oral
Betel nut: The term areca nut is used to denote
submucous fibrosis leads to muscle degeneration
the unhusked whole fruit of the areca nut tree
and term betel nut is used exclusively to refer to and fibrosis.
the inner karnel or seed which is obtained after Nutritional deficiency: The disease is characterized
removing husk. The betel nut has psychotropic by repeated vesiculations and ulcerations of oral
and antihelmintic property due to presence of cavity. A subclinical vitamin B complex
areca alkaloids, predominantly arecoline. These deficiency has been suspected in such cases. The
alkaloids have powerful parasympathetic deficiency could be precipitated by the effect of
properties which produce euphoria and defective nutrition due to impaired food intake
counteract fatigue. Areca nut is found to contain in advanced cases and may be the effect, rather
different types of alkaloids like arecoline, than the cause of the disease.
arecadine, arecalidine, guvacoline, guvacine and Defective iron metabolism: Microcytic hypochromic
isoguvacine. In a habitual betel nut chewer, oral anemia with high serum iron have been reported
submucus fibrosis may be caused by the amount in submucus fibrosis but as such, there is no
of tannic acid contained in the betel nut, the definite proof available to support this cause
influence of mixed calcium powder and the effect relation.
conditional action of arecoline content in betel nut,
Bacterial infections: Streptococcal toxicity is also
affecting the vascular supply of oral mucosa and
a factor in etiology of oral submucus fibrosis, as
causing neurotropic disorder. Nitrosation of
in some other collagen disorder such as
arecoline leads to the formation of areca nut
rheumatic disease. Klebsiella rhinoscleromatis may
specific nitrosamine namely nitrosoguvacoline,
be a factor in cause of submucous fibrosis.
nitrosoguvacine and 3-methyl nitrosomino
pripionitrile, which they alkylate DNA. Collagen disorders: Oral submucus fibrosis is
Metabolism of these areca nut specific nitrosamine thought to be localized collagen disease of oral
will lead to formation of cyanoethyl, which cavity. It is linked to scleroderma, rheumatoid
adducts with o’methyl guanine in DNA. arthritis, Duputreyen’s contracture and
Prolonged exposure to this irritant leads to intestinal fibrosis. A link between scleroderma
malignant transformation. Recently suggested and oral submucous fibrosis has also been
pathogenesis of oral submucus fibrosis is by dual suspected on the basis of similarity of
action of areca nut. It is suggested that arecoline histological characteristics.
196 Textbook of Oral Pathology

Immunological disorders: Raised ESR and globulin Flow chart 12.1: Role of alkaloids in oral submucous fibrosis
levels are indicative of immunodeficiency
disorder. Serum immunoglobulin levels of IgA,
IgG and IgM are raised significantly in oral
submucus fibrosis. These raised levels suggest
an antigenic stimulus in the absence of any
infection. Circulating autoantibodies are also
present in some cases of oral submucus fibrosis.
Genetic susceptibility: The familial occurrence of
oral submucus fibrosis has also been reported.
Altered salivary composition: The study of saliva
in cases of oral submucus fibrosis have shown
increased pH, increase in salivary amylase, low
levels of calcium, increase in alkaline
phosphatase and potassium and normal levels
of salivary immunoglobulin. The fibrin
bFGF. These are fibrogenic growth factors that
precipitating factor in saliva has been attributed stimulate collagen production. Another cytokine
to the increased plasma fibrinogen. This is likely that has anticollagen effect is IFN-α. This is
due to increased dietary content of fibrin. decreased in OSMF. Thus overall there is
Pathogenesis stimulation of collagen synthesis.

Oral submucous fibrosis results from increased Stabilization of Collagen Structure

production of collagen by fibroblasts. In addition Betel nut contains tannin. Tannin has ability to
to this there is decreased breakdown leading to stabilize collagen by cross-linking it. This cross-
accumulation of excessive amounts of collagen. linked collagen is more resistant to degradation.
There are various mechanisms by which this Another component of betel nut that aids this
occurs cross-linking is copper. Copper is present in betel
1. Increased collagen production. nut in high amounts. It is a constituent of enzyme
2. Stabilization of collagen. lysyl oxidase. This enzyme causes cross-linking
3. Decreased collagen breakdown. and makes collagen resistant to degradation.
Increased Collagen Production
Decreased Collagen Breakdown
Under the influence of areca nut fibroblasts
differentiated into phenotypes that produce Due to action of tannin and copper collagen that
more collagen. The alkaloids present in areca nut is produced in OSMF is highly resistant to
arecadine and arecoline are responsible for this. remodeling and phagocytes. It is fibroblast that
Arecadine is more important. Arecoline gets brings about remodeling and phagocytes of
converted in arecadine which is the active collagen. As in OSMF these fibroblasts are
metabolite. There is dose dependent increase in affected they cannot degrade collagen.
production of collagen by fibroblasts under Thus, in oral submucous fibrosis there is
influence these factors (Flow chart 12.2). increased production and decreased degradation
Various cytokines are increased in oral of collagen. This leads to accumulation of
submucous fibrosis. These are TGF-β, PDGF, collagen in oral mucosa (Flow chart 12.1).
 Premalignant Lesions and Conditions 197

Pathogenesis of Oral Submucous Fibrosis and esophagus, the patient may experience
Clinical Features difficulty in swallowing the food. Referred pain
in the ears and deafness, due to occlusion of
It affects both sexes. The age group varies, Eustachian tube and a typical nasal voice has
although majority of patients are between 20 and been reported.
40 years of age. The most frequent location of oral The most common and earliest sign is
submucus fibrosis is the buccal mucosa and the blanching of mucosa, caused by impairment of
retro molar areas. It also commonly involves soft local vascularity. The blanched mucosa becomes
palate, palatal fauces, uvula, tongue and labial slightly opaque and white. The whitening often
mucosa. Some times, it involves the floor of mouth takes place in spots so that the mucosa acquires
and gingiva. a marble like appearance. Blanching may be
The onset of the condition is insidious and is localized or diffuse, involving greater part of the
often of 2 to 5 yr of duration. The most common oral mucosa or reticular, in which blanching
initial symptom is burning sensation of oral consists of blanched area with intervening
mucosa, aggravated by spicy food, followed by clinically normal mucosa, giving it a lace like
either hypersalivation or dryness of mouth. appearance. As disease progresses the mucosa
Vesiculation, ulceration, pigmentation, recurrent becomes stiff and vertical fibrous bands appear
stomatitis and defective gustatory sensation have there.
also been indicated as early symptoms. Gradual Affected sites and their signs—Lips (36%)—mucosa
stiffening of the oral mucosa occurs in few years is blanched, becomes rubbery and is
after the initial symptoms appear. This leads to characterized by the presence of circular bands
inability to open the mouth (Fig. 12.19). Later on around the rima oris like a thin band. In severe
patients experience difficulty in protruding the labial involvement, the opening of mouth is
tongue. When the fibrosis extends to pharynx altered to an elliptical shape (elliptical rima oris),

Flow chart 12.2: Role of areca nut in oral submucous fibrosis

198 Textbook of Oral Pathology

protrude the tongue beyond the incisal edges.

Floor of mouth (29%) —when floor of mouth is
affected, it becomes inelastic. Gingiva—when
affected, it becomes fibrotic, blanched and
inelastic.

Associated Features
• Pigmentation: Hyperpigmentation or
occasional loss of pigmentation is very
common in association with oral submucus
fibrosis. Many a times pigmentation changes
in vermilion border are so striking that this
disease can be suspected even before
examining the patient.
• Vesicle (32%): It is usually found in areas of
redness in the soft palate, the anterior faucial
pillar, buccal mucosa or the mucosal surface
of lip, particularly the lower lip. The vesicles
are painful and they soon rupture leaving
behind superficial ulceration. Often there is
history of vesiculation following the intake
Figure 12.19: Oral submucous fibrosis showing fibrous of spicy food, suggesting an allergic reaction
band and decreased mouth opening
to spicy food.
lips become leathery and it become difficult to • Ulceration (43%): Ulceration often develops
evert them. Buccal mucosa (98%)—the affected in the course of disease, particularly in
mucosa becomes coarse, blanched and inelastic. advanced cases. In advanced cases,
In advanced cases, the mucosa becomes tough epithelium becomes atrophic which render
and leathery with numerous vertical fibrous it fragile and vulnerable to ulceration.
bands. Soft palate (49%) and uvula— • Petechiae: These are small raised reddish blue
involvement of soft palate is marked by fibrotic spots which sometimes occur in oral
changes and a clear delineation of the soft palate submucus fibrosis. It may be few or many.
from hard palate. The mobility of soft palate is They occur most commonly on tongue and
restricted. Uvula, when involved, is shrunken the labial and buccal mucosa. The petechiae
and in extreme cases it becomes bud like. Palatal are transient in nature and do not require any
fauces—in the soft palate the bands radiate from specific treatment.
pterygomandibular raphe to the anterior faucial
Histopathological Features
pillars. The faucial pillars become thick and short
and tonsils may get pressed in between fibrosed Epithelium: In most of the cases, the oral
pillars. Tongue (37%) —the initial change is epithelium is markedly atrophic. The atrophic
depapillation, usually in the lateral margins. epithelium exhibits intercellular edema (18%),
Tongue becomes smooth; its mobility, especially signet cells (13%) and epithelial atypia (7%).
in protrusion, becomes impaired. Patient can not Sometimes atrophic epithelium is associated
 Premalignant Lesions and Conditions 199

with hyperorthokeratosis and pyknotic changes

in the nuclei of basal cell layer. There is
liquefaction degeneration of the basal layer of
cells. Rete pegs are completely lost.
Connective tissue: It shows vesicles which are
caused by subepithelial accumulation of fluid.
The inflammatory cells are mostly mononuclear;
eosinophils and occasional plasma cell may be
seen.

Epithelial Atypia in Oral Submucous Fibrosis

The features most often associated are irregular Figure 12.20: Submucous fibrosis showing flattening of
epithelial stratification, increased number of rete pegs
mitotic figures, nuclear pleomorphism,
hyperchromatism and loss of polarity of cell. • Moderately advanced stage: The collagen is
Another striking feature is spongiosis, especially moderately hyalinized and amorphous
in basal cell layer. A considerable number of changes start from the juxtaepithelial base-
signet cells are also seen, mostly located in basal ment membrane. Occasionally, thickened
layer. Marked reduction in melanin pigmenta- collagen bundles are still seen separated by
tion in basal cell layer. slight residual edema. The fibroblastic re-
Grading of oral submucous fibrosis: sponse is less marked; the cells present be-
Pindborg has described the connective tissue ing mostly adult fibrocytes with elongated,
changes in four consecutive stages as follows spindle-shaped nuclei and scanty cytoplasm.
(Fig. 12.20): Blood vessels are either normal or constricted,
• Very early stage: It is characterized by finely as a result of increased surrounding fibrous
fibrillar collagen dispersed with marked tissue. The inflammatory exudate consists of
edema. The fibroblastic response is strong lymphocytes and plasma cells, although oc-
with plump young cells containing abundant casional eosinophils may be present.
cytoplasm. The blood vessels are sometimes • Advanced stage: Collagen is completely
normal, but more often they are dilated and hyalinized and is seen as a smooth sheath with
congested. The inflammatory cells mainly no separate bundles. Edema is absent. The
polymorphs with occasional eosinophils are hyalinized area is devoid of fibroblasts, al-
present. though a thin elongated cell or vestigial
• Early stage: The juxtaepithelial area shows nucleus is seen in a rare interval along the fi-
early hyalinization. The collagen is still seen ber bundle. Blood vessels are completely oblit-
as separated bundles which are thickened. erated or narrowed. The inflammatory cells
Plump young fibroblasts are present in are lymphocytes and plasma cells (Fig. 12.21).
moderate numbers. The blood vessels are
often dilated and congested. The Staging of Oral Submucous Fibrosis (Haider et al)
inflammatory cells are mostly mononuclear
lymphocytes eosinophils and occasional There are various schemes for staging of oral
plasma cell. submucous fibrosis. These schemes help
200 Textbook of Oral Pathology

phic epithelium first becomes hyperkeratotic and

later, intracellular edema and basal cell hyper-
plasia develop eventually, following epithelial
atypia with moderate epithelial hyperplasia and
then, carcinoma can develop at any time. To sub-
stantiate the precancerous nature of the condi-
tion, following points are noted:
• High occurrence of submucus fibrosis in oral
cancer patient
• Higher incidence of oral cancer in patient
with oral submucus fibrosis
• Histological diagnosis of carcinoma, without
the clinical suspicion of it
• Higher prevalence of leukoplakia among oral
Figure 12.21: Oral submucous fibrosis: Oral submucous submucus fibrosis patients
fibrosis showing abundant deposit of collagen fibers (1) • Higher frequency of epithelial dysplasia.
flattening of rete pegs (2): juxtaepithelial inflammatory
cells (3)
The WHO Collaborating Centre for Oral
Precancerous Lesions has concluded that
clinicians in determining the severity of the although oral submucus fibrosis predisposes to
disease and to modify the treatment accordingly. cancer, it is not absolutely conclusive. It is highly
Clinical staging: It is based on bands seen in the probable that such relationship does exist.
oral cavity (Table 12.2). Following facts support this hypothesis:
• The frequency of oral leukoplakia in oral
Table 12.2: Clinical staging of oral submucous fibrosis
submucus fibrosis patient is 6-8 times higher
Clinical stage Clinical features
than in control group
1 Faucial bands only
2 Faucial and buccal bands
• Carcinoma patients exhibiting oral submucus
3 Faucial, buccal, and labial bands fibrosis have a frequency with exceed the
1.2% of submucus fibrosis in general popu-
Functional staging: Functional staging is based on lation
amount of mouth opeing. It is measured between • Immunological alternations observed in oral
the the central incisors of maxilla and mandible submucus fibrosis are almost similar to that
(Table 12.3). observed in oral cancer.

Table 12.3: Functional staging of oral submucous fibrosis Management m

Functional staging Amount of mouth opening
Restriction of habit/behavioral therapy: The
A 20 mm preventive measure should be in the form of
B 11–19 mm
C 10 mm stoppage of habit, which can be encouraged
through public education. Affected patients
should be explained about the disease and its
Malignant Potential
possible malignant potential. Improvement in
Oral cancer originates in submucous fibrosis clinical features like gradual increase in inter-
from diverse intra-oral location, without any no- incisal opening has been observed in most of the
ticeable predilection for any specific site. Atro- patients who discontinue the habit.
 Premalignant Lesions and Conditions 201

Medicinal therapy: A vitamin rich diet along with epiphora, eyelid infection, urethral anomalies,
iron preparation is helpful to some extent but small testes and hyperhydrosis of the palms and
has little therapeutic value in relieving trismus. soles.
Iodine-B-complex preparation (Injection
Ranodine) is a combination of iodine preparation Oral Manifestations
with synthetic vitamin B complex. The oral lesions have onset between the age of 5
Steroids: Hydrocortisone injection along with to 14 years. Most common sites are tongue and
procaine hydrochloride injection locally in the buccal mucosa. It appears as diffusely distributed
area of fibrosis. Injections are given fortnightly. vesicles and ulcerations, followed by
The early cases show good improvement with accumulation of white patches of necrotic
this therapy. Systemic steroid can also be given epithelium and sometimes, superimposed
in severe cases. monolial infection. After some time, in the age
group of 14 to 20 years, there are repeated
Placental extract: Injection of placentrix can also
recurrent ulceration and development of
be given. Hyaluronidase can be given.
erythroplasia or red mucosal lesion. Finally
Oral physiotherapy: Oral exercises are advised in between the age of 20 and 30 years, there is
early and moderately advanced cases. This development of erosive leukoplakia and
includes mouth opening and ballooning of carcinoma.
mouth. This is thought to put pressure on fibrous
bands. Forceful mouth opening have been tried Histopathological Features
with mouth gag and acrylic surgical screw.
The skin lesion shows increased number of
melanin containing chromatophores and
Dyskeratosis Congenita increased vascularity. Depending upon the stage
It is also called as ‘Zinsser-Engman-Cole of disease epithelium may show dysplasia.
syndrome’. It is a well recognized but rare
Lupus Erythematosus
genokeratosis, which is probably inherited as a
recessive characteristic. Disease manifested has It is characterized by presence of abnormal
three typical signs: oral leukoplakia, dystrophy antibodies and immune complex.
of nails and pigmentation of skin.
Types
Clinical Features • Discoid lupus erythematosus—If it is
It almost exclusively seen in males. Nail changes confined to skin and mucosa.
are the first manifestation, becoming dystrophic • Systemic lupus erythematosus—If multi-
and shedding some time after the age of 5 years. organ involvement occurs.
Grayish brown pigmentations appear in some
Etiology
time which is seen usually on trunk, neck and
thigh. The skin may become atrophic, • Genetic predisposition: Relative of patients
telangiectatic and face appears red. Other minor have higher incidences autoantibodies,
manifestations are frail skeleton, mental immune deficiency and connective tissue
retardation, small sella turcica, dysphagia, disease. This tendency is greatest among
transparent tympanic membrane, deafness, identical twins
202 Textbook of Oral Pathology

• Immunological abnormality possibly mediated by The lesion increases in size by peripheral

viral infection: Immune complex consisting growth. Periphery of the lesion appears pink or
chiefly of nucleic acid and antibody account red, while the center exhibits an atrophic scarred
for majority of the tissue change seen. appearance. Butterfly distribution on macular
• Autoimmune disease: As these patients develop region and across the bridge of the nose.
antibodies to many of their own cells.
• Deposition of antigen: Antibody complexes. Oral Manifestations
• Endocrine (high incidence in females in Most common sites are buccal mucosa, tongue,
pregnancy): Finding of increased estrogen palate and vermilion border of lip. It begins as
level. erythematous area, sometimes slightly elevated,
• Biochemical increase in excretion of metabolic but more often depressed, usually with
products, particularly tyrosine and induration and typically with white spots.
phenylalanine, in certain SLE patient. Occasionally, superficial painful ulceration may
occur with crusting or bleeding, but no actual
Discoid Lupus Erythematosus scale formation. There may be burning and
Clinical Features tenderness which may be intermittent or
disappear if the lesion becomes inactive. The
It occurs in 3rd and 4th decades, female margins of the lesion are not sharply demarcated.
predilection in the ratio of 5:1. Most common Fine white striae radiate out from the margins.
sites are face, oral mucosa, chest, back and Central healing may result in depression.
extremities. It is a circumscribed, slightly Erythematous, atrophic plaque, surrounded by
elevated, white patch that may be surrounded keratotic border may involve the entire lip.
by red telangiectatic halo. Cutaneous lesions are
slightly elevated, red or purple macules; that are
Systemic Lupus Erythematosus
often covered by gray or yellow adherent scales.
Carpet track extension: Forceful removal of scale Clinical Features
results in ‘carpet track extension’, which has
dipped into enlarged pilosebaceous canals. It occurs in 3rd (female) and 4th (male) decades
and has female predilection (8:1). It is
characterized by repeated remissions and
exacerbations with common sites being face,
neck, upper arm, shoulders and fingers. It is
manifested by symptoms of fever and pain in
the muscle and joints. It may present as itching
or burning sensation as well as area of
hyperpigmentation. Severely intensifies after
exposure to sunlight.
Cutaneous lesion: The cutaneous lesion consists
of erythematous patches on the face, which
coalesce to form roughly symmetrical pattern
over the cheeks and across the bridge of the nose,
Figure 12.22: The hair follicles cut in cross section (1),
degeneration of basal cell layer (2), dense aggregate of in a so called butterfly distribution. Skin lesions
chronic inflammatory cells (3), keratin plugging (4) are widespread, bilateral with signs of acute
 Premalignant Lesions and Conditions 203

inflammation. This finding helps to differentiate

between skin lesions of DLE and SLE.
Involvement of various organs including
kidneys and heart. In kidney, fibrinoid
thickening of glomerular capillaries producing
the characteristic ‘wire loop’ which may be
sufficient to result in renal insufficiency. Heart
may suffer a typical endocarditis involving
valves as well as fibrinoid degeneration of
epicardium and myocardium.

Oral Manifestation
The most common sites are buccal mucosa, lip
and palate. Complain of burning sensation,
xerostomia or soreness of mouth. Lesions similar
to DLE, except that hyperemia, edema and
extension of lesion is more pronounced. Greater Figure 12.23: Systemic lupus erythematosis with keratin
plugging (KP)
tendency to bleed and petechiae, suspected
ulcerations surrounded by red halo. The lip and liquefaction degeneration of basal layer.
lesions appear with central atrophic area with Perivascular infiltration of lymphocytes and
small white dots surrounded by keratinized their collection about dermal appendages,
border, which is composed of small radiating basophilic degeneration of collagen and elastic
white striae. There is occasional ulceration of fibers with hyalinization, edema and fibrinoid
central area. The intraoral lesion is composed of change.
a central depressed red atrophic area surrounded Skin lesions show hyperkeratosis, with kera-
by 2 to 4 mm elevated keratotic zone, that tin packed into the openings of the hair follicles,
dissolves into small white lines. called as “follicular plugging”. Degeneration of
the basal cell layers is seen common in both skin
Laboratory Findings
and oral lesions. The underlying connective tis-
LE cell inclusion phenomenon with surrounding sue stroma shows patchy to dense aggregates of
pale nuclear mass apparently devoid of chronic inflammatory infiltrate.
lymphocytes. It is characterized by presence of
Differential diagnosis with Lichen planus: LE shows
abnormal serum antibodies and immune
perivascular infiltrate, PAS positive material in
complexes. Anemia, leukopenia and thrombocy-
the basement membrane zone and subepithelial
topenia, with sedimentation rate increased.
edema which may form vesicle.
Serum gamma globulin increased and Coomb’s
test is positive.
Management
Histopathological Features (Fig. 12.23) It is treated by systemic corticosteroids therapy
Hyperorthokeratinization, hyperpara keratinization and should be managed by physician.
with keratotic plugging, atrophy of the rete pegs Antimalarial drugs can be used in some cases.
204 Textbook of Oral Pathology

CHAPTER
Malignant
13 Tumors

CLASSIFICATION OF MALIGNANT TUMORS

A. Epithelial G. Neural tissue

• Squamous cell carcinoma • Neurosarcoma
• Metastatic carcinoma • Neurofibrosarcoma
• Basal cell carcinoma • Neuroblastoma
• Transitional cell carcinoma • Ganglioneuroma
• Malignant melanoma H. Muscle
• Verrucous carcinoma • Leiomyosarcoma
• Spindle cell carcinoma • Rhabdomyosarcoma
• Primary intra-alveolar carcinoma • Malignant granular cell myoblastoma
• Intra-epidermoid carcinoma I. Lymphoid tissue
• Adenoid squamous cell carcinoma • Hodgkin’s lymphoma
B. Fibrous connective tissue • Non-Hodgkin’s lymphoma
• Fibrosarcoma • Primary reticular cell sarcoma
• Malignant fibrous histiocytomas • Burkitt’s lymphoma
C. Adipose tissue • Leukemia
• Liposarcoma J. Myeloma
D. Cartilage • Multiple myeloma
• Chondrosarcoma • Plasmacytoma
E. Bone K. Tumor of salivary gland
• Osteosarcoma • Mucoepidermoid carcinoma
• Osteochondrosarcoma • Adenocystic carcinoma
• Ewing sarcoma • Adenocarcinoma
F. Vascular • Acinic cell carcinoma
• Hemangioendothelioma • Malignant changes in pleomorphic
• Hemangiopericytoma adenoma
• Angiosarcoma
 Malignant Tumors 205

Tumor is an autonomous new growth of tissue inorganic agents are at increased risk of cancers
or it is an abnormal mass of tissue the growth of of mouth.
which exceeds and is uncoordinated with that of
Orodental factors: It is more prevalent in patients
normal tissue and persists in the same excessive
with poor oral hygiene, faulty restorations, sharp
manner even after the cessation of stimuli which
teeth, ill fitting dentures and those with syphilitic
evoked the changes.
glossitis.
Cancer is a commonly used term for all
malignant tumors. In Latin language, cancer Immunity: The increasing incidence of oral
means crab which has a fat main body and mass cancers is clearly age related, which may reflect
extension which springs and invades the declining immune surveillance with age. It may
surrounding tissues. occur in immunosuppressed patients following
Oral carcinoma is one of the most prevalent organ and bone marrow transplantation. HIV/
cancers and is one of the 10 major causes of death. AIDS patient are at increased risk of oral cancer.
Majority of oral carcinomas are squamous cell
Smokeless tobacco: Taken together; the effect of
carcinomas. It is a disease of increasing age with tobacco use, heavy alcohol consumption and poor
95% cases in people older than 40 years of age.
diet can probably explain over 90% of cases of
oral cancer. Much of tobacco in the world is
Etiology and Risk Factors for Oral Cancer consumed without combustion, by being placed
Actinic radiation: It is a minor etiologic factor in into contact with mucous membrane, through
cases of lip cancer. Lip cancer occurs more which nicotine is absorbed. It contains potent
commonly in fair skinned people who are carcinogens like nitrosamine, polycyclic
generally engaged in outdoor occupation, such hydrocarbons and polonium and metabolites of
as farming and fishing. these constituents, which have been the suggested
etiologic factors in oral cancer. Some common forms
Familial and genetic: There is increasing evidence of oral smokeless tobacco are as follows:
that there is familial and genetic predisposition • Pan/paan/betal quid — It contain areca nut, betel
for the development of oral cancer. Lip cancer is leaf, slaked lime, catechu, condiments with or
amongst the sites which show strongest cancer without tobacco.
clustering within families. But it also reflects the • Khaini — It contains tobacco and lime.
fact that families tend to have same occupation • Mishri — It is burned tobacco.
i.e. fishing and farming, which is related with • Zarda — Boiled tobacco.
ultraviolet exposure. Oral cancers are more • Gadakhu — It contains tobacco and molasses.
prevalent in black as compared to white. • Mawa — Tobacco, lime and areca.
Atmospheric pollution: Parts of the urban/rural • Nass — Tobacco, ash, cotton or sesame oil.
difference in incidence of head and neck cancers • Naswar/niswar — Tobacco, lime, indigo,
have been related to atmospheric pollution. cardamom, oil and menthol.
Sulphur dioxide and smoke concentration in the • Shammah — Tobacco, ash and lime.
atmosphere are positively correlated with • Toombak — Tobacco and sodium bicarbonate.
squamous cancer of larynx and pharynx. The Smoking: Tobacco smoke contains carbon
impact on cancer of the mouth is likely to be less, monoxide. It is an important factor in the
but merits careful study. Blue collar workers development of oral cancer. Study shows that
exposed to dust or inhalation of organic and cigar and pipe smoking increase the risk of cancer
206 Textbook of Oral Pathology

than cigarette smoking. It has been stated that the • Lichen planus: Reports of carcinoma superven-
pooling of carcinogens in saliva gives rise to ing on lichen planus have been made in some
cancer in the floor of mouth and ventral and lateral cases.
tongue. Smoking is strongly associated with soft • Candidosis: It is possible precursor of
palate cancer than anterior sites. carcinoma. Candidal infection is often
associated with acanthosis and parakeratosis.
Alcohol: All forms of alcohol, including hard
• Leukoplakia: It is common premalignant
liquor, wine and beer have been implicated in the condition seen in oral cavity. Nodular
etiology of oral caner. The mechanism by which leukoplakia show higher rates of epithelial
alcohol affects includes the dehydrating effect of dysplasia.
alcohol on the mucosa which increases mucosal • Median rhomboid glossitis: In some cases, it has
permeability and the effects of carcinogens on the followed by cancer.
mucosa. Beverage consumption include nitro- • Plummer-Vinson syndrome: The atrophic
samines and impurities which can act as changes in the mucous membrane of the upper
carcinogens. Most heavy alcohol consumer also alimentary tract that occur in this syndrome
uses tobacco so it is difficult to separate the ill predispose to malignancy.
effects individually. • Oral Submucus fibrosis: It is precancerous
Syphilis: It is traditionally associated with oral condition occuring in oral cavity.
cancer. • Oral melanosis: It appears to be associated with
oral cancers in India.
Diet deficiency and deficiency status: Nutritional • Discoid lupus erythematosus: A number of cases
deficiency and liver dysfunction can also play a of carcinoma of lip developing in the lesion of
role in it. The relationship between sideropenic lupus erythematosus have been reported.
dysphagia and oral cancer is well established. • Epidermolysis bullosa: It is occasionally
Ionizing radiation: Carcinoma of buccal mucosa followed by carcinoma.
may occur as a complication of long term
EPITHELIAL TUMORS
radiotherapy.
Squamous Cell Carcinoma
Trauma: Trauma in combination with other factors
like chronic cheek biting, denture use and It is also called as 'epidermoid carcinoma' or
irregular teeth may act as a co-carcinogen and 'epithelioma'. It represents 90% of all malignant
may promote transformation of epithelial cells. tumors occurring in the mouth and jaws. The
majority of oral cancer cases are of squamous cell
Virus: The possibility of type I herpes simplex
carcinoma. The oral lesion often invades the jaw.
virus being associated with oral cancer has been It arises in gingival tissue, buccal sulcus, floor of
suggested. Other viruses which can lead to oral mouth and some other portion of oral mucosa.
cancer are human papilloma virus and HIV
virus. Clinical Features
Intraoral lesions: Some intraoral lesion can be Predominately, it occurs in males with ratio of
responsible for the oral malignancy. They are as 2:1, older than 50 years with an average age of
follows: approximately 60 years. Most commonly involved
• Chronic ulceration and fissure: It can cause oral are the posterior and lateral borders of the tongue
cancer particularly of lip. and lower lip and less frequently the floor of
 Malignant Tumors 207

mouth, alveolar mucosa, palate and buccal Histopathological Features

mucosa. It may be solitary and multifocal.
The histopathological features are graded into
Patient may present with awareness of a mass
three types depending upon the degree of
in the mouth and neck. Small lesion is
differentiation of the neoplastic proliferating cells.
asymptomatic. Large lesions may cause some pain
1. Well-differentiated or highly differentiated
or paraesthesia and swelling. Patients complain
squamous cell carcinoma.
of persistent ulcer in the oral cavity. Function of
organ is impaired. 2. Moderately differentiated squamous cell
The clinical appearance of a carcinomatous carcinoma.
ulcer is that one of irregular shape, induration 3. Poorly differentiated squamous cell
and raised everted edges. Usually have broad base carcinoma.
and are dome like or nodular. Surface may range All the carcinomas show the following features:
from granular to pebbly to deeply creviced. In 1. Epithelial dysplasia.
some cases, surface may be entirely necrotic and 2. Keratinization varies with degree of
have ragged whitish gray appearance (Fig. 13.1). differentiation.
It may be completely red or red surface may be 3. Local invasion by break in the basement
sprinkled with white necrotic or keratin area. Base membrane and invasion and proliferation into
and borders are firm to palpate and the lesion the underlying connective tissue. This is
may get fixed after infiltration into underlying clinically manifested as fixation and
tissues. induration of the lesion.
Superficial and deep cervical nodes are 4. Metastasis by blood or lymphatic channels
commonly affected. They become enlarged and
histopathologically seen as invasion of tumor
are firm to hard on palpation. The nodes are non-
cells into the capillaries and lymphatic ducts
tender unless associated with secondary infection
permeation.
or an inflammatory response. It may be nodular
5. Perinueral invasion—some of the tumor cells
or polypoid and pink to red and have at least one
may have a typical invasion pattern along the
ulcerated patch on their surface. Fixation of nodes
nerve sheath.
to adjacent tissues occurs later.
Fixation of primary tumor to adjacent tissues, Well-differentiated squamous cell carcinoma
i.e. overlying bone suggests involvement of • It consists of sheets and nest of cells with
periosteum and possible spread to bone. characteristic appearing of squamous origin.
• These cells are generally large and show
distinct cell membrane. They resemble the cells
of squamous epithelium, both structurally and
functionally. The intercellular bridges are
prominent.
• The nuclei are distinctly dark staining.
Increased number of mitotic figures but
relatively few from moderately differentiated
carcinoma. It also shows hyperchromatism
prominent and multiple nucleoli and
increased nucleocytosplasmic ratio
Figure 13.1: Squamous cell carcinoma of mandibular
buccal mucosa showing extraoral growth (Fig. 13.2). Most prominent feature are
208 Textbook of Oral Pathology

Figure 13.3: Well-differentiated squamous cell

carcinoma showing keratin pearl

Figure 13.2: Well-differentiated squamous cell carcinoma cells, highly invasive with poor prognosis. The
showing invasion in connective tissue forming epithelial tumor cells bears little resemblance to their cells
and keratin pearls
of origin and often will present diagnostic
presence of individual cell keratinization and difficulties because of the primitive and
formation of numerous keratin pearls of uncharacteristic histologic appearance. These
varying size. cells show lack of cohesiveness and are extremely
• Each keratin pearl consists of a central area of
vagarious. The mitotic figures are extremely high
keratin surrounded by whorls of prickle cells.
(Fig. 13.6).
• Pleomorphism of cells, keratinization, and keratin
pearls deep to epithelial surface and loss of
Carcinoma of Floor of Mouth
intercellular bridges or cohesiveness may be
seen (Fig. 13.3). It is seen more commonly in men. It is seen most
• Moderately differentiated/ Less well differentiated frequently in the anterior portion of floor. The
squamous cell carcinoma (Fig. 13.4). typical carcinoma of the floor of mouth is an
The tumor cells are less differentiated and indurated ulcer of varying size, on one side of the
have less resemblance to squamous epithe- midline. It may take form of wart like growth,
lium. The characteristic shape of an epithelial which tend to spread superficially rather than in
cell may not be evident. The cell to cell contacts depth (Fig. 13.7). Carcinoma in close relation to
and relation and arrangement are altered. teeth may cause loosening or exfoliation and root
• The greater number of mitotic figures show resorption.
that the growth rate is more rapid (Fig. 13.5). It may or may not be painful. In some cases
This may be varied size and shape. The there may be referred pain in the ears. The
keratinization is absent as the cells cannot in proximity of this tumor to the tongue produces
size and shape. Keratin pearls may not be some limitation of motion of these organs, often
present function to the differentiation point of induces peculiar thickening or slurring of the
keratin formation. Numerous epithelial speech. There may be excessive salivation.
islands of prickle cells with peripheral basal Carcinoma of floor of mouth may invade the
cells may be seen. deeper tissues and may even extend into the
Poorly differentiated squamous cell carcinoma. submaxillary and sublingual glands. Metastasis
This is the tumor with proliferating anaplastic from the floor of the mouth are found most
 Malignant Tumors 209

Figure 13.4: Well differentiated squamous cell carcinoma

showing islands of well differentiated epithelial cells

Figure 13.6: Poorly differentiated squamous cell carcinoma

showing lack of cohesiveness and differentiation

Figure 13.7: Carcinoma of floor of mouth showing

ulcerative growth

Figure 13.5: Moderately differentiated squamous cell It usually occurs opposite to the 3rd molar
carcinoma showing pleomorphism of cells (Fig. 13.8). The tumor begins as small nodules and
enlarges to form a wart like growth which
commonly in the submaxillary group of lymph ultimately ulcerates. The lesion is often painful.
nodes and since the primary lesion frequently There is induration and infiltration of deeper
occurs near the midline where a lymphatic cross tissues. Extension into the muscle of neck, alveolar
drainage exists, contralateral metastasis is often mucosa and ultimately into bone may occur.
present. Some cases appear to be growing outward from
the surface rather than invading the tissues is
Carcinoma of Buccal Mucosa called as exophytic or verrucous growth. The
The lesions develop most frequently along or most common site of metastasis is the
inferior to a line opposite the plane of occlusion. submaxillary lymph nodes (Fig. 13.9).
210 Textbook of Oral Pathology

Figure 13.8: Carcinoma of buccal mucosa extending

extraorally
Figure 13.10: Malignancy involving upper alveolar ridge

Figure 13.9: Carcinoma of buccal mucosa showing

intraoral ulceration Figure 13.11: Malignancy of lower alveolar ridge
showing growth

Carcinoma of Labial Mucosa

It is frequently encountered in person who

habitually keeps a mixture of tobacco lime in the
labial groove (Fig. 13.10). The lower labial mucosa
is more commonly involved than the upper (Fig.
13.12). The most common initial sings and
symptoms are growth or swelling, soreness and
ulceration. Advanced lesion may be ulcerative-
infiltrative type. Lymph node involvement may
occur, which may be unilateral or bilateral.

Figure 13.12: Carcinoma involving the mandible showing

Carcinoma of Palate extraoral swelling

• It is common in area where reverse smoking is defined ulcerated painful lesion on one side
practised. It is seen more amongst women as of the midline. Most of the lesions are
compared to men, in case of reverse smoking. exophytic and with broad base and nodular
Palatal cancer usually manifest as a poorly surface.
 Malignant Tumors 211

Figure 13.13: Maxillary sinus malignancy showing

intraoral ulceration

• It frequently crosses the midline and may

extend laterally to include tonsillar pillars or
even the uvula. The tumor of hard palate may
invade the bone or occasionally the nasal
cavity while infiltrating lesions of the soft
palate may extend into the nasopharynx.

Carcinoma of Maxillary Sinus

Figure 13.14: Extraoral swelling seen in maxillary sinus
• It is caused by sinusitis, snuff and smoke and malignancy

occupational hazards like in boot/shoe

diplopia, proptosis and pain over the cheek
making and nickel worker. Mean age of
and upper teeth. Posterior wall involvement
occurrence is 60 years. Males are commonly
affected more than females in the ratio of 2:1. leads to painful trismus, obstruction of
Eustachian tube causing stuffy ear, referred
It is most common primary tumor of paranasal
pain and hyperesthesia over the distribution
sinuses comprising 80 to 90% of cancers in
this site. There is facial pain, swelling, nasal of second and third division of trigeminal
nerve. It may involved infraorbital nerve and
obstruction and lymphadenopathy. Medial
produces paresthesia of the cheek or erodes
wall involvement leads to nasal obstruction,
discharge, bleeding and pain. Epiphora will blood vessels giving rise to epistaxis.
Paresthesia of mandibular nerve can also
result if the lacrimal sac or naso-lacrimal duct
occur if the tumor invades the cranium.
is obstructed. Involvement of the floor of the
sinus leads to expansion of the alveolus,
Management
unexplained pain, numbness of teeth, loose
teeth and swelling of the palate or alveolar Average timedelay between onset and
ridge and malfitting dentures. It may erode examination is 4 to 9 months. Removal of any
the floor and penetrate the oral cavity (Fig. local irritants, i.e. the 5-S smoking, spices, spirite,
13.14). sepsis and syphylis. Excisional biopsy, if lesions
• Lateral wall involvement leads to facial and are small. Surgery–cryosurgery, laser surgery
vestibular swelling, pain and hyperesthesia and radical surgery. Radiation and chemo-
of maxillary teeth. Roof involvement leads to therapy.
212 Textbook of Oral Pathology

make an invaluable contribution of pathologic

process of bone. Most common sites of origin are
breast, lung, kidney, thyroid, prostate and colon.

Clinical Features
Mandible is involved much more frequently than
maxilla, especially in the region near premolars
and molars as tumor metastasizes to those bones
which are rich in haemopoietic marrow. It is said
that blood flow rate is decreased in areas of
haemopoietic marrow and this predisposes tumor
emboli to settle and grow in these areas. This
Figure 13.15: Keratin pearls
hypothesis is consistent with jaw metastases as
hemopoietic marrow is most routinely found at
this site. The other sites involved are maxillary
sinus, anterior hard palate and mandibular
condyle.
It is found in patients between 40 and 60 years
of age and there may be history of primary tumor.
Early lesion is nodule or dome with shaped
smooth surface and due to trauma may get
ulcerated. There may be pain followed by
paresthesia or anesthesia of lip or chin.
Teeth in this region may become loose or
exfoliate and root resorption may occur. On
occasion, tumor may breach the outer cortical
plate of jaws and extend into surrounding soft
tissue or presents as an intraoral mass.
Figure 13.16: Basal cell carcinoma showing extensive Metastatic tumors are diagnosed only when
destruction of face the sockets of extracted teeth do not heal because
of periodontal disease. If invasion occurs in
Metastatic Carcinoma
muscle then their function is impaired. Loss of
It is also called as 'secondary carcinoma'. It is the bony support, involving one or several adjacent
most common malignant tumor in the skeleton. teeth, in the absence of generalized periodontitis
This tumor is transported to an area distant from is an important symptom. There may be
its origin and establishes a new foot holds and pathological fracture of the jaw or hemorrhage
are said to have metastasized. Although the from the tumor site. Extraction socket fails to heal
metastatic carcinoma of jaw is uncommon, its or enlarged. All the soft tissues curetted from an
recognization is important because the jaw extraction socket, even in absence of clinical
tumors may be the first indication that the patient suspicion should be submitted for histological
has a malignant disease. Oral diagnostician can examination.
 Malignant Tumors 213

Management
Prognosis is poor and death occurs within a short
time. It can be treated by chemotherapy, radiation
therapy, surgery, and immunotherapy and
hormone therapy.

Basal Cell Carcinoma

It is also called as 'basal cell epithelioma' or
'Rodent ulcer'. It arises from basal layer of
epidermis or from the hair follicle.

Etiology
The specific factor in sunlight responsible for skin
carcinogenesis appears to be ultraviolet radiation.
Other factors are also responsible like burn scars
and ionizing radiation. General atrophy
associated with aging process, at least, predispose
to development of skin cancer.

Clinical Features
It develops most frequently on exposed surface of
skin, (Fig. 13.16) middle thirds of face and the
scalp. There is also involvement of lip. The upper
lip is involved more commonly than the lower
lip. In middle aged or elderly person usually in
4th decade of life. Blond people with fair
complexion who have spent much of their lives Figure 13.17: Basal cell carcinoma showing sheets and
nests of hyperchromatic epithelial cells
out doors are often victim of these lesions. It is
much more common in men than women because
size and accounts for its synonym 'rodent ulcer'.
men are exposed to the environmental elements
It is never seen in oral cavity unless it arrives there
more than women.
by invasion and infiltration from a skin surface.
Signs : It begins as a small, slightly elevated papule Occasionally, it may metastasize to lymph
which ulcerates, heals over and then breaks nodes.
down again to form crusted ulcer. It develops a
smooth, rolled border representing tumor cells Histopathological Features
spreading laterally beneath the skin. Untreated It is characterized by appearance of nests, islands
lesion continues to enlarge and infiltrate the or sheets of cells showing indistinct cell membranes
adjacent and deeper tissues and it may even erode with large deeply staining nuclei and variable
deeply into the cartilage or bone. number of mitotic figures (Fig. 13.17).
Due to its invading and destructive infiltration The periphery of cell nests is composed of a
into adjoining tissues, it gradually increases in layer of cells, usually well polarized, that are
214 Textbook of Oral Pathology

strongly suggestive of cell of the basal cell layer of Histopathological Features

skin. The basal cell is a pluripotential cell which
It consists of cells growing in solid sheets or in
may develop in several directions. It may form
cords and nests. The individual cell is moderately
hair, sebaceous glands, sweat glands or large, round or polyhedral and exhibit a lightly
squamous epithelium and eventually keratin. basophilic cytoplasm and indistinct cell outlines.
Histological Types The nuclei appear large and round and they
exhibit varying degree of mitotic activity.
• Adenoid basal cell carcinoma—It represent Keratinization and pearl formation is lacking.
neoplasm which mimics glandular formation.
• Cystic basal cell carcinoma—Presence of many Management
cyst in the lesion. X-ray radiation is most commonly the accepted
• Keratotic basal cell carcinoma—Formation of treatment.
parakeratotic cells and horn cysts and
attempted formation of hair structures to the Malignant Melanoma
trichoepithelioma. It is a neoplasm of epidermal melanocytes. It is
• Solid or primordial basal cell carcinoma—Cells one of the biologically unpredictable and deadly
have little tendency to differentiate. of all human neoplasms. Sunlight is very
important etiological factor in cutaneous
Management
melanoma.
Surgical excision or X-ray radiation can be given.
Types
Transitional Cell Carcinoma • Superficial spreading melanoma
These lesions arise chiefly from the tonsil, base • Nodular melanoma
of the tongue and nasopharynx. It is extremely • Lentigo maligna melanoma.
malignant, running a rapid clinical course,
Clinical Features
metastasizing widely and causing very early
death. Superficial spreading melanoma: It is the most
common cutaneous melanoma. The majority of
Clinical Features case diagnosed in the 5th to 7th decade of life. It is

Mean age of occurrence is 44 years.

Symptoms: There may be sore throat, nasal
obstruction, defective hearing or ear pain,
headache, dysphagia, epistaxis and ocular
symptoms.
Signs: The primary lesions are very small often
completely hidden, usually slightly elevated and
either frankly ulcerated or presenting a granular
eroded surface. The tumor is indurated and in
some instances appears as an exophytic or
fungating growth. Swelling of regional lymph
node is most common occurrence. Figure 13.18: Melanoma showing diffuse border
 Malignant Tumors 215

more common in males as compared to females

in the ratio of 2:1. The lesion presents as a tan
brown, black or admixed lesion on sun exposed
skin, in especially in blacks. It also occurs on skin
of face, head and neck, chest, abdomen and
extremities. It begins as pigmented macule in
superficial radial growth pattern, restricted
mostly to epithelium and junction. In advanced
cases, melanoma present as an ulcerated,
fungating growth which is associated with
bleeding (Fig. 13.18). The radical growth phase
may last for several months to several years. The Figure 13.19: Melanoma in lower mandibular region
showing pigmentation
vertical growth phase is characterized by increase
in size, change in color, nodularity and at times, with the overall age of occurrence being 55 years,
ulceration. Some lesions are devoid of pigmenta- with most cases occurring between 40 to 70 years.
tion which is called as amelanotic melanoma.
Site: It has got definite predilection for palate and
Nodular melanoma: They have a predilection for maxillary gingiva/alveolar ridge followed by
occurrence on head and neck of men. Color varies buccal mucosa, mandibular mucosa, tongue, lips
from mucosal pink through brown and blue to and floor of mouth. Focal pigmentation preceding
black. It is firm on palpation. It has got the development of actual neoplasm frequently
erythematous borders which surround the tumor. occurs, several months to years before clinical
There is rapid infiltration in nodular type of symptoms appear.
melanoma. It may be focal or diffuse (Fig. 13.19).
Signs: The lesions usually appear as deeply
Many times it may ulcerate and hemorrhage may
pigmented areas, at times ulcerated and
be seen. In later stages, it becomes more diffuse,
hemorrhagic, which tend to increase
nodular and tumefactive with foci of hypo and
progressively in size. Oral melanomas are
hyperpigmentation. It presents as a sharply
generally painless. The lesion presents as a soft,
delineated nodule with some degree of
darkish brown or black mass. It may have a
pigmentation.
nodular or a papillary surface. The tumor causes
Lentigo maligna melanoma: It has got predilection extensive destruction of the underlying bone;
for exposed parts. It occurs more often in women loosening of teeth may be a concomitant clinical
than in men. It is pigment macule with ill defined finding. There may be ulcerations and bleeding
margins. It occurs characteristically as a macular of the oral mucosa. There is minimum induration
lesion of the malar skin. It grows slowly in radial and no rolled margins are seen.
phase, with around the central axis and in a
superficial manner. As the disease advances, Histopathological Features
invasion and metastasis are frequent. Superficial spreading melanoma: The intraepithelial
component is characterized by the presence of
Oral Manifestations large, epitehloid melanocytes distributed in a so
It is an uncommon neoplasm of the oral mucosa called "pagetoid manner". When melanocytes
and it is more common in men than in women penetrate basement membrane, a florid host-cell
216 Textbook of Oral Pathology

response of lymphocytes develops. Macrophages Etiology

and melanophages may be present. The cells may Tobacco chewers have high percentage of these
be arranged in cluster. cases. It occurs usually in a person habitual to
Nodular melanoma: It is characterized by large, hold the quid in the buccal sulcus.
epitheloid melanocytes within the connective
Clinical Features
tissue. Small ovoid and spindle shaped cells may
be present. The tumor cell may invade and ulcerate It is generally seen in elder population with mean
the overlying epithelium and penetrate the deep age of occurrences of 60 to 70 years. Men are
soft tissue (Figs 13.20 and 13.21). affected more as compared to women. They are
larynx, external auditory meatus, lacrimal duct,
Lentigo melanoma: It is characterized by increased skin, scrotum, penis, vulva, vagina, uterine cervix,
number of atypical melanocytes with the basal perineum, leg and odontogenic cyst linings. In
epithelial layer. The epithelium is generally the oral cavity, it occurs on buccal mucosa and
atrophic and dermal collagen shows the effect of gingiva or alveolar ridge.
sun damage. If skin appendages are present they Oral lesion is slow growing, chiefly exophytic
are often involved with atypical melanocytes as and only superficial invasive, at least until late in
well. the course of the disease and has a low metastatic
potential.
Histological Grading of Malignant Melanoma
Symptoms: Pain and difficulty in mastication are
It depends upon the depth upto which malignant common complain. Regional lymph nodes are
cells have invaded or infiltrated into the often tender and enlarged simulating metastatic
connective tissue. tumor, but the node involvement is usually
• Grade I: Malignant cells are confined within inflammatory.
the epithelium Signs: They appear papillary in nature with
• Grade II: Malignant cells have invaded into pebbly surface which is sometimes covered by a
the papillary dermis. white leukoplakic film. They have rugae-like folds
• Grade III: Malignant cells have invaded into with deep cleft between them. In some cases, there
the level of reticular dermis. may be warty fungating mass. Large broad lesion
• Grade IV: Malignant cells have completely
invaded the reticular dermis.
• Grade V: Malignant cells have extended into
the subcutaneous fat.

Management
It is treated by surgical irradiation, immuno-
therapy and by chemotherapy or, by combination
of these methods. Survival rate is very poor and is
worse with metastases.

Verrucous Carcinoma Figure 13.20: Vertical growth phase of the malignant

epitheloid cells (arrow) invading the connective tissue
It is a slow growing low-grade carcinoma. malignant melanoma
 Malignant Tumors 217

The epithelium is well differentiated and

shows little mitotic activity, pleomorphism or
hyperchromatism. Cleft like spaces lined by a
thick layer of parakeratin extend from the surface
deep into the lesion (Fig. 13.22).
Parakeratin plugging also occurs in the
epithelium (Fig. 13.23). Parakeratin lining of clefts
with parakeratin plugging is the hallmark of
verrucous carcinoma. Even though the lesion
may be very extensive, basement membrane will
be intact. When the lesion becomes infected, focal
intraepithelial abscesses are often seen. Chronic
Figure 13.21: Malignant melanoma showing spindle inflammatory cell infiltration in the underlying
shaped cells (Courtesy: Dr Sangamesh Halawar)
connective tissue may or may not be present.
All bulbous retepegs of the epithelium tend to
project into the underlying connective tissue, at
more or less the same level and this is called as
'pushing margin' (Fig. 13.24). Down growth of
epithelium in connective tissue show pushing
borders which are rather small that invasive
extension. Abundance of keratin is seen on the
surface and with invaginating epithelium as
keratin plugging.

Management
Prognosis in verrucous carcinoma is very good
because of absence or late appearance of
Figure 13.22: Verrucous carcinoma showing metastases. Excision must be sufficiently radical
parakeratin plugging (Courtesy: Dr Alka Kale, Prof and Head,
Oral Pathology, KLES’s Institute of Dental Sciences, Belgaum)
to remove the entire lesion.

with minimum to extensive elevation above

surface of the mucosa. Margins are well defined
and show rim of slightly elevated normal mucosa.
Lesion on the mandibular mucosa grows into
overlying soft tissue and rapidly becomes fixed
to the periosteum, gradually invading and
destroying the mandible.

Histopathological Features
There is marked epithelial proliferation with
downgrowth of epithelium into connective tissue
but usually without the pattern of true invasion. Figure 13.23: Verrucous carcinoma
218 Textbook of Oral Pathology

Management
Surgical removal of tumor with or without radical
neck dissection and radiation therapy is used.

Adenoid Squamous Cell Carcinoma

It is also called as 'adenoacanthoma'. It arises from
pilosebaceous structures or senile keratosis with
acantholysis.

Figure 13.24: Verrucous carcinoma showing broad Clinical Features

ballooning of rete pegs and epithelial downgrowth (pushing
margin) Females are affected more with age ranging from
20 to 50 years and older. It more commonly occurs
Spindle Cell Carcinoma on lip and also in head and neck region. The lower
It is also called as 'Lane tumor', 'polypoid lip is affected more commonly than upper lip and
squamous cell carcinoma' or 'carcinosarcoma'. It it is also common on the vermilion border of lip.
occurs chiefly in respiratory and alimentary tracts.
Sign and symptoms: It appears as simply elevated
It is a variant of squamous cell carcinoma. There
nodules that may be slow crusting, scaling or
is proliferation of spindle cells believed to be arise
ulcerated. Sometimes, there are elevated or rolled
from the surface epithelium.
borders of the lesion.
Both epithelial and connective tissue is affected.

Clinical Features Histopathological Features

It is more common in male with mean age of There is proliferation of surface dysplastic
occurrence of 57 years. Lower lip, tongue and epithelium into the connective tissue as in typical
alveolar ridge or gingiva with remainder scattered epidermoid carcinoma. Lateral and deep
at other site. extension of this epithelium show the
characteristic solid and tubular ductal structures
Sign and symptoms: There is swelling, pain and
which are lined by a layer of cuboidal cells and
presence of a non-healing ulcer. The initial lesions
often contain or enclose acantholytic or
appear either with a polypoid, exophytic or
dyskeratotic cells. There is heavy chronic
endophytic configuration. The lesion is fleshy.
inflammatory infiltration in the corneum, which
Histopathological Features always show basophilic degeneration: typical of
solar damage.
It will show foci of surface epidermoid carcinoma
or epithelial dysplasia of surface mucosa, usually Management
just at the periphery and often quite limited.
Proliferation and 'dropping off' of basal cell to Prognosis is good and metastasis is rare. It is
spindle cell. treated by surgical excision.
The tissue pattern of tumor is either
Multicentric Oral Carcinoma
fasciculated, myxomatosis or streaming. The cells
are elongated with elliptical nuclei, although Multiple primary tumors occur in about 10% of
pleomorphic cell are also common. Giant cells patients with oral cancer. It is usually seen in
and inflammatory cell infiltrates are often present. alcoholics and heavy smokers. The reason for this
 Malignant Tumors 219

is that the whole mucosa is often in an abnormal

state for long periods, prior to the development of
over cancer. The commonest finding is that there
is area of atrophy, either adjacent to a carcinoma
or randomly distributed.

FIBROUS CONNECTIVE TISSUE

Fibrosarcoma
It is malignant neoplasm composed of malignant
fibroblast that produces collagen and elastin. It
arises in the periosteal tissue or endosteally. It
may arise secondarily in tissues that have received Figure 13.25: Fibrosarcoma showing whorled arrangement
of malignant fibroblasts, (whr) collagen fibers arranged in
therapeutic levels of radiation.
herring bone pattern (herr), abnormal mitosis (mito)
(Courtesy: Dr Sangamesh Halawar)
Clinical Features
Onset at any age with mean age of occurrence of
50 years with male predominance. It is commonly
associated with cheek, maxillary sinus, pharynx,
palate, lip and periosteum of maxilla and
mandible. Mandible is more commonly affected
than maxilla.
It produces fleshy bulky mass of tissue. There
may be pain and loosening of the teeth.
Involvement of TMJ and paramandibular
musculature may subsequently produce trismus.
Sensory neural abnormalities may occur if it
involves peripheral nerves.
Initially they resemble benign fibrous
outgrowth, but they grow rapidly to produce large
tumor. Large tumors are prone to ulceration and
hemorrhage. Secondary infections are seen in
some cases. In some cases, pathological fracture Figure 13.26: High power view of fibrosarcoma showing
may occur. Maxillary lesions are quite destructive high mitosis (arrows) in the fibroblast cells. The cells are
arranged in whorled pattern
and invade the antrum. They tend to penetrate
the cortex and spread along the periosteum. pattern (Fig. 13.25). Associated fibers are generally
arranged in interlacing bands or fascicles. In well
Histopathological Features
differentiated tumors, fibroblasts are regular in
It is characterized by proliferation of fibroblasts size and shape. The prominent collage bundles
and formation of collagen and reticular fibers. tend to be arranged in a herring bone pattern.
Cells are spindle shaped malignant fibroblast and Mitotic figures are prominent in small group of
show elongated nuclei. They form whorled poorly differentiated tumors (Fig. 13.26).
220 Textbook of Oral Pathology

Management Synovial Sarcoma

Radical surgical excision is most commonly used It is usually arises from articular or para-articular
treatment modality. sites, bursae or tendon sheath

Malignant Fibrous Histiocytoma Clinical Features

It is also called as 'malignant fibroxanthoma'.
It is predominately disease of young adults mean
Neoplasm that exhibits both histiocytic and
age being 19 years. There is painless deep seated
fibrocytic features are referred to as histiocytomas.
swelling which may produce difficulties in
Types breathing or swallowing. Intraoral sites are cheek,
tongue, floor of mouth and soft palate.
• Giant cell
• Inflammatory
Histopathological Features
• Myxoid
• Storiform It is characterized by biphasic cellular pattern
• Pleomorphic with cleft like or slit like spaces lined by cuboidal
• Angiomatoid. epithelial like cells. The space may contain PAS
positive mucoid material. There may be
Clinical Features fibrosarcoma like proliferation of cells, with
It is one of the more common soft tissue sarcoma associated collagen or reticulum.
of soft tissues of body. There is slight male
Management
predilection and malignant variety is common in
adults. In the head and neck area, more commonly Early radical resection is the best method of
encountered in the paranasal sinuses or centrally, treatment.
within the jaw. Occasionally it arises in the oral
cavity and in the lateral neck, it appears as ADIPOSE TISSUE
indurated swelling. Metastasis in nearly ¼ of
Liposarcoma
cases has been reported.
It is very uncommon malignant tumor of head
Histopathological Features and neck region.
It presents with variety of patterns. The spindle
Clinical Features
cells are arranged in fascicles with a pinwheel or
storiform pattern. The deep seated tumors contain It most frequently occurs in adults over the age
both epithelioid histiocytes and spindle shaped of 40 years with predilection in males in ratio of
fibroblast. Both cell component display marked 2:1. It has a slow, silent growth, submucosal or
hyperchromatism, pleomorphism and atypical deep in location, producing firm, resilient
mitoses with many multinucleated cells lesions, sometimes lobulated and often
exhibiting angulated cytoplasmic borders. suggestive of cyst.

Management Histopathological Features

It is treated by surgical excision or radiation with Histologically, it is classified into myxoid, round
5-year survival rate in 20 to 60% of cases. cell, adult and pleomorphic types. In general, it
 Malignant Tumors 221

consists of fat cells and lipoblast in varying

degrees of differentiation and anaplasia with
variable stromal component (Fig. 13.27).

Management
It is treated by surgical excision with or without
radiation therapy.

CARTILAGE

Chondrosarcoma

It is also called as 'chondrogenic sarcoma'. It Figure 13.27: Liposarcoma showing large fat cells (L)-
lipoblast cells, surrounded by collagen fibers (cl)
develop from natural cartilage or a benign (Courtesy: Dr Sangamesh Halawar)
cartilaginous tumor. Most of them develop from
may be sensory nerve deficit, proptosis and visual
cartilage located in bone either centrally in bone
disturbances. If swelling erodes through the
(medullary cavity) or peripherally, from the
cortical plate, it tends to be tender, smoothly
cartilage cup of an osteochondroma.
contoured firm mass due to presence of cartilage.
Mucosal covering appears normal in early stage
Types
but later it ulcerates and develops necrotic surface,
• Primary: They directly arise from the if chronically traumatized. If it occurs in/or near
cartilage. the temporomandibular joint region, trismus and
• Secondary: It develops in a preexisting benign abnormal joint function may result.
cartilaginous tumor.

Clinical Features
It develops during 3rd through 6th decades and
male to female ratio is 2:1. Secondary
chondrosarcoma occur at early age than the
primary type of chondrosarcoma. It is rare in jaws
and may occur in maxilla or mandible (Fig. 13.29).
Often found in anterior alveolar process of
maxilla and in mandible it is found at angle and
alveolar ridge of premolar-molar region. It is slow
growing and less malignant.
Symptoms: It is painless in early stages with facial
asymmetry as first complain but as it enlarges
swelling becomes bony hard and painful. There
may be headache (Fig. 13.28).
Signs: Teeth adjacent to the lesion are resorbed,
loosened and get exfoliated. In some cases, there
Figure 13.28: Chondrosarcoma of condyle showing
may be hemorrhage from the neck of teeth. There swelling in condylar region
222 Textbook of Oral Pathology

Figure 13.29: Chondrosarcoma of palate showing

smooth contoured mass

Metastatic spread by vascular channel.

Figure 13.30: Chondrosarcoma showing variable form of
Malignant cells may erode through wall or venules
cartilage cells-normal appearing cartilage cells (nc), clear
and extend along inside the venules without cells of cartilage (cl), binucleate cells in other area (bc),
adhering to vessels wall but still altered at their site normal hyaline cartilage (hy)
of entry. Lung is common region of metastasis
Clinical Features
Histopathological Features
It is most commonly seen in maxilla, skull bone,
Histological appearance of chondrosarcoma is mandible and ribs. It also occurs in tubular bones
more or less same as that of chondroma. It is
and pelvis. It is seen in younger age group than
composed of hyaline cartilage. Cells are of
usual type of chondrosarcoma. It is usually seen
variable size and binucleated cells are present
between the ages of 10-30 years.
(Fig. 13.30).
The signs of malignancy in tumor are Sign and symptoms: There may be pain due to
cellularity with an increased number of cells with compression of nerve. In some cases, there may
plump nuclei. The clear cell chondrosarcoma be swelling. Pathological fracture may also occur.
consist of single or clustered benign cells and
tumor cells with clear cytoplasm. Histopathological Features

Management The mesenchymal chondrosarcoma consist of

Surgery is the only treatment of choice and there sheets of small, round or ovoid, undifferentiated
is 5 years survival rate. cells interspersed by small islands of well
differentiated cartilage which often show
Mesenchymal Chondrosarcoma calcifications and metaplastic bone formation
(Fig. 13.31).
It contains mesenchymal cells. It tends to
metastasize to unusual locations and that too after
Management
long period of time. Most of the tumors arise in
bone but an appreciable number occur in soft The prognosis is poor and most patients die due
tissues. to metastasis.
 Malignant Tumors 223

Histological
• Fibroblastic
• Osteoblastic
• Chondroblastic
• Telangiectatic.

Etiology
There are increased incidences of osteosarcoma
in bone that has been irradiated. Traumatic
irritation may be causative factor for osteosarcoma.
Osteogenic sarcoma may be seen in fibrous
dysplasia and Paget’s disease. Other causes
which can cause osteosarcoma are genetic
mutation and some viral causes.
Figure 13.31: Chondrosarcoma showing binucleated cells

Clinical Features
BONE Mean age of occurrence in the jaws is 33 years
Osteosarcoma and it is more common in males. It is more
common in long bones like femur and tibia and
It is also called as 'osteogenic sarcoma'. It is the in jaw bone. It grows rapidly with a doubling time
most common malignant tumor of bone. It is of 32 days and shows recurrence and early
derived from osteoblasts in which tumor cells metastasis via blood stream to lungs.
contain high level of alkaline phosphatase.
Signs and symptoms: There is exophthalmos,
Classification blindness, nasal obstruction and epistaxis.

Location Oral Manifestations

• Parosteal (Juxtacortical) osteosarcoma: It is It is rare and accounts for 7% of all osteosarco-
uncommon, extremely rare in the jaws and is mas. It is equal in maxilla and mandible. In the
characterized by slow growth. It has good mandible, lesion is seen in body and in maxilla it
prognosis due to low tendency of tumor for occurs in antrum or alveolar ridge.
metastasis. The tumor grows from external
Symptoms: Swelling of a short history and is
surface of the bone. It is more common in
accompanied by pain. Affected tooth may become
femur; as well as in the jaw.
displaced or loose. Numbness of lip and chin
• Periosteal osteosarcoma: It is an aggressive
due to involvement of inferior alveolar nerve.
variant of parosteal type of osteosarcoma but
There may be trismus and hemorrhage.
with better prognosis than conventional
intramedullary osteosarcoma. Signs: There may be history of tooth extraction
• Extra-osseous: Osteosarcoma of soft tissue in with nodular or polypoid reddish granulomatous
absence of primary skeletal tumor and occur appearing mass growing from tooth sockets. Ex-
in breast, liver and kidney. It is highly pansion is very firm due to dense fibrous tissue
malignant type of tumor. which is produced. Initially, swelling is smoothly
224 Textbook of Oral Pathology

contoured and covered by normal mucosa. When

expansion becomes chronically traumatized,
mucositis develops on surface. Later, the surface
gets ulcerated and looks like whitish grey in color.

Histopathological Features
Characterized by proliferation of both, atypical
osteoblasts and their less differentiated
precursors. Neoplastic osteoblasts are spindle
shaped or polyhedral and are generally larger
than normal osteoblasts (Fig. 13.32).
Pleomorphism in size and shape of cells which
show large, deep staining nuclei and are arranged
in disorderly fashion about the trabeculae.
Osteoid and bone formation in irregular pattern
and sometimes, in solid sheets, rather than in Figure 13.32: Osteosarcoma showing abnormal bone or
osteoid (abB), large osteoblasts cells (lo),blood
trabeculae. (Fig. 13.33) Vascular channels can be elements (bl), Cartilage (Ca)
present in the tumor and may be prominent and
this is called as telangiectatic type of tumor
the long bone of extremities although the skull,
Laboratory Investigation clavicle, ribs, shoulder and pelvic girdles are
involved. 10 to 15% occur in jaw, usually in
Serum alkaline phosphatase level is increase in
mandible. It is a very rapidly growing highly
osteosarcoma.
invasive tumor with early and widespread
Ewing's Sarcoma metastasis.

It was first described in 1921 by James Ewing. It is Symptoms: Initially, intermittent pain which later
also called as 'round cell sarcoma' or 'endothelial becomes continuous and is associated with rapid
myeloma'. It is derived from mesenchymal
connective tissue of bone marrow.

Origin
It may arise from endothelial elements in the
marrow. It may be a secondary deposit of
neuroblastoma. It may arise from reticulum cell
lining of the marrow spaces. Some consider it to
be a metastatic tumor, the primary of which is
located in different sites, including bronchus.

Clinical Features
It occurs between the ages of 5 to 25 years with a
male to female ratio of 2:1. The bones affected are Figure 13.33: Osteosarcoma showing bone formation
 Malignant Tumors 225

very little stroma, although few connective tissue

septa can be seen (13.34).
The cells are small and round with little
cytoplasm and relatively round or ovoid nuclei.
Occasionally, the cells are arranged around
blood vessels, giving rise to rosette formation
(Fig. 13.35).

Management
Surgery, X-ray radiation can be used but survival
period is very less. Prognosis is poor, death occurs
within a year of diagnosis.

VASCULAR

Malignant Hemangioendothelioma
It is a neoplasm of mesenchymal origin which
is angiomatous in origin and derived from the
Figure 13.34: Ewing’s sarcoma showing round cells (ro) endothelial cells.
arranged in sheets separated by connective tissue septae (st)
Clinical Features
growth of the tumor and enlargement of bone.
During the attacks of pain the tumor enlarges visibly. It has slight predilection for females. It can arise at
It is associated with febrile attacks and leukocytosis. any age and has been found at birth also. It may
It usually metastasizes to other bones. occur anywhere in the body but most commonly
found in skin and subcutaneous tissues.
Signs: The swelling is hard but occasionally it In oral cavity, it can occur on lips, palate,
may be soft and fluctuant. In the early lesion, gingiva, tongue and centrally within the maxilla
when the tumor is intraosseous, swelling is firm. and mandible.
When the tumor breaks through the cortex, it
spreads extensively in the soft tissues and form a
soft mass which may ulcerate. Swelling is warm
and tender. There may be hyperemia of the
overlying tissues, suggesting inflammatory
condition. The patient may have low grade fever,
facial neuralgias, lip paresthesia. Teeth may
become mobile and paresthesia may develop.
There may be trismus, epistaxis, exophthalmos
and sinusitis.

Histopathological Features
It is a cellular neoplasm which is composed of Figure 13.35: Ewing sarcoma
solid sheets or masses of small round cells with (Courtesy: Dr Sangamesh Halawar)
226 Textbook of Oral Pathology

Symptoms: Localized swelling with pain may be pericytes. There is profuse proliferation of occult
the feature of lesion. capillaries.
Each vessel in turn is surrounded by a
Signs: It appears as flat or slightly raised lesion of
connective tissue sheath, outside of which are found
varying size, dark red or bluish red, sometimes
masses of tumor cells. The tumor cells may appear
ulcerated and show a tendency to bleed even after
large or small, round or spindle shaped and show
slight trauma. Bone may be involved by tumor
tendency for concentric layering about the
producing a destructive process.
capillaries. Cellular pleomorphism can also occur.
Histopathological Features
Management
It composed of endothelial cells often arranged in
columns. Capillary formation is poorly defined, It is treated by surgical removal of the lesion.
although anastomosing vascular channels may
be discerned. The individual cells are pleomorphic NEURAL TISSUE
large polyhedral or slightly flattened, with a faint
Neurosarcoma
outline and a round nucleus with multiple minute
nucleoli. It is also called as 'neurogenic sarcoma'. They arise
from Schwann cells or perineuronal fibroblasts.
Management
Clinical Features
Surgery and X-ray radiation can be given.
It can arise at any age with no sex predilection.
Malignant Hemangiopericytoma Neck, parapharyngeal space, oral cavity and
larynx are involved. Most of these neoplasms arise
It is characterized by the proliferation of
as solitary lesion.
capillaries surrounded by masses of round or
spindle shaped cells which are called as pericytes. Histopathological Features
It resembles to Glomus tumor but lack its organoid
pattern, encapsulation and clinical manifestation They are same as of fibrosarcoma.
of pain.
Management
Clinical Features Wide excision is done. The affected nerve should
be resected over 5 cm from its encasement by the
There is no sex predilection with age ranging from
tumor.
birth to old age. It can occur at any site in the oral
cavity.
Neuroblastoma
Signs and symptoms: It is usually painless. The
It is a rare malignant tumor of the adrenal gland.
lesions are firm, apparently circumscribed and It sometimes arises in the nerves or ganglia of the
often nodular. It may or may not exhibit redness
sympathetic system in the neck, thorax or
indicative of vascular nature. Majority of tumors
abdomen. After leukemia it is the commonest
grow rapidly and are therefore of short duration. malignancy of children.

Histopathological Features Clinical Features

The tumor reproduces the normal arrangement It usually occurs in children under the age of
of capillary endothelial channels surrounded by 5 years. Oral cavity and nasal sinuses are
 Malignant Tumors 227

involved. Oral involvement is usually due to cellular pleomorphism to tumors that are highly
metastasis and it occurs in mandible and maxilla. cellular with pleomorphism and bizarre mitotic
activity.
Signs and symptoms: There is swelling, displacement
of teeth and neurological symptoms. Skull is one of
Management
the commonest sites for metastases. Metastases in
bone tend to be multiple. Surgery and radiation can be given and it has got
marked tendency for local recurrence.
Histopathological Features
The tumor varies very much in its degree of MUSCLE
differentiation. The least well differentiated tumor
consists of small round cells arranged merely in Leiomyosarcoma
diffuse masses. It is a malignant tumor of smooth muscle origin.
In some cases, differentiating cells begin to
form groupings; generally referred to as rosettes Clinical Features
and the cell themselves develop neurofibrillary
processes. These processes become young nerve It can occur at any age with no sex predilection. It
fibers and form tangled masses in the centers of is very rare in oral cavity and if present, it is seen
rosettes. commonly on cheeks and floor of mouth. The
lesion appears as painful swelling. They tend to
Neurofibrosarcoma metastasize through hematogenous route.

It is also called as 'malignant Schwannoma'. It Histopathological Features

arises from nerve tissue, especially from nerve
sheath cells. It is rare in oral cavity. Patients There is presence of mitosis, nuclear
suffering from neurofibromatosis can undergo pleomorphism, hyper-chromatism and bizarre
sarcomatous transformation in some cases, cell forms. Proliferating smooth muscles cells are
usually in deep seated lesions. arranged as interlacing bands or cords.

Clinical Features Rhabdomyosarcoma

It usually occurs in 3rd and 6th decade with no It is a malignant neoplasm of skeletal muscle
sex predilection. It occurs on lips, gingiva, palate origin and it is uncommon in oral cavity.
and buccal mucosa. In central tumor mandible is
more affected than maxilla. Types (histological)
Symptom: Only complain is the presence of mass • Pleomorphic: It occurs most common in
and in some cases, pain or paresthesia may be extremities and in older individuals.
present. • Alveolar: It is found in head and neck region and
in extremities, with early age of occurrence.
Histopathological Features • Embryonal: It is found in the genitourinary tract
It consist of plumps spindle shaped cells arranged and in nasopharynx, with cases reported in
in streams and cords with random nuclei. It oral cavity in the upper and lower labial folds.
exhibits variation in degree of malignancy, i.e. • Botryoid: It is a malignant tumor of vagina,
tumors that are relatively acellular and show little prostate and base of bladder in young children.
228 Textbook of Oral Pathology

Clinical Features dark staining nuclei. Cells floating in the alveolar

spaces are common.
It is most common soft tissue sarcoma in children
and adolescents. It is a mass occurring in any Embryonal rhabdomyosarcoma: Here four types of
region of the head and neck where striated muscle cells are present (i) eosinophilic spindle cells,
or its mesenchymal progenitor cells exist. usually arranged in interlacing fascicles.
Intraorally, the tonsils and soft palate are most (ii) Round eosinophilic cells, large and
frequently involved. Typically, it is a rapidly grow- intermediate in size, with small nucleus and
ing soft tissue mass. It forms polypoid fleshy interspersed among other cell types. (iii) Broad
growth beneath the mucous membrane, with club elongated eosinophilic cells occasionally with
like extensions at periphery. cross striations. (iv) Small, round and spindle cells
It may spread by either lymphatic or hematog- with dark staining nuclei and little cytoplasm.
enous routes.
Management
Signs and symptoms: Depending upon of the size
It is treated by chemotherapeutic agents and
of lesion, there may be divergence of eyes,
radiation. It has got poor prognosis.
abnormal phonation, dysphagia, cough, aural
discharge or deviation of the jaw. The overlying Malignant Granular Cell Myoblastoma
skin is usually erythematous or telangiectatic. The It is also called as 'alveolar soft-part sarcoma'. It
lesions are occasionally ulcerated and may invade is thought to be of striated muscle origin.
the underlying bone and develop distant
metastasis. Clinical Features
It is predominantly a tumor of females, occuring
Histopathological Features
usually in teens or early twenties. Occasional
Pleomorphic Rhabdomyosarcoma: It is composed cases also occur in older patients. The lesion
chiefly of spindle cells in haphazard arrangement. occurs with greatest predilection in muscles of
These cells are generally large and show extremities. They are usually slow growing, well
considerable variation in appearance. The nuclei circumscribed masses.
are ovoid or elongated with packed chromatin.
The nuclei are situated often in an expanded end Histopathological Features
of cells. The 'racquet cell'. 'Strap-like' and 'ribbon' The tumor is composed of large cells with finely
cells typically show process of long streaming granular cytoplasm that is not as eosinophilic as
cytoplasm. The cytoplasm is eosinophilic and the cells of rhabdomyosarcoma. These cells have
intracytoplasmic longitudinal fibrils as well as a uniform pseudo-alveolar or organoid pattern
transverse cross striations may be seen. arranged in relation to numerous delicates
Cytoplasmic vacuoles are present as a result of endothelial lined vascular channels and septa.
large amount of glycogen in the cells. The pattern is reminiscent of that seen in the non-
Multinucleated giant cells are often seen. chromaffin paraganglioma.
Alveolar Rhabdomyosarcoma: It is characterized by
Management
spaces lined by epithelium like cells which appear
to be 'dropping off' from collagen. The cells are Due to the high recurrence rate, radical surgical
often small, monomorphic, round or ovoid with excision is the treatment of choice.
 Malignant Tumors 229

LYMPHOID TISSUE Symptoms: The involved nodes are painless.

Generalized weakness, loss of weight, cough,
Malignant Lymphoma dyspnea and anorexia are seen. Pain in back and
They are group of lymphoproliferative disorders abdomen owing to splenic enlargement, due to
arising from lymph nodes and from lymphoid pressure of enlarged nodes or involvement of
components of various organs. vertebrae.
Signs: The lymph nodes are discrete and rubbery
Definition in consistency with overlying skin being freely
It is defined as a neoplastic proliferative process mobile. Splenomegaly is usually seen in later
of the lymhopoietic portion of reticuloendothe- stage. Some patients may manifest pruritis.
lial system that involves the cells of either the Characteristic features of this disease are Pel-
lymphocytic or histiocytic series, in varying Ebstein fever, a cyclic spiking of high fever and
degrees of differentiation and occurs in an generalized severe pruritis of unknown etiology.
essentially homogenous population of a single Pressure of enlarged lymph nodes on adjacent
cell type. structures may cause dyspnea, dysphagia,
venous obstruction, jaundice and paraplegia.
Types
Clinical Stages (ANN Arbor Staging)
• Hodgkin's lymphoma
• Non-Hodgkin's lymphoma • Stage I: Involvement of single lymph node
region or extra-lymphatic sites.
Hodgkin's Lymphoma • Stage II: Involvement of two or more lymph
It was first described by British pathologist node regions or an extra-lymphatic site and
Thomas Hodgkin in 1832. It is characterized by lymph node region on the same side of
painless enlargement of lymphoid tissue diaphragm.
throughout the body. • Stage III: Involvement of lymph node region on
the both sides or without extra-lymphatic
Etiology involvement or involvement of spleen or both.
Particularly herpes and oncorna virus are being • Stage IV: Diffuse involvement of one of more
investigated as possible etiological agents. extra-lymphatic tissues, e.g. liver or bone
Sometimes, it can occur without any etiological marrow.
factor. • Such stages are subdivided into A and B
categories depending on whether they have
Clinical Features systemic symptoms such as weight loss, fever,
It is characterized by a bimodal age incidence, night sweats.
peak one in young adults and the second in the
Oral Manifestations
5th decade of life with equal distribution between
sexes. The onset is insidious, usually with Primary jaw lesions are uncommon. Secondary
enlargement of one group of superficial nodes. effect can be seen in oral cavity in the form of
The cervical lymph nodes are usually the first to infection due to reduced host immune response.
be involved but the disease may start in the It may appear in the oral cavity as an ulcer or a
mediastinal, axillary, abdominal, pelvic or swelling or as an intrabony lesion which presents
inguinal lymph nodes. as a hard swelling.
230 Textbook of Oral Pathology

Histopathological Features also given after chemotherapy, to sites where

It is characterized by replacement of normal lymph there was originally bulk disease.
node architecture by an admixture of malignant • Chemotherapy: It is given in stage III and IV.
lymphoid cells and non-neoplastic inflammatory Usually combination is given. First
cells. Reed-Sternberg cells—there are sheets of combination is MOPP, i.e. mustine HCl (6 mg/
lymphoid cells with interposed vacuolated spaces meter2 IV on day 1 and day 8), oncovin which
containing characteristic binucleated is also called as vincristine (1.4mg/meter2 IV
mononuclear cells. Multinucleated giant cells are on day 1 and 8), procarbazine (100 mg/
also present. meter2) orally from day 1 to 14) and prednisone
(40 mg/meter2 orally from day 1 to 14). MOPP
Histological Types combination given in six courses with no drugs
• Lymphocyte predominant: Abundant lympho- is given from day 15 to 28. Second combination
cytes, few plasma cells, occasional Reed- is ABVD regimen i.e. adriamycin (25 mg/
Sternberg cell, localized involvement on one meter2 IV bolus on day 1, 8 and 14), bleomycin
side of diaphragm and most favorable (10 mg/meter 12 bolus on day 1,14)
prognosis. vinblastine (6 mg/meter2 IV bolus on day
• Mixed cellularity: Lymphocytes, plasma cells, 1,14) and decarbazine (375 mg/meter2 IV
eosinophils, easily identified Reed-Sternberg bolus on day 1,14). The cycle should be
cell. repeated on 20th day.
• Nodular sclerosis: Sparse lymphocytes, stromal • Combination: A combination of radiotherapy
cell, fibrosis and numerous but bizarre Reed- and chemotherapy may increase the overall
Sternberg cell, poor prognosis. response and long term survival but, it is
• Lymphocyte depletion: Lymphocytes, plasma associated with delayed complications like
cells, eosinophils with localized involvement. leukemia, gonadal atrophy and avascular
necrosis of bone.
Laboratory Investigations • Splenectomy: Splenectomy is advocated in
• Full blood count: Anemia which is normocytic many patients, except with stage IV disease.
and normochromic is a common finding. The
total WBC count is normal but there may be Non-Hodgkin's Lymphoma
mild eosinophilia. In the terminal stage there, It is also called as 'lymphosarcoma'. In this group,
may be leucopenia and thrombocytopenia. there is neoplastic proliferation of lymphoid cells,
• ESR: It is raised. usually affecting the B-lymphocytes. Unlike
• Liver function: This may be abnormal due to Hodgkin's lymphoma, the disease is frequently
infiltration in liver. widespread at the time of diagnosis, often
• LDH: Raised level is an adverse prognostic involving not only the lymph nodes but also bone
factor. marrow, spleen and other tissue. Early
• Chest radiography: It is done to permit staging. involvement of bone marrow is typical of this
lymphoma.
Management
Types
• Radiotherapy: Irradiation treatment 3500-4000
rads/week over the involved region plus all • Nodular
adjacent sites been given in stage I and II. It is • Diffuse.
 Malignant Tumors 231

Etiology in the region of 2nd and 3rd division of 5th cranial

The etiology is unclear but herpes viral etiology nerve. In rare cases necrotic proliferation of palate
has been suggested. There may be induced may also be seen. The swelling may grow rapidly
immunologic effect permitting a malignant clone and ulcerate to produce fungating foul smelling
mass.
to proliferate.
Histopathological Features
Clinical Features
Nodular: In the nodular pattern, the neoplastic
It affects persons of all ages from infants to the
cells tend to aggregate in such a way that large
elderly, but is commonest in middle age group.
clusters of cells are seen.
Males are affected more commonly than the
females. In the oral cavity it frequently occurs in Diffuse: Diffuse pattern is characterized by a
tonsils. The other sites affected are salivary glands monotonous distribution of cells with no evidence
or jaws. of nodularity or germinal center pattern (Fig. 13.36).
Symptoms: The onset of symptoms may be
Laboratory Investigations
insidious. Painless lymph node enlargement of
abdominal and mediastinal region is the most Blood count usually shows normal levels but if
common finding. Very often the first group of there is associated hypersplenism or hemolytic
lymph nodes affected may be cervical, axillary or anemia the reduced WBC and RBC counts are
inguinal. The patient complains of tiredness, loss seen along with reduced hemoglobin levels and
of weight, fever and sweating. Pain is the main reticulocytosis. In some cases there may be slight
symptom of bone involvement which may present increase in lymphocytes and thrombocytopenia.
as a pathological fracture. Patient may complain Moderate degree of anemia will also present when
abdominal pain, nausea, vomiting, diarrhea or there is considerable bone marrow involvement.
intestinal obstruction which may occur due to Some very aggressive high grade non-Hodgkin
involvement of gastrointestinal tract. lymphomas are associated with very high urate
levels which can precipitate renal failure.
Signs: Pressure effect of lymphoma may cause
dysphagia, breathlessness, vomiting, intestinal
obstruction or ascites and paraplegia. If liver and
spleen are involved hepatosplenomegaly is present.
The growth is fleshy and is prone to ulceration.

Oral Manifestations
Occurrence of malignant lymphoma in oral cavity
is rare, when present it is more often found to
arise from the tonsils, although other oral tissue
may also be involved.
Palatal lesions have been described as slow
growing, painless, bluish soft tissues mass which
may be confused with minor salivary gland
Figure 13.36: Diffuse distribution of monotonous abnormal
tumors. Paresthesia of mental nerve has been lymphocytes invading the connective tissue in Non Hodgkin’s
reported. Sometimes there is pain and neuralgia Lymphoma (Courtesy: Dr Sangamesh Halawar)
232 Textbook of Oral Pathology

Management symptoms of nasal obstruction due to superior

No treatment is necessary, if disease is not growth of tumor into the floor of nasal cavity.
advanced. Histopathological Features
Chemotherapy: When diagnosed in late stages, The primary cells of tumor are identical with that
chemotherapy is the treatment of choice. In most of the soft tissue tumors. There may be
cases single agent chemotherapy (chlorambucil) inflammatory cell infiltration which can lead to
is usually given. Combination with prednisolone confusion in the diagnosis of the lesion. The
is also useful. A combination of cyclophospha- individual cells are mixed with both mature
mide, doxorubicin, vincristine, bleomycin with appearing lymphocytes and large histiocytoid
prednisolone is commonly used. lymphoblast.
Radiotherapy: Radiotherapy is used to treat local Management
disease and the patient is given a total dose of
Surgical excision or X-ray radiation, as it appears
150 rads spread over a period of five weeks.
to be radiosensitive. The 5 year survival rate is
Transplantation: Studies of autologous stem cell between 50 to 60%.
transplantation are in progress.
Mycosis Fungoides
Primary Reticular Cell Sarcoma
It is a T cell lymphoma affecting the skin. The
It is also called as 'primary lymphoma of bone'.
disease commences with eczematous lesion,
Clinical Features which gradually develop into thickened plaques.
It varies in size from few millimeters to centimeters
It can occur in any age group but it is common
in diameter and finally spread to lymph nodes,
amongst young adults under the age of 40 years.
spleen and liver.
It is common in male as compared to females. It
Oral lesions can be the first manifestations
most commonly seen in femur, tibia and humerus.
and some times appear after the skin lesions have
Signs and symptoms: It is usually asymptomatic been treated and remitted. It is occur on tongue,
except for the presence of localized swelling of palate, buccal mucosa, lip, gingiva and tonsil. The
the involved bone. Regional lymphadenopathy lesions appear as indurated areas, nodules or
is usually present. erythematous ulceration.
Histopathologically, there is a dense infiltration
Oral Manifestations of lymphoid cells with irregularly shaped nuclei.
It is not common, but it usually occurs more in In some cases, nuclei are so convoluted that they
mandible than maxilla. Pain is common complaint are described as cerebriform.
and usually present for years and more. There is
also presence of expansile mass. The oral mucosa Burkitt's Lymphoma
over the involved bone seldom gets ulcerated but It was described by Dennis Burkitt in 1950. It is
at times, appears diffusely inflamed. The teeth also called as 'African jaw lymphoma'. It is a
usually become exceedingly loose, owing to lymphoreticular cell malignancy. In the African
destruction of bone. form jaw involvement is 75% and in cases of the
When the lesion involves maxilla, there may American form, abdomen involvement is more
be evidence of expansion of the bone as well as common. It is a B cell neoplasm.
 Malignant Tumors 233

Etiology There may be large quantity of mass protruding

into the mouth, on the surface of which may be
EBV virus which also causes nasopharyngeal
seen rootless, developing permanent teeth. Once
carcinoma and infectious mononucleosis is
the tumor perforate the bone it is initially confined
considered to be the etiological factor. There are
by the periosteum, but subsequently it spreads to
higher EBV antibody levels patients of Burkitt's
the soft tissues of the oral cavity and face where
lymphoma patients.
rapid tumor growth soon obliterates the entire face
Clinical Features and skin becomes tense and shiny.
Peak incidences in children between 6 to 9 years. Histopathological Features
Males are affected more commonly than the
females, with a ratio of 2:1. It is more are found in Monotonous sea of undifferentiated
monomorphic lymphoreticular cells, usually
maxilla than in mandible, where it may spread
showing abundant mitotic activity is seen. There
rapidly to the floor of the orbit. Almost always
occurs in molar area. is hyperchromatosis and loss of cohesiveness.
Macrophages with abundant clear cytoplasm
In the African form more than one quadrant is
often containing cellular debris scattered
involved while in the American form, only one
quadrant is involved. The most important throughout the tumor producing a very
characteristic 'starry sky' appearance (Fig. 13.37).
hallmark of this tumor is readily of growth with a
Nuclei are uniform in size, mostly round but
tumor doubling time of less than 24 hours.
occasionally may be ovoid with a slight
Signs and symptoms: The most common presenting indentation. The nuclear membrane is prominent.
features are swelling of the jaws, abdomen and
paraplegia. It is painless. Peripheral
lymphadenopathy is common.

Oral Manifestations
It begins generally as a rapidly growing tumor
mass of the jaws, destroying the bone with
extension to involve maxillary, ethmoid and
sphenoid sinus as well as orbit.
Loosening or mobility of permanent teeth. There is
gross distortion of the face due to swelling.
Paresthesia and anesthesia of inferior alveolar
canal or other sensory facial nerves is common.
Gingiva and mucosa adjacent to the affected
teeth become swollen, ulcerated and necrotic. As
the tumor mass increases, the teeth are pushed
out of their sockets. Swelling of the jaw occurs
and it may cause facial asymmetry.
They are capable of blocking nasal passages, Figure 13.37: “Starry sky” appearance—Burkitt’s lymphoma
showing uniform distribution of lymphocytes (lym) and
displacing orbital contents and eroding through
macrophages with clear cytoplasm (MC STR) interspersed
skin. There is derangement of arch and occlusion. between giving the characteristic appearance
234 Textbook of Oral Pathology

Management In this, absolute neutrophil count remains

It is rapidly fatal in the absence of treatment, with above 30,000/mm3.
death occurring within 6 months. Cytotoxic drugs
like cyclophosphamide 40 mg/kg in single IV Classification
administration and repeated about 2 weeks later. Acute
Vincristine and methotrexate have been
• Acute lymphoblastic leukemia
successful in some cases. Combination of drugs
– L1 - Acute lymphoblastic (principally
such as cyclophosphamide, vincristine and
pediatric): In it, small cells predominate and
methotrexate give better results than any single
nuclei are generally round.
drug. Majority of patients show dramatic response
– L2 - Acute lymphoblastic (principally adults):
to the therapy. The swelling regresses and the
Cells are heterogeneous in size and sharp
displaced teeth return to their normal position
in features, nuclei often show cleft.
within 1 to 2 weeks.
– L3 - Burkitt's: There is homogenous
LEUKEMIA population of large cells. Nuclei are round
to oval with prominent nucleoli.
It is also called as 'leucosis'. It is defined as a • Acute nonlymphoblastic or myeloid leukemia
neoplastic proliferation of WBC in bone marrow, – M -Myeloblastic (without maturation):
usually in circulating blood and sometimes in Myoblasts predominate with distant
other organs such as liver, spleen and lymph nucleoli, few granules are present.
nodes. Presence of leukemic cells in bone marrow – M2-Myeloblastic (with maturation): Myelo-
results in impairment of normal hemopoiesis with blast and promyelocytes predominate and
resultant anemia, granulocytopenia and Auer rods are seen.
thrombocytopenia. Leukemia is a progressive and – M3-Promyelocytic: Hypergranular
fatal condition causing death from hemorrhage promyelocytes often with Auer rods are
and infection. There is presence of excessive seen.
number of abnormal cells in the peripheral blood – M4-Myelomonocytic: Myelocytic and
but leukemia is considered a primary disorder of
monocytic differentiation evident, myeloid
bone marrow.
elements resemble peripheral monocytosis.
Types – M5-Monocytic: Promonocytes or undiffer-
entiated blast.
• Stem' or 'blast' cell leukemia: When the leukemic
– M6-Erythroleukemia: Bizarre, multinucle-
cells are too immature to be classified as to cell
ated, megaloblastoid erythroblast predomi-
type, the leukemia is termed as 'stem' or 'blast'
nate.
cell leukemia.
– M7-Megakaryocytic: Pleomorphic undiffer-
• Subleukemia: When the total WBC is normal
entiated blast cells with antiplatelet anti-
and leukemic cells are seen in the peripheral
bodies, myelofibrosis is present.
blood is termed as subleukemia.
• Aluekemia: When no abnormal leukocytes can
Chronic
be found in the peripheral blood (i.e. they can
be found only in the bone marrow) the term • Chronic lymphatic leukemia (lymphogenous,
aleukemia is used. lymphocytic) involving lymphocytes series.
• Luekemoid reaction: When the peripheral blood • Chronic myeloid leukemia (myelogenous,
picture in non-leukemic patient resembles that myelocytic) leukemia involving granulocyte
of leukemia it is called a leukemoid reaction. series.
 Malignant Tumors 235

Etiology rather than shortened generation time. Thus

accumulation of leukemic blast in acute leukemia
Virus: Epstein-Barr virus, herpes like virus and
results primarily from failure of maturation into
HTLV (human T cell leukemic virus) have been
functional stage. As leukemic blast accumulates
considered to be the etiological agents responsible
in the marrow, they suppress the normal
for leukemia.
hematopoietic stem cells. The mechanism is not
Radiation and atomic energy: If given over the dose fully understood. Suppression part is related to
of 100 rads, it is known to significantly increase physical replacement of normal precursor cells
the risk of leukemia. Leukemia among by expanded clones of leukemic cells. Clinical
radiologists and Japanese exposed to the atomic manifestations result from paucity of normal red
blast are more, as compared to other population. cells white cells and platelets.
It is also common in patients receiving X-radiation
for rheumatoid spondylitis. Clinical Features
Chemical agents: Chronic exposure to aniline dyes; It is more common in children and young adults
benzene and phenylbutazone have been between the age of 15 and 39 years. Males are
recognized to be associated with leukemia. affected more commonly than females with a ratio
Usually in these patients pancytopenia due to of 3:2. There is abrupt stormy onset with pyrexia
marrow hyperplasia occurs prior to leukemia. and enlargement of spleen.
Anticancer drugs: Patient treated with anticancer Symptoms: Usually result from bone marrow
drug like melphalan and chlorambucil have an suppression and infiltration of other organs and
increased risk of developing leukemia, usually of tissues by leukemic cells. Weakness, fever,
acute myelocytic variety. headache, generalized swelling of lymph nodes,
Genetic and chromosomal factors: Philadelphia petechiae or hemorrhage in skin and mucus
chromosome is found in about 15% of cases of acute membrane are seen. Bone pain and tenderness,
lymphocytic leukemia. It suggests that if one set of resulting from marrow expansion, with
identical twins develop leukemia before the age of infiltration of subperiosteum. Central nervous
6 years, the risk of disease in other twins is 20%. manifestations such as headache, vomiting, nerve
palsies resulting form meningeal spread which
Immunological deficiency syndrome: The persons
more common in children than in adults; and
suffering with Wiskott-Aldrich syndrome can
more common in ALL than AML.
develop leukemia.
Signs: The clinical features are due to anemia and
Acute Leukemia thrombocytopenia viz pallor, dyspnea, fatigue,
Acute leukemia is a disorder in which there is a petechiae, ecchymosis, epistaxis and melena.
failure of maturation of leukocytes. As a result Hepatosplenomegaly is present in later stages.
there is an accumulation of immature cells within There is an increased susceptibility to infection.
the bone marrow and later in the blood. It is the Cervical lymphadenopathy, secondary to
most common type of leukemia, except in children pharyngeal sepsis is seen. Intracranial and
(in whom acute lymphoblastic leukemia is more subarachnoid hemorrhage may result from
common). thrombocytopenia and leukastasis (intravascular
clumping of leukemic blasts in the small blood
Pathophysiology vessels of brain). There is recurrent infection of
There is a block in differentiation of leukemic and lungs, urinary tract skin, mouth, rectum and upper
stem cells and leukemic blasts have prolonged, respiratory tract, which may result in fever.
236 Textbook of Oral Pathology

Chloroma: Localized tumors consisting of leukemic Management

cells are called chloromas, surface of which turn
Phase of induction: It is treated using combination
green when exposed to light because of the
of vincristine (1.4 mg/m2 every week of 1 month),
presence of myeloperoxidase. L-asparaginase (600 units/m 2 biweekly for
Oral Manifestations 1 month) and prednisone (40 mg/m2 orally daily
for 1 month). As the leukemic cells regress,
The submental, cervical and pre-and regrowth of normal cells occurs and the patient
postauricular lymph nodes may be enlarged and goes rapidly into remission.
tender. Paresthesia of lower lip and chin may be
present. There may be toothache due to leukemic Phase of consolidation: In it, drugs uses include
cell infiltration of dental pulp. daunorubicin, mercaptopurine, cytarabine and
The oral mucous membrane shows pallor, methotrexate with intrathecal therapy using the
ulceration with necrosis, petechiae, ecchymosis last two drugs, together with irradiation of the
and bleeding tendency. There may be massive cranium to eradicate, the disease from central
necrosis of lingual mucosa with sloughing. There
may be crusting of lip occur due to repeated
bleeding from lip (Figs 13.38 and 13.39).
Gingiva shows hypertrophy and cyanotic
discoloration. The hypertrophy may be due to
leukemic cell infiltration within gingiva or due to
local irritants. The gingiva appears boggy,
edematous and deep red bleed easily due to
ulceration of sulcus epithelium and necrosis of
underlying tissue. Mobility of permanent teeth
may be present. Oral infections (candidal, viral
and bacterial) are serious and potentially fatal
Figure 13.38: Bleeding occur from lip in patient of
complication in leukemic patients leukemia

Hematological Findings
The total WBC count may vary from a very low
count less than 1 × 106 per cu mm to as high as
5000 × 106 per cu mm or more. The peripheral
smear shows significant number of immature
granulocytes or lymphatic precursors or even
stem cells. Bone marrow is hypercellular with
replacement of normal marrow elements by
leukemic blast cells in varying degree. There is an
associated normochromic anemia,
thrombocytopenia and decrease in normally
functioning neutrophils. Figure 13.39: Crusting of lip occur in patient of leukemia
 Malignant Tumors 237

nervous system. Radiation reduces the risk of Chronic Leukemia

relapse in central nervous system when given with
Chronic leukemias are characterized by the
methotrexate.
presence of large leukemic cells and
Phase of maintenance: In this phase, patient receives differentiated WBCs in the bone marrow,
a repeating cycle of above drugs until two or three peripheral blood and other tissues. It has a
years have been completed. prolonged clinical course even without therapy.
Transplantation: Allogenic bone marrow
transplantation from HLA identical twin is done. Chronic Myeloid Leukemia
Ablative therapy has been utilized to eradicate Pathophysiology
the patient's residual leukemic cells and normal
cells with intensive irradiation and It is associated with the presence of a distinctive
chemotherapy. After this reconstruction of the chromosomal abnormality, i.e. Philadelphia
hemopoietic tissue is done with precursor cells chromosome.
from the donor.
Acute nonlymphoblastic leukemia is treated Clinical Features
with daunorubicin, cytarabine and 6- The disease occurs chiefly between the age of 35
thioguanine. All these drugs are very toxic to the to 60 years. The disease may be discovered dur-
normal as well as leukemic cells and therefore, ing routine examination, when splenomegaly or
treatment is given in pulses to reduce toxicity. an elevated count is noted.
Catabolic products of leuekmic cells produce
uric acid and cause hyperuricemia, which is Symptoms: There may be slowly advancing
prevented by allopurinol. anemia with loss of weight, prominence of
abdomen and discomfort in the left upper
Supportive therapy: Transfusion of red cells and quadrant due to splenomegaly. Attacks of acute
platelets may be required in cases of severe anemia left upper abdominal pain may develop due to
and thrombocytopenia. Combination of higher infarction of spleen. Anemia causes weakness,
antibiotics like aminoglycosides with fatigue and dyspnea on exertion.
cephalosporin, allopurinol, before starting
Signs: as the disease progress thrombocytopenia
antileukemic agents are given to prevent
can cause petechiae, ecchymosis as well as
hyperuricemia.
hemorrhage from the skin and mucus membrane.
Topical treatment to stop gingival bleeding
Liver may be enlarged but lymph nodes are
includes removing obvious local irritants and
normal.
direct pressure. Use of absorbable gelatin or
collagen sponge topical thrombin is helpful.
Hematological Finding
The management of oral ulcers includes topical
antibacterials with povidone iodine solution, Examination of blood shows a normocytic and
chlorhexidine rinses or tetracycline rinses. normochromic anemia. WBC count is
Psychological support is very important as considerably increased and may be between 50
delusion, hallucinations and paranoia are not × 106 to 500 × 106 cells per cu mm. Peripheral
uncommon during periods of severe bone marrow smear shows mature leucocytes but, few
failure. immature forms may also be present. The platelet
238 Textbook of Oral Pathology

Symptoms: Tiredness and ill health are common,

although some patients are symptom free and the
disorder is found incidentally. Bone marrow in-
filtration causes anemia and thrombocytopenia
and results in pallor, weakness, dyspnea and
purpura.
Signs: There is a moderate enlargement of lymph
nodes which are firm, rubbery and discrete. Liver
and spleen are usually enlarged and palpable.
There is an increased susceptibility to infection
Figure 13.40: Chronic myeloid leukemia showing
as the leukemic B cells are non-functional.
abundant mature monocytes (mo) in the blood smear Leukemic infiltration results in skin nodules,
intestinal malabsorption, pulmonary obstruction
count is often high initially but with treatment, or compression of the central or peripheral
it comes down.
nervous system. The most common groups of
Management lymph nodes involved are cervical, axillary and
inguinal group.
The treatment of choice is chemotherapy using
the drug busulphan given orally in a dose of 4 mg
Clinical Staging
daily or in large doses of 50-100 mg spaced 2 to 3
weeks apart. The treatment is continued for 12 to • Stage A: Lymphocytosis is less than three areas
18 weeks and should be discontinued when WBC of lymphoid enlargement, no anemia or
count is between 10 × 106 and 20 × 106 /cu-mm, thrombocytopenia.
other wise busulphan may cause aplasia of the • Stage B: More than three areas of lymphoid
bone marrow. enlargement, no anemia or thrombocytopenia.
A combination chemotherapy using a small • Stage C: Anemia or thrombocytopenia,
dose of busulphan 2 mg daily along with regardless of number of area of lymphoid
mercaptopurine 50 mg daily or thioguanine 80 enlargement.
mg daily.
Radiotherapy and splenectomy are other Oral Manifestations
treatment of choice. The most common oral finding is hypertrophy
Chronic Lymphatic Leukemia of gingiva. There may be ulceration with necrosis
and gangrenous degeneration; a dark brown
It is a slowly progressing malignancy involving
exudate and foul fetor oris are present. The
lymphocytes.
tongue is frequently swollen and dark. There is
Pathophysiology regional lymphadenopathy. Rapid loosening of
teeth due to necrosis of periodontal ligament has
It is characterized by the accumulation of long
been reported. Destruction of alveolar bone also
lived, non- functional B lymphocytes.
occurs in some cases.
Clinical Features
Hematological Findings
It occurs more frequently in males and majority of
the patients are over 45 years. The onset is very Peripheral blood smear shows mild anemia and
insidious. a large number of small lymphocytes.
 Malignant Tumors 239

Lymphoblasts are rare but increase in the terminal The characteristic cell is a large lymphocyte with
stages of disease. WBC count may increase up to prominent nucleus.
1000 × 106 per cu mm.
Aleukemic Leukemia
Management It is the subleukemic form of leukemia in which
General measure to maintain good health, the WBC count of the peripheral blood is normal
adequate rest, good food and exercise should be or even subnormal and abnormal or immature
advised. leucocytes may be present.
Chemotherapy: Chlorambucil 6-10 mg/day for 14
MYELOMA
days with break of 14 days and cyclophospha-
mide 2-3 mg/kg IV for 6 days. Multiple Myeloma
Combination therapy: Cyclophosphamide It is also called as 'myelomatosis'. It is a malignant
doxorubicin, vincristine and prednisone have neoplasm of plasma cells of the bone marrow with
been recommended. widespread involvement of the skeletal system,
Radiotherapy: It is useful for large granular masses, including the skull and jaws.
if they cause symptoms. Radiotherapy with very Origin
small doses, of only 150 rads over a period of five
weeks, is very effective and may induce It is thought to be multicentric in origin. There is
satisfactory remission. proliferation of a single clone of abnormal plasma
cells in the bone marrow. Normal plasma cells
Steroids: If the bone marrow is severely involved are derived from B cells and produce
initial treatment with prednisone 40 mg daily and immunoglobulins, which contain heavy and light
25-50 mg daily later should be given. chain. In myeloma, plasma cells produce
immunoglobulin of single heavy and light chain,
Hairy Leukemia
a monoclonal protein commonly referred as para
It a variant of chronic lymphatic leukemia in protein. In some cases, only light chains are
which there is splenomegaly, severe neutropenia, produced and these appear in urine as Bence
monocytopenia and the characteristic appearance Jones proteinuria.
of hairy cells in blood and bone marrow. These
hairy cells appear to be a cross between the Clinical Features
lymphocytes and monocytes. It occurs mainly in
The most common age group affected is between
adults and show male predilection.
40 to 70 years with male to female ratio 4:1. The
Manifestations result from infiltration of bone
skull, clavicle, vertebrae, ribs, pelvis, femur and
marrow, liver, spleen. Splenomegaly is massive
jaws are involved.
and hepatomegaly is less common. Hairy cell can
be identified on the peripheral smear. Symptoms: Skeletal pain associated with motion
or pressure over the tumor masses, is an early
Prolymphocytic Leukemia symptom. Spontaneous pathological fracture
It is another variant of chronic lymphatic leukemia with acute pain may be present. Weakness and
in which there is massive splenomegaly with little pain of back and thorax also may be presenting
lymphadenopathy and a very high WBC count. symptoms. Pain in the involved bone may be
240 Textbook of Oral Pathology

aggregated by exercise and relieved by rest. The Oral amyloidosis: It is a complication of this
patient may also complain of tiredness, bleeding disease. The tongue may be enlarged and studded
tendency and bruising of skin due to anemia and with small garnet-colored enlargements,
thrombocytopenia. The cause of bleeding is that including nodes on lip and cheeks. Tongue
the abnormal globulins bind with coagulation enlargement may cause impairment of speech,
factors which also increase the viscosity of blood. mastication and deglutition. Amyloid can also
Patient may complain of vomiting due to increase deposit in the gingivae, where it can cause
serum calcium level. soreness and inability to wear dentures.
Signs: Swelling over the areas of bone involvement
may be detectable. There is an increased Histopathological Features
susceptibility to infection due to abnormal Microscopically, it can be classified as
immunoglobulin production by the plasma cells. plasmocytic or plasmoblastic.
Hypercalcemia: Mobilization of calcium from the The plasmocytic is characterized by small
skeleton may cause hypercalcemia resulting in normal appearing plasma cells with a low mi-
nephrocalcinosis, lethargy, drowsiness and totic index as is associated with a comparatively
eventually coma, if untreated. better prognosis than the plasmoblastic type.
Complication: The common cause of death is renal The plasmoblastic type shows infiltration by
failure, caused by accumulation of abnormal immature, nucleated plasma cell precursors and
proteins in the renal tissue. has a less favorable prognosis. Plasma cells are
round or ovoid cells with eccentrically placed
Oral Manifestations nuclei exhibiting chromatin clumping in 'cart-
Mandible is more commonly involved than the wheel' or 'checkerboard' pattern. Russell bodies
maxilla and particularly angle of the mandible, are common finding in multiple myeloma.
because of its greater content of marrow. Lesions
have also been reported in temporomandibular Hematological Findings
joints. Bone marrow examination shows an increased
The patient may experience pain, swelling number of abnormal plasma cells. There is
and numbness of the jaw. Epulis formation or usually an associated anemia but, WBC and
unexplained mobility of teeth are also detectable. platelet counts are normal. Increased ESR, serum
Intraoral swelling tends to be ulcerated, monoclonal immunoglobulin with reversal of the
rounded and bluish red similar to a peripheral albumin globulin ratio and increase in total serum
giant cell lesion. Sometimes, swelling may erode protein to a level of 8 to 16 gm percent. Bence Jones
buccal plate and produce rubbery expansion of proteins, which coagulate when the urine is
jaw. Chronic trauma produces an inflamed and heated to 40°C can be demonstrated in the urine
ulcerative necrotic surface. of patients suffering from multiple myeloma. There
Secondary signs of bone marrow involvement may be hyperproteinemia due to increase in
such as pallor of oral tissue, intraoral hemorrhage globulins.
and susceptibility to infection may also be seen.
Excessive hemorrhage may be caused by thromb- Management
ocytopenia, secondary to increased proliferation
of the plasma cells in marrow. On palpation, swell- Chemotherapeutic agents: General disease is treated
ing is tender and eggshell cracking may be elic- with chemotherapeutic agents like melphalan
ited. and cyclophosphamide.
 Malignant Tumors 241

Management of anemia and hypercalcemia: Patients

who present with anemia, hypercalcemia,
evidence of renal damage require urgent
management with alkalization of urine with oral
bicarbonates, high fluid intake, corticosteroids
and possibly mithramycin to reduce calcium level.
Blood transfusion: It may be required if Hb is less
than 10 g/dl.
Cell transplantation: Stem cell autotransplant may
improve quality of life and prolong survival.
Radiotherapy: This is effective for localized pain Figure 13.41: Plasmacytoma showing Russell bodies
not responding to simple analgesics and for (Courtesy: Dr Sangamesh Halawar)
pathological fractures.
Bi-phosphonate therapy: It may reduce bone pain Oral Manifestations
and skeletal events. It is rare in jaws and can occur in mandible or
maxilla. The lesions are described as sessile or
Others drugs: Alpha interferons may prolong the
polypoid reddish masses in mucous membranes,
plateau phase. Inorganic fluoride phosphate to
which become lobulated as they enlarge but exhibits
reduce bone pain.
the tendency to ulcerate. Pain is not prominent
symptom unless bone is invaded. There may be
Plasmacytoma bleeding and ulceration of oral mucosa.
Extramedullary location without bony
Histopathological Features
involvement may occur in the nasopharynx, nasal
cavity, paranasal sinuses and rarely in the oral It contains densely packed plasma cells and is
cavity. It is also called as 'plasma cell myeloma'. indistinguishable from the bony lesion in multiple
myeloma. There may be considerable nuclear and
Clinical Features cellular pleomorphism. Russell bodies can also be
Mean age of occurrence is 51 years and males are found. It is similar to multiple myeloma (Fig. 13.41).
affected more commonly than females. The most
Laboratory Findings
common site of occurrence are nares, tonsil, palate
tongue, gingivae and the floor of mouth. They are Bence-Jones protein are found in urine, There is
also found in pleura, mediastinum, thyroid, ovary, also hyperglobulinemia and anemia.
intestine, kidney and skin.
Management
Signs and symptoms: There is pain and swellings
are the most common complaints. Pathologic It should be treated by conservative process to
fractures are common. Regional metastasis may eradicate the single lesion and this can be
develop in small number of cases. accomplished by surgery.
242 Textbook of Oral Pathology

CHAPTER
Salivary Gland
14 Pathology

DEVELOPMENT OF SALIVARY GLAND sublingual glands arise in the 8th week of

intrauterine life, as a series of about ten epithelial
The three major sets of salivary gland—the buds, just lateral to the submandibular gland
parotid, submandibular and sublingual originate anlage. These branch and canalize to provide a
in a uniform manner by oral ectodermal number of ducts opening independently beneath
epithelium buds invading the underlying the tongue. The minor salivary glands begin their
mesenchyme. The parotid gland buds are the first development during the third month of life. These
to appear at the 6th week of intrauterine life, on glands arise from the oral ectodermal and
the inner cheek, near the angles of mouth and endodermal epithelium. Labial glands arise
grow back towards the ear. In the parotid and ear during the 9th week of intrauterine life and are
region, the epithelial cord of cells branches and morphologically mature by 25th week.
canalizes to provide the acini and ducts of the
gland. The duct and acinar system are embedded CLASSIFICATION OF SALIVARY GLANDS
in a mesenchymal stroma, get organized into
lobules and become encapsulated. The parotid • Exocrine glands: These are the glands whose
gland duct, although repositioned, traces the path products are carried away by the ducts leading
of embryonic epithelial cord in the adult. The from the gland.
submandibular gland buds also appear in the 6th • Merocrine glands: The secretory product pass
week of intrauterine life as a grouped series, through the cell walls losing the cytoplasm.
forming epithelial ridges on the either sides of the • Serous salivary gland: The salivary glands
midline, in the floor of the mouth (Fig. 14.1). which produce a thin watery secretion are
An epithelial cord proliferates back into the called as serous.
mesenchyme beneath the developing mandible to • Mucous salivary gland: These produce a thick,
branch and canalize, forming the acini and ducts viscous substance called mucous.
of submandibular gland. The mesenchymal • Seromucous salivary gland: Salivary glands
stroma is separated off the parenchyma lobules which produce serous and mucous in varying
and provides a capsule for the gland. The quantities.
 Salivary Gland Pathology 243

grams each. It lies between the mastoid process

and vertical ramus of the mandible. The bulk of
the parotid gland is situated in the
retromandibular fossa. It is wedge shaped, with
the broad edge of the wedge lying subcutane-
ously and the apex lying deep between the
parotid fascia. It is divided into superficial and
deep lobes by the facial nerve and its branches.
It forms an irregular lobulated yellowish mass,
lying below the external acoustic meatus, between
the mandible and the sternocleidomastoid. A
small part of it, more or less detached lies between
the zygomatic arch superiorly and the parotid
duct inferiorly is named ‘accessory part’ of the
gland.
Stensen’s duct: The parotid duct which is called
as ‘Stensen’s duct is about 5 cm long and has thick
walls. It emerges from the substance of the gland
to course anteriorly until it reaches the anterior
border of the masseter muscle at the point of upper
and middle thirds. When it crosses the masseter
muscle, it receives the duct of the accessory lobe.
Around the border of the masseter muscle, the duct
turns sharply medially, often embedded in a
furrow of the protruding buccal fat pad. In its
Figures 14.1 A to E: Development of salivary gland medial course, the duct reaches the outer surface
schematic approach of the buccinator muscle, where it perforates in
an oblique direction anteriorly and medially. It
• Major salivary gland: These are large salivary then runs for a short distance obliquely forward,
glands which are located outside the oral between the buccinator and mucous membrane
cavity and convey their secretions through of the oral cavity and opens on the oral surface of
their ducts. the cheek, opposite the upper second molar.
• Minor salivary gland: These are smaller salivary
Blood supply: Parotid gland is supplied by the
glands confined to the mucous and
external carotid artery and its branches near the
submucous coat of the oral cavity.
gland.
MAJOR SALIVARY GLANDS Lymphatic drainage: Drains first to the parotid
nodes and from there to the upper deep cervical
Parotid Gland nodes.
It comes from the word para— around and otic—ear. It Nerve supply: It is supplied by auriculotemporal
is like an inverted flattened pyramid. It is the nerve, plexus around the external carotid artery
largest of the salivary glands weighing about 15 and greater auricular nerve.
244 Textbook of Oral Pathology

Submandibular Gland Nerve supply: Its nerve supply is from the branches
It is a round biconvex salivary gland situated in of submandibular ganglion through which it
the anterior part of the digastric triangle. It is receives fibers from chorda tympani.
irregular in form and about the size of a walnut. It Lymphatic drainage: Passes to the submandibular
is enclosed by two layers of deep cervical fascia. lymph node.
The inner surface of the submandibular gland is
in contact with stylohyoid, digastric and Sublingual Gland
styloglossus muscle, posteriorly and with the
It lies above the mylohyoid and below the mucosa
hyoglossus and posterior border of the mylohyoid
muscle, anteriorly. of the floor of mouth. It is medial to the sublingual
fossa of the mandible, on either side of the
Wharton’s duct: The submandibular duct which symphysis menti and lateral to genioglossus
is called as ‘Wharton’s duct’, is about 5 cm long muscle. It has about 15 ducts which open directly
and its wall is much thinner than that of parotid into the floor of mouth.
duct. It emerges from the middle of the deep
Bartholin’s duct: The duct of sublingual gland is
surface, of the superficial part, of the gland. It runs
called as ‘Bartholin’s duct’. They are eight to
forward, beneath the deep part of the gland,
between the mylohyoid and hyoglossus muscle. twenty in number. Some of the smaller sublingual
ducts open into the sublingual fold, in the floor of
It runs further forward between the medial surface
the mouth, on either side of lingual frenum. Some
of the sublingual gland and the genioglossus
muscle and finally ends by opening into the open into the submandibular duct and others
unite to form the “principle sublingual duct” which
summit of the sublingual papilla, situated in the
opens in the floor of the mouth.
floor of the mouth, on the side of the frenulum.
The last few millimeters of the duct are often Blood supply: It is supplied by sublingual and
slightly widened. submental arteries.
Arterial supply: The arteries supplying the Nerve supply: It is by lingual and chorda tympani
submandibular gland are derived from the lingual nerve.
and facial branches of external carotid artery.
Lymphatic drainage: It passes to the submandibu-
Venous drainage: It drains into facial and lingual vein. lar lymph nodes.
Classification of Salivary Gland Disorders
A. Developmental disorders C. Obstructive disorders
• Aberrancy • Sialolithiasis
• Aplasia and hypoplasia • Mucus plug
• Hyperplasia • Stricture and stenosis
• Atresia • Foreign bodies
• Accessory ducts • Extraductal causes
• Diverticuli D. Cyst
• Congenital fistula • Mucocele
B. Functional disorders • Ranula
• Sialorrhoea E. Asymptomatic enlargement
• Xerostomia • Sialosis
• Allergic • Oncocytoma
• Associated with malnutrition and alcoholism • Monomorphic salivary adenomas
 Salivary Gland Pathology 245

F. Infection Benign but often recurrent

• Viral infection • Pleomorphic adenoma
• Bacterial infection • Mucoepidermoid tumor (low grade)
• Mycotic infection • Acinic cell tumor (some)
G. Autoimmune disorders Malignant
• Sjögren’s syndrome • Carcinoma in pleomorphic adenoma
• Mickulicz’s disease • Adenoid cystic carcinoma
• Uveoparotid fever • Mucoepidermoid tumor (high grade)
• Recurrent nonspecific parotitis • Acinic cell tumor (some)
H. Neoplasms • Squamous carcinoma
Benign but seldom recurrent • Adenocarcinoma
• Warthin’s tumor • Undifferentiated carcinoma

Aberrancy It is asymptomatic and only diagnosed on

radiographical examination. Micros-copically
It is defined as that situation in which the salivary
salivary gland tissue may be seen.
gland tissue develops at a site where it is not
normally found. It is also called as ectopic salivary Gingival salivary gland choristoma: Salivary tissue
gland. is regularly found in lymph nodes within the neck
Ectopic salivary tissue can develop anywhere and can be mistaken for metastatic disease, if
within the territory of the first and second found in a neck dissection specimen. Ectopic
branchial arches, in the lateral neck, pharynx or salivary gland tissue has been reported to occur
middle ear. in the gingiva, where it may be described as
‘gingival salivary gland choristoma’.
Clinical Features
Clinical significance: They may become site for
True aberrant salivary glands are most frequently development of a retention cyst or neoplasm.
reported in the cervical region, near the parotid
gland or body of the mandible. The salivary gland Aplasia and Hypoplasia
tissue in the mandible is found posterior to the
Aplasia or agenesis is the congenital absence of
first molar and often has a small communication
the salivary gland. It was first described by Gruber
with a major salivary gland.
in 1885. Aplasia may occur in association with
Developmental lingual salivary gland depression: The other developmental abnormalities such as atresia
aberrancy of the salivary gland tissue represents of lacrimal puncta and congenital malformation
only an extreme example of the condition known of temporomandibular component.
as the ‘developmental lingual mandibular
salivary gland depression’. It is the developmental Causes
inclusion of the glandular tissue within or more
It results due to regional action of some disturbing
commonly, adjacent to the lingual surface of the
influence in early fetal development. Mac Donald
body of the mandible, in a deep well circumscribed
suggested ectodermal origin for this anomaly.
depression. Mandible develops around the lobe
during development. It was first described by
Clinical Features
Stafne in 1942 and hence referred to as ‘Stafne’s
cyst’. It is rare with incidence of 4 in every adult. Any one of the glands or group of glands is
Males are affected more commonly than females. missing, either unilaterally or bilaterally.
246 Textbook of Oral Pathology

Symptoms: Patient complains of xerostomia, The lesion has an intact surface and is firm, sessile
which may be so severe as to necessitate the and normal in color.
constant sipping of water throughout the day and
particularly, during meal times. The lack of saliva Histopathological Features
results in rampant dental caries and early loss of
The mass appears microscopically as a closely
deciduous and permanent teeth.
packed collection of normal appearing mucous acini,
Signs: The oral mucosa appears dry, smooth, or with the usual intermingling of normal ducts.
sometimes pebbly and shows a tendency for
accumulation of debris. Patients exhibit Management
characteristic cracking of lips and fissuring of the
As it can not be differentiated from minor salivary
corners of mouth. Hypoplasia of salivary glands
gland tumors, it becomes essential to excise it for
is rare but hypoplasia of parotid gland has been
microscopic examination.
reported to be present with Melkerson-Rosenthal
syndrome.
Atresia
Management It is the congenital occlusion or absence of one or
two major salivary gland ducts. Usually, the
Institution of scrupulous oral hygiene is an
submandibular duct in the floor of the mouth fails
attempt to decrease dental caries and preserve the
to canulate during embryological development.
teeth as long as possible.
The newborn infant presents, within 2 or 3 days of
Hyperplasia of Salivary Gland life, with submandibular swelling on the affected
side due to the presence of a retention cyst. It may
Causes produce a relatively severe xerostomia.
• Hormonal disorders: Endocrine disorders and
menopause. Accessory Duct
• Metabolic disorders: Gout, diabetes mellitus. An accessory parotid lobe is the most common
• Autoimmune: Sjögren’s syndrome, Walden- developmental anomaly. It occurs in as many as
strom macroglobulinemia. 20% of subjects. Its position is constant, arising
• Syndrome: Aglossia-adactylia syndrome, from the horizontal component of the parotid duct
Heerfordt’s syndrome and Felty’s syndrome. as it crosses the masseter muscle. Its importance
• Miscellaneous: Hepatic disease, starvation, al- lies in the fact that any of the diseases that can
coholism, inflammation, benign lympho- affect the salivary glands, may involve the
epithelial lesion, adiposity, hyperthermia, oli-
accessory lobe and lead to diagnostic confusion,
gomenorrhea and certain drugs.
as the possibility is not considered. This is because
the symptoms and signs are not within the normal
Clinical Features
anatomical territory of the parotid. Presence of
It is more common in minor salivary glands of the additional duct in some salivary glands has been
palate. It is usually asymptomatic. reported.
Signs: Palatal gland hyperplasia appears as small
Diverticuli
localized swelling of varying size, measuring
from several millimeters to 1 cm, usually on the They are small pouches or out pocketing of the
hard palate or at junction of hard and soft palate. ductal system of one of the major salivary glands.
 Salivary Gland Pathology 247

Their presence leads to recurrent episodes of acute greater size and position of the orifice. The orifice
parotitis. is much smaller than duct lumen.
Physiochemical factors: High mucin content of
Sialolithiasis
saliva. Great degree of alkalinity with high
It is the also called as ‘salivary gland stone’ or percentage of organic matter. Greater
‘salivary gland calculus’. These are stones within concentration of calcium and phosphate salts.
major and minor salivary glands. These are the Low content of carbon dioxide. Richness in
most common calcifications found in soft tissues phosphatase enzyme.
of oro-orbital region.
Clinical Features
Definition
They are usually encountered in middle aged
It is the formation of calcific concretions within patients with slight predilection for occurrence
the parenchyma or ductal system of the major or in men. It usually occurs as a solitary concretion
minor salivary glands. varying in size from a few millimeters up to several
centimeters.
Composition
Symptoms: The symptoms of sialolithiasis vary but
The calculus consists of laminated layers of intra-glandular stones seem to cause less severe
organic material, covered with concentric shells symptoms than the extraglandular or intraductal
of calcified material. The crystalline structure is types. On occasions, there may be complete
chiefly hydroxyapatite and contains octacalcium absence of subjective symptoms. Many patients
phosphate. The chemical composition is complain of moderately severe pain and
principally calcium phosphate and carbon with intermittent transient swelling during meals,
traces of magnesium, potassium, chloride and which resolves after meals. As the calculus itself
ammonium. rarely blocks a duct completely, the swelling
subsides as salivary demand diminishes and as
Etiology and Pathogenesis saliva seeps past the partial obstruction. The
Neurohumoral mechanism: A neurohumoral occlusion of the duct prevents the free flow of
condition, leading to salivary stagnation, results saliva and this stagnation or accumulation of
in a nidus and matrix formation. saliva, when under pressure, produces pain.

Metabolic mechanism: In the presence of coexisting Signs: If no treatment is instituted, it appears as a

inflammation, a metabolic mechanism favors pronounced exacerbation characterized by an
precipitation of salivary salts into the matrix. acute suppurative process with attendant
systemic manifestations such as fever and
Prevalence malaise. Pus may exude from the duct orifice. The
soft tissues surrounding the duct show a severe
Submandibular (83%) calculi are more commonly inflammatory reaction, which may appear as
seen than the parotid (10%) or sublingual (7%)
swelling, redness and tenderness. Stones in the
calculi, due to following factors.
more peripheral portion of the duct may often be
Anatomic factors: The length and irregular course palpated, if they are of sufficient size. Sometimes,
of Wharton’s duct. The submandibular gland and the overlying mucosa may ulcerate over the stone
ductal system lies in a dependent position. The allowing the calculus extends into the oral floor.
248 Textbook of Oral Pathology

No saliva is seen to be coming out through the A metallic duct probe can also be of value.
duct orifice. If stone is present in one duct only Careful probing of the duct with a metallic probe
then saliva will not come out from that duct. It will indicate the existence as well as the location
can be tested by placing two dry swabs one on of calculus.
each orifice and some lemon juice is dropped on Radiographic examination usually reveals the
the dorsum of the tongue. A minute later patient presence of calcific deposits.
is asked to move the tongue up. The swab on the Sialography is an invaluable aid in isolating
orifice of the duct where the stone is impacted the sialoliths which had not been identified on
will remain dry, whereas the other swab will be the standard intraoral and extraoral radio-
wet. graphy.
Stones in minor salivary glands: Sialolithiasis of
Management
minor salivary gland is a rare occurrence. The
most common site is buccal mucosa either near Manual manipulation of stone within the duct
the commissure or in proximity to the mandibular should be carried out.
mucobuccal fold. It is more common after the age
of 39 years. The lesions appear as firm, freely Strictures and Stenosis
movable masses, deeply situated into the mucosal Etiology
surface.
Irritation from prosthetic appliances, maloccluded
Histopathological Features or malpositioned teeth.

It is seen as calcific concretions arranged in Acute trauma: Acute trauma with resultant edema
concentric laminations around the amorphous and/ or scarring.
debris. There is also metaplasia of lining cells Tumor: Intraductal tumor formation.
with inflammatory infiltrates surrounding it.
Sialoliths are also irregular, hexagonal and needle Types
like plate shaped crystals.
Papillary obstruction: It may be either acute
Diagnosis ulcerative obstruction or chronic fibrotic stenosis.
Acute ulcerative obstruction is usually caused by
Palpation is an indispensable tool in the
acute trauma to the papilla and is treated
diagnosis of sialoliths. Palpation of the suspected
conservatively with saline rinses and salivary
gland frequently reveals it to be larger or firmer
gland massage. The ulcer generally heals without
than the normal gland of the opposite side. Digital
scarring and the symptoms will subside in such
manipulation will produce a flow of saliva
cases. In chronic fibrotic papillary obstruction
through the duct orifice and will allow visual
irritations to papilla is recurrent and scarring
inspection of the salivary fluid.
exists.
During examination, the soft tissues overlying
the duct should be manually stretched. Often, the Duct obstruction: It may be due to a variety of
physical distortion caused by the presence of factors. In cases of ductal obstruction secondary
calculus will become apparent. In addition to acute trauma treatment is directed towards
yellowish color of the calcific deposits may be seen providing the duct patency until the edema is
through the distended and thinned mucous resolved. When the ductal obstruction occur
membrane. secondary to irritation or scar contracture,
 Salivary Gland Pathology 249

sialograms are helpful in localizing the status of floor of mouth. Age of the patient varies with peak
gland. If the gland is healthy, progressive and frequency in the 3rd decades but most of the cases
frequent dilatation of involved duct with lacrimal occur before the age of 50. Retention cysts occur
probes is generally successful in relieving the most often in older patients, whereas mucus
symptoms and signs. If this does not prove extravasation cysts occur in younger patients.
beneficial, ductoplasty is indicated. Equal in sex frequency, with most cases are
reported in white.
Mucocele
Definition Symptoms: The lesion may lie fairly deep in the
tissues or be exceptionally superficial. Patient may
It is a term used to describe the swelling caused complain of painless swelling which is frequently
by pooling of saliva at the site of injured minor recurrent. The swelling may suddenly develop at
salivary gland. meal time and may drain simultaneously at
intervals.
Types
Signs: The mucocele may be only 1 to 2 mm in
Mucus extravasation: It is caused by laceration of a diameter (Fig. 14.2), but is usually larger; majority
minor salivary gland duct by trauma resulting in of them being between 5 to 10 mm in diameter.
extravasation of mucus into the connective tissue. Superficial cyst appears as bluish mass, as the
These cysts are not lined by epithelium. thin overlying mucosa permits the pool of mucus
Mucus retention: It is caused by obstruction of fluid to absorb most of visible wave length of light.
minor salivary gland duct which causes the If inflamed, it is fluctuant, soft, nodular and dome
backup of saliva. This continuous pressure dilates shaped elevation. Deeper lesions have the color
the duct and forms a cyst like lesion. It is lined by of normal mucosa and are firmer. The swelling is
epithelium. round or oval and smooth. It is either soft or hard
depending upon the tension in the fluid. It can
Pathogenesis not be emptied by digital pressure. On aspiration,
it yields sticky viscous clear fluid.
Obstruction of salivary gland duct leads to its
dilatation proximal to the obstruction, with the
formation of epithelial lining retention cyst.
Cut or traumatic defect of a salivary gland is
responsible for the production of mucus
extravasation mucocele. There is accumulation
of mucus in the connective tissue and with the
continuous pooling of saliva a clearly demarcated
cavity develops which has no epithelial lining.

Clinical Features
They are very common and occur most
frequently on the inner aspect of lower lip; but Figure 14.2: Mucocele presented as dome shaped
may also occur on the palate, cheek, tongue and elevation on lower lip
250 Textbook of Oral Pathology

Histopathological Features Management

Poorly defined cysts consist of irregularly shaped, Complete excision of the cyst under local
poorly defined pools containing faintly anesthesia. Injection of steroid and cryosurgery
eosinophilic mucinous material and numerous can be tried.
vacuolated macrophages which are sometimes
called as ‘mucinophages’. Ranula
Some of these cysts are smaller and others
extend widely into the connective tissue. In well Definition
defined cysts the periphery consists of The term ranula is used for the mucocele
granulation tissue or condensed fibrous tissue or occurring in the floor of the mouth, in association
both and is infiltrated by vacuolated with ducts of submandibular or sublingual
macrophages, lymphocytes and polymor- glands.
phonuclear leucocytes, including eosinophils
(Figs 14.3 and 14.4). One or more dilated ducts Types
are present and some times a breach may be seen Superficial: The superficial variety may develop
in the duct. as a retention or extravasation phenomenon
The lumen of cyst like cavity is filled with an associated with trauma to one or more of the
eosinophilic coagulum, containing variable numerous excretory ducts of the sublingual
numbers of cells chiefly leukocytes and mono- salivary gland.
nuclear phagocytes. The second group of well-
defined cysts may be partially or completely Plunging: It ramifies deeply into the neck.
lined by epithelium. The epithelium varies, it
Clinical Features
may consist of one or two layer of cuboidal cells
or a thicker pseudostratified columnar epithe- They are usually unilateral. It is usually in
lium. children and young adults with no sex

Figure 14.3: Mucocele showing irregularly shaped area Figure 14.4: Mucocele seen under high power showing
of mucous pool surrounded by inflammatory cell reaction mucinophages
 Salivary Gland Pathology 251

predilection. The typical position is on the floor

of the mouth, below the tongue and on the side of
frenum. They produce blue swelling like a frog’s
belly; hence it was given the term ‘ranula’
(‘ranula’ in Greek mean frog’s belly). The
overlying mucosa is normal in appearance.
Symptoms: It develops as slowly enlarging
painless mass on one side of the floor of mouth.
When the swelling suddenly grows it may be
painful. Big ranula may cause difficulty in speech
or eating.
Figure 14.5: Ranula seen as bluish swelling in
Signs: It is spherical or dome shaped (Fig. 14.5) the floor of mouth
with only top half is visible. It is smaller in early
morning and largest just before meals, due to Management
increased secretory activity in periods of gustatory Surgical excision: They are best treated by surgical
stimulation and water absorption from the pooled excision including a portion of the surrounding
mucus during inactive period. It is soft and tends tissues.
to be fluctuant. It can not empty by pressure and
is nonpulsatile. Partial excision with marsupialization: The major
part of the cyst wall together with its overlying
Fluctuation and transillumination: Both tests are
mucous membrane is excised.
positive. The ranula is typically known as
brilliant translucent swelling. Aspiration yields
Sialosis (Sialadenosis)
sticky clear fluid.
The condition is found in association with
Plunging Ranula systemic diseases especially cirrhosis, diabetes,
When intrabuccal ranula has a cervical ovarian and thyroid insufficiency, alcoholism
prolongation, it is called as deep or plunging and malnutrition.
ranula. It is derived from cervical sinus. It lies It is characterized by non-neoplastic non-
along the posterior border of the mylohyoid inflammatory enlargement of the salivary gland.
muscle and appears in the submandibular region. The enlargement is usually bilateral and may
Sometimes, plunging ranula herniated through present a course of recurrent painless enlargement
the mylohyoid muscle and cause a swelling in of gland. The parotid gland is more frequently
suprahyoid or infrahyoid region. affected and more commonly affects the females.
Bidigital palpation: To inspect plunging ranula, Symptoms: Swelling of the preauricular portion of
bidigital palpation should be performed. One the parotid gland is the most common symptom,
finger is placed inside the mouth on the ranula
but retromandibular portion of the gland may also
and the other finger is place on the swelling in
be affected.
the submandibular region. If pressure on the first
finger causes sense of fluctuation on 2nd finger Laboratory finding: A characteristic alteration in
or vice versa, then it is plunging ranula. the chemical constituents of saliva is a
distinguishing feature of sialosis. Significant
Histopathological Feature elevation of salivary potassium and concomitant
Definite epithelial lining is sometimes present. decrease in salivary sodium is observed.
252 Textbook of Oral Pathology

Allergic Sialadenitis Clinical Features

In some cases, it may not appear as a true Incubation period of 2 to 3 weeks. It is more
hypersensitivity reaction but rather as a toxic or common in boys than in girls and most often seen
idiosyncratic reaction to drugs that cause a between the ages of 5 to 15 years.
deceased salivary flow, resulting in secondary Prodormal symptoms: It is preceded by onset of
infection. Various drugs which have been reported headache, chills’ moderate fever, vomiting and
to cause allergic sialoadentitis include pain below the ear which lasts for about one week.
sulfisoxazole, phenothiazines, iodine containing Symptoms: The parotid gland is most commonly
compounds, mercury, thiouracil and phenyl- involved and it is usually bilateral. Submandi-
butazone. bular gland may also be involved, although this
Mechanism: The exact mechanism of salivary gland is less noticeable and cause less pain. Both the
enlargement and loss of function following parotid glands may involve simultaneously, but
administration of these drugs is not known. Most more commonly one parotid gland swells 24 to
of the drugs cause decrease in capillary 48 hours after the other. It is then followed by
permeability, whereas others cause sodium and sudden onset of salivary gland swelling which is
chloride retention, which subsequently leads to firm somewhat rubbery or elastic and without
edema. purulent discharge from the salivary gland duct.
Signs: The enlargement of parotid gland causes
Symptoms: The clinical appearance of allergic
elevation of ear lobule and it produces pain upon
sialadenitis varies, but in most of the cases there
mastication especially while eating sour food.
is bilateral parotid gland enlargement following
Papilla at the opening of parotid duct is often
the administration of the drug. The enlargement
puffy and reddened.
may be painful and is usually associated with
conjunctivitis and skin rashes. Diagnosis
It is a self-limiting disease and needs no
The presence of parotitis and accompanying
treatment. But in some cases, secondary bacterial
systemic signs of viral infections. Salivary
infection may develop and need treatment.
amylase level is increased. A paramyxovirus may
be isolated from saliva for as long as 6 days before
Mumps
and up to 99 days after the appearance of salivary
It is an acute contagious viral infection, gland swelling.
characterized chiefly by unilateral or bilateral
swelling of the salivary glands. It mainly affects Management
major salivary glands, but also affects the testis, Most of the cases are self-limiting, with salivary
meninges, pancreas, heart and mammary glands. gland enlargement subsiding within a week.
It is also called as ‘endemic parotitis’. Prevention with live attenuated vaccine is the best
method of controlling the disease. Symptomatic
Etiology treatment is given to control pain and swelling.
It is endemic in most urban population. It is
caused by paramyxovirus. It usually spreads from Cytomegalovirus Inclusion Disease
human reservoir, by airborne infection of infected It is also called as ‘salivary gland virus disease’.
saliva and possibly urine. It is caused by cytomegalovirus, a herpes virus. It
 Salivary Gland Pathology 253

is common in immunosuppressed adult. Unilateral involvement of parotid gland is

Although it is congenital in nature, it is usually common.
secondary to concurrent disease which has Symptoms: It begins with the elevation of body
caused debilitation. temperature and sudden onset of pain at the angle
Clinical Feature of the jaw which is intense when the extensive
infection is contained within the confines of the
It affects primarily in newborn infants and
parotid capsule. The localized symptoms are
children, but adults are also affected. In newborns,
accompanied by fever, leukocytosis and other
infection is generalized and is usually fatal with
generalized signs and symptoms of acute bacterial
involvement of liver, lungs and central nervous
infection.
system.
Signs: Parotid gland is tender, enlarged and the
Signs: Infants who survive the infection may have
overlying skin is warm and red. The swelling
permanent central nervous system involvement,
usually causes elevation of the ear lobule and the
including mental retardation and seizures. There
overlying skin is characteristically warm and red.
may be hepatosplenomegaly, hemolytic anemia
and hemorrhagic tendency. It may cause clinical Early in the disease, flecks of purulent material
disease of salivary gland, causing enlargement can be expressed from the salivary duct orifice.
of the gland. Intraorally the parotid papilla may be inflamed
and pus may exude or be milked from the duct of
Laboratory Investigations the affected gland. Cervical lymphadenopathy
Intranuclear and cytoplasmic inclusions in the usually develops.
cells of salivary glands are constant features of
Management
the disease.
Meticulous oral hygiene should be practiced. Soft
Acute Bacterial Sialadenitis diet should be given as chewing is painful. It is
It is also called as ‘acute suppurative parotitis. treated aggressively because even with antibiotics
death can result in debilitated patients. Specimen
Etiology of purulent material should be immediately sent
• Microorganisms: It is most commonly caused to laboratory for sensitivity and culture. Treatment
by penicillin resistant Staphylococcus aureus or usually starts with high dose of parenteral
Streptococcus viridans. antibiotics active against penicillin resistant
• Predisposing factors: It can occur in conditions Staphylococcus.
such as dehydration, malnutrition, cancer and The patient must be adequately hydrated and
surgical infections. the electrolyte balance should be properly
• Oral hygiene: Poor oral hygiene is an important maintained with intravenous fluids. Salivation
contributory factor. should be stimulated to facilitate drainage by
• Drugs: Drugs like anti-Parkinson’s, diuretics sucking the sour hard candy. Oral hygiene should
and antihistaminic have been reported to be a be maintained by debridment and irrigation. If
contributory factor for acute bacterial improvement does not occur, surgical drainage of
sialadenitis. the affected gland should be performed.

Clinical Features Chronic Bacterial Sialadenitis

Most of the cases occur in adults but neonates It is usually caused by Streptococcus viridans, E
and childhood form of the disease may occur. coli or Proteus. As compared to acute parotitis,
254 Textbook of Oral Pathology

it can be seen in normal children or in as in • Secondary Sjögren’s syndrome: It consists of dry

adults. eyes, dry mouth and collagen disorders
usually rheumatoid arthritis or systemic lupus
Clinical Features
erythematous.
The childhood form commonly begins between
the ages of 3 to 5 years and is usually unilateral.
Etiology and Pathogenesis
Recurrent form is most often due to duct
obstruction, congenital stenosis, Sjögren’s Immunological findings: The lesion in this syn-
syndrome, or previous viral infection or allergy. drome is immunologically mediated inflamma-
tory exocrinopathy. It begins with periductal in-
Signs: It appears as a unilateral swelling at the
filtration of the tissue by mononuclear cells.
angle of the jaw in a patient with the history of
similar occurrence. Salivary flow is accompanied Autoantibodies: The B cell hyperactivity may result
by flecks of purulent material. After several recur- from deficiency of suppressor T lymphocytes or B
rences, fibrosis of the glandular parenchyma oc- lymphocytes by producing autoantibodies
curs, which leads to decreased salivary flow. The against them. Antinuclear antibodies found in
pain is usually minimal and antibiotic therapy patients with Sjögren’s syndrome are directed
resolves the infection within a week. against many nuclear antigens, most commonly
to DNA histone. Patients with secondary
Chronic Sclerosing Sialadenitis
Sjögren’s syndrome tend to develop antibodies
It is also called as ‘Kuttner’s disease’ and is against the EBV-associated nuclear antigen
common in submandibular gland. It is a chronic antibodies RANA (rheumatoid arthritis nuclear
inflammatory disease of the major salivary glands antigen).
characterized by enlargement of the glands,
resulting in fibrous tumor like masses. Polyclonal hyperglobulinemia: Serum immunoglo-
It is cause by salivary ductal calculi causing bulin levels of IgG and IgM are also raised.
subsequent pyogenic bacterial infections. It can B 2 microglobulin: Serum salivary level of B 2
lead to chronic inflammation, ensuing in the microglobulin is raised in minority of patients
atrophy of mucous and serous cells and and correlates with salivary lymphocyte infiltrate.
hyperplasia of the involved connective tissue
leading to formation the tumor like swollen Immune complex: Circulating immune complexes
masses. are found in patients with primary and secondary
Sjögren’s syndrome.
Sjögren’s Syndrome
Cell-mediated immune response: Delayed
It is a chronic inflammatory disease that hypersensitivity response to skin testing is more
predominately affects salivary, lacrimal and other
depressed in patients with secondary Sjögren’s
exocrine glands. It was first described by Henrik
syndrome than in patient with primary Sjögren’s
Sjögren in 1933. It predominately affects middle
syndrome. Lymphokine production in response
aged and elderly women.
to antigen present in normal salivary tissue, is
Types increased in patients with Sjögren’s syndrome.
• Primary Sjögren's syndrome: It also called as Natural killer cell activity: Augmented natural
sicca syndrome and it consists of dry eyes killer cell activity is impaired in some patients
(xerophthalamia) and dry mouth (xerostomia). with Sjögren’s syndrome. Although natural
 Salivary Gland Pathology 255

killer cells provide a defense against viral infection may a lack of the usual pooling of saliva in the
and tumor, their role in Sjögren’s syndrome is floor of the mouth and frothy saliva may form
unclear. along the lines of contact with oral soft tissue. In
advanced cases the mucosa is glazed, dry and
Virologic aspect: Culture of saliva does not show
tends to form fine wrinkles. Soreness and redness
any specific microorganisms and serologic
of mucosa is usually the result of candidial
studies have failed to show increased titers of
infection.
antibodies, except to cytomegalovirus (CMV).
In some patients, there may be ‘clicking’
Genetic aspect: Genetic effects of Sjögren’s quality of their speech, caused by sticking of the
syndrome depend on HLA-Linked and non HLA- tongue to the palate. The tongue typically
Linked genes. Relatives of a patient with Sjögren’s develops a characteristic lobulated, usually red
syndrome often show a high incidence of surface with partial or complete depapillation.
connective tissue diseases. Primary Sjögren’s There is also decrease in number of taste buds,
syndrome is associated with HGLA-B8 and DR3 which leads to an abnormal and impaired sense
and secondary Sjögren’s syndrome is associated of taste.
with HLA-B8 DR4 and BW44. Dental caries is severe and gross accumula-
tion of plaque may be obvious. Periodontal dis-
Lymphoproliferative malignancy: Enlargement of
ease can also occur.
salivary glands in patients with Sjögren’s
Sjögren’s syndrome is the most common
syndrome is occasionally massive and associated
underlying cause of acute bacterial sialadenitis
with enlargement of regional lymph nodes, a
in ambulated patients. Such infections are usually
condition known as ‘pseudolymphoma’.
either staphylococcal or pneumococcal and
Malignant B cell lymphoproliferation has been
usually cause swelling of the salivary gland. The
shown to affect patients with Sjögren’s syndrome.
overlying skin is red, tender and shiny. The
regional lymph nodes may be enlarged and
Clinical Features
tender.
Oral
General
Symptoms: Xerostomia is a major complaint in Eyes: the patient usually complains of dry eyes or
most of the patients. But many patients do not continuous irritation in the eyes. Severe lacrimal
complain of dry mouth, but rather of an gland involvement may lead to corneal ulceration
unpleasant taste, difficulty in eating dry food, as well as conjunctivitis.
soreness or difficulty in controlling dentures. Pus
Connective tissue disorder: In patients with
may be emitted from the duct. Angular stomatitis
secondary Sjögren’s syndrome, rheumatoid
and denture stomatitis also occur. Dry mouth may
arthritis is typically long standing and clinically
be accompanied by unilateral or bilateral
obvious. Patients may have small joint and ulnar
enlargement of parotid gland, which occurs in
deviation of fingers and rheumatoid nodules.
about one third of the patients and may be
Dryness of pharynx, larynx and nose are noted
intermittent. Enlargement of submandibular
by some patients. This is accompanied by lack of
gland may also occur.
secretion in the upper respiratory tract, may lead
Signs: Clinically, the mouth may appear moist in to pneumonia. Vaginal dryness may be also
early stages of Sjögren’s syndrome but later, there complained by some females.
256 Textbook of Oral Pathology

Histopathological Features viscosity, leucopenia occasionally may also be

found.
There may be intense infiltration of the glands by
lymphocyte cells replacing all acinar structure. In Microbiological investigations: A swab from oral
some cases there may be proliferation of ductal mucosa should be taken to confirm candidiasis,
epithelium and myoepithelium to form if there is soreness and erythema. Examination of
‘epimyoepithelial island’. Lymphocytic infiltration pus is also of course essential as a guide to
of the glands with ensuing atrophy of glands also antimicrobial treatment, if acute sialadenitis
occurs. develops.
Salivary gland biopsy: The changes in minor glands
Laboratory Investigations
of lower lip show close correlation with those in
Rose Bengal staining test: Keratoconjunctivitis sicca the major salivary glands and provide a safe and
is characterized by corneal keratotic lesion, which convenient source of material.
stains pink when ‘rose Bengal’ dye is used.
Management
Schirmer test: The reduced lacrimal flow rate is
measured by this test. A strip of filter paper is Most of the patients are treated symptomatically.
placed in between the eye and the eyelid to Keratoconjunctivitis is treated by instillation of
determine the degree of tears which should be ocular lubricants, such as artificial tears coating
measured in millimeter. When the flow is reduced methylcellulose and xerostomia is treated by
to less than 5 mm in a 5 minute sample, patient saliva substitutes. Scrupulous oral hygiene and
should be considered positive for Sjögren’s frequent fluoride application is indicated to
syndrome. reduce these problems. Bromhexine can be used
Sialometry: Salivary flow rate estimation is a in some cases of Sjögren’s syndrome. Surgery for
sensitive indicator of salivary gland function. enlargement of salivary gland is only
Parotid glands make the major contribution to recommended when the enlargement is causing
total salivary flow and are the most consistently discomfort to the patient.
affected glands in patients with Sjogren’s
syndrome. Stimulated flow rate in symptomatic Mikulicz’s Disease or
primary and secondary Sjögren’s syndrome is Benign Lymphoepithelial Lesion
usually below 0.5 to 1.0 ml/minute (normal 1 to It was first described by Mikulicz in 1888 as
1.5 ml/minute).
symmetric or bilateral, chronic, painless
Sialochemistry: Parotid saliva in Sjögren’s enlargement of lacrimal and salivary glands. It
syndrome contains twice as much total lipid and exhibits both inflammatory and neoplastic
has elevated content of phospholipids and characteristics. Initially, Mikulicz’s disease was
glycolipids than the normal saliva. The sodium confused with disease processes such as leukemia
chloride and phospholipids levels are higher in and tuberculosis. In 1909, Campbell Howard went
saliva of Sjögren’s syndrome patient. further and limited Mikulicz’s disease to merely a
Immunologic: A routine autoantibody profile can clinical syndrome.
usually be carried out with the particular aim of
detecting rheumatoid and antinuclear factors. Classification
Hematological investigations: It is necessary, • Mikulicz’s disease proper: Lacrimal and salivary
particularly to exclude anemia. ESR or plasma gland swelling only.
 Salivary Gland Pathology 257

• Pseudoleukemia: Lacrimal and salivary gland Management

swelling with lymphatic system involvement.
Surgical excision and radiation can be given.
• Leukemia: Lacrimal and salivary gland
Prognosis is good.
swelling with hematopoietic involvement.
Uveoparotid Fever
Clinical Features
It is a form of sarcoidosis and it is also called as
It more commonly occurs in women in middle
‘Heerfordt’s syndrome’.
and later life. It is manifested as a unilateral or
It consists of a triad of:
bilateral enlargement of parotid and/or
submandibular gland. • Uveitis: Inflammation of uveal tract of the eye.
• Parotid swelling: Firm, painless and bilateral
Symptoms: The onset of the lesion is some times
enlargement of parotid gland.
associated with fever, upper respiratory tract
• Facial palsy.
infection, oral infection, tooth extraction or some
local inflammatory disorders. In some cases there
Etiology
is mild local discomfort, occasional pain and
xerostomia. Tuberculosis was earlier thought to be the
causative agent. Hereditary factor, autoimmune
Signs: There is often diffuse, poorly outlined
mechanisms are also considered etiological factors
enlargement of salivary gland rather than
for it.
formation of a discrete tumor nodule. The
enlargement varies in size but generally few Clinical Features
centimeters in diameter. There is history of
alternating increases and decreases in the size It usually occurs in 3rd and 4th decades of life.
of mass, from time to time. The duration of the Prodormal symptoms lasting from a few days to
tumor mass may be only a few months or many several weeks are the usual initial signs of the disease
years. and complains include fever, malaise, weakness,
nausea and night sweat. It appears as a bilateral,
Histopathological Features firm, painless parotid swelling.

It is characterized by orderly lymphocytic Signs: Submandibular, sublingual and lacrimal

infiltration of the salivary gland tissue, gland swelling may develop independently or
destroying or replacing the acini, with presence during the course of the parotid swelling. The
of islands of epithelial cells, which probably parotid swelling lasts from several months to
represent residua of gland. The epithelium several years. Sarcodial lesion may also be found
consists of ducts showing cellular proliferation in the oral cavity.
and loss of polarity.
Uveitis: It is an inflammation of the uveal tract, is
As the disease persist solid nest or clumps of
a feature of this disease. Although ocular
poorly defined epithelial which termed as
symptoms are usually bilateral, they become more
‘epimyoepithelial islands’ is also present. Such
apparent before the appearances of parotid
islands arise as result of proliferation of ductal cells
swelling.
and peripheral myoepithelial cells. In advanced
cases there is deposition of eosinophllic hyaline Nerve involvement: The most common nerve
material in the epithelial islands. involved is facial nerve. There is unilateral or
258 Textbook of Oral Pathology

bilateral seventh nerve paralysis. The neurological epithelium. Thus the epithelial tumor cells show
signs may precede, follow or appear the features of acinar cells, ductal cells and
simultaneously with parotid swelling. Trigeminal myoepithelial cells.
paresthesia, eyelid ptosis, polyneuritis, intercostal The connective tissue components include
neuralgia and spinal nerve impairment (e.g. fat, fibrous tissue, nerves and blood vessels).
accompanied by weakness and muscle atrophy The complexity of the various cell types leads
have been reported. to variation in the appearance. No other tissue in
the body produces the diversity in
Histopathological Features histopathological appearance as salivary gland
tumor does. Because of these salivary gland
It will reveal a characteristic sarcoid nodule. tumors has always been an area of challenge.
Salivary tumors are said to be relatively
Management uncommon lesions. The incidence is 3 to 5 % of the
It is largely symptomatic as it may under go maxillofacial tumors. In major salivary glands the
spontaneous remission. Corticosteroid can be most common site is parotid (64-80%) whereas
used in cases of acute exacerbation. submandibular gland and sublingual glands
show incidence of 8 to 11% and 1% respectively.
TUMORS OF SALIVARY GLANDS Neoplasms in minor salivary glands are 9 to
23% of all tumors, palate being the most frequent
Salivary gland tumors are tumors arising from site (42-54%).
the major and minor salivary glands.
The three pairs of major salivary glands Histogenesis
extraorally placed are parotid (serous),
The basal cells of the ‘excretory duct’ and
submandibular (mixed) and sublingual
‘intercalated duct’ play a major role in the
(mucous). The minor salivary gland collections
development of salivary gland. Basal cell of
are numerous small glands widely distributed in
excretory duct forms columnar and squamous cell
the mucosa of the oral cavity. The neoplasms
of the duct. The basal cells of intercalated duct give
develop mainly from the gland tissue, i.e. the
rise to acinar cell, striated duct cells and
parenchymal elements and uncommonly from the
myoepithelial cells (Fig. 14.6).
supported investing connective tissue. The
salivary parenchyma is derived from the oral
epithelium andis composed of secretory units.
The secretory units are made of following
component:
• The cellular components
• Serous and mucous acinar cells
• Ductal epithelial cells and
• Myoepithelial cell.
The myoepithelial cells are of particular
interest. They are plentiful in the salivary acini
and intercalated ducts, but much less so in the Figure 14.6: Schematic diagram of salivary tree showing
acinus (A), intercalated ducts (ID-pink), striated ducts (SD-
larger excretory ducts of the major glands. They green), terminal secretory ducts (TSD) and myoepithelial
display features of both smooth muscle and cells (M)
 Salivary Gland Pathology 259

These two basal cells are said to be reserve Excretory duct cells —→ Squamous cell
cells or progenitor cells in salivary gland carcinoma and
mucoepidermoid
development. carcinoma
Intercalated duct —→ Adenocarcinoma
Theories of Salivary Gland Tumor
• Bicellular theory: This theory suggest that
Histogenesis
undifferentiated ‘reserve’ cell, i.e. basal cells
There are two hypothesis suggested in the of the excretory and intercalated duct are
development of salivary gland neoplasms. responsible for formation of majority of
• Well-differentiated cells of the salivary gland neoplasms. According to this theory,
unit form the neoplasm of their differentiated dedifferentiation of already specialized cells
counterparts. According to this concept: such as acinar and striated duct cells duct not
required for development of salivary gland
Acinar cells —→ Acinous cell carcinoma neoplasms.
Striated duct cells —→ Oncocytic tumors

Classification
Old WHO classification (Thackeray and Sobin 1972)
1. Epithelial tumors B. Carcinoma
A. Adenoma • Adenoid cystic carcinoma
• Pleomorphic adenoma • Adenocarcinoma
• Monomorphic adenoma • Epidermoid carcinoma
– Adenolymphoma (Warthin’s tumor) • Undifferentiated carcinoma
– Oxyphilic adenoma (Oncocytoma) • Carcinoma in pleomorphic adenoma
– Other types (Malignant pleomorphic adenoma)
- Basal cell adenoma 2. Non-epithelial tumors
- Canalicular adenoma Unclassified Lesions
• Mucoepidermoid tumor Allied conditions
• Acinic cell tumor • Benign lymphoepithelial lesion
• Sialosis
• Oncocytosis
WHO Classification 1991
1. Adenoma • Monomorphic adenoma
• Pleomorphic adenoma – Adenolymphoma (Warthin’s tumor)
• Myoepithelioma – Oxyphilic adenoma (oncocytoma)
• Basal cell adenoma – Other types
• Warthin’s tumor – Basal cell adenoma
• Oncocytoma – Canalicular adenoma
• Canalicular adenoma • Mucoepidermoid tumor
• Sebaceous adenoma • Acinic cell tumor
• Ductal papilloma 2. Carcinoma
– Inverted ductal papilloma • Acinic cell carcinoma
– Intraductal papilloma • Mucoepidermoid carcinoma
– Sialadenoma papilliferum • Adenoid cystic carcinoma
• Cystadenoma • Polymorphous low grade adenocarcinoma
– Papillary cystadenoma • Epithelial myoepithelial carcinoma
– Mucinous cystadenoma • Basal cell adenocarcinoma
260 Textbook of Oral Pathology

• Sebaceous carcinoma 3. Nonepithelial tumors

• Papillary cystadenocarcinoma and mucinous 4. Malignant lymphoma
adenocarcinoma 5. Secondary tumors
• Oncocytic carcinomna 6. Unclassified tumors
• Salivary duct carcinoma 7. Tumor like lesions
• Adenocarcinoma • Sialadenosis
• Malignant myoepithelioma • Oncocytosis
• Carcinoma in pleomorphic adenoma • Necrotising sialometaplasia
(Malignant pleomorphic adenoma) • Benign lymphoepithelial lesions
• Squamous cell carcinoma • Salivary gland cyst
• Small cell carcinoma • Chronic sclerosing sialadenitis of submandibu-
• Undifferntiated carcinoma lar gland
• Other carcinomas • Cystic lymphoid hyperplasia in AIDS

In addition to epithelial tumors arising in the salivary gland the supporting tissue neoplasms may
be seen as soft tissue tumors of paraglandular origin.
Soft tissue tumors of major salivary gland and paraglandular tissues:
• Vascular – Neuroepithelial sarcoma
– Primary hemangioma of parotid gland – Granular cell tumor
– Lymphangioma – Meningoma
– Arteriovenous fistula • Skeletal muscle
– Angiosarcoma – Rhabdomyosarcoma
• Lymphoreticular – Rhabdomyoma
– Lymphoma – Infantile rhabdomyoma
– Atypical lymphoreticular hyperplasia – Masseteric hypertrophy
– Histiocytosis • Smooth muscle
– Lymphoepithelia lesion – Leiomyoma
– Benign reactive hyperplasia – Leiomyosarcoma
• Neurogenous • Fibroblastic and histiocytic
– Neurofibroma – Fibrous scar or keloid
– Neurofibrosacroma – Fibrosarcoma
– Neurilemmoma – Fibromatosis
– Traumatic neuroma – Histiocytoma

Consistency of the Tumor • Carcinoma in pleomorphic adenoma

• Acinic cell carcinoma
Firm Consistency
• Oncocytoma.
Firmness results from fibrous tissue and hyaline
area as well as cartilage like and bone like tissue. Soft Consistency
Firm consistency is also produced by dense Softness results from fluid produced and
aggregates of nests and cords of closely packed retention phenomenon. Following tumors are soft
tumor. Following tumors are firm on palpation: on palpation:
• Pleomorphic adenoma • Well-differentiated mucoepidermoid tumor
• Adenoid cystic carcinoma • Papillary cyst adenoma/Warthin’s tumor
• Mucoepidermoid tumor of high grade • Mucus producing adenocarcinoma
 Salivary Gland Pathology 261

Clinical Staging of Salivary Gland Tumors • Stage grouping is performed as follows:

– Stage I : T1N0M0 or T2N0M0
By Spiro
– Stage II: T3 N0M0
Staging of salivary gland neoplasms appear to – Stage III: T1 or T2, N1 M0, or T4a or T4b N0 M0
have been initiated by Spiro. It is as follows: – Stage IV: T3 N1 M0, T4a or T4b N1 M0, any T
• T1—0 to 3 cm and solitary and freely mobile any N M1
and CRVII intact.
• T2—3.1 to 6 cm and solitary and freely mobile Pleomorphic Adenoma
or skin fixation and CRVII intact.
• T3—6 cm or multiple nodules or ulceration or The term pleomorphic adenoma was suggested
deep fixation or CRVII dysfunction. by Willis characterizing the unusual histological
• Patient with T1 and T2 lesion are placed into pattern of the lesion (pleomorphic or mixed
stage I and II respectively. appearance) (Fig. 14.7). The morphologically
• Any patient with clinical evidence of diverse appearance of tumor is due to ability of
metastasizes to lymph nodes or with T3 lesion the tumor cells to differentiate into epithelial and
is considered to be in stage III. mesenchymal cells. This leads to formation of
mixed picture showing fibrous, hyalinised,
By American Joint Committee chondroid, myxoid areas along with areas of
epithelial cells with metaplasia. Thus the term
• Primary tumor
mixed tumor means the varied histopathological
– Tx: Tumor that can not be assessed by the
appearance due to differentiation of the tumor
rules.
cells and it does not signify teratomatous origin.
– T0: No evidence of primary tumor.
Other names suggested for this tumor are
– T1: Tumor 2 cms or less in diameter, without
‘iceberg tumor’, ‘endothelioma’, ‘branchioma’, or
significant local extension.
‘enchondroma’, enclavoma.
– T2: Tumor 2-4 cms in diameter without
It is most common of the salivary gland
significant local extension.
tumors.
– T3: Tumor more than 4 cms but not more
than 6 cms in diameter without significant
local extension.
– T4a: Tumor over 6 cms in diameter without
significant local extension.
– T4b: Tumor of any size with significant local
extension.
• Nodal involvement (N)
– Nx: Regional lymph node can not be assessed
– N0: No regional lymph node metastasis
– N 1: Clinical or histologically positive
regional lymph nodes.
• Distant metastasis (M)
– Mx: Distant metastasis can not be assessed
– M0: No distant metastasis Figure 14.7: Pleomorphic adenoma of parotid gland
– M1: Distant metastasis showing huge swelling
262 Textbook of Oral Pathology

Definition It may increase to cricket ball size or even more,

It is defined as a tumor of variable capsulation weighing in pounds and in intraoral cases, not more
characterized microscopically by architectural than 1 to 2 cm in diameter. Its surface is smooth.
rather than cellular pleomorphism. Sometime it is bosselated and is occasionally crossed
by deep furrows. No fixation, either to deeper tissues
Histogenesis and Pathogenesis or overlying skin. It is firm and rubbery to feel.
Three theories of origin have been suggested. Sometimes cystic degeneration may be seen. No
• Pleomorphic adenomas arise from myoepithe- ulceration of skin, no pain, facial discomfort.
lial cell and intercalated duct reserve cells. Metaplasia is seen in liver and long bones.
There are evidences for ultrastructural similari-
ties between the neoplastic cells and the sug- Histopathological Features
gested cells of origin. There is presence of myo- Macroscopy: Encapsulated lesion with rubbery
epithelial cells and reserve cells, arranged in consistency is present. Minor salivary gland
intercalated duct. Also the morphologic diver- tumor may not show a well defined capsule. The
sity of the tumor showing mixed tumor appear-
gross cut surface of the firm mass shows small
ance with areas of fibrous mucinous chondroid
cystic areas with mucoid, hemorrhagic areas.
and osteoid areas seen are suggested due to
myoepithelial cells. The appearance of ductal Microscopy: Capsule separates the tumor tissue
pattern and areas of squamous cells are sug- from normal salivary gland.
gested to be from intercalated duct reserve cells. It is combination of salivary gland epithelium
• Batsakis theory: The reserve cells can differenti- lining the ducts and mesenchyme like tissue. It
ate into ductal and myoepithelial cells and un- can be categorized into four categories depending
dergo subsequent mesenchymal metaplasia. on the proportion of the components (Foot and
Thus, the intercalated duct reserve cell is the his- Fazelle 1954) (Figs 14.8 to 14.12).
togenetic precursor of pleomorphic adenoma. • Principally myxoid
• Dardick’s theory: This theory suggests that any • Myxoid—cellular components in equal
neoplastically altered epithelial cells can differ- proportions
entiate multidirectionally and the origin cannot • Predominantly cellular
be from intercalated and myoepithelial cells. • Extremely cellular.

Clinical Features
The most common type is parotid (90%), followed
by intraoral minor salivary gland (approx 8%).
The palatal gland are the most affected in this
group (60-65%). The incident in submadibular
and sublingual gland is relatively uncommon.
Lower pole of the superficial lobe is most
commonly affected. Women to men ratio is 6:4. It
is common in 4th to 6th decades but also seen in
young adults and children. Small, painless,
quiescent nodule which slowly begins to increase
in size, sometimes intermittently. The tumor tends
Figure 14.8: Pleomorphic adenoma showing presence of
to be round or oval when it is small; as it grows duct like spaces (D), eosinophilic material (E), and mucous
bigger it becomes lobulated. acini (M). Peripherally capsule (C) is seen
 Salivary Gland Pathology 263

Figure 14.9: Pleomorphic adenoma showing chondroid Figure 14.11: Pleomorphic adenoma showing chondroid
areas (C) and island of squamous metaplasia (SM) area (Courtesy: Dr Sangamesh Halawar)

Figure 14.12: Pleomorphic adenoma myxoid area

(Courtesy: Dr Sangamesh Halawar)

intercellular bridges. This also forms ducts, small

cysts containing eosinophilic coagulum in the
lumen. Mucous producing cells and
myoepithelial cells with variable morphology
such as rounded or spindle shape may be seen.
Figure 14.10: Pleomorphic adenoma showing ductal spaces Sometimes they show plasma cell like
(D) filled with eosinophilic material. Chondroid areas (C) are appearance called as plasmacytoid myoepithelial
seen
cells. The background of the tumor is made by
Epithelial component: This forms nests, sheets and stroma containing areas with mucoid deposition
anastomosing chords. These contain epithelial giving myxomatous appearance. Chondroid,
cells showing squamous metaplasia with osteoid and eosinophilic hyalinized areas are also
keratinization and sometimes actual keratin seen. Tumor is always encapsulated, but tumor
pearls formation. These cells exhibit typical cells are often present in connective tissue capsule.
264 Textbook of Oral Pathology

When the pleomorphic pattern of the stroma Clinical Features

is absent and the tumor is highly cellular, it is It occurs primarily in major salivary glands
often refered to as cellular adenoma and when particularly in the parotid gland and intraorally,
myoepithelial proliferation predominates, the upper lip. It is more common in females with 2:1
diagnosis of ‘myoepithelioma’ is made. predilection. Older age group, usually over 60
years of age are affected.
Signs of Malignant Transformation Include
Symptoms: The tumor shows painless slow
• Accelerated growth rate.
growth and presents as freely movable mass like
• Tumor irregularity on palpations.
pleomorphic adenoma.
• Necrosis and painful ulceration.
• Facial nerve involvement. Signs: Size of the lesion is less than 3 cm in diameter.

Management Histopathological Features

Surgical excision should be carried out. Recur- It is encapsulated well circumscribed tumor
rence rate is 5 to 30% due to hypocellularity, in- (Fig. 14.13). The isomorphic cells are arranged into
complete resection and encapsulation. different patterns further classifying tumor into
four types.
Monomorphic Adenoma Solid variant: The tumor cells are arranged into
Monomorphic adenoma is divided into three nests sheets islands with minimal connective
groups by WHO, i.e. adenolymphoma (Warthin’s tissue stroma. The cells have a basaloid
tumor), oxyphilic adenoma (oncocytoma) and appearance and resemble basal cell carcinoma.
other histologic patterns like basal cell adenoma Confirmative diagnosis is made on the basis of
and canalicular adenoma. But Warthin’s tumor clinical behavior and degree of dysplasia (Figs
and Oxyphilic adenoma are recognized separate 14.13 and 14.14).
entity nowadays; so only included in mono-
morphic adenoma are basal cell adenoma and
canalicular adenoma.

Basal Cell Adenoma

It was first described by Kleinsasser and Klein in
1967. It is a neoplasm of uniform distribution of
basaloid epithelial cells.
Characteristic features of monomorphic
adenoma are the composition of the tumor by
isomorphic/monomorphic cells distributed in
various pattern of arrangement.

Histogenesis
The isocellular cells resemble the reserve cells of
intercalated duct. So, the histogenetic source
according to Batsakis is suggested to be from these Figure 14.13: Basal cell adenoma showing uniform
basal cells. appearing basaloid cells (B) in supporting stroma (S)
 Salivary Gland Pathology 265

buccal mucosa and lower lip. It is common in

patients in 3rd to 6th decade. There is
predilection in females.
Symptoms: It presents as a slowly growing, well
circumscribed, firm nodule, which is particularly
on the lip and is not fixed. It may be moved through
the tissues for some distance.

Histopathological Features
It is composed of long strands or cords of epithelial
cells, almost arranged in a double row and usually
showing a ‘party wall’ (Figs 14.15 and 14.16).
Figure 14.14: Basal cell adenoma showing cells arranged
in nests Sheets Island (Courtesy: Dr Sangamesh Halawar) Cystic spaces of varying sizes are enclosed by
these cords. The cystic spaces are usually filled
Trabecular pattern: Epithelial cells form narrow by an eosinophilic coagulum. The supporting
cord like strands in a background of loose fibrillar stroma is loose and fibrillar with delicate
stroma. vascularity (Fig. 14.17).

Tubular variant: Here epithelial cells form small Management

round duct like structures which may be in
It can be treated by simple enucleation and
combination with trabecular pattern.
surgical excision. Recurrence rate is rare.
Membranous variant: Multiple large islands of tu-
mor cells are arranged in zig-saw-puzzle pattern. Warthin’s Tumor
These islands are surrounded by hyaline mate- It is also called as ‘adenolymphoma’ and
rial resembling a double basement membrane. ‘papillary cystadenoma lymphomatosum’ and
‘brachial cyst of parotid’. This tumor was first
Management
It is treated by surgical excision and recurrence is
seldom seen.

Canalicular Adenoma
The name itself implies the histologic appearance
of the tumor. The cells are arranged in canalicular
pattern or branching cord like pattern. It is
an uncommon neoplasm of columnar epithelial
cells.

Clinical Features
Figure 14.15: Canalicular adenoma showing varying size
It originates primarily in the intraoral accessory of cystic spaces surrounded loose stroma (Courtesy:
glands. It occurs in upper lips followed by palate, Dr Sangamesh Halawar)
266 Textbook of Oral Pathology

Figure 14.17: Canalicular adenoma showing double row

of cells of showing party wall appearance. Stroma shows
loose stroma. Epithelial chords (EC), Stroma (S)
Figure 14.16: Canalicular adenoma showing anastomosing
narrow cords of epithelium (E) in loose stroma (S)
Hypersensitivity theory: Allegra suggested that it
is most likely a delayed hypersensitivity response.
described by Hildebrad in 1895, later by Albrecht The lymphocytes being an immune reaction to
and Arzt in 1910 reported few cases. The name the salivary ducts which undergo oncocytic
papillary cystadenoma lymphomatosum was change.
given by Warthin in 1929 in USA. Now it is
recognized as Warthin’s tumor. Clinical Features
The tumor shows papillary projection of the The tumor occurs almost exclusively in the
epithelium into the cystic cavity formed in the parotid gland. It always occurs in the lower
existing adenoma. portion of the parotid gland. The tumor is
Thus, it is a cyst in an adenoma showing generally superficial, lying just beneath the
papillary folds of the epithelial lining. parotid capsule or protruding through it. It is
common in men (male to female ratio is 5:1). It is
Development
common in 6th decade.
Heterotopic salivary rest theory: Tumor arises from
Symptoms: The usual complaint is painless slow
salivary gland tissue entrapped with paraparotid
growing tumor over the angle of jaw. Involvement
or intraparotid lymph nodes during embryogen-
may be bilateral or may be multifocal.
esis.
Another theory suggests that there is Signs: The tumor does not attain a large size and
neoplastic proliferation of parotid ductal the usual size is 1 to 3 cm in diameter. It is spherical
epithelium and concomitant secondary in shape. Its surface is smooth and it is well
proliferation of lymphoid tissue. circumscribed, movable. It classically feels
 Salivary Gland Pathology 267

doughy and compressible on palpation. It is firm or it may actually represent a reactive cellular
on palpation and is clinically indistinguishable infiltrate which involves both, humoral and cell
from other benign lesions of parotid gland. mediated mechanism.

Histopathological Features
The tumor is made up of epithelial and lymphoid
tissue. It is an adenoma exhibiting cyst formation,
with papillary projections into the cystic spaces
and lymphoid matrix forming the connective
tissue core of the papillae (Figs 14.18 to 14.20).
The epithelial cells, covering the papillary
projection, are columnar or cuboidal cells usually
arranged in two rows. Outer cell layer is made up
pseudociliated columnar cells with eosinophilic
granular cytoplasm. Nucleus is polarized away
from the basement membrane. The inner layer is
made up of low cuboidal cells.
Basement membrane distinctly separates
epithelium from the lymphoid tissue. There is
frequently an eosinophilic coagulum present
Figure 14.19: Warthin’s tumor showing cystic space (CS)
within the cystic spaces which appears as a containing papillary projection (PP) lined by epithelium (E).
chocolate colored fluid in the gross specimen. The Lymphoid stroma (LS) containing germinal center (GC) is
lymphoid tissue beneath the epithelium shows present beneath the epithelium
germinal center formation.
The abundant lymphoid component may
represent the normal lymphoid tissue of the
lymph node, within which the tumor developed

Figure 14.20: Warthin’s tumor showing cystic space (CS)

and double row of cells. Outer cells (PC) are tall columnar
with nucleus polarized away from basement membrane.
Figure 14.18: Warthin tumor showing cystic space Inner cells (IC) are cuboidal. These cells enclose lymphoid
(Courtesy: Dr Sangamesh Halawar) stroma (LS)
268 Textbook of Oral Pathology

Management Histopathological Features

Superficial parotidectomy and it seldom recurs Oncocytoma is a well circumscribed tumor. It is
after removal. composed of oncocytes, which are large cells with
eosinophilic granular cytoplasm and distinct cell
Oncocytoma membrane. The cytoplasmic granularity is
because of exclusion of other cell organelles and
Oncocytoma is a tumor of oncocytes. Oncocytes their replacement by abundant uniform
are large epithelial cells that contain brightly eosi- mitochondria which makes the cell to appear
nophilic granular cytoplasm. This is due to abun- swollen.
dant mitochondria packing in the cell cytoplasm. Oncocytes are arranged in solid sheets and
It is also called as ‘oxyphilic adenoma’, ‘acido- sometimes tend to be arranged in narrow rows,
philic adenoma’. It is an uncommon tumor com- cords and may demonstrate alveolar or lobular
posing less than 1% of salivary neoplasms. These pattern. These cells exhibit few mitotic figures are
cells are predominately seen in duct lining of closely packed and there is little supportive
glands in elderly persons. stroma. Lymphoid tissue is frequently present.

Pathogenesis Management

As the tumor is exclusively seen in older age Surgical excision should be carried out. Tumor
group it is suggested that the oncocytes are the does not tend to recur. Malignant transformation
result of degeneration of the salivary gland is very rare.
parenchyma.
Another school of thought is that it is a Myoepithelioma
metaplastic process seen in hyperplasia of It is an uncommon salivary gland tumor. Sheldon
salivary parenchyma. This further could in 1943 separated this as a different entity from
proliferate into neoplastic state. pleomorphic adenoma as these tumors were
Some suggest that it is not neoplasm but composed predominantly of basket cells or
merely a nodular hyperplasia. myoepithelial cells.

Clinical Features Clinical Features

It usually occurs in the parotid gland. It is more It occurs in adults and has equal sex distribution.
common in women than in men and occurs Parotid gland is most commonly involved and
almost exclusively in older persons. the palate is the most frequent intraoral site of
occurrence. The clinical features are same as
Signs: The tumor usually measures 3 to 5 cm in
pleomorphic adenoma.
diameter and appears as a discrete encapsulated
painless mass which is sometimes nodular. Pain Histopathological Features
is generally absent.
The tumor is composed of spindle shaped or
Oncocytosis: An interesting condition called plasmacytoid cells or combination of the two cell
‘oncocytosis’ of parotid gland has been described types (Figs 14.21 and 14.22).
which is characterized by nodules of oncocytes These cells may be set in myxomatous
involving the entire gland or a large portion. background, which vary from scanty to copious.
 Salivary Gland Pathology 269

of nonkeratinized epithelium of the columnar type,

supported by cores of vascular fibrous connective
tissue. It is treated by excision, including the base
and it does not recur after completely removed.
Inverted ductal papilloma: It is present as nodules
of the oral mucosa of adults and has no distinctive
clinical features. Histologically it consists of
squamous, cuboidal or columnar epithelium,
which proliferates into salivary duct to form broad
bulbous masses.
Sialadenoma papilliferum: It is a salivary gland
analogue of the syringocystadenoma papilliferum
of skin. The lesion occurs in adults as an
Figure 14.21: Myoepithelioma showing capsule (C) and
myoepithelial cells (EC) producing hyaline eosinophilic exophytic papillary lesion of the hard palate. It
material (E) consists of luminal layer of columnar cells resting
on a cuboidal basal layer.

Mucoepidermoid Carcinoma
The term mucoepidermoid tumor was introduced
in 1945 by Stewart, Foote and Becker. It is most
common salivary gland neoplasm and ranks
second in frequency after pleomorphic adenoma.
It accounts for 6 to 9% of the salivary gland tumors
and for about 1/3rd of all malignant tumors of
the salivary glands. It consists, of both, mucus
secreting as well as epidermoid type of cells as its
Figure 14.22: Myoepithelioma showing plasmacytoid
cells (P) and eosinophilic material (E) name suggests. The biologic behavior of the
mucoepidermoid carcinoma is graded on the basis
Management of clinical and histological picture.

Surgical excision should be carried out. Clinical Features

Ductal Papillomas About 60% occur in parotid gland (Figs 14.23 and
14.24) and 30% in the minor salivary glands,
It is present in three forms: simple ductal
especially those of the palate. Other common
papilloma, inverted ductal papilloma and
intraoral sites are buccal mucosa, tongue and
sialadenoma Papilliferum.
retromolar area. It shows female predilection. It
Simple ductal papilloma: It is present as an commonly occurs in the 3rd and 5th decade. It is
exophytic lesion with a papillary surface and is usually not completely encapsulated and often
pedunculated. It is usually reddish in color and contains cysts which may be filled with viscid
present on the buccal mucosa or palate. It consists mucoid material.
270 Textbook of Oral Pathology

High grade tumor: The tumor of high grade

malignancy grows rapidly and does produce pain
as an early symptom. Ulceration is seen in cases
which have an aggressive clinical course.
Metastasis: It tends to infiltrate the surrounding
tissues and in high percentage of cases it
metastasizes to the regional lymph nodes. Distant
metastases to lungs, bones, brain and
subcutaneous tissue are common.
Much more various findings such as
Figure 14.23: Intraoral ulceration seen on left side due
lacrimation, trismus, nasal discharge, and blood
high grade mucoepidermoid tumor tinged saliva and facial nerve paralysis are seen
in high grade malignant lesions. Intraoral lesions
with bony erosion may show numbness of teeth.

Histopathological Features
The term mucoepidermoid itself signifies that it is
composed of mucus-secreting cells and epidermoid
type cells. Third type of cells commonly seen is
intermediate cells (Fig. 14.25).
Mucous cells are cells with foamy cytoplasm
filled with mucin.
Epidermoid cells are large polygonal cells
with prominent nuclei, distinct cell membranes,
and intercellular bridges. These cells sometimes
show keratin formation. The intermediate cells
are small, round basaloid cells (Fig. 14.26).
These cells are seldom the dominant cell,
although it appears that it may undergo
transformation into either mucus or epidermoid
cells. Sometimes, cluster of clear cells, often in
abundance, may be present which are generally
mucinous and glycogen-free. Ductal proliferation
adjacent to the tumor is common.
Figure 14.24: Mucoepidermoid carcinoma of left parotid
gland showing ulceration of skin These tumors show sheets or nests of
epidermoid cells and similar nests of mucous cells,
usually arranged in a glandular pattern and
Low grade tumor: The tumor of low grade showing microcyst formation (Fig. 14.28). These
malignancy usually appears as a slowly cysts may rupture liberating mucus, which may
enlarging, painless mass, which stimulates pool in the connective tissue and evoke
pleomorphic adenoma. It seldom exceed 5 cm in inflammatory reaction. In such cases lymphocytes
diameter. are seen.
 Salivary Gland Pathology 271

Figure 14.27: Mucoepidermoid carcinoma showing large

Figure 14.25: Mucoepidermoid carcinoma showing cystic areas containing mucin (Courtesy: Dr Sangamesh
presence of clear cells (CC) Halawar)

Figure 14.26: Low grade mucoepidermoid carcinoma

showing presence of mucous cells (MC) and epidermoid
cells (EC) in equal proportions. Large mucous containing
cystic space (CS) are seen

Tumor is graded on the basis of following

factors: Figure 14.28: High grade mucoepidermoid carcinoma
• Degree of cystic transformation of the tumor showing predominate epidermoid cell (EC) only few mucous
• Degree of cellular atypia cells (MC) and few cystic spaces (CS)

• Proportion of epidermoid and mucous cells.

of intermediate cells although areas of mucous
It graded into three types:
and epidermoid cells are seen. Cellular atypia is
Low grade: It predominantly shows mucous cells minimal.
and abundant extracellular mucin. Several cystic
High grade: The predominant cellularity is of
areas are seen in the tumor mass.
epidermoid cells with varying dysplastic
Intermediate grade: The cystic transformation is less features. These cells show considerable cellular
as compared to low grade. There is predominance atypia.
272 Textbook of Oral Pathology

Management fazelle proposed the term adenoid cystic

carcinoma in 1953. It is a well defined entity which
Surgical excision followed by radiotherapy is
occurs in the major salivary glands and
recommended for intermediate grade tumors and
intraorally mostly in the palate.
high grade tumors. Low grade tumors can be
managed by surgery alone.
Pathogenesis
Central Mucoepidermoid Carcinoma The ultrastructural studies have confirmed that
the tumor originates from ductal and
It is an epithelial tumors originating in the bone
myoepithelial cells.
and is believed to be derived from salivary gland.
Some workers have noted four cell types:
• Intercalated
Origin
• Secretory
It may originate from entrapment of retromolar • Myoepithelial
mucous glands within the mandible, which • Plutipotential reserve cells.
subsequently undergo neoplastic transformation.
It may also form from developmentally included Clinical Features
embryonic remnants of the sub-maxillary gland
Most common glands involved are the parotid,
within the mandible and neoplastic
submaxillary and the accessory glands in palate
transformation of the mucous secreting cells
and tongue. It occurs in the 5th and 6th decades
commonly found in the epithelial lining of
of life.
dentigerous cyst.
Symptoms: The most common initial symptom is
Clinical Features presence of mass followed by local pain, facial
It is more common in females than males. It nerve paralysis in case of parotid tumor and
tenderness. The symptoms may vary like nasal
occurs in mandible in premolar-molar area. It
obstruction, proptosis, sinusitis, ear infection,
does not extend anteriorly beyond the premolar
region. Patient complains of painless swelling. epistaxis, signs of cranial nerve involvement and
visual disturbances.
There may be paresthesia of inferior alveolar
nerve. Swelling may cause facial asymmetry. Signs: Fixation to skin and to surrounding
Tenderness is present. Regional lymph nodes are structures may develop. Some of the lesions
enlarged. exhibit surface ulceration. Other sites where the
adenoid cystic carcinoma can occur is in the
Management mucous glands of paranasal sinuses, luminal
It is treated surgically with en-block resection. glands of the ear and sweat glands.
Neck dissection and postoperative radiation
therapy may be required for control of nodal Histopathological Features
disease.
The tumor is composed of uniform or isomorphic
cells arranged in various patterns. The cells are
Adenoid Cystic Carcinoma
small, deeply staining, having basophilic nuclei
It is also called as ‘cylindroma’, ‘adenocystic having scanty cytoplasm. The different
carcinoma’ and ‘baseloid mixed tumor’. Foot and morphologic patterns seen in this tumor are:
 Salivary Gland Pathology 273

Cribriform or Swiss cheese pattern: This is the classic

and most common pattern which shows
arrangement of tumor cell in ‘Swiss cheese
pattern’. The cells are arranged in small nests of
variable size and shape that contain many
circular or ovoid spaces. These spaces impart the
Swiss cheese pattern. Eosinophilic or mucinous
hyalinized material may be seen in the spaces.
This pattern is also called as honey comb pattern
(Figs 14.29 and 14.30).
Tubular pattern: Tumor cells are similar to those of
the cribriform pattern. Only arrangement differs.
Duct like spaces are seen in the solid nests of the Figure 14.30: Adenoid cystic carcinoma showing tubular
tumor. Cribriform pattern may co-exist with this and cribriform pattern (Courtesy: Dr Sangamesh Halawar)

type of cell arrangement (Fig. 14.31).

Solid pattern: Cells are arranged in nests, sheets of
varying size and shape. The ovoid or duct like
spaces is minimal (Fig. 14.32).
Cylindroma: The stromal connective tissue
becomes hyalinized and surrounds the tumor
cells, forming a structural pattern of cylinder due
to which the tumor is named as ‘cylindroma’.
Perineural invasion: Another characteristic feature
of this tumor is that the tumor invades the
Figure 14.31: ACC showing tubular pattern (T).
perineural spaces of the peripheral nerves (Figs
Hyperchromatic epithelial cells (E) showing no dysplasia
14.33 and 14.34). This is called as neurotropism. form ducts (D)

Figure 14.29: Adenoid cystic carcinoma showing Figure 14.32: Solid pattern of ACC showing sheets of
presence of cribriform pattern uniform appearing cells
274 Textbook of Oral Pathology

Figure 14.35: High power view of adenoid cystic carcinoma

solid type-showing cells arranged in sheets. Cells are
hyperchromatic with little atypia. Few cystic spaces are
Figure 14.33: Characteristic feature of ACC is perineural seen
invasion by tumor cells (TC); (N) Nerves
Acinic Cell Carcinoma
Acinic cell carcinomas are made up of well
differentiated acinar cells. It is also called as
‘acinic cell or serous cell adenoma’.

Histogenesis
It has been suggested that the tumor arises from
the acinar cells. Few suggest that it originates from
cells of the terminal salivary gland ducts.

Clinical Features
It accounts for approximately 1% of all salivary
gland tumors. It occurs in middle age and twice
common in women. It arises exclusively in the
superficial lobe and tail of the parotid gland. The
Figure 14.34: Low power view of adenoid cystic carcinoma most common intraoral sites are the buccal
solid pattern showing large islands or sheets of tumor cells mucosa and lip.
with little tendancy for cyst formation
Symptoms: It is painless and grows slowly.
Signs: Size of lesion varies from 2-4 cm. Exact
Management
delineation of the lesion is difficult and
Surgical and in some cases it is accompanied by attachment to the overlying skin and muscle may
X-ray radiation. Recurrence rate is about 60 to 92 occur. Some of these lesions run a rapid course
%. Long-term follow-up is hence essential. The with hematogenous and lymphatic metastases,
incidence of metastases is more and the organs while others are more slowly progressive. Locally
involved include cervical lymph nodes, lungs, invasive growth may be encountered in some
brain, liver and kidneys. lesions.
 Salivary Gland Pathology 275

It resembles to pleomorphic adenoma and is Adenocarcinoma not otherwise Specific

encapsulated and lobulated in appearance. It is group of unclassifiable lesions. These tumors
Histopathological Features show different growth patterns. It is a malignant
epithelial tumor showing tubules or papillary
The tumor may be well differentiated and less glandular formation. All adenocarcinoma are
differentiated. Well differentiated cells bear
highly malignant and metastasize to regional
remarkable resemblance to normal acinar cells (Fig.
lymph nodes and general viscera.
14.36). Less differentiated cells resemble embryonic
ducts and immature acinar cells. The cells are
Malignant Pleomorphic Adenoma
uniform acinar type cells with dark, round eccentric
nuclei and a basophilic or amphophillic It is also called as ‘malignant mixed tumor’.
cytoplasm. The cells may also contain dark Malignant pleomorphic adenoma involves:
cytoplasmic granules. Despite being a malignant • Carcinoma ex-pleomorphic
tumor mitotic figures are less. It is surrounded by a • Carcinosarcoma
thin capsule, may be composed of cells of varying • Metastasizing pleomorphic adenoma.
degrees of differentiation. The tumor cells can be
arranged in solid masses or in acini like groups. Carcinoma ex-pleomorphic Adenoma
There are four types of growth pattern seen:
Carcinoma ex-pleomorphic adenoma is a tumor
solid, papillary-cystic, follicular and microcystic.
in which malignant transformation is seen in an
Clear cells are also seen sometimes which is
existing benign pleomorphic adenoma.
labeled as clear cell acinic cell carcinoma.
The malignant neoplasia is seen in the
Lymphoid elements are commonly found in
epithelial component of the mixed tumor. The
parotid acinic carcinomas.
epithelial islands shows the dysplastic features
Management such as cellular anaplasia, pleomorphism,
mitosis, abnormal architecture, and destruction
Surgical excision is done. Recurrence rate varies
of normal tissues.
form 8 to 59%.
Other criteria for malignancy are also seen in
the clinical behavior of the tumor such as
invasiveness, induration, fixation to underlying
tissues and metastasis. It is important to note that
when carcinoma ex mixed tumor metastasizes the
benign component is not seen in the metastasis.
It is uncertain that these tumors represent the
previously benign lesions which have undergone
transformation into malignant form or are
malignant lesion right from the onset.

Clinical Features
It accounts for 1% of all parotid tumors and 7% of
all malignant tumors. The tumor occurs from
2nd to 9th decades, but most frequently in 5th
Figure 14.36: Acinic cell carcinoma showing clear cells and 6th decades. The average age of patients
arranged acinar pattern with malignant pleomorphic adenoma is about
276 Textbook of Oral Pathology

ten years older than the patients with benign form Histopathology
of the disease. • PLGA is characterized by infiltrative growth
The tumors are usually larger than benign ones. with diverse morphological features.
There is often fixation of the tumor to underlying • Variety of growth patterns observed growth
structures as well as to overlying skin or mucosa. patterns may be solid, ductal, cystic and
Pain is more frequently a feature of malignant, than tubular (Fig. 14.39)
the benign pleomorphic adenoma. • Tumor composed of cuboidal to columnar
Histopathological Features isomorphic cells that have uniform ovoid to
spindle shaped nuclei.
In some cases malignant component may • Tumor stroma varies from mucoid to hyaline
overgrow the benign component so that the and sometimes fibrovascular.
benign component is difficult to demonstrate and • Perineural invasion noted in few cases
in some cases benign component is more with
few malignant foci may be found. Treatment : PLGA is best treated by conservative
There is presence of nuclear hyperchromatism wide surgical excision.
and pleomorphism, increased or abnormal
mitosis and destruction of normal tissues. There
is destructive infiltrative growth at the periphery,
with excessive hyalinization (Figs 14.37 and
14.38).

Management
Surgical excision should be carried out. These
neoplasms exhibit a high recurrence rate after
surgical removal as well as a high incidence of
regional lymph node involvement.

Polymorphous Low Grade Adenocarcinoma

(PLGA)
PLGA is a malignant epithelial tumor that is most Figure 14.37: Malignant pleomorphic adenoma showing
cellular atypia
commonly seen in minor salivary gland sites. The
tumor is characterized by bland, uniform nuclear
characteristics, infiltrative growth pattern
perineural infiltration.

Clinical Features
• It is most commonly seen at minor salivary
gland site
• Age of occurrence: Older age group
• Sex: Female > Males
Clinical presentation is usually a firm, non-
tender swelling involving mucosa of the hard Figure 14.38: Malignant pleomorphic adenoma
and soft palates, check mucosa or the upper Iip. (Courtesy: Dr Sangamesh Halawar)
 Salivary Gland Pathology 277

carcinomas infiltrate the surrounding structures

and readily metastasize to cervical lymph nodes.

Metastatic Carcinoma
Salivary glands may be a site for tumor metastases,
since the parotid gland contains as many as 20 to
30 lymph follicles. Parotid and paraparotid
lymph nodes are favored sites for metastases from
malignancy of the temple, scalp and ear and
occasionally from the face, neck and palate. The
most common metastasizing tumors are
melanoma and squamous cell carcinoma.
Figure 14.39: Polymorphic low grade adenocarcinoma
(Courtesy: Dr Sangamesh Halawar) Connective Tissue Tumors
Hemangioma is the only common tumor of this
Carcinosarcoma group and the most frequently seen in young
Carcinosarcoma is considered as true malignant infants. The involve gland appears hypertro-
mixed tumor in which both the epithelial and phied. There is blue discoloration of the overlying
stromal components fulfill the histologic criteria skin. Lipoma may occur in the parotid gland. The
for malignancy. When these tumors metastasize facial nerve, occasionally gives rise to a neural
both the components are seen in the metastasis tumor. True sarcoma is extremely rare.
similar to the primary lesion.
Epithelial Myoepithelial Carcinoma
Metastasizing Pleomorphic Adenoma The epithelial myoepithelial carcinoma of
Metastasizing pleomorphic adenoma was intercalated duct is a low grade salivary gland
initially called as “benign metastasizing mixed tumor. It was first described by Donath et al (1972).
tumor”. This tumor for unknown reasons EMC occur as single well circumscribed firm
metastasizes although histologically both lobulated neoplasms of size 2 to 8 cm. They show
epithelial and stromal components are benign in a multinodular growth pattern with irregular
appearance. cystic spaces. The tumor is less infiltrative.
It is a tumor composed of tubular structures
Epidermoid Carcinoma that are lined by two cellular forms—outer clear
It is also called as ‘squamous cell carcinoma of cells resembling myoepithelial cells with rich
salivary glands’. It is thought to be ductal in origin, glycogen, and inner layer of darker cells
since duct may undergo squamous metaplasia resembling the intercalated duct epithelial cells.
with ease. It exhibits infiltrative properties and early This cellular appearance of the tumor is called as
metastasis. It recurs readily. It is not a common biphasic growth pattern.
lesion and arises more frequently in the major
salivary gland. The combined use of surgery and Basal Cell Adenocarcinoma
radiotherapy is more beneficial. Basal cell adenocarcinoma is carcinomas arising
separately as a different entity than monomor-
Undifferentiated Carcinoma phic adenoma. Some authors believe that it is
The tumor is composed of round or spindle cells nothing but malignant counterpart of basal cell
that are poorly differentiated. Undifferentiated adenoma showing arrangement of cells in differ-
278 Textbook of Oral Pathology

ent patterns such as solid, trabecular and mem- erature. It was initially called as mucous cell ad-
branous but in malignant fashion (Fig. 14.40). enocarcinoma, mucous producing adenocarci-
noma, and now mucinous adenocarcinoma.
Sebaceous Carcinoma
Oncocytic Carcinoma
Sebaceous carcinomas are very uncommon
It is a tumor characterized by oncocytes that
tumors having incidence of 0.05% of all salivary
demonstrate histopathological features similar
gland tumors. It is a tumor composed
with adenocarcinoma or clinical evidence of
predominantly of sebaceous cells of varying
metastasis or both. This tumor is one of the least
maturity that are arranged in sheets or nests
common types of all malignant salivary gland
showing different degrees of cellular dysplasia.
tumors. It is characterized by large polyhedral or
Characteristic feature of this is that the tumor has
round cells either eosinophilic granular
biphasic age distribution with peak incidence in
cytoplasm arranged in different patterns showing
3rd, 7th and 8th decades.
malignant histopathological features.
Papillary Cyst Adenocarcinoma
Salivary Duct Carcinoma
These are characterized by large cystic structures
As most of the salivary gland tumors arise from the
with epithelial linings with papillary growth
ductal system many authors believe that salivary
pattern. In some cases the papillary growth
duct carcinoma is not a separate entity but a form of
pattern may be absent which may labeled as cyst
adenocarcinoma. Histologically salivary duct
adenocarcinoma.
carcinoma is composed of epithelial cells arranged
Mucinous Adenocarcinoma in ductal pattern showing intraductal, infiltrative
growth. The basic pattern of arrangement may in
Mucinous carcinoma of the salivary gland is a
solid or cribriform pattern. ‘Comedo necrosis’ is one
rare tumor which is not very well defined in lit-
of the characteristic features.

Small Cell Carcinoma

It is a pulmonary neoplasm which rarely arises
in head and neck region. It has been reported to
arise in the paranasal sinuses, oral cavity, and
pharynx and rarely in salivary glands. They
present as rapidly growing firm swellings in the
parotid gland. They may grow upto 8 cm within
a period of three months. Histologically, they are
composed of infiltrating large sheets, ribbons,
cords or nest of anaplastic round to oval cells with
minimal cytoplasm and hyper chromatic nuclei.

MINOR SALIVARY GLAND TUMORS

Early minor salivary gland tumor is usually
nodular or dome shaped elevation, with smooth
contour. Overlying mucosa is normal or appears
Figure 14.40: Basal cell adenocarcinoma showing cells
arranged in islands. Cells have basaloid appearance smooth and glossier, due to tension. After trauma
 Salivary Gland Pathology 279

ulcer appears which is persistent and becomes Clinical Features

necrotic. Occurrence in an posterior aspect of hard It is more common in males than females. Occurs
palate; next commonly on upper lip, buccal mucosa, in 4th and 5th decade. Most of the cases occur in
retromolar region, tongue and floor of mouth. palate, although cases of lip or retromolar pad
Higher frequency in women with age 30 to 39 for also have been reported. There may be early mild
benign and 40 to 49 for malignant tumors. swelling to more advanced and cancerous
Mucoepidermoid and adenocarcinoma are appearing ulcer.
common variety. Lung is the common site of
Symptoms: It is usually painless or may cause only
distant metastasis.
slight pain. The patient may have numbness in
Necrotizing Sialometaplasia the palate, or area of ‘looseness’ in palate. Pieces
of tissue may fall out from the palate. There may
It is a non-neoplastic inflammatory self-healing be referred pain to ear or pharynx.
reaction of salivary gland tissues, which both
Signs: The lesion begins as a larger ulcer or
clinically and histologically mimics a salivary
ulcerated nodule, which may be unilateral or
gland malignancy. It usually affects the minor
bilateral. The ulcer is well demarcated from
salivary glands. It was predicted that trauma
surrounding normal tissue and often has an
caused ischemia of the minor salivary glands. This
inflammatory reaction around the edge of the
benign self-limiting lesion has been often
lesion. Margins of ulcer in some cases may be
confused with malignancy thereby causing
indurated and inflamed. The lesion itself is most
unnecessary surgery.
often a deep, craterlike, non draining ulcer 1 to 3
Etiology and Pathogenesis (Flow chart 14.1) cm in diameter. The lesion is covered by an
inflammatory exudate and necrotic debris
Local ischemia, which may be due to physical,
however the margins of ulcer are usually clean,
chemical, infective or local vasculitis. Trauma
sharp and the granulation tissue often can be seen
form various factors such as denture wearing and
at the superficial aspect.
recent surgery may be a causative factor. In some
cases, smoking and alcohol intake can lead to this Histopathological Features
condition.
It is characterized histologically by an ulcerated
Flow chart 14.1: Pathogenesis of necrotizing mucosa, pseudoepitheliomatous hyperplasia of
sialometaplasia the mucosal epithelium, acinar necrosis and
squamous metaplasia of salivary ducts. There is
preservation of the lobular architecture, despite
necrosis and inflammation. Inflammatory cells
may be found in and around the lobular areas of
necrosis. Granulation tissue and fibrosis present
in variable amounts.

Management
It is a self-limiting condition. It does not require
any treatment. The healing occurs in six to twelve
weeks via secondary intention. Debridment and
saline rinses may aid the healing process.
280 Textbook of Oral Pathology

CHAPTER

15 Odontogenic Tumors

Odontogenic tumors are group of closely related Ectomesenchyme is the primitive connective
neoplasms that originate from tooth forming tissue surrounding the tooth germ or enamel
apparatus and its remnants. Though the term organ. The primitive connective tissue comes
tumor is used for all lesions or growths from neural crest cells. These migrate to below
originating from the odontogenic tissue, they are the ectoderm along pathways and enter the facial
considered a heterogenous group ranging from region, and form ectomesenchyme. The
hamartomatous or non-neoplastic proliferations neoplasms include the primitive connective
to malignant tumors with metastatic capacities. tissue components of the odontogenic apparatus.
They are derived from epithelial,
ectomesenchymal, or mesenchymal elements of Classification of Odontogenic Tumors
the odontogenic apparatus. Epithelial odontoge- Odontogenic tumors are classified mostly
nic tumors are histologically related to remnants according to the tissue of origin they belong or
of the odontogenic epithelium, which includes: resemble. There are various schemes of classifying
• The dental lamina, odontogenic tumors (Tables 15.1 to 15.4).
• The enamel organ, and Newer WHO classification has included
• The root sheath of Hertwig. odontogenic keratocyst in tumors as it is

Table 15.1: Shafer classification (1984)

Ectodermal tumors of odontogenic origin Mesodermal tumors of odontogenic origin Mixed tumors of odontogenic origin
• Enameloma • Peripheral odontogenic fibroma • Ameloblastic fibroma
• Ameloblastoma • Central odontogenic fibroma • Ameloblastic fibrosarcoma
• Primary intra-alveolar epidermoid • Odontogenic fibrosarcoma • Ameloblastic fibro-odontoma
carcinoma
• Calcifying epithelial odontogenic tumor • Odontogenic myxoma • Odontoma
• Adenomatoid odontogenic tumor • Periapical cemental dysplasia • Ameloblastic odontoma
• Squamous odontogenic tumor • Central cementifying fibroma • Teratoma
• Benign cementoblastoma
• Gigantiform cementoma
• Dentinoma
 Odontogenic Tumors 281

Table 15.2: Histological classification of odontogenic tumors (World Health Organization classification, 1992)
Benign odontogenic tumor
Odontogenic epithelium without Odontogenic epithelium with odontogenic Odontogenic ectomesenchyme with or
odontogenic ectomesenchyme ectomesenchyme, with or without dental without included odontogenic epithelium
hard tissue
Ameloblastoma Ameloblastic fibroma Odontogenic fibroma
Squamous odontogenic tumor Ameloblastic fibrodentinoma Myxoma (Odontogenic myxoma, myxofibroma)
Calcifying epithelial odontogenic Ameloblastic fibro-odontoma Benign cementoblastoma
tumor (Pindborg tumor) Odontoameloblastoma
Clear cell odontogenic tumor Adenomatoid odontogenic tumor
Calcifying odontogenic cyst
Complex odontoma
Compound odontoma
Malignant odontogenic tumor
Odontogenic carcinomas Odontogenic sarcoma
Malignant ameloblastoma Ameloblastic fibro sarcoma Odontogenic carcinosarcoma
Primary intra-osseous carcinoma Ameloblastic fibrodentinosarcoma and
Malignant variants of other Ameloblastic fibro-odontosarcoma
odontogenic epithelial tumors
Malignant changes in
odontogenic cysts

Table 15.3: Histological classification of odontogenic tumors (World Health Organization classification, 2003)
Benign tumors
Odontogenic epithelium with Odontogenic epithelium with odontogenic Mesenchyme and/or odontogenic
mature, fibrous stroma; ectomesenchyme with or without ectomesenchyme with or without
odontogenic ectomesenchyme dental hard tissue formation included odontogenic epithelium
not present
• Ameloblastoma • Ameloblastic fibroma • Odontogenic fibroma
– Solid/Multicystic • Ameloblastic fibrodentinoma • Myxoma (Odontogenic myxoma,
– Extraosseous/Peripheral • Ameloblastic fibro-odontoma myxofibroma)
– Desmoplastic • Complex odontoma • Benign cementoblastoma
– Unicystic • Compound odontoma
• Squamous odontogenic tumor • Odontoameloblastoma
• Calcifying epithelial odontogenic tumor • Calcifying cystic odontogenic tumor
• Adenomatoid odontogenic tumor • Dentinogenic ghost cell tumor
• Keratinizing cystic odontogenic tumor
Malignant tumor
Odontogenic carcinomas Odontogenic sarcomas
• Metastasizing, malignant ameloblastoma Ameloblastic fibrosarcoma
• Ameloblastic carcinoma Ameloblastic fibrodentinosarcoma
– Primary and fibro-odontosarcoma
– Secondary (dedifferentiated), intraosseous
– Secondary (dedifferentiated), extraosseous
• Primary intraosseous squamous cell
carcinoma (PIOSCC)

considered as benign cystic neoplasm. AOT was Ectodermal tumors of odontogenic origin
included in the previous classification under the • Enameloma
category of mixed tumor but recently is • Ameloblastoma
considered as tumor arising from odontogenic • Primary intra-alveolar epidermoid carcinoma
epithelium. • Calcifying epithelial odontogenic tumor
282 Textbook of Oral Pathology

• Adenomatoid odontogenic tumor Tooth is derived from complex interactions

• Squamous odontogenic tumor between ectoderm of the oral cavity and
Mesodermal tumors of odontogenic origin ectomesenchyme. Ectomesenchyme of oral
• Ameloblastic fibroma cavity comes from neural crest cells. These are
• Ameloblastic fibro sarcoma cells derived from neural fold which is formed
• Ameloblastic fibro-odontoma from ectoderm. Certain cells at the tip of this fold
• Odontoma are loose and mobile and have an ability to
• Ameloblastic odontoma migrate. The ectoderm migrate along pathways
• Teratoma
and enter the developing facial region.
Table 15.4: Another classification of odontogenic tumor After reaching the facial region neural crest
I. Benign cells initiate proliferation of oral epithelium at
A. Epithelial certain areas forming a thick epithelial band.
• With inductive changes in the connective tissue This band forms horse-shoe shaped epithelial
– AOT
– Ameloblastofibroma sheet in both the arches. The free end of the arch
– Dentinoma gives two extensions in mesiolateral direction.
– Calcifying odontogenic tumor
The sheet present laterally is known as
– Odonto-ameloblastoma
– Odontoma vestibular lamina and mesial sheet is known as
• Without inductive changes in the connective tissue dental lamina. Growth of dental lamina proceeds
– Ameloblastoma
– CEOT
and results in formation of rounded localized
– Epithelial atypia growth of epithelial cells called as tooth buds.
– Ameloblastic changes in odontogenic cyst Initially 20 tooth buds corresponding to
B. Mesenchymal
• Odontogenic myxoma
deciduous tooth germs develop. Dental lamina
• Odontogenic fibroma gives lingual extension, lingual to the
• Cementoma developing deciduous teeth and forms tooth
• Periapical cemental dysplasia
• Cementifying fibroma buds for all permanent teeth except molars.
• Benign cementoblastoma Molars develop from a direct posterior extension
II. Malignant of dental lamina.
A. Epithelial
• With inductive changes in connective tissue
– Ameloblastic fibrosarcoma Stages of Tooth Development
– Ameloblastic odontosarcoma
• Without inductive changes in connective tissue Tooth development is continuous process. During
– Malignant ameloblastoma development tooth germ takes various shapes.
– Primary intraosseous carcinoma
– Malignant changes in odontogenic cyst Based on these shapes tooth development is
classified into three stages.
Development of Tooth
Dental Lamina Formation
To understand the histogenesis of the
odontogenic tumors, the knowledge of process Certain areas of basal cells of the oral ectoderm
of tooth development is mandatory. proliferate more rapidly than cells of adjacent
area. This leads to formation of dental lamina,
Odontogenesis which is a band of epithelium that has invaded
It is a highly coordinated and complex process underlying ectomesenchyme along each of the
which relies upon cell to cell interactions that horse-shoe shaped future dental arch. Total
result in the initiation and generation of the tooth. activity of dental lamina formation extends
 Odontogenic Tumors 283

atleast over a period of 5 years. The remnants of Four different types of cell are distinguishable
dental lamina persist as epithelial pearls or which are as follows:
islands within the jaw as well as in the gingiva. • Cells of inner enamel epithelium.
• Stratum intermedium.
Bud Stage (round-shaped epithelial condensation) • Stellate reticulum.
At this stage there is no histodifferentiation or • Outer enamel epithelium.
The cells of inner enamel epithelium differ-
morphodifferentiation. Cells are highly
entiate into ameloblast prior to amelogenesis.
proliferative. There is condensation of
ectomesenchyme cells which also proliferate Enamel knot—The cells in the center of the enamel
without differentiation. Peripheral epithelial cells organ are densely packed and form the enamel
are low columnar and central cells are polygonal. knot.

Cap Stage Enamel cord—At the same time, there arises a

vertical extension of the enamel knot, which is
‘Cap’ shape of enamel organ due to invagination called as the enamel cord.
or ‘pushing’ of ectomesenchyme into the tooth These cells are attached to one another by
bud. Tooth bud develops concavity which junctional complex laterally and to cells in
becomes occupied by mesenchymal cells called stratum intermedium by desmosomes.
as dental papilla. The cells of inner enamel epithelium exert an
In this stage morphogenesis of tooth begins organizing influence on the underlying
and continuous till hard tissue formation begins. mesenchymal cells in dental papilla, which then
Histodifferentiation also begins at this stage with differentiate into odontoblasts. The basement
tooth germ composed of four distinct cell layers. membrane that separates the enamel organ and
• Outer enamel epithelium—Layer of cuboidal dental papilla just before dentin formation is
cells covering outer surface of tooth bud. called as ‘membrana preformativa’.
• Inner enamel epithelium—Single layer of low
columnar cells at the concavity of cap. Advanced Bell Stage
• Stratum intermedium—Several layers of
During the advanced bell stage, the boundary
polygonal cells present over inner enamel
between inner enamel epithelium and
epithelium.
odontoblasts outline the future dentinoenamel
• Stratum reticulum—Many layers of polygonal
junction. In addition, cervical portion of enamel
cells occupy space between stratum
organ gives rise to epithelial root sheath of
intermedium and outer enamel epithelium.
Hertwig.
Dental follicle or sac: A layer of mesenchymal cells • 32nd week — dentin and functional
which surrounds the tooth bud and dental ameloblasts of permanent 1st molar.
papilla. This layer gives rise to periodontal Probable sources of epithelium for
ligament. development of odontogenic tumors are shown
in (Fig. 15.1) . They are as follows:
Bell Stage
As the invagination of epithelium deepens and Cell Rest of Malassez and Serrae
its margins continue to grow, the enamel organ The cells rests of Malassez are the remnants of the
assumes a bell shape. Hertwig’s epithelial root sheath. These are the
284 Textbook of Oral Pathology

Figure 15.1: Probable sources of epithelium for development of odontogenic tumors

only odontogenic epithelial cells that remain in tumors especially Adenomatoid odontogenic tumor
the periodontium after the eruption of teeth. and odontogenic keratocyst.
Cell rests of Serrae are remnants of dental
lamina. They are usually present in the gingiva Pericoronal Dental Follicle
few may be present in the periodontal ligament. Dental follicle is a soft delicate tissue which
These cells are believed to behave in a manner covers the crowns of unerupted teeth. This dental
similar to cell rests of Malassez. follicle helps in the protection and eruption of
They possess an inherent potential to newly formed tooth.
diversely transform towards various tumor lines Dental follicles are comprised of nests of
by exerting inductive influence on the adjacent odontogenic epithelium probably derived form
connective tissue. They are believed to give rise the dental lamina, and the reduced enamel
to most of the peripheral odontogenic tumors. epithelium, supported by ectomesenchyme of
Few epithelial rests which are derivatives of developed tooth.
distal extension of dental lamina are present in the It was observed that nests have a greater
molar region. These also give rise to odontogenic proliferative capacity than the cells of the

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 Odontogenic Tumors 285

reduced enamel epithelium and thus they are This may be true in case of tumor that develops
believed to be responsible for development of in first and second decade of life.
odontogenic tumors.
As age advances chances of alteration in Heterotopic Epithelium
pericoronal tissue increases. In advanced age of
Ameloblastoma like tumors are seen in sites
over 40 years it is common to find odontogenic other than jaws. Craniopharyngioma or pituitary
carcinomas in pericoronal tissue. Dental follicle
ameloblastoma arises from craniopharyngeal
of impacted tooth remains in the oral cavity for
duct, a derivative of Rathke’s pouch a deriva-
a very long time. So it is exposed to various tive of oral epithelium. In long bones a tumor
pathological processes for long time. This may
similar to ameloblastoma called Adamantinoma
be one of the explanations why many
develops. But other than resemblance to amelo-
odontogenic tumors are common in molar area blastoma it is not related to it. Pilomatrixoma is
of mandible.
another tumor that shares few features with
The commonly occurring lesions are amelo-
CEOT. It is thought to originate from hair fol-
blastoma, carcinomas, calcifying odontogenic licles.
cysts, calcifying epithelial odontogenic tumor,
and odontogenic myxoma.
Basal Cells of Oral Epithelium
Cyst Lining Tooth germs are developed from oral ectoderm
as a down growth into the connective tissue. Few
It is thought that epithelial lining of odontogenic
cyst is a source of epithelium for a variety of basal cells of oral epithelium retain their
pluripotentiality and in some cases may give rise
odontogenic tumor. This contention is supported
to tumors in adults. Few peripheral odontogenic
by the fact that both cyst and tumor develop from
same odontogenic epithelium, so transition from tumors appear as down growth of basal cells.
In few cases epithelial proliferation known
cyst to tumor is possible.
as odontogenic epithelial hamartias [(OEH: also
Dentigerous cyst is believed to give origin to
ameloblastoma. Calcifying epithelial odontoge- known as odontogenic gingival epithelial
hamartias (OGEH)] believed to be an
nic cyst exists in spectrum where tumor is one
intermediate stage in development of tumors.
extreme end and simple cyst forms the other end.
Few AOT’s are reported in CEOC lining.
Adenomatoid odontogenic tumor has a Gubernacular Dentis
dentigerous relation with the tooth, so it was also
Gubernacular dentis is fibrous tissue that forms
believed to originate from dentigerous cyst.
connection between developing permanent teeth
Unicystic ameloblastoma is a believed to be a and lamina propria of gingiva. These occupy
cystic counterpart of ameloblastoma.
gubernacular canals found in dried skull.
Odontogenic karatocyst is now classified as
Gubernacular dentis contains central strand of
a tumor rather than as a cyst by WHO. epithelial cells derived from the dental lamina,
surrounded by the connective tissue.
Tooth Germ
This connective tissue is organized in two
The similarity between tooth germ and layers. In inner layer collagen fibres show greater
odontogenic tumors has led to the belief that organization and run mainly parallel to long axis
odontogenic tumor develop from tooth germ. of the chord. Outer layer is made up of fewer

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286 Textbook of Oral Pathology

collagen fibres which are less organized. It is Molecular Pathology of Odontogenic Tumors
more vascular than the inner layer. Exact etiology and pathogenesis of odontogenic
It is thought to play a role in eruption of the tumors is unknown. With evolution of immu-
permanent teeth. Epithelial cells occur more nohistochemistry and other advanced diagnos-
numerously in the inner layer like ‘pearls on tic techniques it has been found that various
string’. It hypothesized that these epithelial cells molecules and genes are altered in odontogenic
undergo proliferation to form odontogenic tumors. A series of genetic and molecular alter-
tumors and hamartomatous lesions. But what ations appear to promote the development and
initiates these cells is not known. progression of tumors via multiple steps.
It is thought that different variants of Molecules possibly associated with tumorigenesis and/
adenomatoid odontogenic tumor are derived or tumor cell differentiation (Modified from
from gubernacular dentis. H. Kumamoto)

Molecules involved in tumor genesis and differentiation

Oncogenes Normal cellular genes which promote contribute to neoplastic transformation

oncogenesis if they become overactivated
Ras, Required for functioning of growth factors Expressed in odontogenic epithelial cells
Myc Regulates cell proliferation Expressed in parabasal epithelial cells of
ameloblastoma
Fos Regulates cell proliferation and differentiation Gene altered in ameloblastoma-tumor
genesis via dysregulation of cell proliferation.
Tumor suppressor genes These inhibit cell proliferation. Loss of genes
leads to loss of control of cell proliferation
leading to tumorigenesis
p53 It is called as guardian of genome. It and its Increased expression in ameloblastomas,
products control DNA damage, help in repair malignant ameloblastomas, primary
of damaged DNA, control cell proliferation and intraosseous carcinomas, and ameloblastic
cell death. fibrosarcomas.
Regulators of p53 like MDM, p14ARF are
also expressed in odontogenic tumors
APC This normally inhibits cell proliferation. Decreased APC expression is possibly
Loss of function leads to tumorigenesis involved in oncogenesis orcyto-
differentiation of odontogenic epithelium.
Retinoblastoma Controls cell cycle progression Transgenic mice develop ameloblasstoma
DNA-repair genes Abnormality in these genes leads to genetic Development and progression of these tumors
(hMSH2 and hMLH1) instability. do not depend on a defect in the DNA
repair system.
Growth factors Key regulators of cell growth differentiation TGF-α and EGFR are involved in odontogenic
and proliferation tumorigenesis
TGF-α, -β, FGF-1, -2, TGF-β, HGF regulate epithelial–mesenchymal TGF-β, HGF regulate or dysregulate
interactions epithelial–mesenchymal interactions
HGF HGF essential for morphogenesis of tooth HGF is associated with the malignant
germs potential of epithelial odontogenic tumors
Telomerase Anti-aging enzyme. It is responsible for cell Telomerase activation is associated with
immortalization tumorigenesis of odontogenic epithelium
Contd...

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 Odontogenic Tumors 287

Contd...
Cell cycle regulators Consist of genes and proteins and involved in Proliferation of odontogenic epithelial cells is
regulation of progression in the cell cycle. strictly controlled by these cell cycle regulators
Cyclin D1, p16INK4a, Cyclins, cyclin dependent kinases (CDK)
p21WAF1/ Cip1, and CDK inhibitors (CDKI) are these regulators.
and p27Kip1
Cell proliferation Indicators of cell proliferation. Reflect proliferation activity and
markers malignant potential
PCNA, H3, DNA topoisomerase IIa
Apoptosis-related These factors control physiologic cell death.
factors These help in killing of damaged cells and
check-in proliferation of cells.
Pro-apoptotic signals Expressed in odontogenic tumors but the Apoptosis is decreased in odontogenic tumors
Fas, TNFR I, TRAILR I mediator of these signals known as caspases 8 even though the signals are present
and II is not fully expressed
Anti-apoptotic signals Expressed in various types of tumors There is increased survival of tumor cell
Bcl-2, Bcl-x, survivin
Regulators of Play a role in epithelial–mesenchymal
tooth development
Msx-1, Msx-2, Dlx-2, Involved in positioning and morphogenesis interactions and cell proliferation during the
Barx-1, and Pax-9 of tooth germs growth of odontogenic tumors as well as
during tooth development
SHH, PTCH, SMO, GLI 1 Involved in morphogenesis and
BMP, Wnt, HGF, and cytodifferentiation of tooth germs
FGF
Hard tissue-
related proteins
Enamel related
proteins- Expressed in epithelial components Aberrations of enamel-related proteins are
Amelogenin, enamelin, of odo tumors involved in oncogenesis of odontogenic
tuftelin, enamelsin epithelium.
Ameloblastin Mutation are detected in ameloblastoma
(amelin or sheathlin)
Dentin, Bone and These
cementum related proteins play a role in pathologic
proteins BSP, osteonectin, mineralization and/or tumor formation
osteocalcin, osteopontin, DSP, DPP
Molecules involved in progression of odontogenic tumors
Matrix-degrading Help in tumor remodeling Help in tumor progression
proteinases
MMPs-1, -2, and -9 Expressed in ameloblastoma, myxoma Responsible for invasiveness of odontogenic
MSP and other odontogenic tumors tumors
TIMP, heparanase
Angiogenic factors Necessary for development of vascularity Help in tumor angiogenesis and may
during inflammation and repair be involved tumorigenesis or
malignant transformation
VEGF, FGF, Necessary for tumor angiogenesis
HGF, TGF-β
Osteolytic cytokines
IL-1, IL-6, and Maintain bone homeostasis Responsible for invasion by local resorption
TNF-α, PTHrP, RANKL of bone

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288 Textbook of Oral Pathology

Note: The odontogenic tumors discussed in this

text are according to the most commonly
followed 1992 WHO classification.

BENIGN TUMORS

Ameloblastoma
Ameloblastoma is a benign, locally invasive,
polymorphic neoplasm, presumably derived
from intraosseous remnants of odontogenic
epithelium. It is primarily located (Fig. 15.2)
centrally in the jaw bones. Ameloblastoma
presents as a solid, unicystic, or mixed solid and
multicystic neoplasm. It is second most common Figure 15.2: Ameloblastoma showing huge swelling and
disfigurement
tumor of the odontogenic tissues after odontomas.
Broca in 1868 was first to report to amelo- organ-like islands or follicles of epithelial cells,
blastoma. First detailed description of ameloblas- while in the plexiform type the epithelium forms
toma was given by ‘Falkson’ in 1879. In 1885 continuous anatomizing strands. In both types
Malassez coined the term Adamantinoma. As the epithelial tumor components are embedded
this term suggests formation enamel which is not in mature, connective tissue stroma. Generally,
found in this tumor, Churchill in 1934 suggested a tumor shows one or the other pattern
alternative name of ameloblastoma which be- throughout. However, not infrequently both
came popular and well accepted. patterns are present in the same tumor.
There are different names given to this tumor.
These are ‘adamantine epithelioma’, ‘adaman- Shafer Definition
tinoma’, ‘adamantino-blastoma’, ‘epithelial
odontome’ and ‘multilocular cyst’. The ameloblastoma is a true neoplasm of enamel
organ-type tissue which does not undergo
Definition differentiation to the point of enamel formation.

It has been defined in various ways

Robinson Definition
WHO Definition
Ameloblastoma is a tumor “usually unicentric,
Solid multicystic ameloblastoma is polymorphic nonfunctional, intermittent in growth, anatomi-
neoplasm consisting of proliferating odontogenic cally benign and clinically persistent.
epithelium, which usually has a follicular or
plexiform pattern, lying in a fibrous stroma. Etiology
Reichart and Slootweg Definition • Irritation—Ameloblastoma is common in
A polymorphous neoplasm consisting of mandibular angle region. Here there is
proliferating odontogenic epithelium, usually constant pooling of saliva containing various
occurring in two main patterns. In the follicular irritating factors and microorganisms. This
type of growth the tumor consists of enamel irritation is thought to cause ameloblastoma.

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 Odontogenic Tumors 289

• Oral sepsis—In blacks ameloblastoma more • Papilliferous ameloblastoma

common. In such cases, oral hygiene was found Desmoplastic ameloblastoma
to be poor, so it was thought to be a factor. Peripheral ameloblastoma
• Trauma—Few patients gave history of either Unicystic ameloblastoma
trauma or extraction prior to the Extraoral ameloblastoma
ameloblastoma. So trauma can be a causative – Craniopharyngioma
factor for ameloblastoma. – Adamantinoma
• Dietary deficiency—Dietary deficiency has
Relative frequency of different types
been considered to be a possible factor. For
example, pronounced defect in development Histologic variants Frequency
of tooth germ as seen in rickets may lead to Follicular 33.9
irregularity in the ameloblastic layer. Plexiform 30.2
• Virus—Few ultrastructure studies of amelo- Acanthomatous 11.3
blastoma showed presence of viral particles Granular cell 3.5
which were thought to be polyoma viruses. Basal cell 1.4
Recently various studies indicate that human Desmoplastic 1.4
papilloma virus also plays a role in develop- Keratoameloblastoma 0.1
ment of ameloblastoma. HPV type 16 or 18
and type 6 have been detected. Few studies Pathogenesis
have shown that even Epstein-Bar virus
The resemblance of the tumor epithelium to the
genes are present in few ameloblastoma.
normal enamel organ indicates that ameloblas-
• Chemical carcinogens—Injection of nitrosoureas
toma arises from dental epithelium. The
have been shown to produce ameloblastoma
possibilities for its development are as follows:
like lesion in animals.
Enamel organ—Due to histological similarities
Classification of Ameloblastoma such as in origin, it has been thought the tumor
Ameloblastoma shows different clinical features growth starts at an early age, i.e. during the
and radiographic appearances. These clinical- period of existence of enamel organ but most of
radiographic types are further subdivided on the the patients are middle aged, i.e. in a period
basis of histopathology of the tumor. which is long after regression of the enamel
organ. However, the occasional occurrence of the
Solid/Multicystic Ameloblastoma tumor as a unilocular cystic lesion surrounding
the crown of an unerupted tooth also suggests
Common Variants that in some cases, the enamel organ may give
• Follicular ameloblastoma rise to it.
• Plexiform ameloblastoma
Cell rests—Cell rests of enamel organ either
• Acanthomatous ameloblastoma
remnants of dental lamina or remnants of
• Granular cell ameloblastoma
Hertwig’s sheath, i.e. epithelial cell rests of
• Basal cell ameloblastoma.
Malassez have the potential of transforming into
Newer Variants ameloblastoma.

• Clear cell ameloblastoma Epithelium of odontogenic cysts, i.e. from dentigerous

• Keratoameloblastoma cyst and odontoma—This be possible as the

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epithelium in the wall of the cyst and that of Signs: In later stages, the lesion may show ovoid
ameloblastoma are derived from the same and fusiform enlargement that is hard but non
embryonic source. tender. As tumor enlarges palpation may elicit
a hard sensation or crepitus. Surrounding bone
Oral mucosa—Basal cells of the oral epithelium
may become thin so that fluctuation and ‘egg
of jaws can be the origin. The reason behind this
shell crackling’ may be elicited. If it is left
is that there is communication between the
untreated for many years, the expansion may be
tumors and overlying mucosal epithelium as
extremely disfiguring, fungating and ulcerative
seen in peripheral ameloblastoma.
type of growth characteristic of that of carcinoma
Heterotrophic epithelium—Heterotrophic epithe- can be seen.
lium in other parts of the body especially of Maxillary lesion are more dangerous than
pituitary gland can serve as source of origin. mandibular lesions due to tendency for the
former lesion to spread more extensively in the
Clinical Features more porous maxillary bone and possibility of
the involvement of the cranial base. It may
Ameloblastoma accounts for approximately 1%
extend into the paranasal air sinuses, orbit or the
of all oral tumors and 11% of all odontogenic
nasopharynx.
tumors. It has slight predilection for males and
often seen in blacks. Most patients are between Mural ameloblastoma: Ameloblastoma may form
20 to 50 years of age with mean age of discovery from the epithelial lining of dentigerous cyst and
being 40 years. The tumor can occur in young in such a cases, it is called as ‘mural ameloblas-
children. Unicystic type of ameloblastoma is toma’
more common in the 2nd and 3rd decades and Histopathological Features
the extra-osseous form is more common in the
Macroscopic Appearance
older age group.
It develops in the molar ramus area It is grossly characterized by a fusiform or
(approximately 3/4th of cases) in the mandible cylindrical expansion of bone especially
and also occurs in maxilla in third molar area, involving the lingual plate of the mandible.
followed by the maxillary sinus and floor of the Perforation rarely occurs. On section, it appears
nose. The right side of the mandible is affected as a grayish yellow mass replacing the bone.
slightly more as compared to the left side. Some lesions are completely solid but in most,
cystic spaces are present which are quite small
Preceding factors: Neoplasm is frequently measuring up to 1-2 cm in diameter.
preceded by extraction of teeth, cystectomy and
some other traumatic episodes. Microscopic Feature
Onset: It begins as a central lesion of the bone Histologically ameloblastoma exists in two main
which is slowly destructive but tends to expand forms: follicular and plexiform patterns. Other
bone rather than perforating it. variants are belong to any one of these patterns.
Most of the varieties show follicular pattern.
Symptoms: Patient notices a gradually increasing
facial asymmetry. Teeth in involved region are Follicular Type (Figs 15.3 and 15.4)
displaced and become mobile. Pain and This is the most commonly encountered type of
paresthesia may occur, if the lesion is pressing ameloblastoma. It grows mainly in form of is-
upon a nerve or is secondarily infected. lands, but to a smaller extent chords and strands

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 Odontogenic Tumors 291

pre-ameloblasts and they are called as ameloblast

like cells. Cells that are present at the center of
islands are star shaped, with interconnecting
delicate cytoplasmic processes. These are called
as stellate reticulum-like cells. Sometimes there
is degeneration in central cells resulting
microcystic spaces. These cystic spaces may
enlarge exerting pressure on the peripheral cells
and making the cubical to flat cells.

Plexiform Type
This variant epithelium proliferates in form of the
Figure 15.3: Follicular ameloblastoma showing follicles (F) interconnected chords of epithelial cells. These
of varying size and shape. They are lined by tall columnar chords create a network like structure. These
ameloblast like cells (A). Centrally stellate reticulum like cells chords are lined by ameloblast-like cells. Stellate
(S) are seen. Sometimes cystic degeneration (CS) may be
seen (Courtesy: Dr.Raju Ragavendra T, Dept of Oral
reticulum-like cells are present in the center.
Pathology, Peoples Dental Academy, Bhopal) Stellate reticulum-like cells are not as prominent
as follicular ameloblastoma. Sometimes
ameloblast-like cells are lined back to back with
less or no stroma in-between. Supporting stroma
tends to be loosely arranged and myxoid. It shows
tendency towards cystic degeneration, which is
unlike follicular type where cystic degeneration
is seen mainly in the epithelium (Fig. 15.6).
The growth pattern of plexiform ameloblas-
toma resembles that of dental lamina stage of
tooth development before enamel organ
mophodifferentiation. Differentiation towards
bud stage of tooth development may be
evidenced by rounded nodules of epithelium.
Figure 15.4: High power view of follicular ameloblastoma
showing palisading, reverse polarity and basal vacuolation
of ameloblast like cells (A) surrounding stellate reticulum
like cells (SR) (Courtesy: Dr.Raju Ragavendra T. Dept of
Oral Pathology, People Dental Academy, Bhopal)

are also present. The peripheral cells are tall co-

lumnar. They show palisading arrangement,
where cells are arranged perpendicular to the
basement membrane. These cells show reverse
polarity of the nucleus, i.e. nucleus is placed
away from the basement membrane. Nucleus is
hyperchromatic.
At the basal area of these cells, vacuoles are Figure 15.5: Follicular ameloblastoma
present. Thus, the peripheral cells resemble (Courtesy: Dr Sangamesh Halawar)

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292 Textbook of Oral Pathology

Granular Cell Type

This is also shows follicular arrangement of cells.
This shows transformation of stellate reticulum-
like cells into granular cells. Sometimes a layer
of stellate reticulum may be present at the
periphery of granular cells. These granular cells
may be cuboidal, columnar or rounded. The
granular cells contain no cell organelle except the
presence of coarse eosinophilic granules and
nucleus is displaced to periphery of cells.
The granules pack the cytoplasm and distend
it. The nature of granules is not fully understood.
Ultrastructural studies indicate that these are
lysosomal aggregates. Other studies indicate that
the granularity is caused by increased apoptotic
cell death and associated phagocytes by
neighboring neoplastic cells. Previously it was
Figure 15.6: Plexiform ameloblastoma showing cords (C) of
thought that granular cell ameloblastoma are very
ameloblastic cells creating network like structure in a loose
stroma (L). Cells at the center resemble stellate reticulum (S) aggressive tumors, but this notion is not accepted
now. There are few reported cases of plexiform
Acanthomatous Type granular cell ameloblastoma (Fig. 15.8).
This variant closely resembles the follicular
ameloblastoma. Stellate reticulum like cells show
squamous metaplasia. They loose there star
shape and become polygonal. They produce
parakeratin at the central portions of island.
Sometimes it is present in form of keratin pearls.
The keratin may calcify in some cases (Fig. 15.7).

Figure 15.8: Ameloblastoma showing foci of granular cells

(G) in place of stellate reticulum like cells (SR)
(Courtesy: Dr Sangamesh Halawar)

Basal Cell Type

This type of ameloblastoma shows a close
similarity to basal cell carcinoma. The cells are
arranged in sheet like pattern rather than islands.
Figure 15.7: Acanthomatous ameloblastoma showing All cells including stellate reticulum-like cells
squamous metaplasia (SM) of stellate reticulum like cells have a basaloid appearance (Figs 15.9 and 15.10).

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 Odontogenic Tumors 293

amount of glycogen, which results in clear

appearance. Clear cell ameloblastoma are very
aggressive tumors.

Keratoameloblastoma and Papilliferous

Ameloblastoma
It consists of keratin filled cystic spaces and solid
proliferation of epithelial cells. Cysts are lined
by parakeratotic epithelium. The basal layer
consists of cuboidal cells, and the rest of the
layers of the epithelial lining show loosely
Figure 15.9: Basal cell ameloblastoma showing arranged epithelial cells. Cysts resemble
presence of basaloid cells (B) odontogenic keratocyst. The stroma of the tumor
consists of dense collagenous fibrous connective
tissue septa. Solid component of tumor consists
of large solid masses of parakeratin and keratin,
surrounded by multilayered squamous
epithelium with peripheral, palisaded, cuboidal
basal cells. Epithelium of solid areas differs from
cystic areas. It is not of uniform thickness, and
there is separation and edema between the basal
cells and the rest of the epithelium. Although
the basal cells are palisaded and demonstrate
nuclear polarization, they are cuboidal rather
than columnar.
Keratoameloblastoma is believed to be a link
Figure 15.10: Basal cell ameloblastoma
(Courtesy: Dr Sangamesh Halawar) between a cystic neoplasm-odontogenic keratocyst
and a solid neoplasm having same origin.
This type of ameloblastoma has highest In few cases epithelial cells surrounding the
proliferative rate than any other type of cystic spaces show papillary projections. Such
ameloblastoma, leading to formation of immature keratoameloblastoma are called as papilliferous
cells which have basal cell like appearance. The keratoameloblastoma. In few cases papilliferous
typical cellular morphology is often altered, with ameloblastoma exist independent of keratoamel-
ameloblasts like cells appearing low columnar to oblastoma.
cuboidal. There is loss of reverse polarity and sub
nuclear vacuolation. But cells retain palisading. Hemangioameloblastoma
(adamantinohemangiomas, ameloblastic
Clear Cell Ameloblastoma
hemangiomas, and hemangioameloblastomas)
Some ameloblastoma may contain cells without It is ameloblastoma that shows presence of
eosinophilic cytoplasm and appearing clear. spaces filled with blood or large endothelial lined
Clear cells are usually present in place of stellate capillaries. Presence of increased vascularity is
reticulum like cells. These cells contain high not understood. Various theories have been put

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294 Textbook of Oral Pathology

forward to explain the pathogenesis of the

vascular component in ameloblastomas. It is
probable that excessive stimulation of
angiogenesis during tumor development, by
inductive influences may result in the
overgrowth of vascular elements.
Alternatively, a traumatic incident such as a
tooth extraction may provide the stimulus for
proliferation of epithelial cell rests in the
periodontal ligament and subsequent tumor
development. Tissue damage is usually followed
by repair, involving the formation of granulation
tissue. Thus capillaries become prominent part
of developing tumor. It has also been suggested
that this tumor represents a collision tumor.
Another explanation is that the stroma part
of plexiform ameloblastoma consists of loose
Figure 15.11: Desmoplastic ameloblastoma showing
myxoid tissue which can undergo degeneration, compressed follicles (F) due to deposition of large amount
so blood vessels are left without any support. of collagen fibers in the connective tissue (Desmoplasia D)
They become enlarged, and rupture leading to with squamous cells which may show
escape of blood into empty stroma space. keratinization. Desmoplastic ameloblastoma
appear to have better prognosis (Fig. 15.11).
Desmoplastic Ameloblastoma
This ameloblastoma is different from other types Dentinoameloblastoma
of solid multicystic ameloblastoma, so it is In rare cases, ameloblastoma may be associated
thought to be a separate clinicopathological with odontome showing inclination to form
entity of ameloblastoma and not just histologic dentin and enamel. It consists of cystic and solid
subtype. tissue with ameloblastomatous follicles.
It different clinically, radiologically and Abundant amount of eosinophilic homogenous
histologically from classic ameloblastoma. This extracellular material interpreted as dentinoid
type of tumor is more common in anterior region is present. Dystrophic calcification is also seen
of maxilla. It occurs in older patients than at places.
conventional tumor. Mean age of patients is 42.8
years. The radiological appearance is also
different. Radiologically it appears as a fibro-
osseous lesion. It shows a mixed radio-opaque-
radiolucent appearance.
Histologically, it shows presence of large
amount of collagen fibers (Desmoplasia). These
collagen fibers compress the islands leading to
bizarre ‘animal-like’ shapes. Peripheral cells are
mostly cuboidal, columnar cells are rarely seen.
Central area of island shown’s hypercellularity, Figure 15.12: Desmoplastic ameloblastoma

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 Odontogenic Tumors 295

Melanoameloblastoma Peripheral lesion is relatively innocuous,

It is essentially found in infants before the age of lacks persistent invasiveness and has a limited
6 months. There is proliferation of pigment tendency of recurrence. Prognosis of this tumor
producing odontogenic epithelium. is very good.

Extraosseous/Peripheral Ameloblastoma Unicystic Ameloblastoma/Mural

It is a tumor which occurs in the soft tissue Ameloblastoma
outside and overlying the alveolar bone. It
It is cystic variant of ameloblastoma. It thought
originates from either the surface epithelium or
to develop from dentigerous cyst.
the remnants of dental lamina. It is common in
males and on the mandibular gingiva in the Pathogenesis of unicystic ameloblastoma.
molar-premolar region. Some cases also seen in Three hypothesis are put forward.
buccal mucosa and maxillary tuberosity area. • In dentigerous cyst lining basal cells
Symptoms: The patient complains of a mass undergoes transformation into ameloblasts
which is growing in size and interfering with like cells, which proliferate either into the
speech, mastication and esthetics. lumen or connective tissue forming nodules
of ameloblastoma.
Sign: Mass is mildly tender on palpation at times
• The follicles of ameloblastoma undergo
ulcerted and bleeding may occur on slight touch.
degeneration leading to formation of a cavity
The lesion appears nodular on gingiva varying
or microcystic space, which enlarges forming
in size from 3 mm to 2 cm in diameter.
large cystic space.
Histopathological features: Tumor may exhibit • Unicystic ameloblastoma arises de novo.
in one or more areas, continuity with the It is a unilocular cystic lesion whose clinical
surface epithelium. Histologically, it exhibits features are of those of a non neoplastic cyst. Lin-
the same pattern as seen in intraosseous ing epithelium exhibits alterations virtually iden-
ameloblastoma.
tical with those described as ameloblastomatous
changes in dentigerous cyst; also called as mu-
ral ameloblastoma.
Injection of radiopaque solution into the cyst
cavity will demonstrate presence of mural
nodule of tumor projecting intraluminally as
well as its position and size. Ameloblastomatous
lining epithelium proliferates into the connective
tissue wall. Islands of ameloblastoma occurring
isolated in the connective tissue wall (Fig. 15.14).

Plexiform unicystic type—there is intraluminal

plexiform proliferation of epithelium. It
Figure 15.13: Peripheral ameloblastoma of follicular type proliferates in dentigerous cyst in retiform or
showing stellate reticulum (SR) and ameloblasts like cells
(AC) (Courtesy: Dr Sangamesh Halawar) network pattern.
296 Textbook of Oral Pathology

It is most common in childhood and

adolescents before the age of 25 years. It may be
located within the sella tursica or commonly in
suprasellar space. It grows as a pseudoen-
capsulated mass causing pressure effect and
often destroys the pituitary gland.
It is related due to destruction of pituitary
gland (diabetes insipidus) or due to compression
of nearby cranial nerve.

Histopathological Features
Columnar cells in craniopharyngioma have
central nuclei, longitudinal cytoplasmic fibrils.
The central areas of the trabeculae of cells that
Figure 15.14: Mural ameloblastoma showing cystic lining
compose the tumor consist of squamous cells
showing intercellular bridges, but degeneration
and edema are common and produces an
appearance superficially similar to stellate
reticulum. Frequent presence of calcified
material and keratinization in pituitary
ameloblastoma.

Adamantinoma of Long Bones

It is derived from epithelial rests misplaced
during the course of development. Trauma
causing implantation of epithelium with
subsequent tumor formation.
Figure 15.15: Cystic ameloblastoma It occurs in tibia, ulna, and femur and
(Courtesy: Dr Sangames Halawar) occasionally in others bone.
Some lesions behave aggressively and
sometimes are metastasizing.
Extragnathic Tumors Resembling
Histopathological features: It bears a superficial
Ameloblastoma
microscopic resemblance to ameloblastoma of
Pituitary Ameloblastoma jaws.
It is also called as ‘craniopharyngioma’ or
‘Rathke’s pouch tumor’. It occurs in anterior lobe, Differential Diagnosis (Histological)
which is of ectodermal origin. The lobe is derived • Basal cell carcinoma—Basal cell carcinoma
from Rathke’s pouch, an outgrowth of oral ecto- does not occur interiorly.
derm. The pouch give rise to craniopharyngeal • Squamous cell carcinoma—Acanthomatous
duct, which in due course degenerates itself leav- ameloblastoma may be confused with
ing residues of squamous epithelial cells. From squamous cell carcinoma. But unlike
these squamous epithelial cells ameloblastoma squamous cell carcinoma it does not exhibit
like tumor develops. epithelial dysplasia.
 Odontogenic Tumors 297

• Canalicular adenoma—It may be confused with squamous epithelium in a fibrous stroma. The
plexiform ameloblastoma. epithelial islands occasionally show foci of
central cystic degeneration.
Management
Complete removal of the neoplasm with Origin
resection of the involved segment.
The origin of squamous odontogenic tumor is
Curettage—It is least efficient as it makes not clear. It is believed to originate from the cell
recurrence inevitable. Curettage may leave bone rests of Malassez located in periodontal ligament.
that is invaded by tumor cells. Peripheral variety may originate from gingival
surface epithelium or from the remnants of
Intraoral bloc excision—Excision of a bloc of bone
dental lamina.
may be recommended if the ameloblastoma is
small. The segment in which the tumor is
contained is removed with a safe margin of Clinical Features
normal bone.
There is a wide age distribution in the range of
Extraoral en bloc resection—If the lesion is large 11 to 67 years with mean age 40 years and
and horizontal ramus is involved then this is females are more commonly affected than the
carried out. males. It occurs with equal frequency in maxilla
Peripheral osteotomy—It is a procedure which and mandible. In maxilla, it occurs in incisor-
allows complete excision of the tumor but at the cuspid area and in the mandible; it has got a
same time a part of bone is retained which predilection for the bicuspid-molar area. It is
preserves the continuity of the jaw. The slowly growing tumor.
procedure is based on the observation that Symptoms: It is usually asymptomatic but there
cortical inferior border of the horizontal body, may be mobility of the involved teeth, pain,
the posterior border of the ascending ramus and tenderness to percussion and occasionally
condyle are generally not involved by tumor abnormal sensation.
process. These areas are resistant and strong
because of the dense cortical bone. Bone
regeneration will proceed from such areas with Histopathological Features
considerable restoration of the jaw architecture. It is composed entirely of round to oval islands
of squamous epithelium. Sometimes islands
Squamous Odontogenic Tumor
have irregular or cord like shape without
It is well differentiated odontogenic tumor peripheral palisaded or polarized columnar cells.
composed of islands or sheets of squamous Islands contain peripheral flat to cuboidal
epithelium that lack recognizable features of epithelial cells (Fig. 15.16).
enamel organ differentiation. It is locally Central cells show squamous differentiation.
infiltrative odontogenic neoplasm. They are of very uniform size and shape. They
exhibit no pleomorphism, nuclear hyperchroma-
Peter and Reichart Definition tism or mitotic activity. Sometimes individual
A benign but locally infiltrative neoplasm cell keratinization may be present. But epithe-
consisting of islands of well differentiated lial pearl formation is not seen (Fig. 15.17).
298 Textbook of Oral Pathology

Calcifying Epithelial Odontogenic Tumor

It is also called as ‘Pindborg tumor’, named after

J J Pindborg who described it as ‘calcifying
epithelial odontogenic tumor in 1955. Pindborg
reported few cases of intraosseous CEOT.
Previously it was considered as a variant of
ameloblastoma and was termed as calcifying
ameloblastoma.
WHO accepted and adopted the term calcifying
epithelial odontogenic tumor in 1971. The CEOT is
a benign tumor occurring more commonly
intraosseously and less commonly extraosseously
in the gingiva. The tumor resembles ameloblastoma
Figure 15.16: Squamous odontogenic tumor showing islands
containing keratinized squamous cells (S) arranged in islands for the site and the age. Both the tumors occur
in loose fibrillar stroma background (ST) commonly in the mandible than maxilla by a ratio
of 2:1. They occur three times in molar region than
the premolar.

Origin

Two hypothesis have been suggested for the

pathogenesis of the tumor. It develops from the
reduced enamel epithelium of the embedded
tooth or from stratum intermedium.
The peripheral locations strongly suggested
of an origin from the cell rest of dental lamina.
Another source might be from the basal cells of
the oral epithelium. Most widely accepted origin
Figure 15.17: Squamous odontogenic tumor in current literature is from the disintegration of
(Courtesy: Dr Sangamesh Halawar)
the dental lamina cell rest.
Microcystic degeneration may be seen in few
islands. Intraepithelial calcification may be seen
in few islands. Calcifications tend to be laminar.
Globular, hyaline eosinophilic structures with
the islands which are not amyloid are sometime
found in this tumor. The fibrous stroma of tumor
contains mature bundles of collagen fibers,
devoid of any inductive effect.

Management
Conservative enucleation and curettage is Figure 15.18: Pathogenesis of CEOT arising from
usually curative with a low recurrence rate. reduced enamel epithelium
 Odontogenic Tumors 299

Figure 15.19: Cell rest of malassez and cell rest of serrae

can proliferate either into jaw bone or towards gingiva
Figure 15.20: Pindborg’s tumor showing epithelial cells with
prominent intercellular bridges (ICB) forming eosinophilic
material (EM). Few cells show dysplastic features (D)
Clinical Features
Histopathological Features
Intraosseous CEOT
The tumor consists of sheets, or islands of
This is the most commonly presenting type. It polygonal cells. The cells have eosinophilic
appears as a slow growing painless mass. It cytoplasm, distinct cell borders and very
forms 1% of all odontogenic tumors. It is more conspicuous intercellular bridges (Fig. 15.20).
common in men with an age range of 8 to 92 Nuclei are pleomorphic with prominent
years with a mean age of 42 years. Mandible is nucleoli; cells with giant nuclei and multiple
more commonly affected than the maxilla in the nuclei are also present. The pleomorphism of
ratio of 2:1 and develops in premolar-molar area. nuclei mimics a malignant tumor, but as mitotic
Symptoms: It is asymptomatic and only figures are rare and clinical behavior is slow
presenting symptom is a painless swelling. In growing this possibility is ruled. The connective
rare cases, there is associated mild paresthesia. tissue stroma is scanty and fibrous. Occasionally,
When occurs in maxilla, it may show epistaxis, cells are arranged in cords and rows mimicking
nasal congestion and headache. adenocarcinoma.
Amyloid like material in CEOT—There is presence
Signs: Cortical expansion occurs. Palpation will
of homogenous eosinophilic substance. This
show hard tumor with well defined or diffuse
proteinosis material which has been interpreted
border. Locally invasive with a recurrence rate
as amyloid has also been suggested to be
of about 14%.
comparable glycoprotein, basal lamina, and
keratin or enamel matrix.
Peripheral CEOT
Liesegang rings—There are few deposits of
It occurs as a painless firm gingival mass calcified material which is said to be cementum
clinically diagnosed as fibroma, peripheral giant like material. There is presence of calcification
cell granuloma. The diagnosis is confirmed in large amounts and often in the form of
histopathologically ‘Liesegang rings’ (Fig. 15.21).
300 Textbook of Oral Pathology

Figure 15.21: Pindborg’s tumor showing presence

of Liesengang rings (LR)

Figure 15.23: Calcification seen in CEOT

Management

It has limited invasive potential therefore local

excision with limited margins is indicated.
Simple enucleation or radical resection can be
done.

Adenomatoid Odontogenic Tumor

It is relatively uncommon odontogenic tumor.

It is histomorphologically similar to dental
organ. It is believed to be an attempt at enamel
Figure 15.22: CEOT showing Liesegang ring
(Courtesy: Dr Sangamesh Halawar)
formation by the tumor cells. It is not a tumor
but hamartoma.
Its clinicopathological profile is unique
Clear cell variant—A well-recognized form of this among the odontogenic tumors. It has sometimes
neoplasm is the clear cell variant which exhibits been referred to as the 2/3rd because about two-
a clear vacuolated cytoplasm rather than an thirds occur in the maxilla, two-thirds occur in
eosinophilic cytoplasm. The nucleus may remain young women (preteen and teenage years), two-
round or oval in the center of the cells or be thirds are associated with an unerupted tooth,
flattened against the cell membrane. In some and two-thirds of those teeth are canine teeth.
tumors, clear cells comprise the bulk of the tumor Steenland was first to report AOT in 1905.
cells while in others, it consist of only a few The term AOT was coined by Philipsen and Birn
scattered foci. in 1969. Other terms used for this tumor are epi-
 Odontogenic Tumors 301

thelial adamantinoma, hemangioameloblastoma • Extrafollicular type—There is no association

glandular adamantinoma, adenoameloblastoma. with tooth. twenty one percent AOTs are of
Recently adenomatoid odontogenic cyst has this type.
been coined for this lesion.
Peripheral or Extraosseous Type
Definition
• It is situated in gingiva. Only 4% are
WHO definition— “A tumor of odontogenic peripherally located.
epithelium with duct like structures and with
varying degrees of inductive change in the
Types
connective tissue. The tumor may be partly cystic
and in some cases the solid lesion may be present Terminology suggested for the two central tumor
only as masses in the wall of a large cyst. It is variants serves single purpose of making a
generally believed that the lesion is not a distinction between tumors having and those
neoplasm.” lacking an association with crown of an
embedded tooth. These terms do not indicate or
Classification suggest any pathologic principle.
Peter and Reichart based on clinical and
radiological findings AOT can be subdivided Pathogenesis
into three variants (Fig. 15.24):
Origin of AOT is a debated issue. Initially it was
Central or Intraosseous Variant thought that it is a variant of ameloblastoma as
• Follicular or dentigerous type—It is AOT tall columnar cells similar to that found in
ameloblastoma are also found here. Because of
associated with the crown of unerupted
presence of ductlike structures dual salivary and
tooth. It is usually misdiagnosed as
dentigerous cyst. Seventy one percent of odontogenic origin was suggested. AOT occurs
exclusively within tooth bearing areas of the
AOTs belong to this group.
jaws. This indicates that this tumor is mainly
odontogenic. Transmission electron microscopy
has shown that cells of AOT resemble enamel
organ. Immunohistochemical staining ruled out
salivary gland origin and supported odontogenic
nature.
The epithelium that probably gives rise to
AOT is cell rests of dental lamina. Disintegration
of dental lamina gives rise to numerous epithelial
remnants persisting in the jaws. These rests are
distributed in gingiva and few are present in
gubernacular dentis which is connective tissue
that occupies gubernacular canal. Based on this
Figure 15.24: Schematic diagram showing different types of origin pathogenesis of various clinical forms of
AOT. Follicular (F), extrafollicular (EXF) and peripheral (P) AOT can be explained.
302 Textbook of Oral Pathology

Follicular AOT Peripheral AOT

Cell rests of gubernacular dentis start It is likely to originate from cell rests of Serrae. It
proliferating prior to eruption of permanent is also possible that it develops from cell rest
tooth. As tumor growth continuous and as tooth located at the peripheral most part of the
begins to erupt contact occurs between the two. gubernacular dentis.
This leads to the fusion between tumor and
reduced enamel epithelium of the erupting tooth. Clinical Features
Sooner or later tumor completely embraces the
tooth leading to follicular radiological It represents 3 to 7% of all odontogenic tumors
appearance. and is a developmental outgrowth of
odontogenic tissue. It is found in individuals
Extrafollicular AOT ranging from 3 to 82 years with 70% occurrence
in 2nd decade with an average age of 16 years.
If tumor develops from cell rests situated at the
Females are affected more than males in a ratio
periphery of the gubernacular dentis or if
of 2:1.
eruption is not impeded by the tumor tooth may
bypass it and erupt into the oral cavity. Thus It occurs more commonly in the maxilla than
tumor and tooth are not in contact leading to in the mandible, usually in the anterior region
extrafollicular AOT. It assumes that shape of and especially in the cuspid area. It is commonly
residual, globulomaxillary, lateral periodontal or associated with an unerupted tooth.
radicular cyst on radiograph based on its relation Symptoms: Most are asymptomatic. Few present
to tooth. AOT occurring in molars likely to as an area of swelling over an unerupted tooth.
originate from distal extension of dental lamina. Sometimes it may expand cortical bone but is
Molars do not have gubernacular dentis.
not invasive.
Signs: AOT is frequently associated with an
unerupted tooth in which the epithelial
proliferation is confined within a connective
tissue capsule that is attached to the tooth in
manner similar to the attachment of a
dentigerous cyst. When the tumor occurs
independently of unerupted teeth it is often
encapsulated. It may cause delayed eruption of
tooth. The tumor causes expansion of bone and
fluctuation may be elicited.
It is slow growing and is a gradually increas-
ing painless swelling leading to asymmetry, fre-
quently associated with missing teeth.

Figure 15.25: Schematic diagram showing origin of AOT Extraosseous tumor—This uncommon type
from Gubernacular dentis (GD) which connect gingiva and usually occurs as pink colored mass in
permanent tooth germ (PTG) and contains many cell rests
(ER) gingiva.
 Odontogenic Tumors 303

Histopathological Features
Macroscopy
Cut tumor is soft spherical mass with a
discernible fibrous capsule. Upon section tumor
exhibits white to tan friable tissue nodule lining
one or more cystic spaces may be seen. Cystic
space contains minimal yellow to brown fluid
to semisolid material. Several calcified masses
may be seen. The protein content of cyst is less
than 5.0g/100 ml.
Microscopy
Irrespective of tumor variants histology is Figure 15.26: AOT shows many duct like spaces (D) lined
by single cells and rosette patterns (R)
identical and exhibit remarkable similarity.
Tumor shows highly cellular nodules. These
nodules form scattered duct-like or tubular
structures and rosette patterns.
Eosinophilic material and calcification may
also be seen. Ductlike patterns: These are spaces
lined by single or double row of low columnar
epithelial cells which are reversely polarized. The
lumen of the duct may be empty or contain
eosinophilic material. Size, number and diameter
of ducts may vary (Fig. 15.26).
Many spindle shaped cells occupy space
between these patterns. Tumor also shows
eosinophilic material, calcification in several
forms and fibrous capsule of variable thickness.
Rosette pattern: Few epithelial nodule contain
spindle cells in as swirling pattern. The space
between the duct-like spaces and rosette patterns
is filled by spindle shaped cells (Fig. 15.27).
Figure 15.27: AOT showing many rosette pattern and
Cells of AOT duct like structure

Cells of AOT are divided into three types based Cell type II —These cells surround the epithelial
on morphology. nodules. These are spindle shaped cells with
dark eosinophilic cytoplasm. Nucleus is oval and
Cell type I—These are cuboidal or columnar cells
hyperchromatic. Long axis of these cell are
with pale cytoplasm. They contain vesicular
perpendicular to type I cells.
nucleus with one or two prominent nucleoli.
These are present in solid nodules, rosette Cell type III —These are islands or nodules of
patterns and duct-like structures. polygonal cells having squamous appearance.
304 Textbook of Oral Pathology

They contain prominent intercellular bridges.

These cells surround the type II cells.

Figure 15.29: AOT showing duct-like spaces

Management
Figure 15.28: Different types of cells and their location in
a duct-like pattern Conservative surgical excision and curettage can
Calcifications and eosinophilic material—Some be effective. The recurrence rate is 0.2%.
AOTs contain varying amounts of eosinophilic Odontogenic epithelium with odontogenic
material and calcification especially in the lumen ectomesenchyme with or without dental hard
of duct like spaces and center of rosette patterns. tissue formation.
Exact nature of this is not clear but it is called as
dysplastic dentin, dentinoid, or osteodentin Mixed Odontogenic Tumors
enameled etc.
Mixed odontogenic tumors consist of tumors
Stroma—Supporting stroma of AOT is loose, less where both epithelium and connective tissue
cellular, fibrovascular connective tissue. It shows proliferate. The epithelial component resembles
presence of dilated and congested blood vessels. ameloblasts like cells. Connective tissue
Fibrous capsule—AOT is surrounded by fibrous resembles dental papilla. The clinical features,
capsule of varying thickness. radiological features and to some extent
histopathological features overlap. These tumors
Unusual variants of AOT—Combined epithelial are seen more commonly second decade of life.
tumor: Sometimes AOT and CEOT like may co- Radiologically produce radiolucency containing
exist. In some cases AOT is found in the wall of radio opacities. The treatment and prognosis is
CEOC. Few cases of AOT with melanin almost same for these. Thus these tumors are
pigmentation are reported. AOT can be present thought to be interrelated. Two hypothesis are
along with odontoma (Adenomatoid odontoma). proposed in this regard.
Differential Diagnosis (Histological)
Continuum Concept (Cahn and Blum) (Table 15.5
Most AOT is are distinct so they are not usually
and Flow chart 15.1)
confused with any other tumor. Sometimes it is
confused with ameloblastoma, which can be According to this concept ameloblastic fibroma
excluded by looking for duct-like spaces are is the tumor that develops during active
rosette patterns. odontogenic and it develops similar tooth, i.e.
 Odontogenic Tumors 305

over the time it matures and finally forms But it is clear that odontoma and other lesions
odontoma. During maturation there is gradual cannot exist de novo. They must pass through a
formation of dental hard strucutres. Initially initial stage where there is absence of any
there is formation of dentin like structure. This calcification. This led Philipsen and Reichart put
is called as ameloblastic fibro-dentinoma. Later forth another theory where they propose
enamel also forms leading ameloblastic fibro- existence of two lines.
odontoma. As maturation progresses fibrous • Line I: This is neoplastic line, consisting of
component reduces and calcified structures ameloblastic fibroma occurring after tooth
increase. This stage is called complex odontoma. formation. This does not undergo any
This matures to form compound odontoma. maturation.
• Line II: It is also called as hamartomatous line.
Table 15.5: Two lines of mixed odontogenic tumors This includes the ameloblastic fibroma that
Hamartomatous or Line I Neoplastic or Line II occurs during tooth formation. This matures
Ameloblastic fibroma Ameloblastic fibroma over the time to form complex odontoma. But
Ameloblastic fibrodentinoma this line excludes compound odontoma. It is
Ameloblastic fibroodontoma believed that this lesion has a different
Complex odontoma
pathogenesis than complex odontoma.
Thus, it is believed that few mixed odontogenic
Flow chart 15.1: Continuum concept
tumors are interrelated. They start their develop
from ameloblastic fibroma follow stages similar to
development of tooth. Complex odontoma is
terminal stage after which there is no further
growth. Growth of these mixed tumors stop after
some time and does not progress further.

Ameloblastic Fibroma
Ameloblastic fibroma is mixed odontogenic
tumors in which both epithelial component as
well as mesenchymal components proliferates.
These cells resemble early functional ameloblasts
and primitive mesenchymal components of the
dental papilla. It is believed to be precursor of
other mixed odontogenic tumors. If this tumor
is left undisturbed, it will ultimately differentiate
into other lesions terminating in development
But this theory is not accepted completely. of compound odontoma. There are several
There are various reasons for this. synonyms for this such as ‘fibrous adamantinoma’,
• When residual tumor of ameloblastic fibroma ‘soft odontoma’, ‘soft mixed odontoma’ ‘and
is left in the jaw for long time it is observed ‘fibroadamanblastoma’
that it does not develop into other type of
odontogenic lesion. Pathogenesis
• Few odontoma develop well after tooth Ameloblastic fibroma has odontogenic origin. It
formation. resembles normal tooth germ just before
306 Textbook of Oral Pathology

formation of hard structures. There is failure or cords and small islands, stellate reticulum like
alteration in epithelial mesenchymal interactions tissue is absent so ameloblasts like cells are present
leading to failure of hard tissue formation. in two layers. Cords resemble dental lamina.
Ameloblastic fibroma exists in two forms.
One is neoplastic variety that develops in adults Mesenchymal Components
after formation of tooth is over. Other is Mesenchymal components resemble dental pa-
hamartomatous variety that develops during
pilla. These contain rounded or angular cells. There
tooth formation. This is more common.
is presence of few delicate collagen fibers. One
important feature is presence of cell free zone
Clinical Features
around the epithelial islands. This zone is believed
Most frequently observed during first two to be due to inductive effect of epithelium and con-
decades of life with 40% of patients under the tains excess basement membrane like material.
age of 10 years. Average age of occurrence is 14.8
years. Few cases are seen in adults as old as 60
years. Slight predilection for occurrence in males.
Male to female ratio is 1.4:1. It developed in
premolar molar area of the mandible. Three
times more common is mandible than in maxilla.
Symptoms: It is painless and expands slowly.
There is bulging of the cortical plates rather than
erosion through them. There is also migration
of involved teeth.
Signs: It enlarges by gradual expansion so that
the periphery of bone often remains smooth. It
is associated with unerupted teeth. It reaches
upto an approximate size of 1 to 8.5 cm. It has
got a slower clinical growth than ameloblastoma
and does not tend to infiltrate between trabecule Figure 15.30: Ameloblastic fibroma showing islands (I) and
Cords (C) of odontogenic epithelium made up of ameloblasts
of bone. like cells (A) and stellate reticulum like cells (S). Connective
tissue resembles dental papilla (EM). A cell free zone is seen
Histopathological Features around the islands (CFZ)

This tumor is made up of epithelial and Few ameloblastic fibroma show presence of
mesenchymal components. melanin pigmentation. Sometimes granular cells
similar to those found in granular cell
Epithelial Components ameloblastoma are found in mesenchymal
portion. Such lesions are called granular cell
These are arranged in form islands cords and
ameloblastic fibroma.
stands. Islands show presence of tall columnar
cells with reverse polarity at the periphery thus
Management
resembling the ameloblasts (Ameloblasts-like
cells). The central area of islands contains star Curettage can be done which has successful
shaped cells resembling stellate reticulum. In results in many cases. Surgical excision.
 Odontogenic Tumors 307

hard tissue around the epithelial cells. This is

preceded by cell free zone. The hard tissue is
similar to dentin so it is called as dentinoid.
Rarely hard structure show dentinal tubules
similar to true dentin.

Management
Surgical excision of the lesion should be done.

Ameloblastic Fibro-odontoma
It is very rare neoplasm similar to ameloblastic
fibroma and ameloblastic fibrodentinoma except
Figure 15.31: Ameloblastic fibroma showing ameloblasts- the formation of enamel like material.
like cells (Courtesy: Dr Sangamesh Halawar)

Ameloblastic Fibrodentinoma Pathogenesis

It is very rare neoplasm composed of Continued maturation of ameloblastic

odontogenic epithelium and immature fibrodentinoma leading to formation of enamel
connective tissue and characterized by the gives rise to this tumor.
formation of dysplastic dentin. It is similar to
ameloblastic fibroma showing formation of Clinical Features
dentinoid. It is also called as dentinoma. It is observed during first two decades of life with
99% of cases are seen before 20 years. Average
Pathogenesis
age of occurrence is 9.0 years. It is more common
It is hamartomatous lesion of odontogenic origin. in males. Male to female ratio is 1.4:1. It developed
It is considered to an intermediate stage between in premolar molar area of the mandible.
ameloblastic fibroma and ameloblastic fibro-
odontoma. Symptoms: The most common presenting
complaint is swelling and failure of tooth
Clinical Features eruption. The maxillary tumor if large interferes
It is observed during first two decades of life with with nasal respiration, eating and speech.
75% of cases are seen before 20 years. Average Ameloblastic fibroodontoma consists of elements
age of occurrence is 13.6 years. Few cases are seen of ameloblastic odontoma and is more aggressive
in adults as old as 60 years. It is more common than the common odontoma.
in males. Male to female ratio is 3:1. it developed
in premolar molar area of the mandible. Histopathological Features
Symptoms and signs: It is painless and expands
It contains hard and soft structures. Hard tissues
slowly. It enlarges by gradual expansion so that
are present at the center and they are covered
the periphery of bone often remains smooth.
by the peripheral soft tissue.
Histopathological Features Hard Tissue— It consists of dentin like material
Epithelial and mesenchymal portions are similar and enamel like material. Dentin like material is
to ameloblastic fibroma. There is presence of usually present at the central portion. It is
308 Textbook of Oral Pathology

covered by enamel like material called enamel

oid. Sometimes cementum like material may be
found.
Soft tissue—It is similar to ameloblastic fibroma.
Thus, it contains epithelial islands in dental
papilla like mesenchymal tissue.

Management
Figure 15.32: Ameloblastic fibro-odontoma showing
Surgical excision of the lesion should be done.
ameloblastic follicles (A) surrounded by dentin like
calcifications (D)
Odontoma
It is a hamartoma of odontogenic origin in which • Compound composite odontoma—Multiple
both epithelial and mesenchymal cells exhibit well formed teeth.
complete differentiation with enamel and dentin
laid down in abnormal position. Complex and Compound Odontoma
Definition
Origin
A malformation in which all dental tissues are
They result from extraneous buds of odontogenic represented, individual tissues being mainly well
epithelial cells from the dental lamina. formed but occurring in a more or less disorderly
Odontoma may arise from any of three dental pattern.
tissues, i.e. enamel, dentin and cementum. The
term odontoma was applied to wide variety of Etiology
lesions showing defects in hard tissue formation.
• Trauma—Local trauma or infection may lead
But now they are applied only for mixed
to production of such a lesion.
odontogenic tumor.
• Genetic— They are either inherited or are due
to a mutant gene or interference, possibly
Pathogenesis
postnatal, with genetic control of tooth
Odontoma are hamartomatous proliferation of development.
odontogenic origin. It is thought that local
trauma, infection and genetic mutations cause Mechanism
this proliferation of odontogenic epithelium.
Both the epithelial and mesenchymal cells exhibit
These result in unsuccessful or altered
complete differentiation with the result that
ectomesenchyme interaction during early or
functional ameloblasts and odontoblasts form
later phases of tooth development leading to
enamel and dentin. It is laid down in an
haphazard formation of enamel, dentin and
abnormal pattern because of failure of cells to
cementum.
reach the morphodifferentiation stage.
Lesion is composed of more than one type of
Classification
tissue, for this reason it is called as composite
• Complex composite odontoma—Non odontome. In some composite odontomes, the
discrete masses of dental tissue. enamel and dentin are laid down in such a
 Odontogenic Tumors 309

fashion that the structure bears a considerable which may or may not exhibit normal relation
anatomical resemblance to that of normal teeth to one another. The lesion is surrounded by
except they are often smaller than the typical fibrous capsule which is partially separated by
teeth, which have been termed as compound the fluid; the resultant cyst is usually lined by
composite odontome. squamous epithelium. There is presence of ghost
When calcified dental tissue are simply cells in odontoma (Fig. 15.33).
arranged in a irregular mass bearing no
morphological similarity even to rudimentary
tooth then that form is called as complex
composite odontome.

Clinical Features
It is a non-aggressive lesion that is more likely to
be a hamartoma than a neoplastic growth. Mean
age of detection is 14.8 years. Most begin to form
while normal dentition is developing. There is
slight predilection for occurrence in males.
Compound is twice as common than complex.
Compound occurs in incisor, canine area of
Figure 15.33: Odontoma showing well formed dentin (D)
maxilla and complex occurs in mandibular 1st and enamel [(Enamel space (ES)]
and 2nd molar area. Unusual situation include
the maxillary sinus, inferior border of the Management
mandible, ramus and condylar region.
Mass has to be removed if it is causing periodon-
Sign: Compound odontoma is between 1 to 3 cm tal diseases.
in diameter. It usually remains small and
diameter of the mass only occasionally increases Odontoameloblastoma
than that of the tooth. It is common for a tooth It is also called as ameloblastic odontomas. It is
or teeth to be absent from the arch in the presence a is a very rare mixed odontogenic neoplasm
of an odontome. Teeth may be impacted characterized by the simultaneous occurrence of
malpositioning; diastema, aplasia, malformation an ameloblastoma and a compound or complex
and deviation of adjacent teeth are associated odontoma in the same tumor mass. The epithelial
with 70% of odontoma proliferation form islands or intermingled cords
Symptoms: Occasionally, it may produce that produce the follicular or plexiform patterns
expansion of bone with consequent facial typical of ameloblastoma but, unlike
asymmetry. There may be evidence of swelling conventional ameloblastoma, these induce the
and infection. production of mineralized dental tissues on the
Sometime cyst develops in relation with a adjacent mesenchymal cells and may respond
complex odontome and compound odontome, to this change with the production of enamel.
but it is very rare. The pathogenesis of odontoameloblastoma is
unknown. One possible explanation is that the
Histopathological Features mineralized dental tissues are formed as a
Normal appearing enamel or enamel matrix, hamartomatous proliferation in response to
dentin, pulp tissue and cementum like tissue inductive stimuli produced by the proliferating
310 Textbook of Oral Pathology

epithelium over the mesenchymal tissue. Other Clinical Features

is two tumors developing separately and coming It occurs more frequently in older individual
closer to collide each other (Collision tumor). with mean age of 40 years. It is more common in
maxilla and in anterior region. There is marked
female predilection.
Symptoms: It is generally asymptomatic except
for the swelling of the jaws.
Signs: It may cause localized bony expansion or
loosening of teeth.

Histopathological Features
Odontogenic fibroma are composed of fibrous
tissue of variable cellularity and density; variable
amount of inactive appearing odontogenic
epithelium; and variable presence of
calcifications resembling dysplastic dentin,
cementum-like tissue or bone. Mesenchyme also
varies in fibrosis. Few lesions are highly fibrous
Figure 15.34: Odontoameloblastoma showing dentinoid
(D) and ameloblastic follicle (AF) while few contain less collagen fibers. Few
lesions contain myxoid tissue. Cellularity is
Mesenchyme and/or odontogenic
sparse to moderate. Sometimes eosinophilic
ectomesenchyme with or without included
amorphous globules representing enamel matrix
odontogenic epithelium:
protein are seen. Many calcifications are seen.
These are not specific.
Odontogenic Fibroma
Epithelial islands are few to numerous. They
It is fibroblastic neoplasm containing varying are inactive looking and to not play any role in
amounts of apparently inactive odontogenic the growth of the tumor.
epithelium. It is found around the crown of
unerupted tooth resembling a small dentigerous
cyst. But some say that it is a hyperplastic dental
follicle and not an odontogenic tumor. It may
occur centrally or in the periphery. Peripheral
variant clinically mimics fibroma.

Clinical Types
• Central odontogenic fibroma
• Peripheral odontogenic fibroma.

Pathogenesis
It is said that WHO type of odontogenic fibroma
Figure 15.35: Odontogenic fibroma showing stellate
originates from periodontal ligament and simple fibroblasts and immature collagen fibers thus resembling
type is originating from dental follicle. dental papilla (Courtesy: Dr Sangamesh Halawar)
 Odontogenic Tumors 311

There are two histologic variants: 6% of odontogenic tumors. Mandible is more

• Simple odontogenic fibroma—This lesion commonly affected than maxilla by a ratio of 3:1.
resembles dental follicle. It contains mature Premolar-molar area in mandible and zygoma
fibrous tissue with sparsely scattered inactive in maxilla. Rarely tumor may appear in the
odontogenic epithelial rests. It is composed condylar region.
of stellate fibroblasts with fine collagen fibrils Symptoms: It is associated with congenitally
in which rests of odontogenic epithelium and missing teeth. The growth rate is slow and pain
dystrophic calcification may or may not be is variable. There is a hard swelling which may
present. be sometime large enough to produce facial
• Granular cell odontogenic fibroma or WHO type: asymmetry.
It is more cellular. It consists of large
Signs: Sometimes it perforates the cortical plate
eosinophilic granular cells with cords and
producing a bosselated surface (several small
islands of odontogenic epithelium. In some
areas, calcified material resembling nodules on the surface). It may appear as
fusiform swelling that may be hard and or may
dysplastic dentin or cementum like material
be covered by a layer of bone of only egg shell. It
may be present.
may cause expansion and become huge if
Management unattended. Teeth will be displaced and
loosened but root resorption is rare. It can invade
Treated with enucleation and curettage. Surgical
excision and usually it does not recur. maxillary sinus and cause exophthalmos.

Histopathological Features
Odontogenic Myxoma
It is made up of loosely arranged, spindle shaped
It is also called as ‘odontogenic fibromyxoma’,
and stellate cells, many of which have long
‘myxofibroma’. It is a rare locally invasive
fibrillar processes that tend to intermesh.
neoplasm consisting of rounded and angular
Collagen fibers are sparse and delicate. Some
cells lying in an abundant mucoid stroma.
have a tendency to form large amount of collagen
Pathogenesis fibers and have been designated as fibro-
myxomas (Fig. 15.36).
Origin is not clear. Various tissues are thought
The space between the cells and fibers is filled
to be a source this tumor. This includes dental
by large amount of mucoid material. Some may
papilla, dental follicle, and periodontal ligament.
exhibit marked cellularity and atypia and have
Other possible sources are my fibroblastic or
a more aggressive course.
fibroblastic-histolytic cells.
Few odontogenic epithelial cells in form of
The myxomatous tissue arises as a direct
cords and nests are present. In some tumors they
outgrowth of dental papilla of tooth or as an
are surrounded by a zone of hyalinization or cell
indirect effect of odontogenic epithelium on
free zone. The intercellular substance is mucoid.
mesenchymal tissue.
The tumor is usually interspersed with a variable
Thought to occur due to degeneration of
number of tiny capillaries and occasionally
odontogenic fibroma.
strands of collagen.
Clinical Features Management
It is slightly more common in females with an Tumors may be difficult to enucleate due to their
age range of 10 to 30 years. It accounts for 3 to loose consistency, therefore surgical excision is
312 Textbook of Oral Pathology

down of bone and formation of cementum.

Appears mixed radiolucency.
• Maturation stage—This is inactive stage where
calcification increases.

Clinical Features
It occurs most frequently under the age of 25 years
and with no significant sex predilection. Mandible
is affected three times more frequently than the
maxilla. Mandibular first molar is the most
frequently affected tooth; other involved teeth are
the mandibular second and third molars.
Symptoms: Associated tooth is vital unless
coincidentally involved. In some cases, pain may
be there.
Sign: Lesion is slow growing and may cause
expansion of cortical plates of bone. Periapical
cementomas are multiple.
Figure 15.36 Odontogenic myxoma showing loose
mucoid tissue (Courtesy: Dr Sangamesh Halawar) Histopathological Features
Cementoma contains trabeculae of cementum
indicates. Resection with generous amount of which are lined by plump active cementocytes.
surrounding bone. These trabeculae contain several basophilic lines
called reversal lines.
Benign Cementoblastoma
It is also called as ‘true Cementoma’. There is large
bulbous mass of cementum or cementum like tissue
on roots of teeth. It is rare benign odontogenic
neoplasm which arises from the cementoblast. It
is a true neoplasm of functional cementoblasts,
which forms large masses of cementum or
cementum-like tissue on the tooth root.

Pathogenesis
It is derived from the mesenchymal cells of
periodontal ligament and cementoblasts. It
evolves in three stages:
• Osteolytic stage—There is periapical bone
resorption. This stage radiographically
appears as radiolucent lesion.
• Osteolytic-osteoblastic stage (Cementoblastic Figure 15.37: Cementoblastoma showing excessive
stage)— It is characterized by both break- cementum formation (CT) around root (R)
 Odontogenic Tumors 313

Trabeculae are separated by marrow spaces this mass either enlarges individually or they fuse
which contain dialed blood vessels and few cells. → the fibrous tissue may be replaced entirely by
At the periphery of the lesion a soft tissue bone of greater density than that of normal bone
rimming consisting of numerous cementoblasts → once cementum is formed it remains
and multinucleated cementoblasts is seen. This unchanged without any reduction.
mass is usually attached to the root of the tooth.
Clinical Features
Management
It occurs during the middle age with a mean age
Excision with extraction of the associated tooth. of 39 years. Male to female ratio is 1:9 and is three
Recurrence is not seen. Tumor can be amputed times more common in blacks than in whites. It
from the tooth and tooth is then endodontically is usually discovered as an incidental finding
treated. during routine radiographic surveys. Mandibu-
lar anterior region is commonly affected. Single
Periapical Cemental Dysplasia area of one jaw may be involved or the greater
part or both the jaws is affected.
It is also called as ‘fibrocementoma’, ‘sclerosing
cementum’, ‘periapical osteofibrosis’, ‘periapical Involved teeth are vital with no history of
pain or sensitivity. Occasional lesions localize
fibrosarcoma’. It is a reactive fibro-osseous le-
near the mental foramen and impinge on the
sion derived from the odontogenic cells in the
periodontal ligament. It is located at the apex of mental nerve and produce pain, paresthesia or
even anesthesia. Hypercementosis is usually
the teeth.
associated with it. It rarely enlarges.
Etiology
Histopathological Features
It occurs as a result of trauma, chronic irritation.
Early, small periapical bone is replaced by
There is possibility that female sex hormones
enlarging mass of fibrous tissue. Small, round to
may be an etiologic factor since the disease is
commonly found in women. ovoid calcifications are deposited within the fibrous
tissue. This deposition is composed of bone,
Nutritional deficiency, metabolic distur-
cementum or non-descript cementum like bone.
bances, past history of syphilis, endocrinal im-
balance and anomalous development.
Management
Pathogenesis It requires continuous observation and
subsequent verification through periodic
Bone at the apex of the affected tooth becomes
radiographic examination. Surgical enucleation
replaced by fibrous tissue which may be small in
is indicated for larger lesions which have caused
amount or occupy an area of a square inch or more expansion of the cortical plates or when the
→ the fibrous tissue may remain unchanged for
clinician is unsure of the working diagnosis.
months to years but in most instances there is a
tendency for a change to occur → the affected MALIGNANT TUMORS
area may become partially reduced in size by
Malignant Ameloblastoma
peripheral ossification → bone or cementum
forms either as a single mass which enlarges by Malignant ameloblastoma are those ameloblas-
aggregation at its margin or as several masses → tomas that metastasize but in which the
314 Textbook of Oral Pathology

metastatic lesion do not show any histological Ameloblastic Carcinoma

difference from the primary tumor.
It is lesion similar to ameloblastoma but that
Pathogenesis shows obvious histological malignant
transformation but the metastatic lesion not
Malignant ameloblastomas originate from solid
necessarily present. There are central and
ameloblastoma which have been treated
peripheral variants.
surgically but recur after sometime. Because of
repeated surgeries tumor cells gain entry into
Pathogenesis
blood vessels and lymph channels and get
disseminated to distant organs mainly lung They may originate from ameloblastoma or
where they proliferate forming new tumor. lining of odontogenic cysts.
Other sites include bones such as skull, Some ameloblastic carcinomas originate from
vertebrae and femur, cervical lymph nodes, liver, preexisting solid or multicystic ameloblastoma.
brain spleen and kidney. Cells of such preexisting tumors dedifferentiated
over time forming carcinoma.
Clinical Features Some tumors originate de novo as
It is a rare type of tumor and diagnosis depends ameloblastic carcinoma.
upon presence of metastases, which is in some
cases is seen in lymph nodes and lungs. Male are Clinical Features
affected more commonly than female. It occurs in
These mainly affect elderly persons. Such lesions
1st to 6th decade with mean age of diagnosis 28-32 are more common in males. Majority of lesions
years. It is almost exclusively in the mandible.
are found in mandible.
Symptoms: Swelling followed by pain and/or
Symptoms: These lesions cause swelling, pain,
rapid growth.
trismus and dysphonia.
Sign: Teeth may be displaced and loosened.
Tenderness of overlying soft tissue is present. Sign: These are rapid growing tumors. There may
be paresthesia of nerves depending upon site,
Sites for metastasis: Lungs, spleen, kidney, lymph
such mental nerve, infra orbital nerve
nodes and ileum.
paresthesia. Some are associated hypercalcemia.
Local extension: It may occur in adjacent bone,
connective tissue or salivary gland.
Histopathological Features
Histopathological Features The lesion resembles ameloblastoma to a great
Malignant ameloblastoma resemble ameloblas- extent. Peripheral cells may loose reverse
tomas of jaw from which they have metastasized. polarity and palisading. Cells at the center
Most of the lesions are of mixed variety or of become condensed and hypercellular.
plexiform type. Other type may also produce Tumor shows cellular features of malignancy
metastasis. such as nuclear enlargement, hyperchromatic,
mild pleomorphism, increased nucleus to
Management cytoplasmic ratio increased mitotic activity and
It is treated with en bloc resection. Radiation abnormal mitosis. In some cases keratin pearl
therapy and chemotherapy for pulmonary formation and individual cell keratinization may
metastasis be seen.
 Odontogenic Tumors 315

Types
• Those arising from odontogenic cysts.
• Those arising from ameloblastoma either
well differentiated (malignant ameloblas-
toma) or poorly differentiated (ameloblastic
carcinoma).
• Those arising de novo from odontogenic
epithelium residues, either keratinizing or
non-keratinizing.

Clinical Features
Figure 15.38: Ameloblastic carcinoma mild pleomorphism
There is a wide range of age distribution with
majority of cases occurring in 6th and 7th decade
Management
of life. It is more common in males than in
It is treated with en bloc resection. Radiation females with a ratio of 2:1 and more common in
therapy and chemotherapy for pulmonary mandible than in maxilla.
metastasis
Symptoms: The early symptom is swelling of the
Primary Intraosseous Carcinoma jaw with pain and mobility of the teeth before
ulceration has occurred. Pathological fracture
It is also called as ‘central mandibular carcinoma’, and lip paraesthesia also occurs.
primary intraosseous carcinoma’, primary
epithelial tumor of the jaw, ‘primary intra-alveolar Signs: There is rapid expansion and destruction
epidermoid carcinoma’ and central squamous cell of jaw bones. Tumor invades the periodontal
carcinoma’. It develops within the depth of the ligament and the alveolar bone, destroying it.
jaw. It is rare and may remain silent until they There may be lymphadenopathy. Surface
have reached a fairly large size. epithelium is normal. Occasionally the pulp of
the teeth may be invaded by neoplasm.
Origin Perforation of cortical plate may occur.
Extraction of teeth result in non healing socket
Malignant transformation of epithelial lining of
and sometimes tumor may protrude from the
odontogenic or non-odontogenic cyst. Malignant
non-healed socket.
transformation of ameloblastoma by metastases
from different sites. In case of maxilla, may arise
Histopathological Features
from primary tumor of the maxillary sinus. The
tumor arises from the cell rests of the It has an alveolar or plexiform pattern with
odontogenic epithelium or from the epithelial peripheral cells of the tumor masses showing
remnants at the site of fusion between two palisading arrangement, thereby resembling
embryonic processes. In normal situation for odontogenic epithelium. It is usually of basal cell
some of the original cells of the dental lamina or type although on occasion, spinous cells may be
enamel organ to remain in the jaw long after the found. The tumor cells themselves generally
function of these cells are completed. Malignant exhibit nuclear pleomorphism and hyper-
tumor may develop from these cells. chromatism, mitotic activity.
316 Textbook of Oral Pathology

Excessive proliferation of neoplastic epithe- may be chance that, there are carcinomatous
lium cells either in form of diffuse sheets or epi- changes found in odontogenic cyst. In some cases
thelial islands. Areas of acanthotic changes with adjacent carcinoma may involve otherwise
epithelial pearl formation are often seen. There unrelated cyst.
is presence of rim of ameloblasts like cells in the
position which is usually assumed by the basal Clinical Features
cell in conventional squamous cell carcinoma.
It can affect any cyst in the jaw. But keratocyst is
more likely to undergo malignant changes than
Management
other cysts. The most common complain is pain,
Surgical resection rather than radiotherapy. which is dull and of several months duration. If
upper jaw is involved, sinus pain and swelling may
Clear cell odontogenic tumor or Carcinoma be present. Pathological fracture, fistula formation
It is rare odontogenic tumor with potential for and regional lymphadenopathy may occur.
lymphatic or pulmonary metastases. Its origin
Management
is unknown.
It is same as with primary intraosseous carcinoma.
Clinical Features
Ameloblastic Fibrosarcoma
Most cases reported in women over 60 years of
age. Both maxilla and mandible have been It is the malignant counterpart of the
involved. It may present as asymptomatic or ameloblastic fibroma in which mesenchymal
painful bony swelling. elements has become malignant. It is also called
as ‘ameloblastic sarcoma’.
Histopathological Features
Clinical Features
There are nests of epithelial cells with clear or
It is very rare and most frequently occurs in
faintly eosinophilic cytoplasm which is
young adults with an average age of occurrence
separated by thin strands of hyalinized material.
being 30 years. There is no sex predilection. It
Peripheral cells may demonstrate palisading.
more frequently occurs in maxilla than in
Some consider this tumor to be a clear cell variant
mandible. it is uniformly painful, generally
of the ameloblastoma. Clear cells contain high
grows readily and causes destruction of bone
amount of glycogen similar to cells of dental
with loosening of teeth. There may be ulceration
lamina.
and bleeding of the overlying mucosa. Swelling
is usually soft in consistency.
Management
Tumors demonstrate aggressive local behavior Histopathological Features
and potential lymphatic and pulmonary There are no apparent remarkable changes in the
metastases and therefore should be treated with
odontogenic epithelium in malignant lesions and
extensive resection.
it maintains its benign appearance. In some
lesions, it is diminished in quantity, apparently
Malignant Changes in Odontogenic Cyst
as a result of overgrowth of the malignant
It is also called as ‘carcinoma ex odontogenic mesenchymal portions of the lesion.
cyst’. It is uncommon but in some cases there Mesenchymal tissue exhibits a remarkable
 Odontogenic Tumors 317

increase in cellularity; the malignant fibroblast Clinical Features

being bizarre and pleomorphic with
It is a destructive lesion which produces a fleshy,
hyperchromatic nuclei and numerous atypical
bulky growth. It as such is asymptomatic but
mitotic figures.
pain may be present in many cases.

Management Histopathological Features

Radical resection such as hemimandibulectomy It is identical with that of fibrosarcoma of non-
or hemimaxillectomy can be done. Recurrence odontogenic origin. The cellular element may or
is common and prognosis is poor. may not be prominent than the fibrillar
component. The cells often exhibit considerable
Ameloblastic Odontosarcoma mitotic activity. They resemble immature
fibroblasts and appear as elongated cells
It is similar to ameloblastic fibrosarcoma but
containing ovoid nuclei with varying degree of
limited amounts of dysplastic dentin and enamel
pleomorphism and are situated in a fibrous
are formed. meshwork which may or may not exhibit foci of
odontogenic epithelium.
Odontogenic Fibrosarcoma
Management
It is the malignant counterpart of odontogenic
fibroma. It originates from same mesenchymal Radical surgical removal with resection of the
tissue, as same in central fibroma. jaw. Prognosis is poor.
318 Textbook of Oral Pathology

CHAPTER
Cysts of the
16 Orofacial Region

Cyst has been known to arise in man (ever since General Diagnostic Features of Cysts
he has teeth) and in certain animals. All it takes is Clinical Features
some odontogenic epithelium plus some
unknown initiating factor which stimulates it to It usually depends on the extent and the
proliferate to cause this destructive lesion, which dimension of lesion. A small cyst is unlikely to be
can be found from the mildest form to a greatly diagnosed on routine examination because signs
disfiguring form. will not be demonstrable.
Cysts of jaws are of great clinical importance, As the cyst grows larger, expansion of alveolar
not only because they often attain a large size and bone occurs, usually on the labial and buccal
therefore produce facial asymmetry, disturbance of aspect of the jaw in case of a typical odontogenic
dentition, neurological symptoms and predispose cyst. However, expansion of the lingual aspect
the jaws to fracture but particularly because they alone can occur in cases of cysts of ramus and
have a very high frequency of occurrence. third molar area. The expansion of both inner and
outer bony wall is often indicative of lesion other
Definitions than cyst.
As the cyst increases in size, the periosteum is
• By Killey and Key 1966 — this entity stimulated to form a layer of new bone and it is
constituted an epithelium-lined sac filled with this sub-periosteal deposition which later forms
fluid or semifluid material. the outline of the affected portion of the jaw and
• By some unknown author 1966 — a cyst is an produce enlargement.
abnormal cavity in hard and soft tissue which At an early stage, the lateral expansion
contains fluid, semifluid, or gas and is often causes smooth, hard, painless prominence but as
encapsulated and lined by epithelium. cyst growth proceeds, the bone covering the center
• By Kramer in 1974 — pathologic cavity having of the convexity becomes thinned and can be
fluid, semifluid, or gaseous content but not indented with pressure. The term elastic is used
always is lined by epithelium. to describe this particular consistency.
 Cysts of the Orofacial Region 319

In some cases of expanding dental cyst there distribution. Since an uninfected cyst grows
comes the time when the periosteum appears to slowly and exerts a very slight pressure on the
be unable to maintain the new bone formation, neurovascular bundle. However, in cases of acute
resulting in an area where there is no bone, forms infection there is a sudden increase in pressure
the summit of the convexity. This is called as from pus accumulation in the sac and this can
'window' formation. Window is more or less cause neuroparaxia of the nerve and the
round and has smooth margins which sometimes immediate onset of labial paresthesia or
appears to be very slightly dense at periphery. anesthesia. After surgical drainage of infected
Later the fragile outer shell of bone becomes cyst, sensation returns back to normal.
fragmented and the sensation imparted and The teeth adjoining an odontogenic
sound produced on palpation over the area is keratocyst, fissural cysts or solitary cysts are vital.
described as 'egg shell crackling'. Apical periodontal cyst is associated with non-
In advanced cases, these sample pieces of vital teeth, but in cases of lateral periodontal cysts
bone disappear and are not replaced by new the involved tooth often are vital.
bone. At this point, the cyst lining lies A large anterior cyst may extend under the
immediately beneath the mucosa and fluctuation nasal floor and cause distortion of nostril.
can be elicited. Involvement of the antrum by an infected cyst
Greater distension of the wall of the cyst leads may produce signs of maxillary sinusitis. In the
to an eventual discharge of fluid into the mouth. jaws a cyst enlarging between teeth can cause
This sequence of events is commonly observed their root to diverge and the crown to converge.
with the progressive enlargement of periodontal Afterwards the teeth may tilt over one side.
and dentigerous cysts.
Site of occurrence is also important in Symptoms
considering the diagnostic features. Periodontal Often the first symptom patients experience is
cysts may occur anywhere in the dental arch. pain and swelling, if the cyst becomes infected.
Dentigerous cysts however are mainly associated Some times the patient notices lump in the
with impacted molars and canines. Fissural cysts sulcus.
are confined to the upper jaw and the solitary When the cyst becomes secondarily infected
bone cyst is virtually assigned to the mandible. and discharges into the mouth and when a sinus
Odontogenic keratocysts are most often seen in tract is present, then patient may complain of
the lower third molar area extending into ramus. salty taste or a sinus tract is present;
Cysts rarely cause loosening of adjacent teeth Edentulous patients may seek treatment
until they become extremely large. The clinical because of displacement of denture.
absence of one or more teeth from normal series Discoloration or loosening of tooth may
without a history of extraction may indicate the prompt patients to visit the dentist.
presence of developing dentigerous cyst. A single To confirm the clinical diagnosis, radiological
missing tooth may also invite suspicion of examination provides valuable information.
existence of odontogenic keratocyst or primordial
cyst. Radiographic Examination
Although large mandibular cysts invariably Classically, radiographic appearance of a cyst is
involve the neurovascular bundle and deflect that of a well-defined round or oval areas of
structure into an abnormal position, still it is radiolucency circumscribed by a sharp radiopaque
unusual to find anesthesia of the mental margin.
320 Textbook of Oral Pathology

CLASSIFICATIONS
First Classification (by Robinson, 1945)
A. From odontogenic tissues • Dentigerous cyst
• Periodontal cyst • Primordial cyst
– Radicular or dental root apex type B. From non-dental tissues
– Lateral type • Median cyst
– Residual type • Incisive canal cyst
• Globulomaxillary cyst

Second Classification (by WHO)

A. Developmental • Non-odontogenic
• Odontogenic cysts – Nasopalatine duct (incisive canal) cyst
– Primordial – Globulomaxillary cyst
– Gingival cyst of infants – Nasolabial cyst
– Eruption cyst B. Inflammatory cysts
– Dentigerous cyst (follicular) • Radicular
– Gingival cyst of adults • Apical and lateral
– Lateral periodontal cyst • Residual
– Glandular odontogenic cyst, sialo-odontogenic cyst • Paradental (inflammatory collateral, mandibular
infected buccal cyst).
By Gorlin (1964)
A. Odontogenic cysts • Median mandibular cyst
• Dentigerous cyst • Anterior lingual cyst
• Eruption cyst • Dermoid and epidermoid cyst
• Gingival cyst of newborn infants • Palatal cyst of newborn infants
• Lateral periodontal and gingival cyst C. Cysts of neck and floor of moush and salivary gland
• Keratinizing and calcifying cyst • Thyroglossal duct cyst
• Radicular cyst • Lymphoepithelial (bronchial cleft) cyst
• Primordial cyst • Oral cysts with gastric or intestinal epithelium
• Multiple cysts of jaws and multiple cutaneous nevoid • Salivary gland cyst
basal cell carcinoma and skeletal anomalies • Mucocele and ranula
B. Non odontogenic and fissural cysts D. Pseudo-cyst
• Globulomaxillary cyst (premaxilla maxillary cyst) • Aneurysmal bone cyst
• Nasoalveolar (Nasolabial Klestadt's) cyst • Static (developmental latent) bone cyst
• Nasopalatine (median anterior maxillary) cyst
• Traumatic (hemorrhagic solitary) bone cyst

Classification (by Shear) — Most accepted

I. Cyst of jaws b. Non-odontogenic
A. Epithelial • Nasopalatine duct
1. Developmental • Median palatine median alveolar and median
a. Odontogenic mandibular cysts
• Odontogenic keratocyst • Globulomaxillary cyst
• Eruption cyst • Nasolabial cyst
• Primordial cyst • Midpalatal raphe cyst of infants
• Gingival cyst of adults 2. Inflammatory cyst
• Lateral periodontal cyst • Radicular cyst
• Calcifying epithelial odontogenic cyst • Residual cyst
• Botryoid odontogenic cyst • Inflammatory collateral cyst
• Glandular odontogenic cyst • Paradental cyst
 Cysts of the Orofacial Region 321

B. Non-epithelial • Thyroglossal duct cyst

• Simple bone cyst (traumatic bone cyst) • Anterior median lingual cyst
• Aneurysmal bone cyst • Oral cyst with gastric or intestinal epithelium
II. Cysts of Maxillary antrum • Cystic hygroma
• Benign mucosal cyst of maxillary antrum • Nasopharyngeal cysts
• Surgical ciliated cyst of maxilla • Cyst of salivary glands
III. Cysts of soft tissues of mouth, face and neck • Parasitic cyst, hydatid cyst, cysticercosis cellulosae;
• Dermoid and epidermoid cyst Trichinosis
• Branchial cleft cyst (lymphothelial cyst)

Nevertheless, there is much variation to this Aspiration

standard pattern, which depends not only on the Aspiration of a suspected cyst is a valuable
type of cyst but also its location and the degree diagnostic aid when doubt exists about the
of bone destruction and expansion. nature of lesion after careful clinical and
In the radiographic diagnosis of cysts of jaws, radiographic examination. This examination is
periapical and occlusal views are helpful. In also helpful in distinguishing between maxillary
addition, in case of large cysts, extraoral views sinus and maxillary cyst. Aspirate withdrawn
provide an essential supplement to conventional from the cyst appears as straw colored fluid.
intra-oral radiographs, which include lateral Aspiration of air indicates penetration into the
oblique, PA view for mandible, water's view and antrum. Failure to obtain air or fluid is an
panoramic view[OPG]. indication of a solid lesion.

The Use of Contrast Media for the Radiographs of Theories of Cyst Enlargement
Cysts of the Maxillary Sinus The exact mechanism is not known, but it could
In certain cases, like cysts in the maxillary sinus, be that the mechanism-governing enlargement
use of contrast media, along with routine of cysts of the jaws is the same irrespective of the
radiography is helpful in delineating the size and type of cysts.
precise extent of the cyst. The various steps involved in the formation of
In this technique, the radiopaque medium is a cyst seem to be as follows:
injected into the maxillary sinus with an • The attraction of fluid into the cystic cavity.
aspirating syringe. • The retention of fluid into the cavity.
The mucosa is anesthetized and the needle is • The production of raised internal hydrostatic
introduced into the maxillary sinus through the pressure.
inferior meatus as for an aspiration biopsy of the • The resorption of surrounding bone with an
sinus. increase in the size of bone cavity.
Harries classified the theories of cyst enlargement
Ultrasonic Diagnosis in the following manner:
Ultrasonography can be useful to give adequate • Mural growth
information about the location and extent in – Peripheral cell division
depth of cysts. – Accumulation of cellular content
It is possible to make a diagnosis on the basis • Hydrostatic enlargement
of the echo of an ultrasonic impulse and the – Secretion
margins of bone. It can also provide very – Transudation and exudation
interesting information for the diagnosis of – Dialysis
conditions in the maxillary sinus. • Bone resorbing factor
322 Textbook of Oral Pathology

Mural Growth
Peripheral cell division
Peripheral enlargement is attributed to active cell
division of the lining epithelium in response to
an irritant stimulus.
Cyst regression occurs following the removal
of such stimulus.
The theory has been criticized on the basis
that such regression would lead to an irregularly
thickened inner surface because of the resistance
of the surrounding bone. However, this ignores
the possibility that the cyst wall is not only well
supported by its fluid content but can also Figure 16.2: Enlargement of cyst by hydrostatic pressure
actively resorb bone sufficiently and rapidly to
accommodate the expanding perimeter (Fig. 16.1). extend preferentially along the less dense
cancellous bone with little resorption and
Accumulation of Cellular Content expansion of dense cortex.
Kramer has suggested that keratocyst enlarges by
the increasing accumulation of mural squames Hydrostatic Enlargement
as they are cast off from the living epithelium. Growth is attributed to the distension of the cystic
The characteristic finger like projections of wall by fluid that has accumulated by one or
growth represents local areas of increased cell more of the following processes.
division. Secretion — apart from the occasional goblet cells
An alternative explanation for this elongation usually found in follicular cyst, there is little
is that keratocyst although persistent in their morphological evidence of intra-cystic secretion.
growth are poor bone resorbers and simply Transudation and exudation — these have been
proposed mainly for the enlargement of the
follicular and periodontal cyst respectively. This
conclusion was derived from an examination of
the protein content and specific gravity of the
cystic fluid. The presence of fibrin and cholesterol
in periodontal and follicular cysts suggests that
hemorrhage also contributed to the cystic fluid.
Dialysis — the mean osmolality of the cystic fluid
is 10 miliosmoles higher than that of serum. This
gradient is attributed to the accumulation of the
low molecular weight cells shed from the lining
epithelium and maintained by inadequate
lymphatic access to the cyst lumen, the
Figure 16.1: Cyst enlargement by active proliferation of consequence is net entry of fluid from the capsule
the cyst wall capillaries into the cystic lumen.
 Cysts of the Orofacial Region 323

Bone Resorbing Factor 1. Developmental odontogenic cysts.

2. Inflammatory odontogenic cysts.
Vital cyst tissue in culture has been shown to
release a potent bone resorbing factor, which is
DEVELOPMENTAL ODONTOGENIC CYSTS
predominately a mixture of prostaglandin E2
(PGE2) and prostaglandin E3 (PGE3). Dentigerous Cyst
The source of this resorbing factor appears to
Dentigerous cyst is the developmental
be the capsule and leukocyte content.
Prostaglandin release is reduced in some cysts odontogenic cyst of epithelial origin. It is also
called as 'follicular cyst' or 'pericoronal cyst'.
when the epithelium is removed before culture
It is the most common type of odontogenic
but it is not clear whether the reduction results
from the removal of an epithelial inductive effect cyst which encloses the crown of an unerupted
tooth by expansion of its follicle and is attached
or whether it merely reflects the loss of
to the neck.
prostaglandin produced within the epithelium.
The mechanism of prostaglandin production
Origin
is not known. One possibility is that production
takes place in the capsule under the influence of Can be
epithelial proliferation, lysosomal phospholipase • Intrafollicular
from fibroblasts and polymorphonuclear • Extrafollicular
leukocytes; breaking down of phospholipid cell
membrane to produce arachidonic acid which is Intrafollicular Theory
converted by the ubiquitous enzyme prostaglandin Occurs due to fluid accumulation
synthetase to prostaglandin (Fig. 16.3).
(a) Between the layer of reduced enamel
epithelium, i.e. inner and outer enamel
EPITHELIAL CYSTS
epithelium after formation of crown
Epithelial cyst are classified as:
I. Odontogenic cysts
II. Non odontogenic cysts
Odontogenic cyst are of two categories, based
on their cause of occurrence.

Figure 16.4: Intrafollicular cyst formation occurs by

degeneration of stellate reticulum and accumulation of fluid
Figure 16.3: Cyst enlargement by bone resorption between layers of reduced enamel epithelium
324 Textbook of Oral Pathology

(b) Within the enamel organ itself by the (c) The cyst may occur if an impacted tooth erupts
degeneration of stellate reticulum at an early into an existing odontogenic keratocyst. This
stage of tooth development resulting into cyst incidence is uncommon, the features are more
formation associated with enamel hypoplasia. of OKC than the dentigerous cyst.
Al-Tabani and Smith (1980) (d) Main's theory (1970)
(c) The fluid's pressure incites a proliferation of – Impacted tooth exerts pressure on its
the outer enamel epithelium, which remains follicle due to the eruptive force
attached to the tooth at the cementoenamel – This obstructs the venous outflow and
junction; the inner enamel epithelium is then thereby induces rapid transudation of
pressed onto the crown surface. serum across the capillary walls.
– The increased hydrostatic pressure exerted
Extrafollicular Theory (Fig. 16.5) by this pooling of fluid causes separation
of the crown from the follicle, with or
(a) This theory suggest that the cyst forms by
without reduced enamel epithelium.
accumulation of fluid in between the
unerupted tooth and the reduced enamel Further the osmolality of the cyst fluid is:
• Modified by various factors such as increased
epithelium.
permeability to passage of greater quantities
(b) Other theory says that the crown of a
permanent tooth may erupt into a radicular of proteins,increased amount of glycosami-
noglycans, predominantly hyaluronic acid
cyst of a deciduous tooth. As the incidence
and heparin and chondroitin sulfate in the
of radicular cyst is possibilities of cyst
formation is uncommon with the deciduous cyst wall causes expansile growth rapidly.
Browne and Smith et al (1980)
teeth, the possibilities are rare of these type of
cysts.

Figure 16.5: The cyst forms by accumulation of fluid in

between the unerupted tooth and the reduced enamel Figure 16.6: Development of dentigerous cyst by
epithelium. (Extrafollicular origin) eruption of normal tooth into overlying preexisting cyst
 Cysts of the Orofacial Region 325

• Thus in each theory, the fluid generates the Expansion: The most important feature of these
cystic proliferation by its hyperosmolar lesions is its potential to expand.
content created by cellular breakdown and cell Cystic involvement of an unerupted third
products, causing an osmotic gradient to molar may result in hollowing out of the entire
pump fluid into the cyst lumen. ramus extending up to the coronoid process and
condyle as well as the body causing expansion
Clinical Features of cortical plates.
Most of them discovered by radiographs, when In the case of a cyst associated with maxillary
these are taken because a tooth is missing or teeth cuspids, expansion of the anterior maxilla often
are tilted or out of alignment. occurs and may superficially resemble acute
sinusitis or cellulitis.
Age and sex: As it arises from the follicle of an
unerupted tooth, it is usually found in children Associated disease: Bilateral cysts are found in
and adolescents with a higher incidence in 2nd association with basal cell nevus syndrome,
and 3rd decades. Equal in both sexes. cleidocranial dysplasia and a rare form of
Site: Most commonly associated with mandibular amelogenesis imperfecta.
3rd molars and maxillary canines which are most Blue domed cyst: When it contains blood, then it is
commonly impacted. called as 'blue domed cyst'.
It may also be found enclosing a complex com-
pound odontome or involving a supernumerary Radiographic Features
tooth.
Radiodensity — well defined radiolucency usually
Size: They vary in size from a little more than the
associated with hyperostotic borders unless they
diameter of the involved crown to an expansion
are secondarily infected and is seen around an
that causes progressive but painless enlargement
unerupted tooth (Fig. 16.7).
of jaws and facial asymmetry.
Internal structure — usually, it is unilocular but
Teeth: Teeth adjacent to the developing cyst and
some times it may appear multilocular, this image
involved teeth may get severely displaced and
is caused by ridges in the bony wall and not by
resorbed.
the presence of bony septa.
There may be displacement of third molars to
such an extent that it sometimes comes to lie
compressed against the inferior border of the
mandible.
Symptoms: Generally, it is painless but may be
painful if it gets infected.
When dentigerous cyst expands rapidly to
compress sensory nerve it produces pain which
may be referred to other sites and described as
headache.
Signs: The dentigerous cyst has a potential to
become an aggressive lesion with expansion of
bone and subsequent facial asymmetry.
In some cases pathological fracture can occur. Figure 16.7: Radiograph of dentigerous cyst
326 Textbook of Oral Pathology

Margins — The bony margins are well defined

and sharp.

Teeth
It may envelope the crown symmetrically, but it
may expand laterally from the crown.
Associated tooth may be displaced in any
direction.
It is usual for those unerupted teeth which
become surrounded by the growing cyst to retain
their follicle for a time at least, which serves to Figure 16.8: Central variety of dentigerous cyst
indicate that the tooth is actually outside the cyst.
The large cysts are always confined to the
mandible. There may be resorption of roots of
adjacent teeth.
Teeth may be displaced in the coronoid
process, high up above the tooth bearing region
of maxilla.
When maxillary canine is involved, it may get
displace into the maxillary sinus or adjacent to
nasal fossa or even in the floor of orbit. Figure 16.9: Lateral variety of dentigerous cyst
Outline may expand from the ramus into the
coronoid process or condyle.

Radiographic Types
According to Thoma
Central variety — in it crown is enveloped
symmetrically. In this instance pressure is applied
to the crown of the tooth and may push it away
from its direction of eruption. In this way the
mandibular third molar may be found at the lower Figure 16.10: Circumferential variety of dentigerous cyst
border of the mandible and in the ascending
ramus and a maxillary canine in the sinus or as Circumferential type — in it the entire tooth appears
far as the floor of the orbit. The maxillary incisors to be enveloped by the cyst. The entire enamel
may be found below the floor of the nose (Fig. 16.8). organ around the neck of the tooth becomes cystic
Lateral type — in it dentigerous cyst is a often allowing the tooth to erupt through the cyst
radiographic appearance which results from (Figs 16.10 to 16.12).
dilation of the follicle on one aspect of the crown.
According to Mourshed
This type is commonly seen when an impacted
mandibular molar is partially erupted so that its Class I — dentigerous cyst associated with
superior aspect is exposed (Fig. 16.9). completely unerupted teeth
 Cysts of the Orofacial Region 327

Figure 16.11: Bilateral dentigerous cyst

Figure 16.13: Dentigerous cyst

Figure 16.12: Multiple dentigerous cyst

• Dentigerous cyst associated with unerupted

teeth, who failure to erupt is due to lack of
space in the dental arch. Figure 16.14: Dentigerous cyst
• Dentigerous cyst associated with unerupted
teeth, who failure to erupt is due to mal-
positioning of the tooth germ.
• Dentigerous cyst associated with unerupted
supernumerary teeth.
Class II — dentigerous cyst associated with
partially erupted teeth (Figs 16.13 to 16.16).

Histopathological Features

It is composed of thin cystic wall (Fig. 16.16)

• The lining is a thin layer of non-keratinized
stratified squamous epithelium.
• In very few instances the lining may be
keratinized and it may be mistaken as Figure 16.15: Dentigerous cyst
328 Textbook of Oral Pathology

Figure 16.17: Inflamed dentigerous cyst with lining of variable

thickness. The stroma of connective tissue is organized,
fibrous and shows vascular proliferation and juxtaepithelial
Figure 16.16: Dentigerous cyst with thin lining resembling inflammatory infiltrate
reduced enamel epithelium with no rete pegs the connective
tissue is delicate as of primitive type Rushton bodies within the lining epithelium
which are peculiarly seen as linear or hairpin
keratocyst or keratin may be produced rarely shaped curved bodies have been reported as a
as due to metaplastic changes. result of inflammation.
• As the lining is derived from reduced enamel
epithelium it is 2-4 cell layer thick primitive Metaplasia in Dentigerous Cyst
type of epithelium. The cell lining may show metaplastic changes in
The cells are cuboidal or low columnar. the form of:
Retepegs formation is absent except in cases • Mucous producing cells or secretory cells
that are secondarily infected such as goblet cells
As the connective tissue wall is derived from • Pseudostratified ciliated columnar
the dental follicle of developing enamel organ, it epithelium also has been reported.
is a loose connective tissue stroma. • Rarely sebaceous glands in the walls are
Young fibroblast are present in the stroma observed.
which is rich in acid mucopolysaccharides. The content of the cystic lumen is usually thin
watery yellow fluid and is occasionally blood
Inflamed Dentigerous Cyst (Fig. 16.17) tinged.

The epithelial lining shows variable thickness in Differential Diagnosis

response to inflammation
Ameloblastoma and ameloblastic fibroma — they are
Budding or proliferation of the epithelium
multilocular and not associated with crown of
into the connective tissue wall may be seen.
an unerupted tooth. They will grow laterally
Occasional islands of odontogenic epithelium
away from the tooth in comparison to
are observed.
dentigerous cyst, which envelope the tooth
The connective tissue wall is frequently quite
symmetrically and it is more common in
thickened and composed of organised fibrous
premolar and molar area.
connective tissue.
Inflammatory cells infiltrate the connective Adenomatoid odontogenic tumor — they are rare and
tissue mostly juxtaepithelial. occur in the maxillary anterior region.
 Cysts of the Orofacial Region 329

Calcifying odontogenic cyst — it may occur as Mucoepidermoid carcinoma — the development

pericoronal radiolucency and may contain of mucoepidermoid carcinoma, which is a
evidences of calcification. malignancy of salivary glands, associated with
Developmental primordial and follicular primordial the lining epithelium or dentigerous cyst which
cyst — it can also be considered in the differential contain mucous secreting cell as a result of mucous
diagnosis. It occurs in close proximity to the crown metaplasia.
of unerupted teeth and superimposition of its
image may cause the cyst like radiolucency to Eruption Cyst
appear as dentigerous cyst on the radiograph.
A specific type of cyst, which must be classified
However, in cases of follicular primordial cyst the
as a form of dentigerous cyst, is frequently
cystic lining surround the crown, which in
dentigerous cyst is attached to the neck of tooth. associated with the erupting deciduous or
The diagnosis of primordial cyst can be confirmed permanent teeth in children.
by taking a radiograph with different angulations. An eruption cyst is in fact a dentigerous cyst
occurring when a tooth is impeded in its eruption
Management within the soft tissues overlying the bone, whereas
the dentigerous cyst develops around the crown
Treatment of dentigerous cyst depends upon the of an unerupted tooth which is lying in the bone.
size of the cyst. This cyst has often been termed as 'eruption cyst'
Surgical — smaller lesions can be surgically or 'eruption hematoma'.
removed with little difficulty. The larger cyst It is essentially a dilation of the normal tooth
involves surgical drainage and marsupialization. caused by accumulation of tissue fluid or blood.
This procedure results in relief of pressure and In 11% of cases it occurs during the eruption of
gradual shrinking of the cystic lesion by peripheral incisors and in 30% of cases it occurs during
opposition of new bone. eruption of canines and molars.
Appearance — clinically the lesion appears as
Decompression — small acrylic button or short
a circumscribed, fluctuant, often translucent
section of rubber - is placed in preformed surgical
opening in cyst which keeps opening open and swelling of the alveolar ridge over the site of
permits drainage. eruption of the tooth.
Recurrence is relatively uncommon unless Eruption hematoma — when the circumscribed
there has been fragmentation of the cystic lining cystic cavity contains blood the swelling appears
with remnants allowed to remain. purple or, deep blue, hence it is termed as 'eruption
hematoma'.
Potential Complication The lesion is called 'eruption cyst' because of
There are several potential complications besides their association with erupting teeth. The cause
the possibility of recurrence following for development of this form of dentigerous cyst is
incomplete surgical removal. These include— not known.
Ameloblastoma — the development of an Radiographic features — radiographically
ameloblastoma either from the lining epithelium expansion of the normal follicular space of
of the cyst or rests of odontogenic epithelium in erupting tooth crown. In some cases there is saucer
the wall of the cyst which is also called as mural shaped excavation of bone projecting very slightly
ameloblastoma. into the cavity.
330 Textbook of Oral Pathology

Odontogenic Keratocyst (OKC) strongly hypothesized that the keratocysts might

express proteins not commonly expressed in
Odontogenic keratocyst is a developmental
normal epithelia.
odontogenic cyst of epithelial origin.
Other ultrastructural and histochemical
OKC was previously termed as primordial cyst
studies have proved that
by Robinson (1945). According to Pindborg's and
1. altered gene expression of the epithelial cells
Hansen the designation keratocyst was used to
as suggested by gp38 positivity (epithelial-
described any jaw cyst exhibiting keratinization
specific cell surface glycoprotein) which is seen
in their lining which may occur in follicular,
in cells with neoplastic potential.
residual and very rarely in a radicular cyst.
2. p53 protien -a mutant product of the tumor
suppressor gene p53 was shown to have an
Odontogenic Keratocyst as a
increased expression. this is seen in malignant
Benign Neoplasm
cells and not in normal cell cycle.
Odontogenic keratocyst is considered in newer 3. Ki-67 is an antigen or cell specific marker for
classifications of the odontogenic neoplasms (see proliferating cells; it is also increased in
chapter of odontogenic tumors) as a neoplasm than parakeratinized OKC.
a cyst for the following reasons: 4. PCNA- Proliferating cell nuclear antigen, is
Aggressive clinical behaviour than any other widely regarded as a marker for replication
cyst and is associated with DNA repair processes
1. asymptomatic till it reaches a large size and stimulation growth factors. This
2. tends to extend through the medullary cavities immunohistochemical expression is also
rapidly as in some reported cases complete raised in the lining cells.
hollowing of the ramus of mandible, including 5. AgNOR-Argyrophillic nucleolar organizing
the condylar and coronoid process has been regions are shown to be more. The mean
recorded before any symptoms. In maxilla, if it number of AgNORS per nucleus was
occurs, it may involve the entire maxillary significantly more in the inflamed
sinus in a similar fashion. odontogenic keratocyst than in any other
High mitotic count of basal and para basal inflamed cyst.
layers. Origin of Cyst
Highest turnover rate of the epithelium than
any other cyst. The OKC is originating from the the odontogenic
The studies for mean mitotic counts- shows epithelium;
the proliferative index OKC (mean = 8), Dental lamina or it's remnants. Which
nonodontogenic cyst (mean = 2.3) and radicular possesses marked growth potential or
cyst (mean = 4.5). Alternatively from proliferation of basal cells
The proliferative index is more similar to those as 'basal cell hamartias' which are offshoots of
seen in ameloblastoma and dental lamina. the basal cells.
(Richard Jordan, oral and maxillofacial surgery
Clinical Features
clinics of north America -2003)
Active collagenase aids to rapid growth and Age — odontogenic keratocyst occurs over a wide
extention of the cyst. age range and cases have been recorded as early
In view of the established association of OKC as the first decade and as late as the ninth with
and Nevoid basal cell carcinoma syndrome it was age group 4 to 84 years. Initiated early in life,
 Cysts of the Orofacial Region 331

during the period of tooth development with a Occurrence of satellite cyst, which is a bud-
peak incidence in 2nd and 3rd decades. like projection of basal cell layer into the connective
tissue, i.e. retained during the enucleation
Sex — it is found more frequently in males than in
procedure.
females and this sex predilection is more
Some instances of recurrence are likely
pronounced in blacks than in whites.
because of new cyst formation rather than true
Site — it is more common in mandible with a recurrence.
greater incidence at the angle and extending for Secondarily, its lining is very thin and fragile
varying distance into the ascending ramus and particularly when the cyst is large and therefore
forward into the body. more difficult to enucleate than a cyst with thick
Symptoms wall. Portion of the lining may be left behind and
• Asymptomatic unless they become secondarily constitute the origin of recurrence.
infected, in which case patient complains of Enucleation in one piece may be more
pain, soft tissue swelling and drainage. difficult with cysts which have a scalloped
• The maxillary sinus gets infected in the initial margins and this may explain the higher
phase of cyst enlargement, so these OKC may recurrence rate than those with smoother
manifested earlier than the duration taken by contour.
the mandibular cyst to manifest. Toller suggested that there may be an
• Occasionally, paraesthesia is experienced of intrinsic growth potential in the epithelial
the lower lip or teeth with mandibular cases. lining which may be responsible for a higher
• Paraesthesia can also occur, if there is a recurrence rate.
pathological fracture. It may arise from proliferation of the basal
Teeth — teeth may be displaced, if it expands cells of the oral mucosa particularly in the third
through cancellous bone and the body of the molar area and ascending ramus of the mandible.
mandible. It is referred that there is often a firm adhesion
of the cyst to overlying mucosa and it is
Signs
recommended that mucosa should be excised with
• The lesion can lead to pathologic fracture.
them in an attempt to prevent possible recurrence
• Those that occur in the maxilla causes buccal
from the residual basal cell proliferation.
expansion.
• On aspiration there is odorless creamy or
caseous content. Radiographic Features
Multiple odontogenic keratocyst are found in Site — more than 90% are seen posterior to the
following syndromes: canines in the mandible and more than 50% at
• Gorlin-Goltz syndrome. the angle of the mandible.
• Marfan syndrome.
• Ehler's Danlos syndrome. Characteristic — 40% have a characteristic
• Noonan's syndrome. suggestive of dentigerous cyst, 25% suggestive of
primordial cyst, 25% lateral periodontal cyst and
Recurrence 10% of globulomaxillary cyst.
Rate of recurrence of odontogenic keratocyst is Internal structure — undulating borders cloudy
very high. There are number of reasons for it. interior appearances suggestive of multilocularity.
332 Textbook of Oral Pathology

Size — size varies and may be 5 cm or more in

diameter. Maxillary lesions are smaller and
rounder than those in the mandible.
Shape — shape of cyst is usually oval extending
along the body of the mandible with little
mediolateral expansion.
Margins — margins are hyperostotic and tooth
displacement is seen.
Majority of lesions are unilocular with smooth
borders but some unilocular lesions are large with Figure 16.18: Odontogenic keratocyst
irregular borders. Radiolucency is usually hazy
due to keratin filled cavity and it is surrounded
by thin sclerotic rim due to reactive osteocytes.

Effect on bone— (Figs 16.18 and 16.19)

• It can expand and perforate the buccal and
lingual cortical plates of bone and involve the
adjacent soft tissue.
• Downward displacement of the inferior
alveolar canal and resorption of the lower
cortical plate of the mandible may be seen as
well as perforation of bone and a pathologic
fracture may occasionally occurs. Figure 16.19: Odontogenic keratocyst
• There may be extensive involvement of the
body and ascending ramus of the mandible
with little or no bony expansion.
Teeth — as the keratocyst enlarges it may
produce deflection of unerupted teeth mostly in
the region of the angle of the mandible and
occasionally, on the ascending ramus and
towards the orbital floor and in some cases root
resorption is also seen.

Radiological Types of Keratocyst (Fig. 16.20)

Envelopment type — it is referred to a variety of Figure 16.20: Varients of odontogenic keratocyst

keratocyst which embraces an adjacent unerupted 1-Replacemental, 2-envelopmental, 3-extranueous,
4-collateral
tooth.

Replacement — those which forms in the place of Extraneous — those in the ascending ramus away
normal teeth. from the teeth.
 Cysts of the Orofacial Region 333

Collateral — those adjacent to the root of teeth

which are indistinguishable radiologically from
the lateral periodontal cyst.

Histopathological Features
Macroscopic Features
The odontogenic keratocyst's gross pathological
features are characteristic as the cysts are hardly
received intact in the laboratory.
The small cysts may have a well defined cystic
wall, but the large cyst almost always show thin
walled, collapsed folded or detached linings.
The lumen of keratocyst may be filled with thin
straw colored fluid or with thick creamy material.

Microscopic Features (Figs 16.21 to 16.24) Figure 16.22: Higher magnification view showing severe
epithelial dysplasia
The odontogenic keratocyst shows two types of
linings:
1. Parakeratinised stratified squamous
epithelium.
2. Orthokeratinised stratified squamous
epithelium.

Figure 16.23: Inflamed odontogenic keratocyst

The parakeratinised epithelium is more

common, (80-90%) cases.
The orthokeratinised OKC shows less common
occurrence.
The characteristic feature of the lining of is
pathognomic corrugated,with a regular thickness
Figure 16.21: OKC lined by corrugated parakeratinized of the epithelium between 5-8 cell layers.
stratified epithelium. The epithelium is collapsed and folded The lining is without rete ridges.
334 Textbook of Oral Pathology

Some times lumen contains a great deal of

keratin, while at other times it has little.
Cholesterol as well as hyaline bodies at the site
inflammation, may also be present.

Differential Diagnosis

It is can be given in two ways:

Most likely

Ameloblastoma — This usually occurs in older age.

Figure 16.24: OKC lined by orthokeratin
It is generally multilocular though a unilocular
The basal cell layer is columnar with lesion may also occur. Mostly, it appears with
palisaded arrangement of the nuclei. paresthesia. As benign it may show resorption
The nuclei tend to be placed away from the of root with displacement. In cases of cyst, it
basement membrane. shows amber colored fluid on aspiration.
The nuclei of the basal cells are darkly staining, Primordial cyst — it is also common in third molar
show basal cell hyperplasia, this is not present in area. Absence of a tooth without a history of
other keratocyst. extraction favors primordial cyst. It also shows
Highly aggressive OKC shows moderate to amber colored fluid on aspiration.
severe epithelial dysplasia.
The fibrous capsule is thin with relatively few Residual cyst — in cases of residual cyst, patient
cells widely separated by a stroma which is often gives a history of extraction of tooth. Most of
in mucopolysaccharide and resembles residual cysts are unilocular. A thin radioopaque
mesenchymal connective tissue. margins is common, although those infected will
In the inflamed cases the epithelium might not have such a well defined margins. It shows
lose the keratinization and develop rete ridges. amber colored fluid on aspiration.
Connective tissue shows islands of Traumatic cyst — the most characteristic
odontogenic epithelium forming small duplicate radiographic feature of the cyst is the scalloped
daughter cysts or small satellite cysts. margins. Mostly, it appears unilocular. The lesion
The satellite cysts are more common in rarely causes cortical plate expansion but when
patients with multiple cysts and naevoid basal this occurs it is mostly buccal. There may be a
cell carcinoma syndrome. history of trauma. In most cases, it is
Another most important feature of these cyst asymptomatic. Needle aspiration is usually non-
is that there is a weak epithelial-connective tissue productive. Only a few milliliter of straw colored
attachment, this causes the detachment of the or serosanguinous fluid can be withdrawn.
epithelium and further recurrences as it becomes
difficult during removal of the cyst.
Less likely
Other less common findings are — Mucous
metaplasia, Rushton bodies and cholesterol clefts Benign odontogenic tumor and cementifying or
in inflamed cysts, ossifying fibroma — these are not common lesions.
 Cysts of the Orofacial Region 335

Giant cell granuloma — it usually is found in the Age and sex — it is found in children and young
anterior region of the jaw. adult between 10 to 30 years of age, although it
The giant cell lesion of hyperparathyroidism — it can may persist in older age group and occurs with
often be ruled out by studies on serum chemistry. equal frequency in both the sexes.

Tooth crypt — the possibility of radiolucency of Site — it can arise in any portion of the jaw, but
tooth crypt is less likely as calcification of the tooth most often seen in the ascending ramus of the
starts in the 8th year. The halo has thin outer mandible and in from third molar area. It is
radioopaque border which is continuous with the occasionally associated with an over retained
lamina dura in the area of the cemento enamel erupted deciduous tooth.
junction. Symptoms — It has a tendency to painlessly enlarge
Odontogenic myxoma — the rare myxoma also must and slowly replace large portions of cancellous
be considered in such circumstances because the bone before expansion of the cortical plate by way
tooth has failed to develop and may be seen as a of which it reveals its presence.
cyst like radiolucency. Pain which is associated with a large cyst is
caused by infection that may follow the
Management perforation of the expanded cortical plate.
Enucleation of entire cyst with vigorous curettage Aspiration — when aspirated, they yield a thick
of the cystic wall. granular yellowish material.
Periodic post treatment examination.
Histopathological Features
Primordial Cyst
This is similar to that of other odontogenic cysts.
It is one of the less common types of odontogenic It is lined on the inner surface facing the
cysts. As described earlier, it was named by lumen by an intact or interrupted layer of stratified
Robinson in 1945. He defined a primordial cyst squamous epithelium.
as "a cyst which arises by breakdown of the In some cases, the epithelium is non-keratinized
stellate reticulum of the enamel organ before any and exhibits a very prominent spinous layer with
hard tissue is formed and hence which may be elongated and some times confluent retepegs. Basal
one of the normal series or a supernumerary". cell layer is present but is not prominent.
In other cases, the epithelium may exhibit a
Origin surface layer of orthokeratin, while the spinous
It originates when cystic changes take place in layer may be relatively thin or be of moderate
the stellate reticulum of the tooth germ before thickness. The basal cell layer is not prominent
any calcified enamel or dentin has been formed. and is sometimes with an atypical corrugated
So it is found in place of a tooth rather than appearance, while the remainder of the
directly associated with it. epithelium is exceptionally uniform in thickness,
usually 6 to 10 cells thick with extremely
It may originate from the enamel organ of
prominent basal layer. The cells are arranged in
supernumerary tooth or from the remnants of
a 'picket fence' or 'tombstone pattern' and
dental lamina.
showing no rete peg formation.
Clinical Features Radiographic Features
Occurrence — less common and account for only Radiodensity — cyst like radiolucency that is well
5% to 6% of cysts of the odontogenic variety. defined and have hyperostotic borders.
336 Textbook of Oral Pathology

Internal structure — it may be unilocular or have a Buchner and Hansen also suggested them to
scalloped outline that gives it a multilocular be distinct entities based on its pathological origin.
appearance. The gingival cyst may certainly occur without bone
involvement and may produce a gingival
Teeth — it produces deflection of adjacent tooth
swelling. Most of the times, the swelling goes un-
root, but seldom has it caused any root resorption.
noticed. It is improbable though not impossible
The involved tooth is missing because of failure
that a cyst originating in the gingival soft tissue
of the tooth to develop.
could enlarge sufficiently to produce radiolucency
Those in maxilla are smaller than their
by obvious bone erosion without producing any
mandibular counterparts.
gingival swelling. In the case of a lesion, which
has produced both gingival swelling and
Differential Diagnosis
radiolucency, faint shadow (which is due to
Same as odontogenic keratocyst. surface depression) indicates gingival cyst. Where
Missing permanent tooth without history of the radiolucency is dark and sharply demarcated
extraction favors primordial cyst. and a communication with the periodontium is
indicated then the lesion is more likely to be lateral
Management periodontal cyst that has eroded outward. These
Surgical enucleation. assumptions based on radiological features and
Regular check up due to a high recurrence rate. can be confirmed by surgical exploration when
the lesion is being removed.
Lateral Periodontal Cyst and
Gingival Cyst of Adult Lateral Periodontal Cyst
They are named so, due to its location. Lateral The lateral periodontal cyst is uncommon but a
periodontal cyst arises in the peridontium and well recognized type of developmental
located in the interproximal bone between the apex odontogenic cyst. The designation lateral
and the alveolar crest. Gingival cyst appears as a periodontal cyst is confined to that cyst, which
dome shaped swelling in the attached gingiva. occurs as a result of inflammatory etiology and
Although, it has been customary to consider the diagnosis of collateral keratocyst has been
these cysts as a distinctly different entity, their excluded on clinical and histological ground.
clinical appearance and behavior, morphologic
and histochemical features and site of occurrence Pathogenesis and Etiology
are so similar in appearance that they are in
reality the intraosseous and extraosseous Origin initially as a dentigerous cyst developing
manifestations of the same pathoses. along the lateral surface of the crown and as the
Bhasker grouped the gingival and lateral tooth erupt the cyst assumes a position in
periodontal cysts together as gingival cyst and approximation to the lateral surface of the root.
considered that they both arise from extra- Origin from proliferation of cell rests of
osseous odontogenic epithelium. Some cases Malassez in the periodontal ligament although
have shown a circumscribed radiolucency the stimulus for this proliferation is unknown.
indicative of lateral periodontal cyst, which he Origin simply as a primordial cyst of a
believed were because of cup shaped depressions supernumerary tooth germ, since the
on the periosteal surface of cortical plates produced predilection for occurrence of the lateral
by enlargement of the gingival cysts. periodontal cyst in the mandibular bicuspid area
 Cysts of the Orofacial Region 337

corresponds well with the known high incidence The associated tooth is vital.
of supernumerary teeth in the same region. If the cyst becomes infected, it may resemble a
Origin from proliferation and cystic lateral periodontal abscess.
transformation of rests of dental lamina (which is
in post functional state and therefore has only Radiographic Features
limited growth potential), that is in accordance
Shape — the intra-bony lateral periodontal cyst is
with the usual small size of the cyst.
seen as a round or ovoid well defined radiolucency
Recent theory suggests that the lateral
with hyperostotic borders.
periodontal cyst and gingival cyst of adult share
the common histogenesis from post functional Site — it is usually found between the cervical
dental lamina rests. These two cysts represent margins and the apex of adjacent root surfaces
basically the central or intra-osseous and and may or may not be in contact with root
peripheral or extra-osseous manifestations of the surfaces.
same lesion.
Size — most of them are less than 1 cm in diameter
except the botryoid variety which may larger and
Types
multilocular.
Inflammatory — it occurs near alveolar crest. Pocket
content may irritate and stimulate rest of malassez. Histopathological Features
Developmental — it is associated with developing
It is lined by a layer of stratified squamous
tooth germ.
epithelium with connective tissue wall.
Clinical Features Cuboidal and columnar cell may be found
compassing the lining. Many of the lining cells
Age — the lateral periodontal cyst occurs chiefly have clear vacuolated glycogen rich cytoplasm.
in adults with an age range from 22 to 85 years The lumen cyst show focal thickened plaque
with a mean age of 50 years. of proliferating lining cell. These are especially
Sex — it shows a male predilection for occurrence. prominent in Botryoid cyst.
Site — the most frequent location of lateral
periodontal cyst reported on lateral surface of the Differential Diagnosis
roots of vital teeth in mandibular canine and The location and radiographic appearance of the
premolar region and is followed by the anterior lateral periodontal cyst results into the following
region of the maxilla. lesions to be included in the differential diagnosis:
Symptoms — gingival swelling may occur on the
Lateral radicular cyst — In radicular cyst, the lamina
facial aspect and in these types of cases, it must be
dura will not be intact and it is associated with
differentiated from the gingival cyst. In gingival
pulpal infection and a non vital tooth.
cyst the overlying mucosa is blue but in lateral
periodontal cyst the overlying mucosa appears Lateral periodontal abscess — it is very difficult to
normal. differentiate between abscess and cyst, but if it is
When the cyst is located on the labial surface less than 1.5 cm then it is considered as an
of the root, it appears as a slight obvious mass, abscess.
overlying the mucosa.
338 Textbook of Oral Pathology

Lateral dentigerous cyst — it is generally associated A cyst with typical features, which has some
with an impacted tooth i.e. third molars and characteristics in common with lateral
canines. periodontal cyst and botryoid odontogenic cyst,
Residual cyst arising from the primary or permanent has recently been reported.
dentition — there will be a history of extraction of The term most descriptive of the lesion is
the tooth. probably mucoepidermoid odontogenic cyst
Primordial cyst — if primordial cyst arising from because of the presence of both secretory elements
supernumerary tooth is superimposed on adjacent and stratified squamous epithelium.
root surface, then it may be considered in The typical feature of the cyst is that it is
differential diagnosis of lateral periodontal cyst. intrabony radiologically and which can recur if
However, primordial cyst is more common at a not adequately excised. Histologically, shows
younger age. So to confirm the diagnosis of cystic space lined by non-keratinized epithelium.
primordial cyst, radiographic examination from The mucous and cylindrical cells form an
integral part of the epithelial component with
a different angulation should be carried out.
mucinous material within the cystic space.
Globulomaxillary cyst — it is seen in between
Gardner, who favors the name glandular,
maxillary lateral incisor and canine region
suggests that the features and biological behavior
common in young age and appears as a pear
are sufficiently distinct for it to be regarded as a
shaped radiolucency.
distinct entity.
Median mandibular cyst — it occurs in the midline
It occurs over a wide age range in either of
of mandible.
the jaws and has the propensity to grow to a large
size and to recur.
Management
Radiologically, it is a unilocular or
The lateral periodontal cyst must be surgically multilocular radiolucency with either smooth or
removed if possible without extracting the scalloped margins.
associated tooth. If this can not be accomplished,
the tooth must be sacrificed. There is a tendency Gingival Cyst of Adult
for recurrence for this type of cysts following its It is an uncommon cyst occurring either on free or
surgical excision. attached gingiva.

Botryoid Odontogenic Cyst Etiology and Pathogenesis

This term was proposed by Waldron, which refers Degenerative changes in proliferating epithelial
to a multilocular periodontal cyst. tissue.
It resembles a cluster of lateral periodontal Remnants of dental lamina, enamel organ or
cysts exhibiting some difference. epithelial islands of periodontal membrane.
The lesion is multilocular with thin fibrous Traumatic implantation of epithelium.
connective tissue septa. It is clear from numerous Arise from post functional rests of dental
reports of recurrence that the botryoid lamina.
odontogenic cyst requires careful excision.
Clinical Features
Glandular Odontogenic Cyst
Age — the gingival cyst may occur at any age, but
It is also called as 'Sialo odontogenic cyst', or it is most common in adults in the 5th and 6th
'mucoepidermoid cyst'. decade of life.
 Cysts of the Orofacial Region 339

Site — the location of the lesion closely follows Epstein's Pearls, Bohn's Nodules and Dental
that of lateral periodontal cyst. It is more common Lamina Cyst.
in mandible in premolar and canine region with
Pathogenesis
predilection for males.
They are multiple, occasionally solitary nodule
Symptoms — it is slowly enlarging, painless
on the alveolar ridge of newborn originating from
swelling, usually less than 1cm in diameter and
remnants of the dental lamina.
may occur in attached gingiva or the interdental
papilla. Epstein pearls — these are cystic keratin filled
nodules found along the midpalatine raphe
Appearance — the surface may be smooth and the
probably derived from entrapped epithelial
color may appear as that of normal gingiva or
remnants along the line of fusion.
bluish and may appear red when it is blood filled
as a result of recent trauma. Bohn's nodules — these are keratin filled cyst
scattered over the palate most numerous along
Signs — the lesions are soft and fluctuant and
the junction of hard and soft palate and
adjacent teeth are usually vital during surgical
apparently derived from palatal salivary gland
exploration. Slight erosion on the surface of the
structure. It is formed along the buccal and
bone may be observed without extension into the
lingual aspect of the dental ridge and on the
periodontium.
palate away forms the raphe. The nodules are
considered remnants of mucous gland and are
Radiographic Features
histologically different from Epstein's pearls.
There may be no radiological changes or only a
Dental lamina cyst of newborn — it is derived from
faint round shadow indicative of superficial bone
the remnants of the dental lamina. It is found on
erosion.
the crest of the maxillary and mandibular dental
ridges. The cyst apparently originated form
Histopathological Features
remnants of dental lamina.
The lining epithelium is generally identical to that
found in lateral periodontal cyst. Clinical Features
In cases of traumatic implantation type of
gingival cyst, calcification or ectopic ossification, Age — these cysts are rarely seen after 3 months of
age.
on rare occasions, it may be associated with cystic
lesion. Site — they tend to cluster along the junction of
The epithelium ranges in thickness from the hard and soft palate in a linear fashion or are
simply one layer to several layers of cells. The scattered over the hard palate.
layer consists of flat or cuboidal cells with thin
Symptoms — occasionally, they become large to
stratified squamous epithelium.
be clinically obvious as small discrete white
Management swellings of the alveolar ridge.
• Surgical excision of the lesion in adults is Appearance — clinically it appears as small
usually recommended and the lesion does not whitish projection of the alveolar ridge of the
tend to recur. jaws of infants giving rise mistaken appearance
• A neoplastic potential has never been of a tooth. Sometimes appearing blanched as
reported. though from internal pressure.
340 Textbook of Oral Pathology

Size — it is raised nodules, usually multiple, Clinical Features

measuring a fraction of a millimeter to 2-3 mm in
Site — 3/4th of the lesions occur centrally, with
diameter.
about equal in both jaws and 75% occuring
These probably correspond to those structures
anterior to the first molar.
described as 'pre-deciduous dentition' in older
literature. Age and sex — age distribution from 10 to19 years
with a mean age of 36 years. However, there may
Histopathological Features be another peak incidence through the seventh
decade. It is slightly more prevalent in women.
These are true cysts with thin epithelial lining
and show lumen filled with desquamated Symptoms — it is slow growing, painless, non-
keratin, occasionally containing inflammatory tender swelling of the jaws. Occasionally some
cells. patients may complain of pain.
Dystrophic calcification and hyaline bodies
Signs — in some cases, cortical plate over the
of Rushton, commonly seen in dentigerous cyst,
expanding lesion may be destroyed and cystic
are also sometimes seen her.
mass may be palpable with patients reporting of
discharge. Aspiration yields viscous granular
Management
yellow fluid.
No treatment is required as these lesions almost
Teeth — adjacent teeth may be displaced.
invariably disappear by opening onto the surface
of the mucosa or through disruption by erupting
Radiographic Features
teeth.
Radiodensity — the central lesion may appear as a
Calcifying Epithelial Odontogenic Cyst cyst like radiolucency.

It is also called as 'keratinizing and calcifying Margins — it presents with variable margins
odontogenic cyst' and 'Gorlin's cyst' as it was first which may be quite smooth with a well defined
described by Gorlin in 1962. The lesion is outline or irregular in shape with poorly defined
unusual in that it has some features suggestive borders.
of cyst but also has many characteristic of a solid Teeth — It is not unusual to find these associated
neoplasm. Most cases of calcifying odontogenic with unerupted teeth and in some cases as
cyst arise centrally within the bone, but it may pericoronal radiolucency.
occur peripherally in the soft tissue overlying Roots of adjacent teeth may resorb.
the tooth bearing area. Central calcifying
odontogenic cyst is also known as intra-osseous Internal structure — It may contain small foci of
odontogenic cyst. calcified material that are only microscopically
apparent. In some cases, it is completely
radiolucent while in other cases an increasing
Praetorius Divided it into three Types
amount of calcified material may be seen as white
• Simple unicystic type. flecks.
• Unicystic odontome producing type. • It may be unilocular or multilocular.
• Unicystic ameloblastomatous proliferating • In some cases, calcified material may occupy
type. most of the lesions.
 Cysts of the Orofacial Region 341

Histopathological Features
The epithelium of the cystic lining is chiefly low
cuboidal or squamous type and is two or three
layers thick (Fig. 16.25).
Focal areas of stellate reticulum and ghost cell
may be present.
Ghost cells according to some authors are
abnormal keratin, they are pale eosinophilic
swollen epithelial cells, that have lost the nucleus
and nuclear membrane. They have affinity for
calcification (Fig. 16.26).
More accepted is the concept that they are
product of coagulative necrosis of the epithelial
Figure 16.26: Ghost cells are pale eosinophilic swollen
cells as some amount of calcified material may epithelial cells that have lost its nucleus and nuclear
be seen. As the cells lack some qualities for special membrane
staining methods for cytokeratin, it is suggested.
Calcifications are seen as dentinoid or
basophilic dystrophic calcifications in the cyst
lining (Figs 16.27 and 16.28).

Differential Diagnosis
The radiographic image of this lesion is so variable
in form, outline and radiodensity that a
differential diagnosis for such lesion may include
the following. Figure16.27: Basophilic dystrophic calcifications in
ghost cells

Figure16.25: Unicystic CEOC showing Figure 16.28: Dentinoid material induced by the adjacent
ameloblastomatous proliferation of the epithelial lining odontogenic epithelial islands
342 Textbook of Oral Pathology

Fibrous dysplasia (initial stage) — it appears as The epithelium may be derived in some cases
mottled or has smoky defined border on from:
radiographs. It has poorly defined borders. It is • Respiratory epithelium of the maxillary sinus,
more common in the maxilla. when the periapical lesion communicates
with the sinus wall.
Partially calcified odontoma — it appears within
• Oral epithelium proliferates apically from the
the capsule.
periodontal pocket.
AOT — in intermediate stage of development of • Oral epithelium forms a fistulous tract.
AOT, radiographically, it appear like CEOC.
Pathogenesis
Ossifying fibroma (initial stage) — the fibro osseous
lesions are likely to be situated in more inferior Peri-radicular inflammatory changes cause the
position in the mandible. It may show root epithelium to proliferate. As the epithelium
resorption. Histologically, it shows Chinese letter grows into a mass of cells, the center losses the
shaped islands of bone or calcification distributed source of nutrition from the periapical tissue.
throughout the connective tissue. These changes produce necrosis in the center and
a cavity is formed and cyst is created.
Odontogenic fibroma — histologically, it shows
Another theory is that an abscess cavity is
odontogenic tissue like cementum.
formed in the connective tissue and is lined with
Cementoblastoma — the radiographic image is proliferating epithelial tissue.
well define and attached to the root of tooth.
Clinical Features
Management
Age — peak frequency occurs in the 3rd decade
• Enucleation and curettage should be done. and there are large numbers of cases in the 4th
• Malignant transformation has been recorded. and 5th decades after which there is a gradual
decline. Although dental caries is more common
INFLAMMATORY in deciduous teeth in the first decade but radicular
cysts are not often found in deciduous teeth may
Radicular Cyst
be because teeth tend to drain more readily than
It is also called as 'apical periodontal cyst', the permanent teeth and the antigenic stimuli
'periapical cyst', or 'dental root end cyst'. It is a which evoke the changes leading to the formation
common sequel in progressive changes of radicular cyst may be different.
associated with bacterial invasion and death of
Site — maxillary anterior are more commonly
the dental pulp. It most commonly occurs at the
affected. Also in addition to caries, maxillary
apices of teeth.
teeth are more prone to traumatic injuries which
The radicular cyst is classified as an
lead to pulp death.
inflammatory cyst because this process is thought
to initiate the growth of the epithelial component. Sex — male predominance, as females are less
It may be classified as odontogenic cyst because likely to neglect their maxillary anterior teeth and
of its origin in cell rests of Malassez which are males are more likely to sustain trauma to their
remnants of Hertwig's root sheath and is a product maxillary teeth. It shows a higher frequency of
of the odontogenic epithelial layer. occurrence in whites.
 Cysts of the Orofacial Region 343

Symptoms — it represents an asymptomatic phase

in periapical inflammatory process following
death of the dental pulp. It is associated with non
vital tooth.
Expansion — it rarely causes non tender expansion
of the overlying cortical bone.
Teeth — the associated tooth is not sensitive to
percussion.
Consistency — swelling may be bony hard or
crepitations may be present as bone is thinned
or it may be rubbery or fluctuate, if the bone is
completely destroyed.
Figure 16.30: Radicular cyst
Complication — ameloblastoma, epidermoid
carcinoma and mucoepidermoid carcinoma may
arise in the epithelial lining of periapical cyst.

Radiographic Features
Shape — it appears as a rounded or pear shaped
radiolucency at the apex of non sensitive tooth or
with non vital tooth.
Size — radiolucency is more than 1.5 cm in
diameter but usually less than 3 cm in diameter
(Fig. 16.30). It has got well defined outline with
thin hyperostotic borders (Fig. 16.31).
Margins — in uncomplicated cases, margins are
smooth, corticated and the cortex is usually well

Figure 16.31: Radicular cyst in the palate

defined, well etched and continuous, except in

some cases there may be window formation. There
is also thin white line surrounding the margins of
the bone cavity. This thin layer of cortical bone is
almost always present unless suppuration
supervenes in the cyst (Fig. 16.32).
Image of radiopaque borders is continuous
with lamina dura around the associated tooth.
Infection may cause the borders to become less
Figure 16.29: Radicular cyst distinct.
344 Textbook of Oral Pathology

Figure 16.33: Lining of radicular cyst showing arcading

pattern of rete pegs

respiratory type of epithelium, when it occurs in

the maxillary teeth that involve the maxillary
sinus.
The lining shows variation in thickness with
rare keratin formation. The lining is proliferating
into the connective tissue forming rings and
Figure 16.32: Radicular cyst arcades by enclosing the connective tissue
(Fig. 16.33)
Radicular cysts of long duration may cause Hyaline bodies or Rushton bodies often found
resorption of roots. in great numbers in the epithelium of apical
Adjacent teeth — adjacent tooth are usually periodontal cyst (Figs 16.34 and 16.35).
displaced and rarely resorbed. There is also Connective tissue that makes up the wall of
buccal expansion and if it involves maxillary area apical periodontal cyst is composed of parallel
then displacement of antrum can occur. bundles of collagen fibers with variable number
of fibroblasts and small blood vessels.
Gross Pathology
• The contents of radicular cysts are usually a
soft brown material, often with glistening, oily,
yellowish flecks.
Nodules of opaque yellow crystalline material,
representing cholesterol, maybe seen protruding
into the lumen or within the wall.

Histopathological Features
The apical periodontal cyst is usually lined by
stratified squamous epithelium. Occasionally, it Figure 16.34: Lining of radicular cyst showing presence
is lined by pseudo-stratified, columnar or of Rushton bodies
 Cysts of the Orofacial Region 345

Figure 16.35: Linear, straight or curved, hairpin shaped

Figure 16.36: Cholesterol clefts in radicular cyst
Rushton bodies. Some show concentric lamellations

associated with periapical granuloma on the other

The universal occurrence of an inflammatory
hand shows only a faint to moderate pattern in
infiltrate in the connective tissue immediately
the albumin zone.
adjacent to the epithelium is characteristic feature.
Collection of cholesterol cleft with associated Periapical scar — it can be eliminated on the basis
multinucleated giant cells is found in the wall of of history and location.
the lesion (Fig. 16.36).
Surgical defect — on the basis of history.
The lumen of the cyst usually contains fluid
with a low concentration of protein. Periapical cementoma (early stages) — in cementoma
Lumen may contain cholesterol or keratin. the tooth is vital whereas the tooth with
granuloma or cyst has a non-vital pulp. Periapical
Differential Diagnosis cementoma mostly involves the incisors.
Traumatic bone cyst — the pulp of associated tooth
Periapical granuloma — if the radiolucency is
is usually vital. 90% of traumatic cysts occur in
smaller than 1.5 cm then it is most likely to be a
granuloma. Cysts show a straw colored fluid on mandible i.e. in molar and premolar area where
the periapical cyst has no predilection for
aspiration. Cyst is more apt to have thin
occurrence.
hyperostotic borders than granuloma. Morse and
coworkers described chair side method for Periodontal abscess — it usually shows moderate
differentiating cysts` and granuloma. This to severe crestal bone loss. Mostly the tooth is
method involves the use of alkaline copper vital.
tartarate in cystic fluid aspirate. A consistent
Mandibular infected buccal cyst — it is more common
color difference related to difference in protein
in young patients. First molar is frequently
content was observed for cyst and granuloma.
involved. Pulp is usually vital. The lamina dura
Aspirate of root canal fluid from patients with
around the tooth is intact.
cyst showed an intense albumin pattern and
definite pattern in globular zones on Benign tumor — presence of septum inside the
polyacryalamide gel electrophoresis and fluid cavity is suggestive of benign tumor.
346 Textbook of Oral Pathology

Management Radiographic Appearance

Root canal treatment — it is the treatment of choice Pre-extraction radiographs show tooth with an
as in many cases, radicular cyst resolve after root evidence of deep caries or fracture adequate for
canal treatment. The reason behind it is that as pulp involvement and/or an associated cyst.
drainage is established, the inflammatory process
Shape — it is round or ovoid radiolucency that is
subsides and the fibroblast start producing
usually well circumscribed.
collagen. The pressure of proliferating- collagen
reduces the blood supply to the epithelium lining Margins — thin radiopaque margins are common
and causes it to degenerate. Macrophages remove with unilocular appearance although the infected
the degenerating epithelial tissue. cyst will not have such well defined margins.
Due to resistance, cyst enlarges in elliptical
Extraction — if the lesion fails to resolve, extraction
shape with hyperostotic borders resulting from
of associated tooth is carried out.
minimal pressure of expanding cyst.
Enucleation and marsupialization — enucleation or
marsupialization of a larger lesion is done. Differential Diagnosis

Primordial cyst — in it, tooth is missing without a

Residual Cyst
history of extraction. It is more common in
It is a cyst that either remained as such in the jaw mandibular posterior teeth and at a younger age.
when it's associated tooth was removed or was On aspiration, it shows straw colored fluid.
formed in residual epithelium of cell rests from a
Keratocyst — it is more common in mandibular
periodontal ligament of the lost tooth. Low grade
posterior area and at an early age. It may appear
inflammation of parent cyst might predispose
multilocular. The size of the cyst is larger when
formation of residual cyst.
compared to residual cyst.

Clinical Features Traumatic cyst — usually in the mandible, in apical

region (if it is associated with tooth) and it shows
Age and sex — cyst is common in patients older scalloped outline. It is common at younger age.
than 20 years with an average age of about 52 Aspiration is non-productive.
years. It is twice more common in male than
female. Ameloblastoma (in initial stages/unilocular) — usually
the lesion appears larger compared to the residual
Site — higher incidence in the maxilla. Mostly cyst. There is no history of extraction of the tooth.
found on alveolar process or body of the tooth
bearing area with some cysts described in the
Management
lower ramus of the mandible.
Enucleation — if the cyst is not large and patient's
Symptoms — it is asymptomatic with a previous
age and health will tolerate the insult, the cyst
history of pain in the tooth.
wall should be completely enucleated. The extent
Size — it is seldom more than 5 to 10 mm in of repair of the defect will depend on the size of
diameter. the cyst and health of the patient.
 Cysts of the Orofacial Region 347

Marsupialization — in the cases where the surgical Site — usually associated with the third molar on
procedure must be as atraumatic as possible, the buccal surface and covers the bifurcation. It
marsupialization or decompression may be used. may occur bilaterally.
Excision — complete excision and replacement Signs — the involved tooth is vital.
with autogenously bone graft or single segment
of bone fixed in it. Radiographic Features

Inflammatory Collateral Cyst Radiodensity — there is well demarcated

radiolucency occurring distal to the partially
It is cyst which arises in the periodontium on the erupted tooth but there was often buccal
lateral aspect of an erupted tooth as a result of superimposition.
inflammatory process in the periodontal pocket. The radiolucency sometimes extends apically
They arise by proliferation of cell rests of but an intact periodontal ligament space provided
Malassez in the lateral periodontium. the evidence that the lesion did not originates at
They are rare as drainage occurs more readily the apex.
from the lateral periodontium which is closely
associated with gingival crevice.
Histopathological Features

Paradental Cyst It is lined by proliferating, non keratinized

squamous epithelium of varying thickness.
It was first described by Craig in 1976. It is a cyst The fibrous capsule is the seat of an intense
of inflammatory origin occurring on the lateral
chronic or mixed inflammatory cell infiltrate.
aspect of the root of partially erupted mandibular
third molar with an associated history of
Management
pericornitis.
The lesion is treated by surgical enucleation.
Origin

Cell rests of malassez can be the origin, but Mandibular Buccal Infected Cyst
argument is that if rests of malassez were It is an inflammatory cyst occurring on the buccal
responsible then the lesion should be equally surface of mandibular molars in young children.
distributed around the root surface.
Second theory is that it originates from the
Clinical Features
reduced enamel epithelium as the presence of
reduced enamel epithelium over the enamel Age — a younger age group than the paradental
projection might be the source and could explain cyst.
the common location of the cyst.
Site — it affects the mandibular first and second
molars more commonly rather than the wisdom
Clinical Features
teeth. The cyst always is situated on the buccal
Age and sex — it occurs between 10 to 39 years of surface of the mandibular molar most frequently
age. It is most commonly in the third decade of the first permanent molar after their partial or
life. It shows predilection for males. complete eruption.
348 Textbook of Oral Pathology

Symptoms — there may be discomfort, pain, Management

tenderness and rarely suppuration. Swelling
It is generally agreed that enucleation of the cyst
particularly, if inflamed, is the clinical feature
without removal of the associated tooth is the
most likely to induce the patient to seek advice.
treatment of choice.
Facial swelling may follow and this may be
inflamed. Healing of Cyst
Signs — usually, a vital pulp and intact lamina A cyst that is caused by an infected tooth often
dura. heals after removal of that tooth.
Diagnostic features — the diagnostic features are One of earliest changes is the gradual loss of
the young age of the patient, the mandibular molar the cortical layer of bone which lines most of the
sites, buccal periostitis, usually vital pulp and cysts.
intact lamina dura. At the same time, if the cyst becomes infected,
the bone in the immediate vicinity of the walls
Teeth — the associated tooth is usually tilted, so becomes radiolucent as a result of hyperemia.
that the apices are adjacent to the lingual cortex, After the period of time, there is gradual
a feature which is demonstrable in occlusal lessening of radiolucency of the cavity and a
radiographs. return of the surrounding bone to a normal
Size — the size of cyst varies and may extend density.
beyond the limit of the involved tooth and The dark shadow of the cavity is replaced by
impinge upon and displace the crypt of the a gray one and it is apparent that the cavity is
adjacent unerupted tooth. becoming smaller.
A large cyst may fail to heal completely,
The cyst extension in buccal direction is variable, resulting in a smaller cyst. This is due to the
but frequently the outer bony cortex is lost. retention of some part of the epithelial wall of the
cyst, which has continued its activity.
Radiographic Features
NON ODONTOGENIC CYSTS
Periosteum — with involvement of the periosteum
new bone may be laid down either as a single Nasopalatine Cyst
linear band or laminated, if there two or more
layers. Sometimes, the new bone may be It is also called as 'nasopalatine duct cyst', 'incisive
homogeneous. canal cyst', 'median anterior maxillary cyst' or
'vestigial cyst'. It is the most common non-
Site — the cysts will appear on the buccal aspects odontogenic cyst in the oral cavity.
of the affected molars.
Bone — usually there is involvement of the bone Origin
in the furcation and the entire inter-radicular bone
It is developmental in origin and arises in the
may be lost.
incisive canal when embryonic epithelial
Margins — the inferior margins of the cyst is remnants of the nasopalatine duct undergo
concave and rarely, the cyst may extend to the proliferation and cystic transformation.
inferior border of the mandible but not leading to They are found to form in the canal or at the
any external deformity. oral terminus of the canal.
 Cysts of the Orofacial Region 349

It is generally agreed that the nasopalatine duct • Patients complain of salty taste in mouth
cyst is an entity. It may occur with in the produced by small sinus or remnant of
nasopalatine canal or in the soft tissue at the nasopalatine duct that permits cystic fluid to
opening of the canal where it is called as cyst of drain into oral cavity.
the palatine papilla. • Burning sensation and numbness may be
experience due to pressure on the nasopalatine
Etiological Factors nerve.
• Sometimes cystic fluid may drain and patient
Trauma: In the form of direct blow to the incisive
reports a salty taste.
canal or indirectly from mastication, particularly
Signs —
when ill-fitting dentures are involved, have been
• Swelling is fluctuant and bluish if it is near
suggested. But if this is true, the cyst would likely
the surface.
be found more often and there would not be a
• It opens by a tiny fistula on or near the palatine
male predilection.
papilla. In such cases a tiny drop of watery
Bacterial infection: Either from the nasal cavity or fluid or pus may be elicited by pressure in this
from the oral cavity, stimulates to the epithelial area.
remnants to proliferate has been suggested as a • Deeper cysts are covered by normal mucosa,
cause. However, since an open connection with unless it is ulcerated.
the oral cavity or the nasal cavity is extremely • If cyst expands, it may penetrate the labial plate
rare, an evidence for the bacteria is lacking. and produce a swelling below the maxillary
labial frenum.
Retention phenomenon: Blocked duct of mucus
glands and an accumulation of secretion would Teeth — roots of central incisors diverge. It may
be responsible for the cystic expansion. bulge into the nasal cavity and distort nasal
septum.
Epithelium: Some workers believe that it is derived
from the epithelium included in the lines of fusion Cyst of palatine papillae — Sometimes cyst formed
of embryonic facial processes. in palatine papilla will be evident as an elevation
or a soft round swelling of palatine papilla which
Clinical Features extends posteriorly along the midline of the
palate. It occurs anterior to the incisive foramen
Occurrence — it is the most common non
below the periosteum and does not enter invades.
odontogenic cyst.
Age — most cases discovered in the 4th and 6th Radiological Features
decades and it is also frequently found in
Site — typical cyst like radiolucency superimposed
edentulous patients.
with the apices of the central incisors.
Sex — it is three times higher in males than in Appearance —
females and found equally in blacks and whites. • Image of radiopaque anterior nasal spine may
Symptoms — superimpose over the dark cystic cavity giving
• There is a small well defined swelling just it a heart shape or shape of inverted tear drop.
posterior to the palatine papilla. • Two separated cysts may develop in two
• Sometime it may become infected, producing canals and cause paired cysts like
pain radiolucency.
350 Textbook of Oral Pathology

• Cyst in canal cavity also erodes the bone

posterior to the canal and creates impression
of midpalatal cyst.
Internal structure — if cyst is formed in one of the
branches of the canal, image will be displaced on
one side of the midline.
Shape — in many cases, cyst is situated
symmetrically in the midline. It may be rounded,
oval or irregularly shaped.
Size — size of cyst is variable but usually from 6
mm to several centimeters in diameter.
Teeth —
Figure 16.37: Nasopalatine cyst lined by respiratory
• Adjacent teeth usually show distal epithelium and connective tissue capsule with prominent
displacement but they are rarely resorbed. neurovascular bundle
Divergence of central incisor roots and external
root resorption is common.
Cyst of palatine papillae — the cyst in the palatine
papillae is not usually apparent because it does
not cause enough pressure on the bone to cause
discernible bone resorption but it can produce
some bony erosion.

Histopathological Features
The cystic epithelial lining depends upon the
proximity of the lesion to the nasal cavity. The
most superficially located cysts are lined with
respiratory epithelium while those in the inferior Figure16.38: High power view shows pseudostratified
ciliated columnar epithelium
position close to the oral cavity are composed of
epithelium of squamous variety (Figs 16.37 to 16.39).
shaped. Radiolucency in the area less than 6 mm
The connective tissue wall of this cyst
wide should be considered as a normal fossa in
frequently shows inflammatory infiltration.
the absence of associated symptoms. Incisive fossa
Collections of mucus glands are also often
sharply defines at the lateral margins in contrast
present as well as several large blood vessels and
to the cyst, which has a well defined boundary on
nerves.
all margins. Aspiration will help to distinguish
In the cystic fluid, erythrocytes, leukocytes,
between cyst and incisive fossa.
desquamated epithelial cells, tissue debris and
bacteria can be found. Radicular cyst — Pulp is non-vital with loss of
lamina dura in the radicular cyst.
Differential Diagnosis
Dentigerous cyst with mesiodens — Radiographic
Incisive fossa — The shape of the fossa varies from evidence of association with supernumerary teeth
round to oval to triangular to diamond to funnel will establish the diagnosis of dentigerous cyst.
 Cysts of the Orofacial Region 351

Clinical Features
Site — it is very rare and develops in midline of
the hard palate posterior to pre-maxilla.
Symptoms — if it becomes larger, then it bulges
into the oral cavity and produces a swelling in
the roof of mouth.
Signs — it is fluctuant and non-tender. Overlying
mucosa is normal.
Corticated plate is rapidly perforated as the
cyst grows.
If floor of nasal fossa is eroded, cyst may be
superiorly displaced.
Teeth — maxillary teeth are vital and aspiration
Figure16.39: Stratified squamous epithelium lining
nasopalatine duct cyst produces amber colored fluid.

Median palatine cyst — radiolucent lesion is behind Radiographic Features

the incisive canal in premolar-molar area. Site — radiolucent lesion is behind the incisive
Primordial cyst from supernumerary teeth — more canal in premolar- molar area.
common in the posterior teeth. Margins — well defined borders which are
hyperostotic.
Management
Appearance — nasal septum image crosses the
• Its removal is not indicated unless there are
septum and appears on occlusal radiographs.
clinical symptoms.
• Removal is indicated in edentulous patients Histopathological Features
before dentures are introduced.
The lining of such cyst usually consists of
• Enucleation.
• If it is large, then marsupialization. stratified squamous epithelium overlying
relatively dense fibrous connective tissue band
Median Palatine Cyst which may show chronic inflammatory cell
infiltration.
It is very difficult to distinguish from the
In some cases, it may be lined by pseudo-
nasopalatine cyst. Normally there is septopalatal
stratified ciliated columnar epithelium or even a
contact, fusion and lamination of adult epithelial
'modified' squamous epithelium.
surfaces followed by breakdown (of opposing
epithelial surface) into epithelial remnants and Differential Diagnosis
actual fusion by inter-shelf mesenchymal bridge.
Nasopalatine cyst — not distinguishable.
In cases, due to continued differentiation of these
epithelial remnants pathological median palatine Radicular cyst — vitality test and periapical
cyst is formed. It may be the only true fissural cyst radiographs demonstrate intact lamina dura and
in oral region. It is now felt that it represent normal periodontal ligament space in median
posterior extension of incisive canal cyst. palatine cyst.
352 Textbook of Oral Pathology

Plexiform neurofibroma — aspiration non Sex — women are more commonly affected.
productive.
Site — it is unilateral but may be bilateral.
Palatal space abscess — soft, fluctuant and yield
Symptoms — it may cause pain and difficulty in
pus on aspiration and non vital teeth found breathing through the nose. There is swelling of
adjacent which give rise to infection.
the nasolabial fold and nose.
Incisive canal cyst — it occurs in canal above Signs —
palatine papillae while midpalatine occur in Swelling is fluctuant.
midline of palate posterior to palate. There is flaring of ala and distortion of nostril
and fullness of upper lip below the nasal vestibule.
Retention phenomenon — seen laterally and not in
It may bulge into the nasal cavity and cause
midline. Aspiration won't yield amber colored
fluid but viscous clear sticky liquid. some obstruction. Infection may drain into the
nasal cavity.
Malignant and benign tumors of salivary gland — Superficial erosion of the outer surface of the
laterally and not in midline. maxilla may be produced by pressure of the
nasoalveolar cyst.
Management
Surgical excision is done. Care should be taken Radiographic Features
not be perforate the nasal mucosa. Site — increased radiolucency of alveolar bone
Healing defect is protected by a acrylic stent beneath the cyst and above the apices of incisors.
prefabricated.
Appearance — cyst causes erosion of the underlying
Nasoalveolar Cyst bone by virtue of its presence and pressure.
It is also called as 'nasolabial cyst' or 'Klestadt's Margins — usual outline of inferior border of nasal
cyst'. It is a soft tissue cyst, which involves the fossa is distorted resulting in posterior
bone secondarily. convergence of the margins.
Shape — the actual shape and position of the cyst
Pathogenesis
can be demonstrated by aspirating the typical cyst
Some state that it is a fissural cyst arising from fluid and replacing it with radio-contrast material.
epithelial rests in the fusion lines of globular A tangential view then demonstrates the kidney
process, lateral nasal process and maxillary shaped lesion below the nasal fossa and above
process. the apices of the incisors.
Others state that it is actually a merging of
mesenchymal processes and not a fusion per se, Histopathological Features
so there is no epithelial entrapment in the naso- It may be lined by pseudo-stratified columnar
optic fissure. They state that the location of epithelium which is sometimes ciliated often
nasoalveolar cyst strongly argues in favor of its with goblet cells or by stratified squamous
development from the embryonic nasolacrimal epithelium.
duct that initially lies on the surface.
Differential Diagnosis
Clinical Features
Acute dento-alveolar abscess — radiographically
Age — it ranges from 12 to 75 years with a mean cystic radiolucencies is seen in case of
age of 41 with peak in the 3rd decade. nasoalveolar cyst.
 Cysts of the Orofacial Region 353

Nasal furuncle — same as above. Signs — they seldom produce obvious

expansion of the cortical plate of bone.
Mucus extravasation cyst — same as above.
Teeth — associated teeth react normally to pulp
Cystic salivary adenoma — same as above. .
vitality test.
Management
Radiographic Features
It should be excised using an intraoral approach.
Appearance — it is unilocular, well circumscribed
There is no tendency to recur.
radiolucency, although it appears multilocular.

Median Mandibular Cyst Shape — the image is well defined, round or ovoid
radiolucency that may be regular or irregular in
The median mandibular developmental cyst is an shape.
extremely rare lesion occurring in the midline of
the mandible. Lamina dura — the lamina dura around the lower
incisor teeth is intact.
Origin Teeth — as it expands, it diverges the roots of the
Some authors consider it a true developmental mandibular incisors.
cyst originating from proliferation of epithelial
Histopathological Features
remnants entrapped in the median mandibular
fissure during fusion of bilateral mandibular It shows thin stratified squamous epithelium often
arches. with many folds and projections lining a central
Lesions may represent some type of lumen.
odontogenic cyst such as primordial cyst In some cases, it may be lined by a
originating from supernumerary enamel organ pseudostratified ciliated columnar epithelium.
in anterior mandibular segment, particularly
since bone uniting at the mandibular symphysis Management
originate deep within the mesenchyme and These types of cysts should be surgically excised
thereby provide little opportunity for inclusion by preserving the associated teeth, if possible.
and subsequent proliferation of epithelial rests
deep within the bone. Globulomaxillary Cyst
It is also said that it represents lateral It is also called as 'intra-alveolar cyst'. It occurs in
periodontal cyst in the midline although the globulomaxillary area. It was considered to be
origin of this latter lesion is also obscure. an inclusion or developmental cyst that arises
from entrapped non-odontogenic epithelium in
Clinical Features globulo-maxillary suture which occurs at the
junction of globular portion of the medial nasal
Site — it has got predilection for the inferior part
process and maxillary process.
of the mandible, so that it does not come in close
There is evidence that the cyst acutely forms
relationship with the roots of lower incisors.
in the bone suture between the premaxilla and
Symptoms — most are clinically asymptomatic and maxilla, the incisive suture, so that location may
are discovered only during routine be different. Due to this Ferenczy has suggested
roentgenographic examination. the term 'premaxilla-maxillary cyst'.
354 Textbook of Oral Pathology

Previously existence of globulo-maxillary cyst

was disputed by Christ who said that
development of anterior maxilla occurs by
merging of growth center and not by fusion of
facial processes so no entrapment occur so it can
not exist.
Recent embryonic studies have demonstrated
that Christ's view of facial development is
incorrect. Fusion of facial process does occur and
epithelium is entrapped in areas that will later lie
between the maxillary lateral incisors and
canines. In conclusion, recent evidence favors the
reintroduction of the globulomaxillary cyst as a
specific pathologic entity with varying histologic
features. Figure16.40: Globulomaxillary cyst in between lateral and
canine of maxilla. (Courtesy: Dept of Pediatric dentistry,
Clinical Features Peoples Dental Academy, Bhopal)

Symptoms
• It is asymptomatic and is discovered during Radiographic Features
routine radiographical examination. Shape — it appears as pear-shaped or tear-shaped
• If cyst becomes infected, patient may complain radiolucency between roots of maxillary lateral
of local discomfort or pain in that area. incisors and canines. Small end of the pear is
Teeth directed toward the crest of alveolar ridge. The
• As it enlarges, it expands the buccal cortical upper border may invaginate the floor of the nasal
plate between maxillary lateral incisors and fossa or the antrum.
canines.
• It may diverge the roots of two teeth and their Size — the size is variable and may reach the
crown may rotate causing the contact point to maximum level of diameter of 3-4 cm.
move incisally. Teeth —
• Adjacent teeth are usually vital. • It may cause divergence of the roots adjacent
Signs teeth. Displacement of the teeth is common.
• Mucosa over the expanding cortex remains • It is well defined and lamina dura of the
normal in color. adjacent teeth are usually intact.
• If cortical plate is eroded then fluctuant • There may be root resorption.
swelling develops. Palpation will produce • The rotation and separation of the lateral
crepitus (Fig. 16.40). incisor and canine roots will usually be
• If it becomes secondarily infected, the apparent on the radiograph.
expansion will mimic lateral periodontal
Histopathological Features
abscess.
• Aspiration of the swelling is productive of It is lined by stratified squamous epithelium or
typical amber colored cystic fluid. ciliated columnar epithelium.
 Cysts of the Orofacial Region 355

The remainder of the wall is made up of Clinical Feature

fibrous connective tissue usually showing
Site — it arises near the labial surface of the
inflammatory cell infiltration.
premaxilla between the two incisors.
Symptoms — there may be swelling at the site.
Differential Diagnosis
Lateral periodontal cyst — radiographically, it Radiological Features
appears a dome shaped radiolucency more
Site — it occupies the position more close to the
commonly seen in mandibular lateral incisor and
shadow of the crowns of the incisors than the
first premolar region occurring in an older age
root.
group.
Radiodensity — it appears as area of radiolucency
Lateral dentigerous cyst — it is commonly associated
in the midline.
with impacted teeth. The radiolucency is
associated with the crown (attached to the neck of Margins — the borders may be sharply defined or
the tooth). not.

Primordial cyst — it is more common in mandibular Periphery — there may or may not be a thin layer
posterior region. of cortical bone at the periphery of the cavity.
Teeth — in some cases, when the cyst comes in
Giant cell granuloma — it is more common in
contact, there is symmetrical resorption of the roots
anterior region. Usually it appears as a
mandibular multilocular radiolucency. of teeth.

Traumatic bone cyst — it appears as round shaped NON EPITHELIAL CYSTS

with moderately defined outline. Needle
aspiration is non-productive. Traumatic Bone Cyst

Adenomatoid odontogenic tumor — radiolucency is It is also known as 'solitary bone cyst', 'hemorrhagic
associated with unerupted teeth. In mature stage, bone cyst', 'extravasation cyst', 'simple bone cyst',
it appears as radiolucent with radiopaque foci. 'unicameral cyst' and 'idiopathic bone cavity'. It is
an unusual lesion which occurs with
Surgical defect — patients give history of surgery.
considerable frequency in the jaws as well as in
Odontogenic myxoma — in myxoma, mandible is other bones of the skeleton. The traumatic bone
more commonly affected. It shows a typical cyst is a misnomer, since these intra-bony cavities
honey comb pattern. are not lined by epithelium. It results from
Anatomical variation like prominent incisive trauma induced intra-medullary hematoma with
fossa. subsequently result in bone resorption and
cavitations during hematoma resolution.
Management
Pathogenesis
Enucleation.
It originates from intra-medullary hemorrhage
following traumatic injury.
Anterior Alveolar Cyst
Hemorrhage occurring within the medullary
It is also called as 'median alveolar cyst'. This cyst space of bone after trauma heals in most cases by
arises in the intermaxillary suture anterior to the organization of the clot and eventual formation of
site of anterior palatine cyst. connective tissue and formation of new bone.
356 Textbook of Oral Pathology

According to the traumatic injury theory, Symptoms — it is asymptomatic in most cases but
however it is suggested that after traumatic injury occasionally, there may be evidence of pain and
to an area of spongy bone containing hemopoietic tenderness.
marrow enclosed by layer of dense cortical bone,
Signs — cortical swelling or slight tooth movement
there is failure of organization of blood clot and
are not the usual finding and the teeth are vital.
for some unexplained reasons subsequent
degeneration of the clot that eventually produce Aspiration — needle aspiration is actually
an empty cavity within the bone. unproductive and if it is productive it contains
In the development of the lesion, the trabeculae either a small amount of straw colored fluid shed
of bone in the involved area become necrotic after off necrotic blood clot and fragment of fibrous
degeneration of the clot and bone marrow, connective tissue.
although some viable marrow tissue must persist
to initiate resorption of the involved trabeculae. Radiographic Features
The lesion then appears to increase in size by Appearance — it appears as a radiolucent lesion
steady expansion produced by a progressive with a spectrum of well defined to moderately
infiltrating edema on the basis of restriction of defined borders (Fig. 16.41).
venous drainage. Margins —
This expansion tends to cease when the cyst • Most cases are unilocular with a fairly regular
like lesion reaches the cortical layer of the bone, border.
so that expansion of the involved bone is not a • There is evidence of hyperostotic borders
common finding in the solitary bone cyst. around the entire lesion but occasionally such
Origin from bone tumors that have undergone border is lacking.
cystic degeneration. • Most characteristic radiographic feature of this
A result of faulty calcium metabolism such cyst is scalloped superior or occlusal margins
as that induced by parathyroid disease. where it extends between the roots of the teeth.
Origin from necrosis of faulty marrow due
Size — some cyst may be only a centimeter in
to ischemia.
diameter while others may be so large that they
The end result of low grade chronic infection.
involve most of the molar area of the body of the
A result of osteoclastic activity resulting from
mandible as well as part of the ramus.
disturbed circulation caused by trauma thereby
creating an unequal balance of osteoclastic activity
and repair of bone.

Clinical Features
Age and sex — the traumatic bone cyst occurs most
frequently in young persons at an age of 6 to 20
years with a male predominance as they are
exposed to traumatic injury most frequently than
females with the ratio being 3:2.
Site — it is usually found in mandible anywhere
from the symphysis to the ramus, but about one
third are found in the maxilla, usually in the
anterior region. Figure16.41: Simple bone cyst
 Cysts of the Orofacial Region 357

Site — they may be found in dentulous as well as Management

edentulous arch.
This lesion must be surgically exposed to rule out
Teeth —
the possibility that the lesions of serious nature
• They may be superimposed with the root or
are not being confronted.
'scallop' superiorly between the roots.
When the correct diagnosis is determined,
• Occasionally lamina dura of the tooth may be
enucleation and curettage are carried out.
absent and even less frequently the root may
show resorption.
Aneurysmal Bone Cyst
Alveolar bone —
• It rarely causes cortical expansion but if it It is an uncommon hemorrhagic lesion of the bone
occurs it is mostly buccal. which is rarely seen in the jaw. It was first
• Surface is smooth, the cortical plates are not described as a clinicopathological entity by Jaffe
disrupted and pathological fracture does not and Lichtenstein in 1942. It is most often
result. categorized as to be a tumor like reactive lesion
• The cavity occupies the position in the body of of bone. The name of this entity is misleading, in
the jaw, but it may extend to involve the that, it does not contain vascular aneurysms and
alveolar process. it is not a true bony cyst.
It represents an exaggerated localized
Histopathological Features proliferative response of the vascular tissue. It may
The simple bone cyst consists of loose vascular be similar to peripheral and central giant cell
granuloma to which it resembles in some ways
fibrous tissue membrane of variable thickness
including the histological presence of giant cells.
with no epithelial lining.
Fragments of fibrin with enmeshed red cells
Pathogenesis
may be seen.
Hemorrhage and hemosiderin fragments are Persistent local alteration in hemodynamics leads
usually present and scattered small cells are often to increased venous pressure and development of
found. dilated and engorged vessels in transformed bone
area. Resorption of bone occurs, to which giant
Differential Diagnosis cells are related and this is replaced by connective
Radicular cyst — in radicular cyst, tooth is usually tissue, osteoid and new bone.
non-vital. Further all the true cysts tend to have Exuberant attempt at repair of hematoma of
a more rounded appearance. bone, similar to that of central giant cell
granuloma. But, in the case of aneurysmal bone
Central giant cell granuloma — it usually shows cyst, it is postulated that hematoma maintains a
evidence of internal bony septa which traumatic circulating connection with the damaged vessel.
bone generally lacks. It is more common in This would lead to a slower flow of blood through
mandibular anterior region. the lesion and account for a clinical "welling" of
Ameloblastoma and odontogenic myxoma — are blood. This is the only difference between the
usually multilocular. aneurysmal cyst and the giant cell granuloma that
is in later lesion, the blood vessels fail to retain
Lesions of eosinophlic granuloma — these lesions are
circulating connection with lesion.
not well corticated as that of traumatic bone cyst.
Biesecker and his associates have proposed a
Fibrous dysplasia — not so corticated. new hypothesis for etiology and pathogenesis of
358 Textbook of Oral Pathology

this lesion that a primary lesion of the bone bone and is projected as a definite radiolucency.
initiates an arteriovenous fistula and thereby Internal structure — invariably, fine septa are seen
creates, via its hemodynamic forces, a secondary crossing through the lesion in a random pattern.
reactive lesion of the bone. Thus occurrence of this
Appearance — the term 'soap bubble' may be applied
cyst is secondary in association with osseous
to describe an occasional multilocular
lesions like non-osteogenic fibroma, benign
radiographic appearance.
osteoblastoma and hemangioma of bone.
Margins — are somewhat less regular and distinct
Clinical Features than odontogenic cyst but more discrete than a
Age — although, it may be seen in adults, it is central malignancy.
more commonly seen as abnormalities of older Expansion — as the cyst enlarges to more than a
children and adolescents with more than 90% of few centimeters in anterior-posterior dimension,
lesions occuring in individuals younger than the it also produces expansion of the buccal and
age of 30 years. lingual cortical plates.
Sex — it is more common in females than in males. Teeth —
Site — it usually involves the mandibular molar • Simple tilting and bodily displacement of
region as compared to anterior region. erupted teeth.
• Some degree of external root resorption may
History — history of traumatic injury and of recent
be seen though it will not devitalize the tooth.
displacement of teeth which remain vital.
Cortex — there is also buccal and lingual
Symptoms —
expansion of the cortex often marked and
Aneurysmal cyst of the jaw produces a firm
described as ballooning or blowing out.
swelling which may be painful and tender on
motion. Histopathological Features
Swelling becomes progressively worse and the
rate of development is often described as rapid. The lesion consists of mainly capillaries and blood
Some times patient may complain of difficulty filled spaces of varying sizes, lined by flat spindle
in opening the mouth i.e. if there is impingement shaped cells and separated by delicate loose
of the lesion on the capsule of TMJ. textured fibrous tissue (Fig. 16.42).
Signs — It contains many cavernous sinusoidal blood
Usually there is tilting or bodily displacement filled spaces which may or may not show
of teeth in the affected areas though it does not thrombosis.
devitalize the affected tooth. Young fibroblasts are numerous in the
Excessive bleeding may occur. connective tissue stroma.
When the lesion perforates the cortex and is Most of the cysts contain small multinucleated
covered by periosteum or only a thin shell of bone, giant cells with a patchy distribution (Fig. 16.43).
it may exhibit springiness or egg shell crackling, Scattered trabeculae of osteoid and woven
but it is not pulsatile. Bruit is not heard. bone.

Radiographic Features Differential Diagnosis

Radiodensity — the aneurysmal bone cyst is an Ameloblastoma — it is more common in older age
expansile osteolytic process within the affected group and in the mandibular posterior region.
 Cysts of the Orofacial Region 359

unerupted tooth. It shows a typical honey comb

appearance.
Cherubism — it occurs in a younger age group and
it is a bilateral lesion.
Metastatic tumor — it occurs in older age group.
Giant cell lesion of hyperparathyroidism — more
common in the older age group. Serum shows
high levels of alkaline phosphatase.

Management
Surgical curettage or partial resections are the
primary forms of treatment for aneurysmal bone cyst.
Cryosurgery and immediate packing with
Figure 16.42: Aneurysmal bone cyst with cavernous bone chips is the treatment of choice.
sinusoidal blood filled spaces The recurrence rate is fairly high, ranging form
5 to 19% after curettage. Thus indicate the need
for careful follow-up.

Cysts of the Maxillary Sinus

It is discussed in chapter of maxillary sinus

CYSTS OF SOFT TISSUES OF THE FACE

AND MOUTH
Dermoid and Epidermoid Cyst
It may occur as developmental anomalies and
about 1 to 2% occurs in oral cavity. The lumen of
simple cyst filled with cyst fluid or keratin and no
other specialized structure, is called as 'epidermoid
Figure 16.43: Giant cells of aneurysmal bone cyst cyst'. If lumen contains sebaceous material as well
as keratin then it is called as 'dermoid cyst'. If
Giant cell granuloma — more common in the anterior lumen contains elements such as bone, muscle or
region. teeth from various germinal layer is called as
Central hemangioma — it is more common in the 'teratoma'. Dermoid cyst is lined by epidermis and
mandible and it shows profuse hemorrhage if skin appendages are present in the fibrous wall.
aspirated. Bruit is heard in such lesion. Epidermoid cyst is lined by epidermis, but contains
no appendages.
Multilocular cyst — it is more common in the
mandibular posterior area and also the borders of Pathogenesis
the lesion are well defined.
Most likely site for their origin is anteriorly,
Odontogenic myxoma — it is more frequently between the contributions from the mandibular
associated with congenitally missing or arches to the tongue.
360 Textbook of Oral Pathology

Implantation keratinizing epidermoid cyst Radiographic Appearance

may occur in other parts of the mouth as a result No specific appearance. CT scan may be useful.
of trauma. In some cases, if you want to see the extent of
the cyst, then we have to remove some content
Types
of the cyst to enable introduction of an opaque
Median variety substance such as lipiodol, which is chemical
• Supramylohyoid variety combination of iodine and poppyseed oil. After
• Inframylohyoid variety this, radiograph is made from every position
Lateral variety necessary to enable opaque material find its way
• Supramylohyoid variety by gravity to each portion of the cyst.
• Inframylohyoid variety
Histopathological Features
Clinical Features
They are lined by keratinized epidermis.
Age and sex — it occurs at any time from birth to Cysts of the floor of the mouth lined
adolescence and it is small in infancy and large predominately by secretory epithelium are
in adolescence. Mainly it is apparent between 12 probably of salivary duct origin.
to 25 years of age and occurrence is equal in both Dermoid cyst is characterized by presence of
sexes. one or more dermal appendages such as hair
Site — midline of the floor of mouth is the follicles, sweat glands or sebaceous glands in the
commonest location of these cysts which may wall with the lumen usually filled with keratin
cause a swelling in the midline of the neck or some (Fig. 16.44).
times, it may be lateral.
Symptoms — swelling is slow and painless. It may Differential Diagnosis
interfere with breathing, speaking, closing the Ranula — it is not in midline and appears bluish.
mouth and eating. Transillumination test is positive.
Size — the size may vary up to several
Unilateral or bilateral blockage of Wharton's duct —
centimeters in diameter.
pain during meals.
Transillumination test — it is usually negative.
Fluctuation test — it is generally positive. Thyroglossal duct cyst — it lifts when the patient
Appearance — swallows or protrudes the tongue.
– If superficial, it is yellow to white and surface
is smooth and non ulcerated until
traumatized.
– Inframylohyoid variety - it causes swelling in
the sub-mental area and it give rise to a 'double
chin' appearance.
Signs —
– Soft to firm, fluctuant, rubbery or cheesy
sharply delineated with straw colored fluid.
– It may be fluctuant and doughy depending on
the content.
Figure 16.44: Dermoid cyst lined by keratinized epidermis
– It does not move with protrusion of the tongue and shows dermal appendages.The lumen is filled with
or deglutition. keratin
 Cysts of the Orofacial Region 361

Cellulitis — the swelling is diffuse and widespread. Histological features — histologically, consists of
Submandibular lymph nodes swelling — it is solid. epithelial lined multilocular cystic spaces
enclosed by dense lymphoid tissue composed of
Management small lymphocytes, plasma cells and germinal
Surgical excision is the treatment of choice. centers (Fig. 16.45).
It should be excised through the floor of mouth
by retracting the posterior border of the mylohyoid Intra-oral Lymphoepithelial Cyst
muscle. There is cystic degeneration of epithelial inclusion
in lymphoid aggregate in the oral cavity.
Lymphoepithelial (branchial cleft) Cyst
Its location in the neck, parotid gland and intra- Clinical Features
orally will be dealt separately. Site — it affects mainly the floor of mouth and the
tongue.
Pathogenesis
Age — the age range is 15-65 year with a male
It originates from cystic transformation of
predominance.
glandular epithelium entrapped within the oral
lymphoid aggregates during embryogenesis. Appearance — the cyst appears as a non-ulcerated
Epithelium in lymphoepithelial cyst might be freely movable mass which has been present for a
derived from the ductal epithelium of salivary period ranging from 1 month to a year.
glands. • Symptoms — patients may complain of
swelling and discharge.
Lympho-epithelial cyst of the neck • Size — size may vary from few mms to 2 cm.
Clinical Features • Signs — fairly mobile, superficial soft fluctuant,
sharply delineated swelling.
Site — location is superficially in the neck, close
to the angle of the mandible, anterior to the • Shape and color — the usual observation was
of round or oval swelling of the oral mucosa
sternocleidomastoid muscle.
of normal color but when large swelling they
Age — occurs at all ages with a fairly equal
were yellow or white in color.
distribution form first to sixth decade.
Size — the neck lesions vary in size from small to
very large (about 10 cm in diameter).
Symptoms — there is swelling, which may be
progressive or intermittent and pain may also be
a feature.
Histological features — cyst is lined by stratified
squamous epithelium.

Lymphoepithelial cyst of parotid gland

Clinical Features
Age — ranges from 16 to 69 years with a male to
female ratio of 3:1.
Figure 16.45: Branchial cyst lined by stratified squamous
Symptoms — there is a nodule in the parotid gland. epithelium. The cytic wall typically contains lymphoid tissue
362 Textbook of Oral Pathology

Histopathological Features Site — most commonly located in the area of hyoid

bone and when they occur in the mouth, they do
• Cyst lined with thin stratified squamous
so either in the floor or at the foramen caecum.
epithelium, usually parakeratinized devoid
Symptoms —
of rete pegs.
– Pain may occur if the cyst is infected.
• The lining epithelial is quite thin; lacking rete
– If they are located high in the tract they may
pegs and is usually parakeratinized and cause dyspnea.
occasionally orthokeratinized.
• The lumen of the cyst often contains sloughed Shape — it is spherical or oval with a long axis
epithelial cells, lymphocytes and eosinophilic along the thyroglossal tract.
amorphous coagulum. Size — the cyst varies in size from 0.5 to 5 cm in
• Occasionally, lining epithelium is of columnar diameter.
type with or without goblet cells.
Fluctuation and transillumination — it is usually
Management positive.

Local surgical excision. Complication — complication like fistula

formation, recurrent infection can occurs.
Thyroglossal Duct Cyst Signs —
– They lift when the patient swallows or
The analoge of the median lobe of the thyroid gland
protrudes the tongue.
develops at about the fourth week of the intra-
– The cysts are usually in the midline and
uterine life from a site at the base of tongue which
produce a softer, movable sometimes fluctuant
is recognized later as the foramen caecum. A
or tender swelling.
hollow epithelial stalk known as the thyroglossal
– Consistency is often form or hard depending
duct extends caudally and passes ventral to the
upon the tension of fluid within the cyst.
hyoid bone to the ventral aspect of the thyroid
cartilage where it joins the developing lateral lobe. – Cyst moves with deglutition as swelling is
The thyroglossal duct disintegrates by about tenth attached to hyoid bone by fibrous tissue. It
week, but cyst may form from residue of the duct also moves with protrusion of tongue.
at any point along its line of descent.
Histopathological Features

Pathogenesis They are lined by pseudo-stratified columnar

epithelium which may be ciliated or by stratified
Inflammatory conditions which lead to reactive squamous epithelium.
hyperplasia of the lymphoid tissue adjacent to The connective tissue wall of the cyst
the remnants of thyroglossal tract may stimulate frequently contain small patches of lymphoid
the epithelial remnants themselves, have been tissue, thyroid tissue and mucous glands
mentioned, as has a blocked thyroglossal duct (Fig. 16.46).
with an accumulation of secretion.
Differential Diagnosis
Clinical Features Subhyoid bursal cyst — it moves with deglutition
Age and sex — it is slightly more common in women but not with protrusion of tongue.
and occurs commonly in the first, second and third Sublingual dermoid — it does not move with
decades of life. protrusion of the tongue.
 Cysts of the Orofacial Region 363

Oral Cyst with Gastric or Intestinal Epithelium

It is also called as 'oral alimentary tract cyst'.

Pathogenesis
Gorlin pointed out that in the 3-4 mm embryo, the
undifferentiated primitive stomach lies in the mid-
neck region, not far from anlage of the tongue.
Gastric mucosa has been shown to occur in
the esophagus of 7.8% of infant and in 51% of
these, heterotrophic tissues were located in the
upper third.
They suggested that in the sublingual region
of the oral cavity and in the region of apex and
Figure 16.46: Thyroid follicles, mucous glands and duct dorsum of tongue, the ectodermal and endodermal
in the wall of thyroglossal duct cyst
epithelia fuse and this will explain presence of
heterotrophic gastric or intestinal mucosa.
Management
Surgical excision is usually the treatment of Clinical Features
thyroglossal duct cyst. Age and sex — most cases occur in infants and
Sistrunk operation involves the removal of a 1 young children between the ages of 2 to 11 years.
cm block of tissue surrounding the duct and the The male to female ratio is 3:1.
duct should be traced down to the pyramidal lobe Site — common location in the anterior part of
of thyroid gland and to the foramen caecum at the the tongue, floor of mouth and submandibular
base of tongue. gland.
The cyst may be enclosed entirely within the
Anterior Median Lingual Cyst tongue or floor of mouth or may communicate
It is also called as 'intra-lingual cyst of foregut with the surface.
origin'.
Histopathological Features
Clinical Features The cyst may be lined partly by stratified
Age — it is a rare lesion and is commonly found in squamous epithelium and partly by gastric or
children. It can be present since birth and intestinal mucosa.
recurrence is common.
Site — it is a fluctuant swelling in anterior half of Management
the tongue. Surgical excision. Recurrence is rare but has been
Symptoms — there may be difficulty in feeding. reported.

Histopathological Features Cystic hygroma

It is lined by pseudostratified ciliated columnar It is a developmental abnormality in which there
epithelium and by cuboidal epithelium. is progressive dilatation of lymphatic channels.
364 Textbook of Oral Pathology

Clinical Features Thymic Cyst

Site — it frequently involves the neck and face, They are rare clinical entities, which arise, in
although it can occur anywhere in the body. persistent thymic tissue, which may occur in any
location between the angle of the mandible and
Age — it is often present at birth and most cases
midline of the upper neck to the sternal notch.
are diagnosed before the age of 2 years.
Histologically, the cyst is lined by squamous
Symptoms — those that involve facial tissue and cuboidal epithelium and thymic tissue is
produce a swelling often painless and usually present in the wall.
compressible.
Cysts of Salivary Glands
Signs — the overlying skin may be blue and the
swelling transilluminates. It is described in chapter of salivary gland
There may be a history of gradual or sudden disorders
enlargement.
Parasitic Cyst
Histopathological Features
Hydatid Cyst
Histologically, the cystic hygroma consists of
It is also called as 'hydatid disease' or 'echinococcosis.
dilated cystic spaces lined by endothelial cells.
It is caused by the larvae E. granulosus, the dog
tapeworm and E. multilocularis.
Management
Complete surgical removal of the mass. Pathogenesis
This tapeworm lives in the intestinal tract of the
Nasopharyngeal Cyst
dog.
They are rare clinical entities. Its ova are excreted in the faeces of the dog and
They may be classified as congenital or may be ingested by the intermediate host like cattle
acquired and in midline or lateral. sheep and pigs. Man is also susceptible as an
They are lined by ciliated or non ciliated intermediate host as dogs are common household
columnar epithelium with areas of squamous pets, so may accidentally ingest the ova.
metaplasia in response to inflammatory stimuli. The ingested ova hatch in the upper
Lymphoid follicles are present in the wall. gastrointestinal tract, from where the small
Congenital midline cyst arises either from the embryo permeate the intestinal mucosa and are
pharyngeal bursa or from Rathke's pouch. distributed through the blood vessels and
Cysts arising from Rathke's pouch are lymphatics to all parts of the body.
exceedingly rare.
They have a median base attached to the Clinical Features
nasopharyngeal vault and lie anterior to the
Site — it is more common in liver, but others are
usual site of origin of retention and pharyngeal
found in lung, bones and brain.
bursa cyst.
They are lined by stratified squamous Symptoms — usually they are asymptomatic but
epithelium, in keeping with their ectodermal they enlarge progressively to cause pressure
origin. symptoms.
 Cysts of the Orofacial Region 365

Signs — liver involvement causes hepatomegaly The outer layer which is derived from the host
and jaundice. Pulmonary hydatid cyst may cause is made up of fibrous tissue and is infiltrated by
shortness of breath and hemoptysis. chronic inflammatory cells, eosinophils,
lymphocytes and giant cells.
Complications — rare complication of hydatid cyst
The intermediate layer is white, non-nucleated
includes rupture, suppuration and calcification
and consists of numerous delicate laminations. It
of the cyst.
usually shrinks away from the outer fibrous layer
when the tension within the cyst is relieved.
Oral Manifestation
Innermost layer is a nucleated germinal layer.
Site — intra-orally, found in tongue and angle of The cystic fluid is usually clear, albumin free
mandible. and contains the so called 'hydatid sand' consisting
Appearance — oral hydatid cyst presents as of brood capsule and scolices.
painless, progressively increasing, soft, elastic The brood capsules or daughter cysts develop
and well circumscribed fluctuant swelling. originally as minute projections of germinative
Symptoms — they may pose speech and layer which develops a central vesicle and become
mastication difficulties. a minute cyst.
Scolices of the head of the worm develop in
Radiographic Features the inner aspect of the brood capsule. It is when
There are four stages in the radiographic evolution they separate from the germinative layer that they
of the disease. They are as follows— form the 'hydatid sand'.
• First stage — there is cystic bone destruction
involving medullary portion of the bone. The Management
cysts are rounded and are described as 'grape The ideal treatment for hydatid cyst is surgical.
like'. The medulla is expanded and disease is Limiting contact between dogs and humans
sharply demarcated. and deworming dogs at regular intervals are
• Second stage — it is of secondary infection. In useful steps towards prevention.
this stage osteitis is the main change and new
bone form beneath the periosteum. Septa Cysticercosis Cellulosae
between the cysts become coarser and the Pathogenesis
margins become less distinct. Pathologic
fracture may occur. Man develops cysticercosis through the larval
• Third stage — the third stage is characterized form, cysticercus cellulosae, of the pork tapeworm
by confluent abscesses which break into the Taenia solium. He can act as both the intermediate
soft tissue. and the definitive host.
• Fourth stage — in fourth and final stage, bone The adult worm may be ingested from
is destroyed. The whole of affected portion inadequately heated or frozen pork. Alternatively,
of the bone is represented by a 'meaningless man may ingest the cysticerci themselves from
jumble of calcareous bubbles'. The patient is infested pork and these develop into adult worms.
likely to be crippled and bedridden. These lives attach to the wall of the small
intestine where they fully grow and at times
Histopathological Features reach a length of 7 mm.
The mature cyst consists of three layers, one of Gravid proglottids or eggs begin to drop off
host and two of parasitic origin. and are passed in the faeces.
366 Textbook of Oral Pathology

In this way, man through contaminated food dense inflammatory cell infiltrate consisting
or from their own dirty hands may ingest them or predominantly of lymphocytes, plasma cells and
they may be regurgitated into the stomach. In the histiocytes.
stomach, their covering is digested off and the Few foci of dystrophic calcification are present
larval forms are hatched. These penetrate the in this capsule and some of these are concentrically
intestinal mucosa and are then distributed laminated.
through the blood vessels and lymphatics to all Within the capsule is a delicate double layered
the parts of the body where they develop into membrane consisting of an outer acellular hyaline,
cysticerci. eosinophilic layer and an inner sparsely cellular
layer. This layer has a loose attachment to the
Clinical Features fibrous capsule and is readily torn away from it.
It depends upon the site and the number of the The cyst lies within this membrane and
cysticerci in the body. contains larval form of T solium.
During the stage of invasion there are no
symptoms or slight muscular pain and mild fever Management
may be present.
Cutaneous cyst should be surgically removed.
CNS involvement produces serious effects.
Mebendazole has some value in the treatment.
Oral Manifestations Pork should be properly cooked as preventive
measures.
There is very little report about cysticercosis of
oral region. In oral cavity, most common site Syndromes associated with odontogenic cysts
involved is the tongue. Other sites involved are
cheek and lips. Jaw cyst-Basal Cell Nevus-bifid Rib Syndrome
There is a firm mass and contains watery fluid It is also called as 'nevoid basal cell carcinoma',
and coiled white structure apparently attached to 'Gorlin's and Goltz's syndrome'. It is transmitted as
the inner aspect of cyst. autosomal dominant trait with poor degree of
It is a painless, well circumscribed, elastic and penetrance and variable expressivity.
fluctuant swelling.
Clinical Features
Radiological Features
Age — it appears early in life after 5 years and
When the larvae die in the soft tissue, calcification
takes place and it appears on the radiograph. before 30 years.
The shadow of calcific density are rod shaped Cutaneous anomalies —
or resemble a "drop". • Basal cell carcinoma, dermal cyst and tumors,
Some appear more of less rounded when the palmar pitting, palmar and plantar keratosis
projection of the rays happen to strike the larva and dermal calcinosis.
in its long axis. • Nevoid basal cell carcinoma is brownish
The size of the shadow is 1 mm more in width colored papules predominant on skin, neck
and -1.5 cm in length. and trunk.
• Skin lesions are small, flattened, flesh colored
Histopathological Features or brownish papules occurring anywhere in
It shows dense fibrous outer capsule which is the body, but are prominent on the face and
derived from host tissue. This contains a fairly trunk.
 Cysts of the Orofacial Region 367

• Basal cell carcinoma is less aggressive in this Management

syndrome than in solitary basal cell carcinoma.
• Complete enucleation of the lesion should be
Skeletal abnormalities — rib anomalies and done.
brachymetacarpalism, bifid rib, agenesis, • Periodic examination.
deformity and synostosis of rib; kyphoscoliosis,
vertebral fusion, polydactyly and shortening of TREATMENT OF CYSTS
metacarpals.
Regression of Cysts without Surgical
Ophthalmologic abnormalities — hypertelorism Treatment
with wide nasal bridge, dystopia canthorum,
There is good evidence that some small radicular
congenital blindness and internal strabismus.
cyst will regress if the necrotic pulp remnant and
Neurological anomalies — mental retardation, bacteria are removed from the root canal of the
calcification of falx cerebri and other parts of dura, causative tooth and the canal is effectively filled.
agenesis of corpus callosum, congenital Circumscribed rounded zone of periapical
hydrocephalus and occurrence of bone destruction perhaps up to 1.5 cm in
medulloblastomas. diameter will be seen in some patients to reduce
in size and resolve over a period of month.
Sexual abnormalities — Hypogonadism in males
and ovarian tumors. Marsupialization of Dental Cysts
Oral Manifestations It is known as 'Partsch's operation' as it is
discovered by Partsch. It involves making an
Jaw lesions appear as multiple odontogenic
opening into the cyst as large as practical and
keratocysts usually appearing in multiple
packing the cavity. It is a more effective way of
quadrants.
emptying a cyst.
Mild mandibular prognathism.
Indication
Radiographic Features
Age — in a young child, with developing tooth
Jaw cysts appear as a multiple cyst like
germs.
radiolucency of variable size varying from few
millimeters to several centimeters. Proximity to vital structure — when proximity of
They occur most frequently in premolar-molar the cyst to vital structure can create an oronasal
region. or oroantral fistula or injure the neurovascular
Radiopaque lines of calcified falx cerebri are structure, this treatment modality should be
prominent on PA projection. considered.

Differential Diagnosis Eruption of teeth — in a young patient with a

dentigerous cyst, marsupialization will permit the
Multiple myeloma — Bence-Jones protein in the
eruption of the unerupted tooth or any other
urine.
developing tooth which has been displaced.
Metastatic carcinoma — history of primary tumor.
Vitality of teeth — when the apices of many adjacent
Histiocytosis X — does not have characteristic erupted teeth are involved within a large cyst,
borders as seen in cyst. enucleation can prejudice the vitality of these
Cherubism — jaw expansion is common in it. teeth.
368 Textbook of Oral Pathology

Advantages The need for greater attention to the details of

surgical technique than is at first apparent, to
The procedure at least for large cysts, is technically
avoid complications.
simple.
Risk of invagination and new cyst formation.
Even quite large cysts can be dealt with under Prolonged follow-up visits and periodic
local anesthesia. As anesthesia of deeper recesses irrigation.
is not essential and this is particularly an Regular adjustments of plug.
advantage in the maxilla.
Because the deeper part of the lining is not Enucleation
disturbed, adjacent important structures are not Enucleation allows for the cystic cavity to be
put at risk i.e. the blood vessels to the apices of covered by a muco-periosteal flap and the space
adjacent vital teeth, the inferior dental fills with blood clot which will eventually
neurovascular bundle and the integrity of lining organized and form normal bone.
of the antrum or nose are well preserved.
Marsupialization may be the best way to Indications
conserve the tooth of origin of a dentigerous cyst • Treatment of odontogenic keratocyst.
and to permit its eruption. • Recurrence of cystic lesion of any cyst type.
It is the simplest way to treat a fracture
complicating a large cyst of the mandible. Advantages
Prevents oronasal and oroantral fistula. • Primary closure of the wound.
It reduces operating time and blood loss and • Healing is rapid.
helps in the shrinkage of cystic lining. • Postoperative care is reduced.
Alveolar ridge is preserved. • Thorough examination of the entire cystic
cavity can be done.
Disadvantages
Disadvantages
The need for regular postoperative care, possibly • After primary closure, it is not possible directly
over a substantial period of time. It is necessary to observe the healing of the cavity as with
supervise healing so that opening remains large marsupialization.
in proportion to the underlying cavity. • In young persons, the unerupted teeth in a
A rapid reduction in the size of the opening dentigerious cyst will be removed with the
may be difficult to prevent. Indeed an opening lesion in this mode of treatment.
into a large cyst in the ramus may present • Removal of large cysts will weaken the
significant problems. mandible making it prone to jaw fracture
Uneven reduction in size of the cavity may • Damage to adjacent vital structures.
result in slit like cavity, difficult to keep clean. • Pulpal necrosis.
 CHAPTER
Chemical and
17 Physical Injuries

LINEA ALBA veloped rete-pegs and densely collagenous

lamina propria that merges with periosteum.
Linea alba refers to the line of keratinization, found Increased thickness or hyperorthokeratosis is
on the buccal mucosa parallel to the line of seen. Sometime intracellular edema of the epi-
occlusion expanding to a triangular area inside thelium and mild chronic inflammation of un-
each labial commissure. derlying connective tissue can occur.
Etiology
Treatment
It occurs due to variation in dietary and oral
hygiene practice, frequent frictional contact with No treatment is required.
food and teeth. Effect of smoking, food texture
and other environmental irritants can cause linea Habitual Cheek or Lip Biting
alba. It is also called as ‘Morsicatio buccarum’.
Morsicatio comes for Latin word ‘morsus’
Clinical Features
meaning bite.
Common sites are buccal mucosa at line of Superficial lesions produced by frequent and
occlusion and where the mucosa overlies the repeated rubbing, sucking or chewing
bone as in hard palate and gingiva. Palate and movements that abrade the surface of a wide area
gingiva appear whiter than the adjacent mucosa of lip or cheek mucosa without producing
of the soft palate and alveolar gingiva, buccal and discrete ulceration.
lingual sulcus. It is more prominent in people
with little overjet of molars and premolars. It is
Etiology
line extending horizontally from commissures to
most posterior teeth. Unconscious nervous habits, uncontrolled
tongue thrusting and neuromuscular disorders
Histopathological Features such as tardive dyskinesia. Occlusal discrepancies,
Epithelium is keratinized under normal condi- rough tooth surface, stress and anxiety can also
tions and exhibits stratum granulosum, well de- lead to habitual cheek biting.
370 Textbook of Oral Pathology

Clinical Features tooth brush bristles, sharp margins of crown,

fillings, denture, orthodontic appliances and
It can occur at any age with no sex predilection. It
faulty instrumentation.
is usually occur on the buccal mucosa at the level
of occlusion. Usually, there is opaque white Chemical—It results from caustic substances such
appearance and is homogenous. In some cases, as silver nitrate, phenol, TCA, formocresol,
there is macerated and reddened area usually eugenol, eucalyptus oil, phosphorus and
with patch of partly detached surface epithelium. acetylsalicylic acid.
It may have sharply delineated borders or in
Thermal—Excessive heat in the form of hot fluid
some cases it may be poorly outlined. In some
or food, on rare occasion the application of the
cases, contused margins present with transient
dry ice, reverse smoking and hot instrumenta-
whitish tags of necrotic tissue around the ulcer.
tion.
It feels rough to examiner’s fingers. Area
becomes thickened, scarred and paler than Electrical current application to the oral tissues
surrounding mucosa. may result in destruction and consequent
ulceration, e.g. galvanism.
Histopathological Features
Others—Radiation burns, self-inflicted and
Long established lesion may be associated with iatrogenic.
areas of keratosis and increased thickness of
epithelium. There may be hyperorthokeratosis, Clinical Features
hyper-parakeratosis, and acanthosis. Depending
It occurs in persons of any age, with equal
upon degree of irritation, the underlying
frequency in both the sexes. It may involve any
submucosa may show an inflammatory cell
region of the mouth but is common on tongue,
infiltrate. In some cases clusters of vacuolated
in mucobuccal fold, gingiva and palate. The
cells are present in the superficial portions of the
appearance of the traumatic ulcer varies
prickle cell layer.
markedly depending on the site of the injury,
Management the nature and severity of trauma and the degree
of secondary infection present.
Small doses of diazepam 5 to 10 mg at bed time
for the management of neurological disorders. Single uncomplicated ulcer—The most common
Plastic occlusal night guard to control the habit variety of traumatic ulcer is single
of cheek biting. uncomplicated ulcer which generally is of
moderate size (from several millimeters to a
Traumatic Ulcer centimeter or more in diameter), round, oval or
elliptical in shape and flat or slightly depressed.
It is a frequently encountered ulcerative lesion Its surface consists of a serosanguineous or
of mouth. The common term used to denote a grayish serofibrinous exudate. It may be
traumatic ulcer is ‘decubitus ulcer’, ‘tropic ulcer’, composed of a grayish necrotic slough which
‘neutrons-tropic’ and ‘Bednar’s ulcer’. when removed, reveals a red raw tissue base.
The lesion is surrounded by a narrow border of
Causes
redness. There is tenderness and pain in the area
Mechanical or physical—It includes biting, sharp of lesion and it will be helpful to identify the
or mal-pozed teeth or roots, sharp food, stiff cause of lesion. It may persist for few days and
 Chemical and Physical Injuries 371

may last for weeks. Ulcer on vermilion border may Vascular connective tissue deep into the ulcer
have crusted surface due to absence of saliva. can become hyperplastic which may results in
Painful regional lymphadenopathy also occur. surface elevation.
Complicated ulcer—In still other instances, the
Management
traumatic lesions are large and irregular. These
are the result of unusually sever traumatic Usually the simple and uncomplicated traumatic
episodes, such as a blow or a fall and are often ulcer heals uneventfully in 5 to 10 days after
accompanied by considerable edema, inflamma- onset and even without treatment. However, in
tion and swelling of the neighboring tissues. the presence of secondary infection or repetitive
trauma longer healing period is required.
Infected ulcer—The infected ulcer is larger more
Causative agent should eliminate.
irregular and more protruding than the non-
infected one and often it is covered with a thick Persistent ulcer—triamcinolone acetonide in
layer of necrotic slough through which purulent emollient base before bedtime and after meals.
exudate may be observed. Chlorhexidine mouth wash or even topical local
anesthetic should be given to relive acute
Radiation burns—Acute reaction occurs during
symptoms of pain. A persistent ulcer, not
the course of radiotherapy due to direct tissue
responding to the foregoing regimen, should be
toxicity. Ulcer resolves over several weeks
surgically excised and the entire tissue must be
following the completion of therapy. Chronic
sent for histopathological examination.
complication or late radiation reaction occurs
due to change in the vascular supply, fibrosis in
Electrical and Thermal Burns
connective tissue and muscle and change in
cellularity of tissues. Mucositis occurs when the Burns cause transient non-keratotic, white
rate of epithelial growth and repair are affected appearance of the mucosa which is attributed to
by radiation, resulting in epithelial thinning, superficial pseudomembrane composed of
erosion and ulceration. The first sign of mucositis coagulated tissue with an inflammatory exudate
may be whitish appearance of the mucosa due (saliva protects oral mucosa). Chronic mild burns
to hyper keratinization and intra-epithelial cause keratotic white lesions while intermediate
edema or a red appearance due to hyperemia. burns cause localized mucositis and severe burns
Pseudomembrane formation, most likely coagulate the surface of the tissue and produces
represents ulceration with fibrinous exudate, diffuse white lesions. If coagulation is severe,
oral debris and microbial component. Radiation tissue can not be scraped off easily leaving raw
has more marked effect on rapidly proliferating and bleeding painful surface.
epithelium and therefore, mucositis involves the Electrical burns can be contact type (electrical
non-keratinized mucosa first. current passing through the body from the point
of contact to the ground site) or arc type (saliva
Histopathological Features act as conducting medium and an electrical arc
Simple ulcers are covered by fibropurulent flow between the electrical source and mouth).
membrane that consists of firing intermixed with Electrical burns usually occur in children
neutrophils. Adjacent surface epithelium may younger that 4 years of age with oral
show hyperplasia with or without hyper- commissures is commonly involved. Edema is
keratosis. Inflammatory infiltrate consists developed which is followed by necrosis of
lymphocytes, neutrophils. affected area after 4 days. Bleeding may occur
372 Textbook of Oral Pathology

due to exposure of vasculature which should be Ethyl alcohol burns—Topical application of ethyl
closely monitored in this case. alcohol solution which results in sloughing of
Thermal burns can be cause by hot food, the oral mucosa. Some of the mouth wash
beverages. There is pain which lasts for short contain 25% ethyl alcohol.
duration. It is seen anterior 1/3 of tongue and Vitamin C tablet—In some cases, it can causes
palate. It may produce coagulation necrosis of burns of oral mucosa.
superficial tissue that appears whitish. In some
cases, there may be frank ulceration and Acid burns—If the rubber dam placement is
ineffective then acid used to etch tooth surface
stripping of mucosa. Red area is tender to
may come in contact with the oral mucosa.
painful; it may blanch on pressure and there is
bleeding on manipulation. Surface layer of Ingestion—Chemical burns of the oral cavity can
epithelium is desquamated. It can be manage by result when the children mistakenly drink
systemic analgesics and topical hydrocarbon in household chemical and with suicide attempts
emollient base. by ingestion of caustic material.

Pizza burns—Central palatal burns, whitish grey Hydrogen peroxide—It is use for the treatment of
or ulcerated lesions of the middle third of the periodontitis. Concentration higher than 3% will
hard palate. These also present superficial result in mucosal damage.
necrosis and ulceration due to combination of Silver nitrate—It is the treatment modality of aph-
heat of the cheese and its adhesion to blister thous ulceration. It will also results more mu-
epithelium. cosal damage so its use should be discouraged.
CO2 burns (snow) (dry ice)—Children affected Clinical Features
commonly. Tongue and lip are most common
sites. Frost bite of lip—it is also called as ‘Popsicle Irregularly shaped, white pseudomembrane
covered lesion develops. The lesion is usually
panniculitis’. There is persistent swelling and
painful. Gentle lateral pressure causes the white
redness. It occurs due to prolonged contact of
ice cream and other frozen confectionaries or material to slide away exposing an exquisitely
painful central ulceration and more adherent
very cold metal, glass object, with child’s lip.
patches of white material the periphery. Aspirin
Epithelium becomes dry and rougher than
surrounding tissues. burns are focal with sharply delineated borders.
Diffuse border may present if the burn is more
Chemical Burns extensive. In the toothache drop burn there may
be sloughing of oral mucosa.
Burns due to caustic chemical agents will
produce coagulation necrosis of the epithelium Cotton roll burns—Sometime when cotton is used,
caustic material can leak in it and it held against
with subsequent inflammation.
the mucosa for longer period of time. This will
Causes lead to injury to mucosa. In some cases mucosa
can adhere to dry cotton role which will results
Aspirin and aspirin-containing compounds—It in striping of the epithelium in the area.
occurs when it is kept in mucobuccal fold to
relieve toothache. Histopathological Features
Tooth-ache drop burns—Such drops contain It causes coagulation necrosis of the epithelium.
creosote, guaiacol and phenol derivatives. The upper layer shows a homogenous
 Chemical and Physical Injuries 373

appearance with loss of structure and failure of

nuclei to take stain. Inflammatory cell infiltrates
with the underlying connective tissue can be
seen. Hyperkeratosis and acanthosis of oral
mucosa.

Management
Palliative care with a topical anesthetic or a bland
coating suspension is used. These are
accomplished by the use of Orabase gel or
palliation may result from anesthetic effects of
an antihistaminic mouth rinse in combination
with kaopectate. Painful lesion heals quickly.

Radiation Injury
Painful mucositis and dermatitis are the most
frequent manifestation of radiation injury.
Figure 17.1: Dermatitis in patient after receiving
Dermatitis—Excessive exposure will cause radiotherapy
dermatitis. Repeated exposures have a
cumulative effect (the tissue does not return to
original state due to irreparable damage). There
is dryness, erythema, thickening, desquamation
and cracking of hands may also occur. Epilation
can be cause by radiation. It is often seen in
association with dermatitis. Hair loss can be
permanent (Fig. 17.1).
Mucositis—It is redness and inflammation of the
mucous membrane and it is commonly seen
during radiation therapy. It occurs due to death
of the cells of oral mucous membrane. By the end
of therapy, the mucositis is very severe, with
maximum discomfort and difficulty in intake of
food (Fig. 17.2). Microscopic features—the surface
epithelium may show ulceration or atrophy. Later
changes show an inflammatory cell infiltrates in
the submucosa with an attenuated spinous cell
layer.
Figure 17.2: Mucositis in patient receiving radiotherapy
Pseudomembrane formation—After this the mucous
membrane begins to break down and leads to the Taste buds—Dose in therapeutic range can cause
formation of white to yellow pseudomembrane extensive degeneration of the histological
(Fig. 17.3). architecture of taste buds. Patient usually notices
374 Textbook of Oral Pathology

Children receiving radiation therapy to the jaws

may show defect in the permanent dentition
such as retarded root development, dwarfed
teeth or failure to form one or more teeth. Pulp
will show fibro- atrophy after radiation.

Osteoradionecrosis—It is the most serious clinical

complication that occurs in bone after irradiation.
When the changes in a bone are so severe that
bone death results, the condition is known as
osteoradionecrosis. Bone infection occurs either
due to radiation induced breakdown of the oral
mucous membrane, mechanical damage to the
Figure 17.3: Pseudomembrane formation in patient who
is receiving radiotherapy weakened oral mucous membrane, through
periodontal lesion or from radiation caries.
loss of taste during 2nd or 3rd week after
Osteoradionecrosis is more common in mandible
radiotherapy. Bitter and acid flavors are severely
affected when the posterior third of the tongue is than in maxilla due to rich vascular supply of
the maxilla.
irradiated and taste of salt and sweet are affected
when anterior third of tongue is irradiated.
Bruxism
Xerostomia—The parenchymal component of
salivary gland is more radiosensitive (parotid The word bruxism is taken from the Greek word
gland is more radiosensitive than sub- brychein: gnashing of teeth. Although the term
mandibular or sublingual gland). Exposure to bruxism is not generally known to lay people, it
radiation leads to injury of these parenchymal is shorter and more convenient than teeth
cells leading to xerostomia. The extent of reduced clenching or grinding. Bruxism can perhaps be
salivary flow is dose dependent and it is zero best defined as the involuntary, unconscious and
when dose reaches 60 Gy. The mouth becomes excessive grinding, tapping, or clenching of
dry, tender, and swallowing is difficult and teeth. When it occurs during sleep, it may be best
painful since the residual saliva also loses its called as sleep or nocturnal bruxism. A few
normal lubricating properties. Because of small people, on the other hand, brux while they are
amount of thick, viscous, acidic saliva such awake, in which case the condition may be called
patient are prone to carries which is known as wakeful or diurnal bruxism.
radiation carries. All forms of bruxism entail forceful contact
between the biting surfaces of the upper and
Histopathological features — After months, the
lower teeth. It is also called as ‘night grinding’ or
inflammatory response becomes more chronic
‘bruxomania’. It is a habitual grinding of the teeth,
and the glands demonstrate progressive fibrosis,
either during sleep or as an unconscious habit
adiposis, loss of fine vasculature and
during waking hours. It is term used both for
concomitant parenchymal degeneration,
clenching habit during which pressure is exerted
resulting in xerostomia.
on the teeth and periodontium by the actual
Teeth—Irradiation of teeth during develop- grinding or clamping of the teeth and, also to
mental stage retards their growth severely. repeated tapping of the teeth.
 Chemical and Physical Injuries 375

Etiology time, the condition may lead to bridges, crowns,

root canals, implants, partial dentures and even
Local—Mild occlusal disturbances, unconscious
complete dentures. As long as bruxism continues,
attempt by the patient to established a greater
the situation keeps getting worse. Thus, "by 40 or
number of teeth in contact or to counteract a local
50 years of age, most patients have worn their
irritating situation.
teeth to the degree that extensive tooth restorations
Systemic factors—Gastrointestinal disturbances, have to be performed.
subclinical nutritional deficiency and allergy or As the teeth wear out, they become shorter. As
endocrine disturbances have been reported to be a result, when the mouth is closed, the upper and
the causative factors. lower jaws are nearer than they used to be and so
Psychological factors—Emotional tension in which are the nose and chin. The skin now may bag
patient is unable to express his emotion due to below the eyes and curl around the lips, causing
fear, rage, rejection and it becomes hidden in the lips to seemingly disappear. The chin recedes
subconscious and later expressed by variety of and the person looks comparatively old.
way like by grinding the teeth. Facial muscles—Bruxism involves excessive
Occupational—Like in cases of watchmaker, muscle use leading to a build-up or enlargement
persons who chew gum, tobacco or objects such (hypertrophy) of facial muscles, especially those
as toothpicks or pencils. of the jaws (where the masseter muscle—the
muscle that raises the lower jaw and enables
Clinical Features closing the jaws—is located). In long-term
bruxers, this build-up may lead to a characteristic
In grinding and tapping, this is contact which
square-jaw appearance. Some patients resort to
involves movements of the lower jaw and
removing part of the masseter muscle by surgery
unpleasant sounds which can often awaken
or injections of toxic materials to reduce muscle
housemates. Clenching (or clamping) on the
size and thus partially regain their former, more
other hand, involves inaudible, sustained,
aesthetically pleasing look.
forceful teeth contact unaccompanied by
mandibular movements. Salivary glands—Another example of this spiral
involves the occasional inflammation and
Teeth—Chronic bruxism may lead to sensitive,
blockage of some salivary glands. In this case, the
worn-out, decayed, fractured, loose or missing
masseter muscle becomes disproportionately
teeth. Grinding or clenching breaks down the
overdeveloped and blocks the opening of the
enamel; sometimes in long-term bruxers,
nearby parotid glands. They, thus interfere with
reducing the teeth to stumps. Instead of a white
the flow of saliva into the mouth, causing the
enamel cover, one often sees the more yellowish
saliva to accumulate in the glands. This in turn
and softer dentin. The posterior teeth of some
may lead to periodical swelling, pain,
chronic bruxers often loose their cusps and
inflammation and abnormal dryness of mouth.
natural contours, appearing instead flat, as if they
had been worked over with a file or sandpaper. TMJ—Bruxism may also damage the
When anterior teeth are affected, their biting temporomandibular joints. First few signs of
surfaces are damaged. The absence of enamel temporomandibular joint disorders are TMJ
makes it easier for the bacteria to penetrate the discomfort or pain, soreness of jaws and muscles,
softer part of the teeth and produce cavities. With clicking or popping sounds when opening the
376 Textbook of Oral Pathology

jaws or while chewing and difficulties in opening develop techniques for reducing that tension and
the mouth fully. hence, bruxism.
Malocclusion—Malocclusion or bad bite is more Exercise—Quinn suggested isokinetic and
common among bruxers than in the general stretching exercises of the mandible. Such
population. Bruxism may often involve more exercises may or may not help alleviate bruxism,
pressure on one side of the mouth than on the but perhaps may be used to complement other
other, thereby causing malocclusion. As the teeth approaches. However, but it seems unlikely that
wear out, the distance between the upper and they could ever be used as the sole therapeutic
lower jaw decreases and overclosure may approach. Evidence that this approach is
develop. effective are non-existent.
Effect on periodontium—There may be loss of Drugs—Both, the stress and brain malfunction
integrity of the periodontal structures resulting etiological theories give at times, rise to the use
in loosening or drifting of the teeth and even of anti-anxiety agents, muscle relaxant and other
gingival recession occurs. drugs. Most authorities however, feels that at
best, drugs in use now are of limited value in
Management the treatment of great majority of chronic bruxers
and that they often involve moreover untoward
Psychotherapy—The belief that bruxism is
traceable to stress and other emotional and side effects. Evidence that this approach is
effective: are non-existent.
psychological factors give rise to a variety of
psychotherapeutic approaches. For instance, Equilibration therapy—Some people believe that
listening to progressive relaxation or bruxism is traceable to malocclusion (bad bite).
autosuggestion tapes just before going to sleep They therefore suggest eliminating this cause
may foster calmness and self-confidence. through orthodontic adjustment.
Wakeful EMG feedback—Another psychological Splints—By far, the most common treatment re-
approach to stress reduction resorts to gime for bruxism relies on the time-honored pro-
instrumentation. During bruxing, the relevant cedure of splints like nightguards, biteguards,
muscles are active and this increased activity or occlusal splints, biteplates, removable appliances
tension can in turn be measured with an or interocclusal orthopedic appliances and use of
electromyograph (EMG: electro—electric; myo— manufactured customized appliances. Removable
muscle; graph—record). During treatment splints are worn at night to guide the movement
sessions at home or the laboratory, the patient so that periodontal damage is minimal.
sits or reclines comfortably. One or more pairs
of recording electrodes are then attached to the Traumatic Lesion due Sexual Habit
surface of the skin in close contact to appropriate
Orogenital practice is common nowadays.
muscles (e.g., masseter muscles). These
electrodes transmit information about the level
Clinical Features
of muscle activity to a computer monitor. The
patient is instructed to consciously lower that There is submucosal palatal hemorrhage
level below a threshold line (also visible on the secondary to fellatio. It appears as erythema,
screen). Gradually, by becoming alert to the petechiae, purpura, and ecchymosis of soft
presence of muscle tension, patients may palate.
 Chemical and Physical Injuries 377

Oral lesion also occurs due to cunnilingus, slightly extruded. If fracture line is close to the
resulting in horizontal ulceration of the lingual apex then tooth will be more stable. If only
frenum. As the tongue is thrust forward the taut movement of crown is detected, root fracture is
frenum rubs or rakes across the incisal edges of likely. There is temporary loss of sensitivity. It
the mandibular central incisor. Linear fibrous returns to normal within 6 months.
dysplasia also in person which repeatedly
perform the act. Crown/Root Fracture
Such fracture is likely to be intra and extra-
Histopathological Features alveolar. They are a result of direct trauma. They
There is subepithelial accumulation of red blood have labial margin in the gingival third and
cells which may separate the surface epithelial course obliquely to exist below the gingival
from underlying connective tissue. Patchy attachment on the lingual surface. It frequently
degeneration of epithelial basal cell layer can involves pulp. There is pain during mastication.
occur. Tooth is sensitive to occlusal force.

Vertical Root Fracture

Management
It is also called as ‘cracked tooth syndrome’. It
No treatment is required. runs length wise from crown towards apex of
Fracture of Teeth tooth. It occurs in endodontically treated tooth
as there is weakening of tooth in such cases. It
Dental Crown Fractures may be caused by traumatic occlusion. It is
Anterior teeth are commonly involved. It may usually seen in posterior teeth in adults,
be caused by fall, accident and blows from especially in mandibular molars. Dull pain of
foreign bodies. long duration which may vary from non-existent
to mild. It may have periodontal lesions
Cracks—It can be seen in indirect light (directing
resembling chronic lesion. History of repeated
the beam along the long axis of tooth). failure of endodontic treatment.
Uncomplicated fracture—It does not involve
dentin and is usually found on mesial or distal Perforation of the Root
corner of maxillary central incisors. Dentin The root canal is sometimes penetrated during
involvement is identified by contrast of color operative procedures and injury may extend to
between it and peripheral layer of enamel. cause a perforation of the root. The usual site of
Exposed dentin is very sensitive to chemical, it is at the apex and the side of the root, but the
thermal and mechanical stimulation. floor of the pulp chamber and even the side of
Complicated fractures—There is bleeding from the crown; near the neck of the tooth may be
exposed pulp or atleast drop of blood oozes from perforated. In some cases, the shadow of root
the pinpoint exposure. Exposed pulp will be canal can be seen approaching the side of the
sensitive to most forms of stimulation. root and this can be the evidence of a perforation.

Dental Root Fracture Histopathological Features of Tooth Fracture

It is common with maxillary central incisors. The clot between root fragments is organized and
Coronal fragment is displaced lingually and is this connective tissue is subsequently the site of
378 Textbook of Oral Pathology

new cementum or bone formation. There is always enter the mucosa lacerated by rotary instruments
some resorption of the ends of the fragment but during removal of old amalgam fillings or crown
these resorption lacunae ultimately are repaired. and bridge preparations of teeth with large
amalgam restorations. Broken pieces may be
Tooth Ankylosis introduced into the socket or beneath the
Ankylosis between tooth and bone is an periosteum during extraction of the teeth.
uncommon phenomenon in the deciduous Particles may enter the surgical cut during root
dentition and even more rare in permanent teeth. canal treatment with retrograde amalgam filling.

Causes Clinical Features

It occurs when partial root resorption is followed The most common sites are on gingiva and
by repair; with either cementum or bone that alveolar mucosa with mandibular region being
unite the tooth root with alveolar bone. affected more commonly than maxillary region.
Ankylosis does occur after traumatic injury It can occur at any age but it is rarely seen below
particularly occlusal trauma. It may follow root the 12 years as amalgam restorations are not used
canal therapy, if the apical periodontal ligament before the age of 12 years. Females are affected
is irritated or seriously damaged. more commonly than males in ratio of 1.8:1. It is
described as a flat macule or sometimes slightly
Clinical Features raised lesion with margins being well defined
It seldom manifests any clinical symptoms unless or diffuse in other. Pigmentation is blue black in
there is concomitant pulp infection. If the extensive color. It may gradually increase in size.
area of the root surface is involved, the tooth give
Histopathological Features
a dull muffled sound on percussion rather than
the normal sharp sound. There is considerable Amalgam presents as discrete fine dark growth
difficulty while doing extraction of the teeth, and irregular solid fragments. Dark granules
sometimes necessitating surgical removal. arranged mainly along collagen bundles and
around blood vessels, nerve sheath, elastic fibers
Histopathological Features and acini or minor salivary glands. Dark
An area of root resorption which has been granules are present intracellularly within
repaired by a calcified material, bone or macrophage multi-nucleated giant cell,
cementum which is continuous with the alveolar fibroblasts.
bone. The periodontal ligament is completely
Management
obliterated in the area of ankylosis.
Treatment is not necessary. However, if required,
Management excision is done.
It has good prognosis and as such no treatment
Bismuthism
is required.
Causes
Amalgam Tattoo
Medicinal use of bismuth containing
Causes preparation. Many proprietary drugs contain
It may be condensed in the abraded gingiva bismuth salt and bismuth containing pastes may
during routine amalgam restorative work. It may result in bismuth pigmentation.
 Chemical and Physical Injuries 379

Mechanisms papilla and marginal gingiva in a patient

receiving oral or anal administration of bismuth
This pigment is produced by the action of
compounds.
hydrogen sulfide on the bismuth compound. The
hydrogen sulfide is formed through bacterial Paper test—it will indicate whether the
degradation of organic material of food pigmentation is actually in gingival tissue. If the
retention. pigmentation persists, when small piece of white
paper is inserted in the gingival sulcus, the
Clinical Features presence of pigmented area is verified.
Vague gastrointestinal tract disturbances,
Plumbism
nausea, bloody diarrhea, ‘bismuth grippe’ and
jaundice. Sometimes in the long bone, white It occurs due to lead poisoning.
bands of increase density appear in the ends of
the diaphyses immediately adjacent to the Causes
epiphyseal lines. This is called as ‘bismuth line’. It is caused by lead in the paints, glazes, cooking
vessels, batteries, ointment and containers.
Oral Manifestations ‘Moonshine’ an illicit alcoholic beverage distilled
Patients often complain of a metallic taste with in car radiators has been shown to cause acute
lead poisoning. Use of tetraethyl lead, an
burning sensation in the oral cavity and
antiknock compound in gasoline, has introduced
annoying gingivostomatitis with symptoms
similar to ANUG. Large, extremely painful, a new source of lead.
Excessive absorption of the lead from
shallow ulcerations are seen at times on the cheek
automobile exhaust and dust and dirt derived from
mucosa in molar region. Regional
lymphadenopathy may be present. Tongue is house paint is known to affect a large number of
poor children living in urban areas.
frequently enlarged and sore. Blue black’
Acute exposure can occur in foundries,
bismuth line appears to be well demarcated to
eye on gingival papillae. Blue black bismuth smelters, battery plants, munitions and garages.
sulfide granules formed by action of H 2 S Mechanism of Action
produced by action of bacteria on organic
material remaining in areas of poor oral hygiene. Absorption of lead from alimentary tract, lungs
and gut → modulated by vitamin D and calcium
Histopathological Features status of the individual → lead is taken up by
circulating erythrocytes and bound to reactive
The granules of sulfide are seen in the tissue sulfhydryl group of proteins → from the
section as small irregular black collection of circulation, lead is transferred to all the soft tissues
pigment, sometimes perivascular in location. The and in high concentration it will inhibit metabolic
material may be present in endothelial cells or pathways → in the red cells, lead inhibits
in mononuclear phagocytes in the tissue, but enzymes associated with hemoglobin synthesis
usually in intercellular tissue. → hence abnormal activity of the enzymes occur.

Diagnosis Clinical Features

An ulcerative gingivostomatitis accompanied by Nervous system—Lead has high affinity for cells
discrete blue black pigmentation of interdental in central as well as peripheral systems. In acute
380 Textbook of Oral Pathology

poisoning, demyelination and axon degeneration Neurological value—Abnormal neurological value

occurs. Lead encephalopathy, cerebral palsy, for the both peripheral and central nervous
mental retardation, seizures, wrist or foot drop system.
and fatigue can occur.
Management
Gastrointestinal tract—There may be serious
gastrointestinal disturbances like nausea, Lead can be removed from body by using a
constipation, vomiting, and colic. chelating agent such as calcium edetate (EDTA)
or penicillamine.
Bone—When incorporated in the bone, it can
interfere with cellular metabolism and changes Mercurialism
the rate of bone resorption and apposition.
It may be chronic or acute. It is also called ‘pink
disease’, ‘swift’s disease’, ‘dermato-polyneuritis’,
Oral Manifestations
and ‘acrodynia’. It is an uncommon disease
Oral tissues are exposed to lead through direct caused due to a mercurial toxicity reaction, either
contact with ingested lead and through secretion actual mercury poisoning or, more likely, an
of lead in the saliva. There is a metallic taste idiosyncrasy to the metal. Exposure of young
which is accompanied by excessive salivation children to minute amounts of mercury is
and dysphagia. When exposure to lead is very responsible for a condition. The exact etiology
high and oral hygiene is very poor, a line known of it is unknown but it is thought that it may
as ‘burtonian line’ is seen which is gray black in occur due to mercury toxicity.
color and is present along the gingival margin.
Lead line is more diffuse than bismuth line. Causes
There is pallor of lip; poor muscle tone and the As a result of occupational contact, drug
face appears ashen in color because of associated
overdose, suicide attempt or self-medication
anemia. There is bilateral parotid gland
with mercurial compounds. Mercury hazards
hypertrophy. exist in paints containing mercurial salts such as
phenylmercuric propionate. Prolonged admini-
Laboratory Finding stration of mercurial diuretics can also result in
Blood changes—These are those of hemolytic mercurialism. Frequent use of night cream
anemia with basophilic stippling of the RBCs. containing inorganic salts may produce
distinctive discoloration. Mercurial fumes which
Lead level—In children, the level of exposure can are produced industrially also result in bone
be gained by measurement of tooth lead level changes in the jaws if inhaled in quantity.
and in adults the hair lead concentration is Improper use of dental amalgam alloy can also
commonly used as an indicator. cause mercurialism.
Enzymes level—Measurement of aminolevulinic
Clinical Features
acid and aminolevulinic acid dehydratase and
synthetase level in blood are decreased in lead It occurs most frequently in young infants before
poisoning as these enzymes are associated with the age of 2 years although children can
hemoglobin synthesis and lead inhibits occasionally affected up to age of five years or
hemoglobin synthesis. six years.
 Chemical and Physical Injuries 381

Gastrointestinal symptoms—Intestinal colic and Sound teeth may exfoliate and one or more
diarrhea. There is also pharyngitis, dysphagia, teeth may be found on bed in the morning as
nausea, abdominal pain. patients awake. The reason for it is that there is
Nervous symptoms—Long continued exposure to marked periostitis with loosening of the teeth
mercury vapor can result in permanent which may lead to exfoliation of teeth. Many
neurological changes. Headache, insomnia, times children extract their own teeth with the
tremors of fingers and tongue and mental help of their finger. There may be loosening and
depression. premature shedding of teeth often occurs. The
loss of teeth sometimes followed by necrosis of
Renal symptoms—Severe intoxication and it can
bone and there may be sequestrum formation.
be the cause of death. Bruxism is a common finding.
Hands, feet, nose and cheeks assume pink
color. The nails are shed at the same time with Management
teeth lost prematurely and alopecia is also Bed rest and suitable dietary regimen should be
present. The children will frequently tear their adjusted for renal damage. Atropine or
hair out in patches. The skin over the affected belladonna can be prescribed to lessen the
area peels frequently during the course of the salivary flow. Due discontinuation of possible
disease. The patients also have maculopapular exposure to mercury and administration of BAL
rash which is extremely pruritic. (British anti-lewisite) and dimercaprol has been
Raw beef appearance—The skin of hands, feet, proven successful in most cases unless the
nose, ears and cheek becomes clammy red or disease is of long duration.
pink and has a cold clammy feeling. The
Argyria
appearance is described as resembling raw beef.
Severe sweating, extreme irritability, photo- It is also called as ‘argyrosis’ which occurs due
phobia with lacrimation, insomnia, muscular to chronic exposure to silver compound.
weakness, tachycardia and hypertension.
Causes
Oral Manifestations It is cause from local and systemic absorption of
There is marked increase in inflow of ropy viscid silver compounds. It may result from the use of
saliva, hot mouth, itching sensation and metallic silver containing nasal drops or sprays or silver-
taste are experience. Patient will exhibit profuse arsphenamine injection used to treat syphilis.
salivation and often much ‘dribbling’. Mastica- Chewing pieces of photographic films over an
tion is difficult due to pain. The gingiva becomes extended period can also result in argyria.
extremely sensitive or painful and it may exhibit Localized argyria can develop following long
ulceration. continued use of silver preparation.
Oral mucosal ulceration occurs and spreads
to the palate, throat and pharynx. Salivary glands Clinical Features
and lymph nodes may be swollen and tongue is The exposed body surfaces including the nail beds
enlarged, painful and ulcerated. Tongue trem- are deeply discolored. Skin is slate gray, violet or
ors may be present. Faint diffuse grayish pig- cyanotic and in marked cases, there is even
mentation of alveolar mucosa. The gums are of suggestion of metallic luster.
a deeper hue than normal. Lips are dry, cracked Pigmentation is distributed diffusely
and swollen. throughout the gingival and mucosal tissue.
382 Textbook of Oral Pathology

Management Management
Source of contact should be eliminated. Discontinuation of gold therapy and alkaline
mouth washes.
Arsenism
It occurs due to arsenic poisoning. Fibrous Hyperplasia
It is also called as ‘inflammatory fibrous
Causes hyperplasia’, ‘denture injury tumor’ and ‘epulis
Industrial exposure or intentional use or due to fissuratum’.
therapeutic consumption.
Causes
Clinical Features
Ill fitting dentures are the most common reaction
Chronic gastritis and colitis, keratosis of palms to a chronically ill-fitting denture. It occurs due
of the hands and soles of feet. Dermatitis,
to overextended denture flanges. Other factors
pigmentation or ulceration of the skin.
which are responsible are ragged margins of
teeth, overhanging restorations, sharp spicules
Oral Manifestations
of bone, badly fitting clasps and chronic biting
Oral tissues are extremely painful, become of cheek and lips.
intensely inflamed and severe gingivitis may
develop. The mouth is dry. Tissues are deep red Clinical Features
in color. Local contact with arsenic trioxide often
There is development of elongated rolls of tissue
produces ulceration.
in the mucolabial or mucobuccal fold area, into
Management which the denture flanges conveniently fit. The
proliferation of tissue is usually slow.
Surface anesthetic ointment or rinses such as
There may be small nodular or polypoid
lidocaine or dyclonine solution. overgrowth of fibrous tissue due to gingival
irritation. When the lesions occur in buccal sulcus
Auric Stomatitis
due to denture flanges, it is called as epulis
Causes fissuratum. In it, there is concomitant
Gold is useful for the treatment of Rh arthritis, overgrowth of surrounding fibrous tissues with
a groove in it.
lupus erythematous and leprosy.
The excess folds of tissue are not usually
Clinical Features inflamed clinically, although there may be
irritation or even ulceration in the base of the
Dermatitis is the most common complaint. fold, into which the denture flange fits. The lesion
Purpura and malignant neutropenia can also is firm on palpation.
occur. Faint blue or purple discoloration.
Histopathological Features
Oral Manifestation
The hyperplastic mass of tissue is composed of
It is the most common complaint of the patient an excessive bulk of fibrous connective tissue
who is receiving gold therapy. Vesiculations and covered by a layer of stratified squamous
ulcerations of the oral mucosa. epithelium, which may be of normal thickness
 Chemical and Physical Injuries 383

or show acanthosis. Pseudoepitheliomatous base. In rare cases, cheek may be involved. Whole
hyperplasia, hyperorthokeratosis or parakera- palatal mucosa under the denture may be
tosis are often found. covered with numerous small polypoid masses.
The connective tissue is composed chiefly of The lesion presents as numerous closely
coarse bundles of collagen fibers with few arranged, red, edematous papillary projections
fibroblasts or blood vessels, unless there is an often involving nearly all of the hard palate and
active inflammatory reaction. There is moderate imparting to it a ‘warty appearance’. In some
degree of chronic inflammatory infiltration in the cases, it is swollen and papillary projection
subepithelial connective tissue. There is also resembles the surface of ‘overripe berry’. In some
mucopolysaccharide keratin dystrophy, also cases, it produces a ‘cobblestone’ appearance.
referred as plasma pooling. Lesions are friable, often bleed with minimum
trauma and may be covered with thin whitish
Management exudate. The tissue exhibits varying degrees of
inflammation, but seldom there is ulceration.
It should be treated with excisional biopsy.
Elimination of irritation should be done. These are seldom over 0.3 cm in diameter. The
lesion may extend onto the alveolar mucosa. The
Inflammatory Papillary Hyperplasia individual papillae are seldom over a millimeter
or two in diameter. When complicated by
It is also called as ‘palatal papillomatosis’ and Candida albicans lesion appears as red to scarlet,
‘palatal epithelial hyperplasia’. It occurs in 3 to soft and bleeds easily in the inflammatory or
4% of dentures wearers. granulomatous stage.

Causes Histopathological Features

Frictional irritation produced by loose fitting It has got exophytic nature. There is varying
dentures on palatal tissues. It occurs in patients degree of branching and polypoid proliferation
who sleep with their dentures on. Full dentures on the epithelial surface. It shows numerous
in which relief areas or suction chambers are cut small vertical projections, each composed of
in palatal seating surface appear to be the parakeratotic or sometimes orthokeratotic
strongest stimuli for the lesion. It is much stratified squamous epithelium and a central
common in acrylic dentures than in those with core of connective tissue. Pseudoepitheliomatous
metallic dentures. It is more common in patients hyperplasia is seen.
with poor oral hygiene. In some cases, irritation
due to amalgam filling may produce these Management
lesions.
Remove the denture at night to provide rest to
the tissue. Conditioning liner should be applied.
Clinical Features
In cases of fibrosis, surgical removal and
It can arise at any age in adults and has no curettage, electrosurgery and cryosurgery
definite sex predilection. It occurs exclusively on should be done. In case with superimposed
palate beneath the complete or partial denture. candidal infection, topical application of
It occurs predominately in edentulous patients antifungal agents should be used. Most
and the site of lesion corresponds to the denture commonly used is nystatin ointment.
384 Textbook of Oral Pathology

Epulis Granulomatosum results in a loose textured feathery appearance.

There is frequently patchy chronic inflammatory
It is reactive hyperplasia that develops within a
infiltration, consisting of lymphocytes and
tooth socket after the extraction or exfoliation of
occasional plasma cells and macrophages.
tooth. It is caused by sharp spicules of bone in
Fibroblasts tend to be larger than their normal
socket.
size. The nuclei are plump with prominent
nucleoli.
Clinical Features
It can occur at any age and becomes apparent Management
within 2 weeks after the loss of a tooth. It is
Local excision will yield excellent result.
exuberant dark red granulation tissue extruding
from a tooth socket. It is painless growth. The
Uremic Stomatitis
enlargement is soft, hemorrhagic with an
erythematous to white, smooth surface. Nonkeratotic white lesions caused due to
elevated creatinine or blood urea nitrogen. It has
Management been suggested to be consequence of strongly
It is done to remove the granulation tissue and alkaline saliva due to ammonia formation from
smoothing the socket borders is indicated. It retained urea secreted in the saliva. It is common
should be done. in young and middle aged adults with no sex
predilection.
Nodular Fascitis There is extensive pseudomembranous white
lesion. There is ammonical odor to breath. In
It is also called as ‘pseudosarcomatous fasciitis’.
some cases, there is appearance of ulcer occurs.
It is a proliferative fibroblastic lesion and
presents as a tumor like mass with infiltrative Oral ulceration varies in size and is irregular in
shape and usually shallow.
properties. It appears to be an inflammatory
reactive phenomenon. Microscopic features—The epithelium is lacking,
have been replaced by an eosinophilic coagulum
Clinical Features with an adjacent inflammatory cell infiltrates.
Half of the cases are reported on upper limb. Oral ulcer, if painful may be treated by
Intraorally, the affected sites are subcutaneous prescribing a palliative oral rinse such as an
tissues overlying the mandible, zygoma, parotid antihistamine oral suspension with Kaopectate.
sheath and oral mucosa. It can occur at any age,
but 3rd to 5th decades are common. It appears Traumatic Keratosis
as a small lump, which may be painful. The It refer to isolated area of thickened whitish oral
lesion often enlarges rapidly, but only to mucosa that is clearly related to identifiable local
maximum size of 4 cm, where it remains irritant and resolves following elimination of
stationary or regresses. irritant.

Histopathological Features Etiology

There is proliferation of fibroblast in a rather Local irritants like ill fitting denture, sharp clasp
haphazard manner in a vascular myxoid matrix and rough edges of restoration. Heavy cigarettes
which is rich in acid mucopolysaccharide. It smoking.

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 Chemical and Physical Injuries 385

Clinical Features hard palate. It is restricted to the area which is

exposed to heavy cigarette smoke.
Most common sites are lip and buccal mucosa.
In the early stages, mucosa is reddened. It
There is isolated thickened whitish area.
subsequently become grayish white, thickened
Glassblower’s white patch – it is variant of
and fissured. Fissures and cracks may appear
traumatic keratosis affecting the cheek and lips,
producing a wrinkled, irregular surface. In some
which occur in glass factory.
cases there may be papular or multinodular
appearance. Papules do not coalesce and are
Histopathological Features
separated from one another by intervening
There is varying degree of hyperkeratosis, normal appearing mucosa. Tonsillar pillars are
parakeratosis and acanthosis. usually erythematous. Discoloration is
homogenous with the exceptions of numerous
Management erythematous spot. It represent focal thickening
Upon removal of the offending agent, the lesion surrounding the orifice of the salivary gland
should resolve within 2 weeks. Biopsies should which appears as white umblicated nodule with
be performed on lesions that do not heal to rule red center that may be stain brown by deposit of
out a dysplastic lesion. tar. There is discrete elevated striae with an
appearance somewhat similar to pumice.
Stomatitis Nicotina
Histopathological Features
It is also called as ‘smoker’s palate’ ‘stomatitis Epithelium shows acanthosis and hyper-
nicotina palati’. keratosis. Epithelium lining of minor salivary
It refers to a specific white lesion that gland often shows squamous cell metaplasia and
develops on the hard and soft palate in heavy hyperplasia. Obstruction of duct may lead to
cigarette, pipe, and cigar smokers. formation of retention cyst. Periductal
inflammatory cell infiltrate composed of
Etiology lymphocytes and plasma cells accompanied
Heavy pipe, cigarette, cigar smokers and reverse capillary proliferation and dilation in zone.
smoking. It is also common in individual with Management
long history of drinking extremely hot beverages.
It is completely reversible once the habit is
discontinued. The lesions usually resolve within
Clinical Features
2 weeks of cessation of smoking. Biopsy of
It is usually seen in men who are pipe smokers. nicotine stomatitis is rarely indicated. But biopsy
It is common in middle age and elderly adults. should be performed on any white lesion of the
Most commonly affected site is palate. The lesion palatal mucosa that persists after one month of
is well developed and prominent on keratinized discontinuation of smoking habit.

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386 Textbook of Oral Pathology

CHAPTER Allergic and

18 Immunological
Diseases of Oral Cavity

Ulceration of the oral mucous membrane is a • Raised and pearly white, beaded edge—It is a
common occurrence in patients and may challenge feature of rodent ulcer. This type of edge
the diagnostic acumen of the clinician. When develops in invasive cellular disease and
observed in mouth, all these lesions have a similar becomes necrotic in the center.
appearance. Ulcer is a Greek word meaning • Rolled out (everted) edge—It is a characteristic
‘wound’ or ‘sore’. It has focus of necrotic tissue on feature of malignant ulcer. This ulcer is caused
the surface and a destroyed overlying epithelium. by fast growing cellular disease, the growing
An ulcer consists of margins, edges, floor and portion at the edge of the ulcer heaps up and
base. A margin is the junction between normal spills over the normal skin to produce an
epithelium and the ulcer; so it is the boundary of everted edge.
the ulcer. An edge is the junction between a margin • Floor is the exposed surface of the ulcer. The
and the floor of the ulcer. Activity is maximum at floor is composed of connective tissue fibrin
the margins and edges of the ulcer. with polymorphonuclear leukocyte
Five common types of ulcer edges are seen. infiltration. One must understand the
• Undermined edge—It is mostly seen in difference between the floor (exposed surface
tuberculosis ulcer. The disease causing the within the ulcer) and the base (on which the
ulcer spreads in and destroys the ulcer rest) which is better felt than seen.
subcutaneous tissue faster than it destroys the
skin. The overhanging skin is friable reddish Microscopic Changes Occurring in Ulcer
blue and unhealthy. There is complete thickness of surface epithelium
• Punched out edge—It is mostly seen in is missing and the exposed connective tissue is
gummatous ulcer or in deep tropic ulcer. The necrotic on surface and covered by fibromatous
edges drop down at right angle to the skin exudate. Ulcer shows acute inflammation with
surface. polymorphonuclear leukocytes in the connective
• Sloping edge—Sloping edge is seen mostly in tissue of its borders. Less acute phase shows
healing, traumatic or venous ulcer which is greater concentration of chronic inflammatory
reddish purple in color and consists of new cells such as lymphocytes, plasma cell and
healthy epithelium. macrophages. In the healing phase of the ulcer,

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 Allergic and Immunological Diseases of Oral Cavity 387

granulation tissue with fibroblastic proliferation acute signs may be relived by administration of
is predominant and few macrophages, plasma anti-histaminic drugs or cortisone.
cells and lymphocytes are seen.
Contact Allergy
Drug Allergy
It is caused by delayed type of hypersensitivity
It is also called as ‘drug idiosyncrasy’, ‘drug reaction to topical antigen. On the skin, it is
sensitivity’ and ‘stomatitis or dermatitis referred as ‘dermatitis venenata’ and oral lesions
medicamentosa’. They may cause acute multiple are referred as ‘stomatitis venenata’.
ulcers and vesicles of oral mucosa or lichenoid
reaction. It includes a variety of sensitive Causes
reactions. Some patients have greater It is caused by poison ivy, leather, rubber, nickel,
susceptibility to drugs and manifest reactions medication or other chemicals. Contact allergy to
more readily than others. Drugs which can most dental amalgam is caused by mercury, which is
commonly cause drug reactions are aminopyrine, released during condensation.
barbiturates, gold, bromide, penicillin, strep- Dental and cosmetic preparation like
tomycin, etc. dentifrices, mouthwashes, denture powder, lip
stick, cough drop and chewing gums.
Clinical Features
Dental materials like vulcanite, acrylic, metal
It is characterized by inflammation, ulceration alloy base.
and vesicle formation with arthralgia, fever and Dental therapeutic agents like alcohol,
lymphadenopathy. The skin lesion is often of antibiotics, chloroform, iodide, phenol, procaine
erythematous type, as in erythema multiforme or and volatile oils.
they may be urticarial in nature.
Fixed drug reactions may occur in those who Clinical Features
are administrated on repeated occasions, a drug There are typically an itching erythematous areas
to which they are sensitive. It consists of with superficial vesicle formation, directly at the
appearance of same reaction at the same site each site where allergen contacts skin. The skin may
time. become thickened and dry. After the rupture of
vesicle, erosion may become extensive and if
Oral Manifestations
secondary infection occurs, the lesion may be
The oral lesions are diffuse in distribution and serious. Burning is a commoner complaint rather
vary in appearance from multiple areas of than itching of skin. Localized area of erythema,
erythema to extensive areas of erosion or edema and vesiculation in specific areas of skin
ulceration. In the early stages of reaction, vesicle or mucosa whenever specific allergen is
or even bullae may be found on the mucosa. administered.
Occasionally purpuric spots appear and
angioneurotic edema is seen. Ulceration and Oral Manifestations
necrosis of gingiva often resemble ANUG. Oral lesions are rare due to number of Langerhans
cells, saliva which dilutes the allergens and
Management
washes them from the surface of the mucosa and
The signs and symptoms of drug allergy regress digest with enzymes and a thin layer of keratin
with discontinuing of the causative drug. The present on the oral mucosa.

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Allergy to cinnamon oil or formalin present Angioneurotic Edema

in tooth paste appears clinically as swelling,
It is also called as ‘angioedema’, ‘Quincke’s
cracking and fissuring of lips, perioral
edema’, ‘and ‘giant urticaria’. It is common form
desquamation and edema, angular cheilitis,
of edema occuring in both hereditary and non-
swelling of gingiva and oral ulcerations.
hereditary forms. It is one form of acute
Plasma cell gingivitis—Another oral manifestation anaphylactic reaction representing response
is plasma cell gingivitis, which is characterized allied to urticaria, allergic rhinitis and asthma.
by erythematous edematous attached gingiva
accompanied by chelitis and glossitis. Mechanism
The mechanism of development of swelling is due
Diagnosis to vasodilation brought about by the release of
histamine like substances with subsequent
Patch test—Suspected allergen is placed on normal
non-hairy skin, i.e. on upper portion of back. It transudation of plasma.
remains in contact with skin for 48 hours. Then
Causes
the patch is removed and after 2 to 4 hours, the
area is examined for persistent erythema. It appears closely related to general urticaria. It
occurs most commonly due to food allergy. In some
Management cases, it is thought that some drugs, endocrine
disturbances or focal infection play an important
Removal of allergen. Application of topical etiologic role.
corticosteroids.
Clinical Features
Secondary Vaccinia
It affects both sexes equally, but it is infrequent in
It is also called as ‘vaccinia autonoculata’. children while some cases originate at puberty.
Undesired skin or mucosal lesion, after smallpox Most often, the face and lips are involved, but
vaccination, is caused by transfer of the contents sometimes the tongue also becomes swollen.
of vaccination pustule to other parts of the body. Edema may develop gradually in a matter of hours,
It is followed by formation of secondary lesions but can also progress in minutes. It typically
usually with weaker reaction than the one seen manifests as a smooth, diffuse edematous swelling,
in case of primary inoculation. particularly involving the face, around the lips,
Secondary vaccinia may develop at the site of chin and eyes, the tongue and sometimes, the hands
scratching possibly in already existing epithelial and feet.
defect. Eyes, ears and areas of lips and tongue are Parotid gland may be affected in some cases.
possible sites. In this area, a patch develops that The eyes may be swollen, shut and lips may be
becomes vesicular than pustular. After the extremely puffy. Symptoms may appear
development of crust, there is repair with scar suddenly sometimes may present in morning. A
formation. Other ulcerative lesions such as feeling of tenderness or an itching or prickly
primary lesion of syphilis, tuberculosis, aphthae sensation sometimes precedes the urticarial
of the major type should be differentiated. The swelling.
history is important as secondary vaccinia appear The skin is of normal color and/or slightly
about 5 to 7 days after inoculation. pink. The condition usually last for 24 to 36 hours

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 Allergic and Immunological Diseases of Oral Cavity 389

although some cases persist for several days. The Iron deficiency or folic acid deficiency—Small
hereditary forms are more dangerous because percentage of patients with recurrent aphthae
there is visceral involvement. Vomiting and have certain nutritional deficiency. Presence of a
abdominal pain may occur and especially, deficiency allows the expression of an unrelated,
dangerous edema of glottis can result in death underlying tendency to ulceration.
through suffocation. Hereditary—Increased susceptibility to RAS is
seen among the children of RAS positive parents.
Management
Specific HLA antigen has been identified in RAS
When etiological agent such as food can be patients. There is familial tendency for the
discovered, its elimination from diet will prevent occurrence of the disease.
recurrent attacks. Antihistaminic drugs (50 to 75
Hematological deficiency, serum iron or vitamin B12
mg diphenylhydramine hydrochloride) can give
deficiency, secondary malabsorption syndrome
prompt relief.
such as celiac disease.
Aphthous Stomatitis
Precipitating Factors
It is a common disease characterized by
Trauma—Local trauma including self-inflicted
development of painful, recurrent, solitary or
bites, oral surgical procedures, tooth brushing,
multiple ulcerations of the oral mucosa, with no
needle injections and dental trauma.
other signs of any other disease.
Endocrine conditions—There is some relation
Classification between occurrence of apthous ulcer and
• Minor aphthae—It is also called as ‘canker pregnancy, menstruation and menopause. There
sores’ in which the ulcers are less than 1 cm is remission of ulcers during pregnancy.
in diameter and heal without scar. Incidences of aphthae are greatest during
• Major aphthae—It is called as ‘Sutton’s menstruation. Ulcerations are maximum during
disease’ or ‘peri-adenitis mucosa necrotica postovulation period.
recurrent’ and the ulcers are over 1 cm in Psychic factors—Acute psychological problems
diameter and heal with scarring. appear many times, to precipitate the attacks of the
• Herpetiform ulcers—Recurrent crops of dozens disease. Anxiety can also precipitate the attack.
of small ulcers throughout the oral mucosa. Cessation of smoking increases the frequency
• Recurrent ulcers associated with Behcet’s and severity of RAS.
syndrome.
Allergic factor—Patients may have a history of
Etiology asthma, hay fever and food or drug allergy.

Bacterial infection—A pleomorphic transitional L- Clinical Features

form of α-hemolytic Streptococcus and
It usually occurs between second and third
Streptococcus sanguis has been implicated as the
decades of life. It is common in women than men.
causative agent of the disease.
It may be precipitated by minor trauma,
Immunological abnormalities—IgG and IgM menstruation, URTI or contact with certain foods.
binding of the epithelial cells of the spinous layer It occurs most commonly on buccal and labial
of oral mucosa is seen in patients suffering from mucosa, buccal and lingual sulci, tongue, soft
recurrent aphthous ulcer. palate, pharynx and gingiva.

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390 Textbook of Oral Pathology

It begins with prodormal burning for 24 to 48 erosions that gradually enlarge and coalesce.
hours, before the ulcer appears. It begins as a Lesions are more painful than would be
single or multiple superficial erosion covered by suspected by their size. Present continuously for
gray membrane. Localized areas of erythema one to three years, with relatively short remission.
develop and within hours small white papules Patient gets relief immediately with 2%
form, ulcerate and gradually enlarge over next 48 tetracycline mouthwash.
to 72 hours.
Lesions are round, symmetric and shallow but Histopathological Features
no tissue tags are present from the ruptured
Fibrinopurulent membrane covers the ulcerated
vesicles. The lesion is typically very painful so, it
area. Occasional superficial colonies of
commonly interferes with eating for several days.
microorganisms may be present in this membrane.
Multiple lesions are present but the number and
size are frequently varied. Most patients have Intense inflammatory cell infiltrate is present in
between 2 to 6 lesions at each episode and connective tissue, beneath the ulcer, with
experience several episodes a year. The ulcers considerable necrosis of tissue near the surface of
themselves generally persist for 7 to 14 days the lesion.
(Fig. 18.1). Neutrophils are predominant immediately
below the ulcer but lymphocyte prevailing
Minor aphthae—Size is 0.3 to 1 cm, heal without adjacent to this. Epithelial proliferation along the
scarring, within 10 to 14 days. margins of the lesion.
Major aphthae—Develop deep lesions, larger than Anitschkow cells—Consist of cells with elongated
1 cm and may reach upto 5 cm in diameter. They nuclei, containing a linear bar of chromatin with
interfere with speech and eating. Large portions radiating processes of chromatin extending
may be covered with deep painful ulcers. The towards the nuclear membrane.
lesions heal slowly and leave scars, which result The epithelium at margin of the lesion
in decreased mobility of uvula and tongue and demonstrates spongiosis and numerous
destruction of portions of oral mucosa. mononuclear cells in the basilar one third. A band
Herpetiform ulcers—Multiple small shallow ulcers of lymphocytes intermixed with histiocytes is also
often up to 100 in number. Found on any intraoral seen in superficial connective tissue.
mucosal surface. Begin as small pinhead size
Management
Mild cases—Topical protective emollient base
(Orabase). Topical tetracycline mouth wash (250
mg per ml) use four times daily for 5 to 7 days
produces good response in nearly 70% of the
patients. Topical corticosteroid preparation
topical corticosteroid triamcinolone acetonide 3
to 4 times daily.
Severe cases—Fluocinolone gel, clobetasol cream
or beclomethasone spray. Injection of
Figure 18.1: Aphthous ulcer presented as well defined
corticosteroid directly in lesion in, combination
lesion of systemic administration of cortisone.

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 Allergic and Immunological Diseases of Oral Cavity 391

Chlortetracycline as mouth rinse to be flushed over Eye lesions—Consist of uvetitis, retinal internal
the affected region, for at least 2 minutes provides edema and vascular occultation, optic atrophy,
relief from pain. In some cases, dapsone or conjunctivitis and keratitis.
thalidomide can be used. Interferon alpha,
Skin lesions are generally small pustules or papules
nicotine tablets and colchicine can be used, but it
on the trunk or limbs and around the genital.
is under investigation.
Pathergy test—Cutaneous hypertrophy to
Behcet’s Syndrome intracutaneous injection or needle sticks
(pathergy) with the finding of pustule forming 24
It is a disease of uncertain etiology that may
hours after needle puncture.
resemble an infectious origin. It is triad of
recurring oral ulcers, recurring genital ulcers and Arthritis—Arthritis is common. Affected joint is
eye lesions. red and swollen.
CNS involvement—It may occur and it may include
Classification
brain stem involvement of cranial nerve and
• Mucocutaneous—Oral, genital and skin lesions. neurologic degeneration.
• Arthritic—Arthritis, in addition to
Others—Thrombophlebitis, intestinal ulceration,
mucocutaneous lesions.
venous thrombosis, renal and pulmonary disease.
• Neuro-ocular—Neurologic, ocular and
mucocutaneous lesions.
Diagnostic Criteria
Etiology • Recurrent oral ulcerations at least 3 times in
It is caused by immune complexes that lead to one 12 months period, plus at least two of the
vasculitis of small and medium sized blood ves- following four manifestations.
sels. There may be inflammation of the epithe- • Recurrent genital lesions.
lium caused by immunocompetent T- lymphocytes • Eye lesions including uveitis or retinal
and plasma cells. vasculitis.
• Skin lesions including erythema nodosum,
Clinical Features pseudo-folliculitis and papulopustular lesions.
• Positive pathergy test.
It begins between 10 to 45 years of age, with a
mean age of occurrence of 30 years. It is five to ten Laboratory Findings
times more common in males.
Hypergammaglobulinemia, leukocytosis with
Recurring oral ulcers—It may be mild or may be eosino-philic and elevated ESR.
deep, large scarring lesions and may appear
anywhere on the oral and pharyngeal mucosa. Histopathological Features
They are painful lesions. They may range from
It has got similar appearance as that of aphthous
several millimeters to a centimeter in diameter.
stomatitis. Endothelial proliferation with
These ulcers have erythematous borders and are
vasculitis is seen which is called as
covered by gray or yellow exudate.
leukocytoclastic vasculitis. Small blood vessels
Genital lesions—It includes ulcers of scrotum and demonstrate intramural invasion by neutrophils,
penis in males and ulcers of labia in females. The extravasation of red blood cells and fibronoid
genital ulcers are small and painful in females. necrosis of the vessels wall.

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392 Textbook of Oral Pathology

Management Conjunctivitis—Conjunctivitis is often so mild as

Patients with life threatening or slight threatening to be overlooked.
vasculitis are managed with combination of Skin lesion—The skin lesions consist of red or
immuno-suppressive drugs and systemic yellow keratotic macules which eventually
corticosteroids. desquamate.

Reiter’s Syndrome
Oral Manifestations
It is a disease of unknown etiology and is considered
Oral lesions occur in less than 5% to about 50%of
as an important complication of non-gonococcal
the patients with the disease. It is seen on the
urethritis and is often acquired sexually.
buccal mucosa, lips and gingiva. The lesions
It consists of tetrad of:
appear as painless, red, slightly elevated areas,
• Urethritis
sometimes granular or vesicular with a white
• Arthritis
circinate border.
• Conjunctivitis
The palatal lesions appear as small, bright
• Mucocutaneous lesions.
red, purpuric spots which darken and coalesce.
Etiology Lesions on the tongue closely resemble
geographic tongue. They may be mistaken as
It is may be due to pleuropneumonia like organism.
recurrent apthous ulcers.
Variety of infectious agents like bedsonia,
mycoplasma, chlymadia, virus, etc. It can be
Laboratory Findings
associated with staphylococci and in that case, it
is called as staphylococcal scalded skin syndrome. Leukocytosis. The findings show a mild
leukocytosis and an elevated ESR.
Clinical Features
It is totally confined to men, usually between the Histopathological Features
ages of 20 to 30 years. The disease begins abruptly Consist of parakeratosis, acanthosis and
with diffuse erythema and fever. Large flaccid polymor-phonuclear leukocyte infiltration of
bullae are formed which contain a clear yellowish epithelium, sometimes with microabscess
fluid. The bullae rupture very easily leaving large formation. Connective tissue shows lymphocytes
areas of skin devoid of superficial epidermis. The and plasma cell infiltration.
urethral discharge is usually associated with an
itching and burning sensation. Management
Arthritis—Arthritis is often bilateral, symmetrical Spontaneous remission. It is treated by antibiotics
and usually polyarticular. and corticosteroids.

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 CHAPTER
Granulomatous Diseases
19 and Collagen Disorders

GRANULOMATOUS LESIONS Glomerulonephritis, which develops

ultimately to uremia and terminal renal failure.
Wegener’s Granulomatosis
It is a disease of unknown etiology which basically Oral Manifestations
involves the vascular, renal and respiratory
The disease usually starts with tumor like
systems. It is a granulomatous involvement of blood
vegetations in mouth and nose. Inflammatory
vessels resulting in necrosis of tissue. It is generally
process starts in the interdental papilla, spreading
thought that the disease is aberrant hyper-
rapidly into the periodontium. The lesion
sensitivity reaction to an unknown antigen.
undergoes necrosis with formation of large
Types perforating ulceration.
Involvement of gingiva is the most common
• Generalized—It involves super respiratory
manifestation, which is characterized by
tract, pulmonary and renal lesions.
• Localized—It affects oral and nasal cavity and ulceration, friable granular lesions or simple
the lungs. enlargement of gingiva. Inflamed, hyperplastic
appearing and hemorrhagic gingiva may be
Clinical Features found. Strawberry gingivitis is the characteristic
It usually occurs in 4th or 5th decade of life, with feature of it.
slight predilection for males. The most common Oral lesions typically include ulceration of the
symptom of Wegener’s granulomatosis is nasal palate by extension of nose lesions and
stuffiness with chronic discharge, which is destruction of nasal septum, poorly healing
sometimes bloody. Patient soon develops cough, extraction sites or oroantral fistulae. There may
hemoptysis, fever and joint pains. There is also be perforation of palate.
presence of rhinitis, sinusitis and otitis or ocular There may be loosening of teeth, spontaneous
symptoms. There are also nonspecific symptoms exfoliation of teeth diffuse ulcerative stomatitis,
of malaise, arthralgia and weight loss. post- extraction poor healing, cranial nerve palsies
Hemorrhagic or vesicular skin lesions are also and parotid swelling. There are often signs of
commonly present. alveolar bone loss.

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394 Textbook of Oral Pathology

Laboratory Findings spleen and bones. Mild malaise, fever, weight loss,
fatigue and cough can be the chief features of the
It includes anemia, leukocytosis, elevated
disease. It presents most frequently with hilar
sedimentation rate and hyperglobulinemia.
lymphadenopathy, pulmonary infiltration and
Hematuria with finding of albumin, casts and
skin and eye lesions.
leukocytes in urine.
Cutaneous lesions appear as multiple, raised
red patches that occur in groups, grow slowly
Histopathological Findings
and does not ulcerate or crust.
It reveals acute and chronic inflammatory cells,
with areas of multinucleated giant cells. Oral Manifestations
Demonstration of neutrophils, cytoplasmic It is rare in oral cavity, but cases are reported on lip,
autoantibodies in serum has very recently showed palate and buccal mucosa. It appears as small
great promise for the immunodiagnosis of this papular nodules or plaques or resembles herpetic
disease. Involved vessels demonstrate lesions or fever blister. On the palate and buccal
transmural inflammation with heavy mucosa, it is described as bleb like, containing a
neutrophilic infiltration, necrosis and nuclear clear yellowish fluid or as solid nodules. There is
dust. The oral epithelium may demonstrate increased incidence of dental caries. Ulceration of
pseudoepitheliomatous hyperplasia and buccal mucosa due to salivary insufficiency. It also
subepithelial abscess. appears to produce diffuse destruction of the bone.

Diagnosis Histopathological Features

It should be suspected on the basis of clinical It resembles proliferative noncaseating nodules

symptom and signs. Definitive diagnosis is made of tuberculosis. Nests of epithelioid cells with
by histological examination. multinucleated giant cells are also present.
Fibrinoid necrosis and hyalinization are
Management occasionally found in the center of granuloma.
The giant cells may contain Schaumann bodies,
Cotrimoxazole, and corticosteroids.
which are concentrically or eccentrically
laminated, calcified bodies (Fig. 19.1).
Sarcoidosis
It is also called as Boeck’s sarcoid, Besnier-Boeck-
Schaumann’s disease. It is a disease of unknown
etiology. It is a multi-system granulomatous
disease. It is characterized by depression of
delayed type of hypersensitivity, suggesting an
impaired cell-mediated immunity and raised or
abnormal serum immunoglobulin, suggesting
lympho-proliferation.

Clinical Features
It commonly affects young adults and shows more
prevalence in blacks. Lesions are most common Figure 19.1: Sarcoidosis showing circumscribed
in lungs, skin, lymph nodes, salivary glands, collection of histiocytes, lymphocytes

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 Granulomatous Diseases and Collagen Disorders 395

Asteroid bodies are delicate spiderlike radiating ulceration spreads from the palate to the inside of
structures that are sometimes seen in the the nose and then to the outside.
macrophages of sarcoid granuloma. The palatal, nasal and malar bones may
become involved, undergo necrosis and
Diagnosis eventually sequestrate. Destruction is a prominent
Kveim-Siltzbach test is an intracutaneous test for feature and loss of entire palate is common. The
the diagnosis of sarcoidosis. ‘Kveim-Siltzbach patient may exhibit purulent discharge from the
test’ is positive in some cases. In this test, eyes and nose; perforating sinus tracts may
intradermal injection of a saline suspension of develop and soft tissues of face may slough away
known human sarcoid tissue used as an antigen leaving a direct opening into the nasopharynx
is given to a patient suspected to have sarcoidosis. and oral cavity.
One month after the injection, any palpable
nodule is excised and examined histologically for Histopathological Features
evidence of a sarcoid reaction, or epithelial
There is extensive necrosis with infiltration of
tubercle.
some inflammatory cells and formation of
Management occasional new capillaries.

Asymptomatic patient requires no treatment but Management

in cases where symptoms are present,
corticosteroids can be given. Corticosteroid therapy has proven beneficial in
some cases.
Midline Lethal Granuloma
Chronic Granulomatous Disease
It is also called as ‘malignant granuloma’,
‘midline lethal granulomatous ulceration’ and It is an uncommon hereditary disease with X-
‘midline non-healing granuloma’. It is described linked mode of transmission. It is characterized
as idiopathic progressive destruction of nose, by severe recurrent infections as a result of defect
palate, face and pharynx. of intracellular leukocytes enzymatic function,
Etiology with decreased oxidative metabolism, in which
there is failure to destroy certain catalase-positive
Lethal granuloma is due to a dysfunction of the
microorganisms, including staphylococci.
immune mechanism normally responsible for
granuloma formation. Nowadays, it represents a
Clinical Features
fulminant hypersensitivity response to an
unidentified antigen. Vascular allergy like arthus Majority of the patient are males, but females are
phenomenon or periarteritis nodosa. also commonly affected. It is commonly found in
children and infants. There is widespread
Clinical Features infection from infancy; usually affecting lymph
It is commonly involved sites are nose, palate, face nodes, lung, liver, spleen, bone and skin. Skin
and pharynx. It begins as superficial ulceration lesions occur on face, leading to tissue necrosis
of the palate or nasal septum, often preceded by a and granuloma formation. Abscess, septicemia,
feeling of stuffiness in the nose. This may persist pneumonia, pericarditis, meningitis and
for a month or two to several years. It eventually osteomyelitis are common features.
396 Textbook of Oral Pathology

Oral Manifestations – Osteogenesis Imperfecta (OI)

– Marfan syndrome (MS)
The lesions are chiefly of diffuse stomatitis, with
– Systemic lupus erythematosis
or without solitary or multiple ulcerations. In some
– Oral submucous fibrosis
cases, benign migratory glossitis is also present.
– Scurvy
– Rheumatoid arthritis
Histopathological Features
– Polyarteris nodosa
It consists of small granulomas with mononuclear – Sjögren syndrome
histiocytes and multinucleated giant cells. Cen- – Dermatomyositis
tral necrosis with polymorphonuclear leukocytes – Behcet’s syndrome
may also be present. – Rheumatic fever.

Ehler-Danlos Syndrome
Diagnosis
• Type I (gravis): Gross hypermobility, skin
It is established by neutrophil function test. hyperextensibility, skin splitting, skin
bruising.
Management • Type II (mitis): Symptoms of type I with a
moderate scale
Vigorous treatment of the infection.
• Type III (benign hypermobile): Gross
hypermobility, variable skin hyperextensi-
COLLAGEN DISORDERS bility, minimal skin splitting and marked
Disorders of Collagen Deposition bruising.
• Type IV (ecchymotic): Minimal hypermobility
• Insufficient collagen content and skin hyperextensibility, moderate skin
– Presence of chemically and/or morpho- splitting, marked bruising.
logically abnormal collagen • Type V (X-linked): Moderate skin hyperexten-
– Excessive collagen content sibility, minimal hyperflexibility, minimal
– insufficient collagen resorption splitting and bruising.
– Excessive collagen resorption. • Clinical manifestations
• Genetic abnormalities of collagen – Joint hypermobility
– Mutation that lead to amino acid deletions – Skin hyperextensibility
or additions – Skin tends to split with minor trauma
– Deficient synthesis of a portion – Nodules
– Disorders in post-translational modifica- – Tendency to bruise.
tion of (hydroxylation of lysine, hydroxy-
lation of proline) Osteogenesis Imperfecta
– Defects in enzymes essential for post- • Tendency of bone and fracture
translational modification. • Type I:
• Collagen is the building blocks; thus, its – Diseases moderate, early onset of fractures,
disorders leads to significant deterioration in hearing impairment.
the mechanical integrity of tissues. • Type II:
• Collagen Diseases – Onset in utero, often stillborn or death soon
– Ehlers-Danlos syndrome (ED) after birth, marked long bone bowing.
 Granulomatous Diseases and Collagen Disorders 397

• Type III: results in difficulty in movements of the affected

– Progressively deforming, multiple fractures, parts. Hyperpigmentation, telangiectasia and
hearing loss, kyphoscoliosis. subcutaneous calcification may occur, leading to
• Type IV: deformity and severe cosmetic problems along
– Variable age of onset and variable deformity with involvement of internal organs. Skin area
• Increased level of reducible crosslinks has thickened hide bound cavity with lack of
• Increase level of type III/type I collagen. mobility of skin, limited mouth opening and renal
involvement.
Scleroderma
Localized Form (Circumscribed or morphea)
It is also called as ‘systemic sclerosis’, or
‘Hidebound disease’. It is a disease which It usually occurs on the sides of the chest and
involves connective tissue, blood vessels and lead thighs. It begins with violaceous patches on the skin.
to fibrosis. It is also called as Progressive systemic These lesions enlarge; become indurated and
sclerosis. eventually loose hair and ability to sweat. They
may be present for several months to many years.
Types Progressively, these lesions turn into hypo or
hyper- pigmented areas depressed below the level
• Diffuse systemic or progressive systemic
of skin. They may become stiff and hard and are
sclerosis
usually asymptomatic.
• Localized form
• Circumscribed or morphea Localized Form (Linear)
• Linear scleroderma.
A linear form of disease develops as a thin band
Etiology of sclerosis that may run the entire length of
extremities involving underlying muscle, bones
It is caused by an endocrine dysfunction, vascular and joints. A band made up of furrow with an
disease, basically an endarteritis obliterans, elevated ridge on one side is often termed as a
nervous disorder, toxic or infectious agents such coup de sabre since it resembles the mark produced
as shock or pneumonia, influenza, diphtheria and by the blow of saber.
exanthematous disease.
Oral Manifestations
Clinical Features
The tongue, soft palate, lips and larynx are
Progressive systemic sclerosis: It generally begins in
commonly involved. These are characterized by
childhood or young adult and greatest incidence
mild edema, which is followed by atrophy and
is between 30 to 50 years of age. Females are more
induration of mucosal and muscular tissue.
commonly affected with a ratio of 3:1. It usually
The lips become thin, rigid and partially fixed,
begins on face, hand or trunk.
producing microstomia and the oral aperture
Symptoms: There is development of indurated narrows considerably. Skin folds are lost around
edema of skin, neuralgia and paresthesia. the mouth.
Signs: Initial sign of PSS is frequently Raynaud’s Tobacco pouch mouth: It can be seen periorally
phenomenon, a paroxysm vasospasm of finger. where furrow rows radiate from the atrophic
In several months the edema is replaced by vermilion borders, creating the so called tobacco
tightening and hardening of the skin, which pouch mouth.
398 Textbook of Oral Pathology

Tongue: Tongue can become hard and rigid, thickening and organ involvement by interference
loosing its mobility and papillary pattern, making with cross linking of collagen and immuno-
speaking and swallowing difficult. The color of suppression.
tongue changes to a livid appearance. In the end
stages, the tongue lays as a stiff, reduced body in Kawasaki Disease
the floor of mouth. The lingual frenum, which It is also called as mucocutaneous lymph node
usually reflects the first oral change, shortens, syndrome. Etiology is unknown but has been
becomes tendinous and finally disappears. suggested to be or collagen or vascular disease.
Involvement of esophagus causes dysphagia.
Involvement of soft tissues around the TMJ Clinical Features
leads to restricted movement of mandible, causing It most commonly occurs in children between 3
a pseudo-ankylosis. When the facial tissues and months and 12 years of age.
muscles of mastication are involved the pressure Symptoms: There is fever for five days or more,
exerted will cause resorption of mandible at the with no response to antibiotics, diarrhea and
attachment of masseter muscle. Gingival arthralgia may be present.
hyperplasia may result from calcium channel
blocker. Signs: There is bilateral congestion of ocular
conjunctiva, indurative edema, erythema of
Histopathological Features palms, soles and membranous desquamation of
fingers and toes. In some cases there is presence
There is thickening and hyalinization of the of polymorphous exanthema of torso without
collagen fibers in the skin, with loss of dermal vesicles or crusts. Acute non-purulent swelling
appendages, particularly the sweat glands. There of cervical lymph nodes occurs.
is atrophy of the epithelium with loss of rete pegs
and increased melanin pigmentation. Oral Manifestations
Subcutaneous fat disappears and the walls of the Changes in lip and mouth including dryness,
blood vessels become sclerotic. In the periodontal redness and fissuring of lips occurs. Strawberry
ligament, there is an increase in collagen and like reddening and swelling of tongue papillae
oxytalan fibers, as well as appearance of and diffuse reddening of oral and pharyngeal
hyalinization or sclerosis of collagen with mucosa, sometimes with gingival ulceration.
diminution in the number of connective tissue cells
is usually found. Laboratory Findings
There is proteinuria, leukocytosis, increased
Management sedimentation rate and positive C-reactive protein.
D-penicillamine, a drug has shown promise in Other collagen disease are discussed in
the management by decreasing both, the skin Chapter Skin.
 CHAPTER

20 Dental Caries

Dental caries is a complex, continuous, dynamic • Miller's acidogenic theory

biological process of tooth decay with • Proteolytic theory
multifactorial etiology. The process of dental • Proteolysis-chelation theory
decay comprises of periods of progression • Sucrose chelation theory
alternating with periods of arrest and repair of • Phosphatase theory
affected dental tissues. • Sulfatase theory.

DEFINITION The Legend of Worm

Dental caries is a microbial disease of the calcified In ancient Sumerian text it is stated that dental
tissues of the teeth, characterized by caries is caused by a worm that drank the blood
demineralization of the inorganic portion and of the teeth and fed on the roots of jaws. This is
destruction of the organic substance of the tooth. known as the legend of worms. It was obtained
from the Mesopotamian area which dates to
THEORIES OF CARIOGENESIS about 5000 BC. This theory is supported in the
ancient literature of India, China, Finland,
Old theories Scotland and the writing of Homer (Fig. 20.1).
• Exogenous theories
– The legend of worm Chemical Theory
– Chemical theory
– Parasitic or septic theory Parmly (1819) proposed that an unidentified
• Endogenous theories chemical agent was responsible for caries. He
– Humoral theory stated that caries began on enamel surface where
– Vital theory. food putrefied and acquired sufficient dissolving
power to produce the disease chemically.
New Theories
These are the current concepts of caries
Parasitic or Septic Theory
pathogenesis. These are based on sound
experimental evidences. Among these Miller's Erdl in 1843 described filamentous parasite in
acidogenic theory is most accepted one. plaque. Ficinus identified filamentous
402 Textbook of Oral Pathology

Miller's Acidogenic/Chemoparasitic Theory

W D Miller proposed this theory in 1890. He based

his theory on observations of following
investigators:
• Pasteur discovered that microorganisms can
transform sugars into lactic acid.
• Emil Magitot demonstrated that fermenta-
tion of sugars can cause dissolution of tooth
mineral in vitro.
• Underwod and Miles stated that caries is
absolutely dependent on the presence of
organisms that create an acid which removes
the lime salt.
• WD Miller's chemoparasitic theory states,
"Dental decay is a chemoparasitic process
Figure 20.1: Legend of worm
consisting of two stages, the decalcification of
microorganism which he termed "Denticolae", enamel, which results in its total destruction
and thought that these are responsible causation and the decalcification of dentin as a
of dental caries. preliminary stage, followed by dissolution of
the softened residue."
Humoral Theory The chemoparasitic theory can be better
described with the role of the following factors
According to this ancient theory the four humors together causing decay:
of the body are blood, phlegm, black bile and 1. Role of carbohydrates.
yellow bile and any change in the relative 2. Role of microorganisms.
proportion of these elements causes disease. 3. Role of acids.
Similarly dental caries is caused by the change 4. Role of dental plaque.
in these humors. According to Greek physician
and philosopher Gallen, "Dental caries is Role of Carbohydrate
produced by the internal action of acrid and
corroding humors." Hippocrates the father of Various food substances can directly or indirectly
medicine, while favoring the concept of humoral affect dental caries. Food substances act as
pathology also referred to the accumulated substrates for microorganisms of dental plaque,
debris around teeth and to their corroding action. which form acids, thereby causing dental caries.
He also stated that the stagnation of food and Other substances may affect microflora in
juices was the cause of the decay. negative way thus protecting the tooth from
caries. Among these carbohydrates are main
cause of dental caries.
Vital Theory
Various carbohydrates have been examined
The theory stated in 18th century says that tooth for cariogenic potential. Among these
decay originated like bone gangrene, from carbohydrates are most important group as
within the tooth itself. caries causing food component. There is
 Dental Caries 403

considerable increase in caries incidence after Thus, sucrose is most potent cariogenic
exposure of modern civilization to refined foods. substance.
This is mainly because these substances contain • Other polysaccharides synthesized by the
more amount of sticky carbohydrates. cariogenic bacteria are glucan (from glucose)
The cariogenicity of carbohydrate varies with and levan (from fructose). glucan and levan
the : are weakly adhering substances so are less
i. Frequency of ingestion cariogenic.
ii. Physical form
Route of administration: Glucose or sucrose
iii. Chemical composition
administered intravenously or by gastric tube do
iv. Route of administration, and
not contribute to decay as they are unavailable
v. Presence of other food constituents.
for microbial breakdown, thus oral intake of
Frequency of ingestion: More is the frequency of sticky carbohydrates are found to be the cause
ingestion, more is the chance of dental caries. The behind the dental decay.
risk of caries incidence increases greatly if Presence of other food constituents: Refined pure
carbohydrates are taken repeatedly in between
carbohydrates are more caries producing than
two major meals. It provides an almost constant
crude carbohydrates complexed with other food
supply of carbohydrate to the plaque bacteria for elements capable of reducing enamel solubility.
fermentation and subsequent production of
acids. Role of Microorganisms
Physical form: Sticky, solid carbohydrates are It is generally agreed that dental caries is caused
more cariogenic than in any other forms. Sugars by acid resulting from action of microorganisms
that are easily cleared are less damaging than on carbohydrates.
soft retentive ones.
Microbiology of Dental Caries (Table 20.1)
Chemical composition: Cariogenicity among
carbohydrates also varies. Initiation of Dental Caries
• The carbohydrates in the form of glucose,
Animal studies with sophisticated experiments
sucrose and fructose, etc. rapidly diffuse into
and sterile conditions have confirmed the
the plaque due to their low molecular weight
and therefore make themselves easily
Table 20.1: Microbiology of dental caries
available for fermentation by plaque bacteria.
• Polysaccharides are less fermented by plaque Initiation of dental caries Progression of dental caries
bacteria than the monosaccharides and Streptococci Streptococcal species:
• S. mutans Streptococcal species in
disaccharides.
• S. milleri deep dentinal caries and
• Sucrose is utilized by Streptococcus mutans to • S. mitior root caries
synthesize an extracellular insoluble • S. sanguis Lactobacilli in dentin
polysaccharide (dextran) with the help of • S. salivaris • L. acidophillus
glucosyl transferase enzyme. Dextran helps Lactobacilli • L. casei
• L. acidophillus Actinomycoses
the plaque to adhere firmly onto the tooth
• L. casei • A. Israeli
surface and helps a direct contact between Actinomycoses • A. odontolyticus
the acids liberated by microorganisms and • A. viscosus
the tooth, thus causing demineralization. • A. naeslundii
404 Textbook of Oral Pathology

essential role of microorganisms in the initiation establishment. These are S. oralis, S. lactis, S.
and progression of dental caries. The absolute faecalis and S. bovis.
requirement of the microorganisms which are
said to be inducing dental caries, specifically Lactobacilli
bacteria that can produce acids, particularly
Lactobacilli form a small proportion of the oral
lactic acid, from the substrate of host's diet and flora at any part of the mouth. These are gram-
can tolerate very low pH (<5) are suspected of
positive rods that may be straight or curved and
initiating caries.
usually have blunt ends. Most of them are
The most important organism [(according to facultative anaerobes but some are strict
Clarke (1924) and Orland and co-workers (1954)]
anaerobes. Increased Lactobacillus counts with
is Streptococcus mutans.
the increase in caries activity shows that these
Streptococcus mutans organisms play an important role in the initiation
as well as progression of caries. They are:
The role of S.mutans has been proved for the
initiation of caries on the following basis: Acidogenic: They produce acids which mainly
• Increases the amount of sticky plaque as it is cause the initial demineralization of the enamel
capable of producing extracellular polysac- surface
charides. Aciduric: They particularly grow well in an
• Generates acid rapidly from sucrose and environment of low pH.
helps in initial establishment of the lesion. Though the role as a organism for initiating
• Multiply in abundance in low pH plaque or inducing carious lesion is not specific as of
thereby colonizing and increasing the plaque the S. mutans strain but it is definitively a co-
bulk. cariogenic.
• Provides a favorable substrate for other The lactobacilli strains which are responsible
cariogenic microorganisms. for formation of carious lesions include:
The other cariogenic organism responsible for • L. casei
the induction and establishment of the carious • L. acidophillus.
lesion are:
• S. salivaris: This is present in saliva, and Actinomycoses
epithelial surfaces mostly on tongue,throat
and establishes itself in dental plaque. The Actinomycoses form a major proportion of
• S. sanguis: Present in dental plaque and is the plaque flora at most sites on the teeth. The
capable of producing adhesive extracellular species which are responsible for the initiation
polymers which aids in colonization of of carious lesions on the basis of acidogenic
cariogenic organisms. properties and they also produce extracellular
• S. mitior: Most commonly isolated in polysaccharides.
plaque,this is present in smooth surface and The strains in initiation of caries are:
pit and fissure caries along with other strains. • A. viscosus
• S. milleri: This is also present in dental plaque • A. naeslundii.
and produces enzymes which help in sucrose
Progression of Dental Caries
degradation.
• Other streptococcal strains which are present The organisms responsible for progression of the
in the carious lesions may aid in its initial lesion are usually present in the advancing front
 Dental Caries 405

of the dentinal caries. These are mostly facultative Table 20.2: Localization of the oral flora related to caries
and strict anaerobes in advanced dentinal caries. Pit and fissure Streptococcus mutans
They are: Lactobacillus species
• Streptococcal species in deep dentinal caries Smooth surface caries Streptococcus mutans
and root caries Root caries Actinomycosis viscosus
• Lactobacillus casei and acidophillus in dentin. Actinomyces naeslundi
Other filamentus rods
Role of Acids Streptococcus mutans
Streptococcus sanguis
Acids play most important role in the pathogenesis Streptococcus salivarius
of dental caries. It has been noted that pH of
Deep dentinal caries Lactobacillus species
plaque decreases within 2-4 minutes of rinsing Actinomycosis viscosus
oral cavity with glucose. Actinomyces naeslundi
Stephan curve Robert M Stephen conducted Other filamentus rods
Streptococcus mutans
experiments to determine the plaque pH in
relation to dental caries and carbohydrate intake. The rapid fall in pH is mainly due to
Plaque pH was measured using Antimony touch microorganisms metabolising food substances
electrodes. and producing acids. This fall in pH depends
The Stephan curve describes the change in pH upon amount of diffusible carbohydrates, nature
at interface of dental plaque and tooth surface in of microorganisms and rate of diffusion of
response to various dietary components. The microorganism. The rise in pH is slower and it
Stephan curve reveals a rapid drop in plaque pH, depends upon ability of saliva to neutralize acids
followed by a slower rise until the resting pH is and diffusibility of acids out of plaque.
attained. The initial drop is usually rapid with The pH 5.5 is called as critical pH because
the lowest pH being attained within 2-4 minutes. below this pH demineralization of tooth substance
However, pH recovery takes about 40 minutes. begins.
This recovery depends saliva's ability to neutralize Flow Chart 20.1: Formation of acids by carbohydrates
acids (Fig. 20.2). and microorganisms

Figure 20.2: Stephan's curve: rapid fall and slow rise of

pH after oral rinse of glucose
406 Textbook of Oral Pathology

Acids are produced due to enzymatic Plaque is found on uncleaned tooth surfaces
breakdown of the sugar and the acids formed are and appears as tenacious, thin film which may
chiefly lactic acid and butyric acid (Flow accumulate within 24 to 48 hours.
chart 20.1).
Acquired pellicle: This form is a glycoprotein that
Other acids which are produced are acetic
is derived from the saliva and is adsorbed on
acid, propionic acid, glutamic acid, aspartic acid.
tooth surface. It is on this component of dental
These acids cause demineralization of
plaque the bacterial colonization takes place.
inorganic portion (initially enamel and later
Thus, the pellicle serves as a nutrient for plaque
dentin) and eventually cause tooth decay.
microorganisms.
These acids result in highly localized drop in
the pH at the tooth surface and plaque interface. Microbial homeostasis: Plaque develops naturally
pH below 5.5 causes demineralization . on teeth, and gives benefit to the host by
The tooth minerals act as buffers which providing colonization resistance. Once
initially help to maintain the local pH at 5.5. established at a site, the plaque flora remains
Further drop in the pH causes subsurface relatively stable with time despite regular
demineralization, i.e surface enamel remains environmental challenges. This stability
intact and demineralization is below the surface. (microbial homeostasis) is not due to any
When the pH is decreased further, i.e more metabolic indifference by the resident microflora
acidic pH of about 3.0 to 4.0, causes etching of but is due to a dynamic balance being established
enamel surface and resorption, this leads to among the resident microbial colony (Fig. 20.3).
cavitation. Breaks down in the homeostasis and
imbalances in the microflora can occur which
Role of Dental Plaque predispose a site to disease.

Dental plaque is a general term for the diverse

microbial community (predominantly bacteria)
found on the tooth surface, embedded in a matrix
of polymers of bacterial and salivary origin.
GV Black (1899) defined cariogenic plaque
as follows—"The gelatinous plaque of the caries
fungus is a thin, transparent film that usually
escapes observation, and which is revealed only
by careful search. It is not the thick mass of
materia alba found on the teeth, nor is it the
whitish gummy material known as sordes,
Which is often prominent in fevers and often
present in the mouth in smaller quantities in the
absence of fever."
Figure 20.3: Role of dental plaque in dental caries
The dental plaque is variable in both chemical pathogenesis. Plaque harbors microorganisms which
and physical composition, but usually consists ferment dietary carbohydrates producing lactic acid and
of salivary components such as mucin and dextran. Dextran forms matrix of plaque helping
microorganisms to adhere to tooth. Lactic acid causes
desquamated epithelial cells and micro- demineralization of tooth structure. Saliva and fluoride have
organisms. inhibitory effect on demineralization and microfloral of plaque
 Dental Caries 407

The repeated intake of fermentable sugar in Subsurface demineralization cannot be

the diet produces frequent conditions of low pH described by this acidogenic chemicoparasitic
in plaque which inhibits the growth of many of theory. Acute caries progression also is not
the species associated with highly acidogenic explained by this concept.
(acid-producing) and aciduric ("acid-loving")
species, such as mutans streptococci and Proteolytic Theory
lactobacilli, associated with dental caries. As
This theory suggests the role of the proteolysis
already stated, pH of 5.5 is critical threshold for
of the organic components of the tooth as an
the demineralization. initial process than the actual demineralization
Cariogenic plaque contains 2 × 108 bacteria
and dissolution of inorganic substances.
per milligram weight.
The organic degradation is said to be the
The filamentous organisms grow in long initial pathway of invasion of the micro-
interlacing threads and have the property of
organisms.
adhering to smooth enamel surfaces.
It has also been proposed that the enamel
Smaller bacilli and cocci become entrapped lamellae or rod sheath (proteins) may be lysed
in this reticular meshwork. which means proteolysis as the first event in the
The adhering property and acidogenic further progression of the bacterial invasion and
property of streptococci and aciduric property demineralization carious lesions.
of lactobacilli help in further development of Some authors suggest that the Nasmyth's
cariogenicity of the dental plaque. membrane and enamel proteins produce
Early colonizers (pioneers) of the tooth sulphuric acids on hydrolysis. This formed
surface are mainly Neisseria spp. and strep- calcium sulfate compounds when combined
tococci. with sulfatase enzymes liberated by
The growth and metabolism of these pioneer microorganisms. This subsequently leads to
species changes local environmental conditions formation of the carious enamel.
(e.g. Eh, pH, coaggregation, substrate availabil- The possibility of the events may be as
ity). thereby enabling more fastidious organisms follows:
to colonise, e.g. obligate anaerobes tend to be late • The enamel may be intact or clinically
colonisers in plaque produce chalky area only by alteration in
According to some authors, the pioneering structure and underlying dentin may be
organisms are S. sanguis, A. viscosus and involved through this pathway of degraded
Peptostreptococcus. proteins though in very minor quantities.
S. mutans further colonize in this • Minor variation in the organic and inorganic
noncariogenic plaque and grows in an acidic structure of the tooth determines the pattern
environment. of the rate and progression of the carious
Enzyme glucosyl transferase helps in the lesions. Thus, caries penetrates through
synthesis of the extracellular matrix and aids in enamel rods or along inter rod areas available
adhesion with the tooth surface for degradation.
Thus, dental plaque provides environment This theory could not be accepted for the fact
for the cariogenic microorganisms, a medium that the induction of the caries in experimental
for acid produced and their adhesion to the tooth animals was seen in the absence of proteolytic
surface for longer period. organisms.
408 Textbook of Oral Pathology

Proteolysis Chelation Theory active individual, there can be formation of

Chelation is a process of combining a metallic complex substances like calcium saccharates and
ion to a complex substance with the help of calcium complexing intermediaries, etc. by the
coordinate covalent bond which results in a action of phosphorylating enzymes. These
highly stable, weakly ionized and poorly complexes cause release of calcium and
dissociated compound. For example, phosphorus ions from the enamel and thereby
Haem (iron) + 4 pyrrole globin = haemoglobin. resulting in tooth decay.
The four pyrrole nuclei are attached to iron This theory is unlikely to be a significant
by covalent bond. because once the sucrose is in the oral cavity, it
The proteolytic chelation theory suggest that readily gets metabolized to form acids, and there
the caries is caused by simultaneous events of is hardly any scope for the formation of calcium
proteolysis and chelation. saccharates, a very high level of pH is required,
Proteolysis is destruction of the organic the range of which is never achieved in the oral
portion of the tooth by the proteolytic micro- cavity.
organisms.
Chelation is removal of calcium by forming Autoimmune Theory
soluble chelates by biologic chelators such as Jackson and Bunch suggest that zones or regions
certain citrates, amino acids, phosphatases, of odontoblasts in specific sites with the pulp of
tartrates, oxalates and enzymes etc. specific teeth are damaged by an autoimmune
It is postulated that oral bacteria attack process so that the defense capacity of the
organic component of enamel (proteolysis) and overlying dentin and enamel is compromised
that the breakdown products have chelating and concluded that caries should be regarded
ability and this dissolves the tooth minerals. as a degenerative process. Initially disease event
This results in formation of soluble chelates corresponds to a form of somatic gene mutation
with the minerals of the enamel and thereby in central growth control stem cells. Descendent
decalcifies it at a neutral or even at an alkaline mutant cells synthesize autoantibody which
pH. (Chelation). damage specific groups of odontoblasts and thus
Some authors suggest that the dental caries determine the sites of caries susceptibility.
is not merely an event due to proteolysis or
chelation but it occurs due to sequential events.
Secondary Contributing Factors in
1. Simultaneous degradation of organic
Dental Caries (Fig. 20.4)
substances and demineralization of the
mineralized tissues A. Saliva
2. This leads to low pH which leads to • Salivary flow rate
promotion of the growth of the lactobacilli. • Composition
3. The combined effects of the organic degra- – Inorganic
dation, mineralized substances decalcifica- – Organic
tion and increased microbial population lead • pH
to the formation of carious lesion. • Viscosity
• Antibacterial substances
Sucrose Chelation Theory B. Teeth
This theory states that if there is a very excessive • Structural composition
concentration of sucrose in the mouth of a caries • Morphology
 Dental Caries 409

• Arrangement in the arch • Occasionally, a rapid wearing of the incisal

• Presence of dental appliance and the occlusal surfaces will be seen.
C. Diet • Alterations in the amount and bacteriological
• Physical nature composition of the plaque are also reported.
• Composition Streptococcus mutans, lactobacilli, yeast,
D. Other factors Actinomyces and Staphylococci increase in
• Heredity. number while Veillonella, Streptococcus
sanguis, Niesseria, bacteroids and
Fusobacterium have been observed to
decrease in number.
• Post-radiation therapy there are changes in
the main salivary glands with altered
composition and flow of saliva.
pH and buffering capacity
• A buffer is a solution that tends to maintain
a constant pH.
• In saliva, the chief buffer systems are the
bicarbonate ions and phosphate ions.
• High concentrations of bicarbonate ions
neutralize the acids produced by the
cariogenic bacteria.
• Urea secreted in saliva helps in the formation
Figure 20.4: Parameters involved in pathogenesis of
of ammonia by action of the plaque
dental caries
microorganisms. Ammonia acts as buffer in
Saliva
maintaining the salivary pH.
Salivary flow rate
Viscosity
• Decrease or lack of salivary secretion results
• Thick and gluey consistency tends to attach
in increased rate of dental caries and rapid
more number of the microorganisms and
destruction of tooth as the cleaning or flushing
more plaque also.
of the bacterial deposits is hampered.
• Xerostomia (Greek: xeros—dry, stoma— Antibacterial substances
mouth) was first described by Bartley in 1968. • Lysozyme along with sodium lauryl sulfate,
Synonyms for xerostomia include oligosalia, a detergent, can lyse many cariogenic
asalia, and stomatitis sicca. Conditions streptococci.
leading to xerostomia are discussed in • Lysozyme cleaves the linkage between N-
diseases of salivary glands. acetylglucosamine and N-acetylmuramic
• In patients with xerostomia the caries is acid, which are responsible for the repeating
atypical. Decay often attacks the cervical area, units of the bacterial cell wall peptidoglycans.
involves cementum and dentin, and
progresses inwardly until the crown is Teeth
destructed. It involves those teeth that are less Structural composition
commonly affected by caries such as incisors • Teeth are usually susceptible to caries during
and canines. first 2 years after eruption as additional
410 Textbook of Oral Pathology

2 years are required for completion of encourage the retention of food debris and plaque
calcification after eruption. material and have been shown to result in an
• Concentration of higher number minerals in increase in the bacterial population.
the surface enamel renders it more resistant
to decay. Diet
• Hypomineralised and hypoplastic enamel Physical nature: Fibrous foods help to keep the
has more incidence of caries. Increased tooth surfaces clean and stimulate the salivary
permeability of the enamel surface increases flow which reduces the incidence of caries. Soft
the risk of the caries activity. and sticky foods tend to be retained on the tooth
• Teeth which have high percentage of fluoride surface and thereby predispose for more
are more resistant to caries process. bacterial accumulation and decay.
• Accumulation of elements like fluoride,
chloride, zinc, lead, and iron in the surface Composition
enamel with aging takes place .Thus, this • Carbohydrate content plays an important
enamel is more resistant to dissolution to role in the causation of caries as stated in the
acids. Miller's acidogenic theory.
Morphology • Presence of phosphates in the diet can reduce
• Pits and narrow fissures allow retention of the caries by increasing the remineralization.
food debris and thus are prone to develop- • Among other components proteins are less
ment of decay. cariogenic and lipids are least cariogenic.
• These areas are also not easily approachable Lipids help in preventing retention of food
to routine oral hygiene practices. The substances. Medium chain fatty acids have
formation of plaque at the base of the defect some antibacterial properties at acidic pH.
further aids in the progression of the caries • Although vitamin A deficiency results in
in these areas. defects of developing teeth, but it has no
• The most susceptible permanent teeth are the definitive role in formation of dental caries.
mandibular first molars followed by the Similarly vitamin D, vitamin C deficiency
maxillary first molars and the mandibular result in tooth defects but their association
and maxillary molars. with dental caries is not definitive one.
• The second premolars, maxillary incisors and • Traces of molybdenum, strontium, and
first premolars are the teeth next in sequence vanadium in diet are found to reduce the
for occurrence of caries. incidence of dental caries.
• The mandibular incisors and canines are the • Selenium in diet and drinking water is found
least likely teeth for caries incidence. to increase incidence of dental caries. These
findings are not confirmed.
Arrangement in the arch: Crowded, malaligned,
• Fluoride in drinking water is proven to be
rotated and irregular teeth are not readily cariostatic. But dietary fluoride is less
cleansed during the natural masticatory process.
important because of metabolic unavailability.
Such teeth favor the accumulation of food and
debris and may be susceptible for caries. Other Factors
Presence of dental appliance: Partial dentures, space Heredity: It has been suggested that genetic
maintainers and orthodontic appliances often factors contribute the causation of dental caries.
 Dental Caries 411

Another possibility suggested is that inheritance Based on number of surfaces involved

of tooth form or structure can also be a factor • Simple: Only one surface is involved by caries
which predisposes to caries susceptibility or • Compound: Two surfaces are involved.
immunity. • Complex: More than three surfaces involved.
As dietary habits, cooking habits and tooth
GV Black classification based on treatment and
brushing frequency are passed on generation to
restoration design
generation from parents to offspring, the exact role
of inheritance cannot be assessed. Class I: These begin in pits, fissures and defective
grooves. These are seen in occlusal surface,
Classification occlusal two-thirds of molars and lingual pits of
incisors.
Depending on nature of attack
• Primary caries (Incipient, initial): First attack Class II: These are lesions seen on proximal
on tooth surface aspects of molars and premolars.
• Secondary caries (Recurrent): Caries Class III: These are lesions involving proximal
occurring at the margins or walls of existing aspects of incisors that do not involve or
restorations. necessitate removal of incisal edge.
Depending on the progression of the caries Class IV: These are lesions involving proximal
• Acute: It rapidly invading process that aspects of incisors that involve or require
involves several teeth. Lesions are soft and removal of incisal edge.
light colored. Usually pulp is involved at the Class V: These are lesions present on gingival
early stage. third of all the teeth.
– Rampant caries
Class VI: Lesions found on incisal edges and cusp
– Nursing bottle caries
tips.
– Radiation caries
• Chronic: These lesions are long standing and Based on location of the lesion
fewer in number. • Pit and fissure caries
– Occlusal
Depending on surfaces involved
– Buccal or lingual pit
• Pit and fissure caries
• Smooth surface caries
• Smooth surface caries.
– Proximal
Based on direction of caries attack – Buccal or lingual surface
Forward Caries: Caries that proceeds from enamel • Root caries.
to dentin. The lesion is triangle shaped with base
Based on tissue involved
of the triangle at the enamel surface and apex
• Enamel caries
towards dentin. In pits and fissures base is at
• Dentinal caries
dentinoenamel junction and the apex is in the
• Cemental caries.
pit.
Senile caries: It is caries associated with aging
Backward Caries: Caries that proceeds from
process. These are almost exclusively seen on
dentino enamel junction towards enamel
root surface.
surface. This is also triangle shaped with base at
the dentinoenamel junction and apex towards Residual caries: It is caries that is not removed
enamel surface. during restorative procedure.
412 Textbook of Oral Pathology

Arrested caries: Sometimes progress of caries is

halted because of treatment or change in
conditions. Such lesions are mineralized but
retain brown color.

SMOOTH SURFACE CARIES

Interproximal caries
Clinical Features
It takes 3 to 4 years to manifest clinically as loss of
enamel transparency resulting in opaque chalky Figure 20.5: Labial caries showing destruction of tooth
region (white spot). In some cases it appears as a surface
yellow or brown pigmented area but it is usually
As the caries process results in demineraliza-
well demarcated. Spots are generally located on
tion, the affected area of the tooth appears more
the outer surface of enamel between contact point
radiolucent than unaffected area.
and height of free gingival margin. The early white
Carious area attenuates less radiation than
chalky spot becomes slightly roughened owing
intact tooth substance so that the area of the film
to superficial decalcification of the enamel.
on which remnant beam from the deminerlized
As the caries penetrates the enamel, the enamel
area falls receives higher exposure and thus
surrounding the lesion assumes bluish white
appears darker on the processed radiograph.
appearance which is usually apparent as
laterally spreading caries at the dentinoenamel Histopathological Features of Smooth
junction. Caries does not initiate below free
Surface Caries
gingival margin. It is common for proximal caries
to extend both buccally and lingually (Fig. 20.5). Enamel Changes
The earliest changes are loss of the inter-
Cervical, Buccal, Lingual or Palatal Caries
prismatic or inter-rod substance of enamel with
Clinical Features increased prominence of the rods. In some cases
It usually extends from the area opposite the the changes are roughening of the ends of the
gingival crest occlusally to the convexity of the enamel rods, suggesting that the prisms may be
tooth surface. It extends laterally towards the more susceptible to early attack. Another change
proximal surfaces and on occasion extends in early enamel caries is the accentuation of the
beneath the free margin of the gingiva. It usually incremental striae of Retzius (Fig. 20.6).
occurs in cervical area and the typical cervical As the process advances and involves deeper
lesion is a crescent shaped cavity beginning as layers of enamel, it will form triangular or
slightly roughened chalky area which gradually actually a cone shaped lesion with apex towards
becomes excavated. the dentinoenamel junction and the base towards
the surface of the tooth. Enamel feels rough to
Radiographic Features hand in advanced caries which may be due to
The initial lesion appears as opaque white or disintegration of the enamel prisms after
brown spot beneath the plaque layer. decalcification of the inter-prismatic substance
 Dental Caries 413

and the accumulation of debris and micro- • Zone 3: The body of the lesion lies between the
organism over the enamel rods. relatively unaffected surface layer and the
Several zones can be identified in the enamel dark zone. It is the area of greatest
which are as follows (Fig. 20.7): demineralization.
• Zone 1: The translucent zone lies at the • Zone 4: The surface zone, when examined by
advancing front of the enamel lesion. By use polarizing light appears relatively unaffected.
of polarized light it is learnt that this zone is
slightly more porous than sound enamel. Dentin Changes
• Zone 2: The dark zone lies adjacent and The initial penetration of the dentin by caries
superficial to the translucent zone. It has been
may result in alteration in the dentin called as
referred to as positive zone because it is
'dentinal sclerosis'. Dentinal sclerosis is a
usually present. This zone is present as a reaction of vital dentinal tubules and a vital pulp
result of demineralization.
in which there results a calcification of the
dentinal tubules which finally seals them
against further penetration by microorganisms.
(Fig. 20.8) The initial decalcification involves the
walls of the tubules allowing them to distend
slightly as they become packed with masses of
microorganisms. The decalcification of the walls
of the individual tubules leads to their confluence
(Fig. 20.9).
A thickening and swelling of the sheath of
Neumann may sometimes be noted at irregular
intervals along the course of the involved dentinal
tubules, in addition to increased diameter of the
Figure 20.6: Smooth surface caries with intact enamel dentinal tubules due to packing of the tubules by
surface microorganisms (Fig. 20.9).

Figure 20.7: Various zones of dental caries: The intact

surface zone (4) covers the body of lesion (3). At the
advancing front of caries translucent zone (1) separates Figure 20.8: Dentinal caries—ground section (Courtesy:
dark zone (2) from normal enamel Dr Sangamesh Halawar)
414 Textbook of Oral Pathology

Figure 20.9: Decalcified and H and E stained section dentinal Figure 20.11: Pioneer bacteria responsible for dental
caries showing Miller's liquefaction foci an area of coagulation caries
necrosis and coalescence of dentinal tubules parallel to the
dentinal tubules
As the carious lesion progresses various zones
beginning pulpally at the advancing edge of the
Tiny 'liquefaction foci' are formed by focal lesion adjacent to the normal dentin are seen
coalescence and breakdown of a few dentinal which are as follows:
tubules. This 'focus' is an ovoid area of destruction • Zone 1: Zone of fatty degeneration of Tomes
parallel to the course of the tubules and filled with fibers.
necrotic debris which tend to increase in size by • Zone 2: Zone of dentinal sclerosis
expansion. This produces compression and characterized by deposition of calcium salts
distortion of adjacent dentinal tubules so that their in dentinal tubules.
course is bent around the liquefaction focuses • Zone 3: Decalcification of dentin, a narrow
(Figs 20.10 and 20.11). zone preceding bacterial invasion.
• Zone 4: Zone of bacterial invasion of decalcified
but intact dentin.
• Zone 5: Zone of decomposed dentin.

Pit and Fissure Caries

It is also called as 'occlusal caries'. It is primary
type and develops in the occlusal surface of molars
and premolars. Deep narrow pits and fissures
favor the retention of food debris and
microorganisms and caries may result due to
fermentation of this food and the formation of
acids.

Clinical Features
It usually occurs in pits and fissures with high
Figure 20.10: Advanced dentinal caries in a decalcified steep walls and narrow bases. It appears brown
section showing clefts (CFT), coagulation necrosis of dentinal
tubules (CN), isolated Miller's liquefaction foci (M), pioneer or black and will feel slightly soft and catch a
bacteria (pb) fine explorer point. The enamel directly bordering
 Dental Caries 415

the pit and fissure may appear opaque, bluish Freshly exposed root are more vulnerable to an
white as it becomes undermined. The lateral acid attack because of higher porosity and
spread of caries at the dentinoenamel junction smaller crystal.
as well as penetration into the dentin along the
dentinal tubules may be extensive without Clinical Features
fracturing away the overhanging enamel. Thus, It appears as slowly progressing chronic lesion.
there may be large carious lesion with only a
It is usually found in mandibular molar and
tiny point of opening.
premolar region. Tooth surface involved in
decreasing order of frequency are buccal, lingual,
Histopathological Features
interproximal. Gingival recession is associated
Enamel changes: They are more or less same as with root surface caries.
smooth surface caries. Enamel at the bottom of
the pit or fissure may be very thin so that early Histopathological Features
dentin involvement can occur. Enamel rods flare
It is a soft progressive lesion that is found
laterally at the bottom of the pits and fissures.
anywhere on the root surface that has lost
Lesion forms a triangular or cone shaped lesion connective tissue attachment and is exposed to
with its apex at the outer surface and its base
the oral environment. Microorganisms appear
toward the dentinoenamel junction (Fig. 20.12).
to invade the cementum either along Sharpe's
fibers or between bundles of fibers, in a manner
comparable to the invasion along dentinal
tubules.
As cementum is formed in concentric layers
and presents a lamellated appearance, the mi-
croorganisms tend to spread laterally between
the various layers.The carious lesion assumes the
shape of a saucer (Fig. 20.13). After decalcifica-
tion of cementum destruction of the remaining
matrix occurs similar to the process in dentin.

Recurrent Caries

Figure 20.12: Pit caries Dental caries that occurs immediately adjacent
(Courtesy: Dr Sangamesh Halawar) to the restoration is referred to as recurrent caries.
It may be caused by inadequate extension of
Root Caries restoration and if there has not been careful and
It is also called as 'cemental caries' and involves complete excavation of original carious lesion.
both dentin and cementum. Nowaday there is
Clinical Features
greater prevalence of root caries due to longer
life span of persons, with the retention of teeth Sixteen percent of restored teeth have recurrent
into the later decades of life and increase in the caries. Restoration will show poor margins
number of people exhibiting gingival recession which permitted leakage and the entrance of
with clinical exposure of cemental surface. both bacteria and substrate.
416 Textbook of Oral Pathology

Clinical Features
Prolonged bottle feeding beyond the usual time
when the child is waned from the bottle and
introduced to solid food may result in early and
rampant caries. There is early carious involvement
of the maxillary anterior teeth, the maxillary and
mandibular first permanent molars, the
mandibular canines. The carious process in the
teeth is so severe that only the root stumps remain.

Prevention
The infant should be held while feeding. The
child who falls asleep while nursing should be
burped and then placed in bed. Parent should
start brushing the child teeth as soon as they
erupt in the oral cavity. Discontinue bottle
Figure 20.13: Cemental caries forming a saucer shape at feeding as soon as child can drink from a cup, at
the advancing dentinal front approximately 12 to 15 months of age.

Nursing Bottle Caries Rampant Caries

Etiology It is defined as a suddenly appearing,
It occurs due to nursing bottle containing milk or widespread, rapidly burrowing type of caries,
milk formulae, fruit juice or sweetened water. resulting in early involvement of the pulp and
Sometimes it occurs due to sugar or honey- affecting those teeth usually regarded as immune
sweetened pacifier. to ordinary decay. Some believe that the term
rampant caries should be applied to those carious
Pathogenesis lesions with 10 or more new lesions per year.

The reasons for this are that the child is put on

Etiology
bed at afternoon nap time or at night with
nursing bottle containing milk or a sugar Under laboratory conditions sucrose is more
containing beverage. The child falls asleep and likely to cause rampant multisurface cavitations
the milk or sweetened liquid becomes pooled than glucose, fructose etc. It may occur due to
around the maxillary anterior teeth. The nutritional deficiency, malnutrition, emotional
carbohydrate containing liquid provides an disturbances.
excellent culture medium for acidogenic Various forms of stress in both children and
microorganisms. adults, as well as various medication (such as
Salivary flow is decreased during sleep and tranquilizers and sedatives) commonly taken
clearance of the liquid from the oral cavity is to help persons cope with stress, are associated
slowed. Lactose content of human milk, as well with decreased salivary flow and decreased
as that of bovine milk, can be cariogenic if the caries resistance caused by impaired
milk is allowed to stagnate on the teeth. remineralization.
 Dental Caries 417

Clinical Features overlying primary tooth or by surgical exposure.

Restoration of the teeth.
It usually occurs in children with poor dietary
habits. It demonstrates extensive inter-proximal Control of Dental Caries
and smooth surface caries. Rampant caries can
occur suddenly in teeth that were for many years Control of All Active Lesions
relatively immune to decay. Initial treatment of all active lesions. Gross
excavation of all carious lesions followed by
Management systematic manner of restoring a tooth to normal
Extensive dental care and parent education. contour.

Nutritional Measures for Caries Control

Arrested Caries
Group of patients whose diet is high in fat, low
It has been described as caries which becomes
in carbohydrate and practically free from sugar
static or stationary and does not show any
have low caries activity. In a study, when refined
tendency for further progression. sugar was added to the diet in the form of a
mealtime supplement there was little or no caries
Clinical Features
activity. Phosphates diet causes significant
Both deciduous and permanent dentitions are reduction in incidence of caries.
affected by this condition. It occurs exclusively
in caries of occlusal surfaces. It is characterized Mechanical Measures for Caries Control
by large open cavities in which there is lack of Tooth Brushing
food retention and in which the superficially Tooth brushing reduces the number of oral
softened and decalcified dentin is gradually
microorganisms, particularly if the teeth are
burnished and has taken a brown-stained
brushed after each meal. Tooth brush also
polished appearance and is hard. This has been removes gross amounts of food debris and
referred to as 'eburnation of dentin'. In some
plaque material.
cases there is brown stained area at or just below
the contact of the affected tooth. Mouth Rinsing
The use of mouthwash for the benefit of its action
Pre-eruptive Caries
in loosening food debris from the teeth has been
Occasionally defect on the crowns of developing suggested as a measure of caries control.
permanent teeth are evident radiographically,
even though no infection of the primary tooth Dental Floss
or surrounding area is apparent. It is called as 'pre- Dental flossing has been shown to remove
eruptive caries'. Such lesions do resemble caries plaque from an area gingival to the contact areas
when it is observed clinically and the destructive on proximal surfaces of teeth, an area impossible
nature of lesion progresses if it is not restored. to reach with toothbrush.

Treatment Detergent
As soon as the lesion is reasonably accessible, Some workers have related the high caries
the tooth should be uncovered by removal of the incidence among modern civilized races to the
418 Textbook of Oral Pathology

unrestrained use of soft, sticky, refined foods, Fluoridation of the communal water supply
which tend to adhere to the teeth. It has been stated is the most effective method of reducing the
that fibrous food prevents lodging of food in pits dental caries problems in the general population.
and fissures of teeth and in addition acts as
Fluoride containing dentifrices: It contains stannous
detergent.
fluoride in combination with calcium pyrophos-
phate as the cleaning and polishing system and
Pit and Fissure Sealants
was accepted as the first therapeutic dentifrice.
Pits and fissures of occlusal surface are among the
Fluoride mouth rinses: It should be given
most difficult areas on teeth to keep clean and from
cautiously in children under 4 years of age as
which to remove plaque. The pit and fissure
they may not have full control over the
sealants generally used in conjunction with an
swallowing reflex.
acid pretreatment to enhance their retention,
contain either cyanoacrylate, polyurethane or the Dietary fluoride supplement: The administration of
adduct of bisphenol A and glycidyl methacrylate. fluoride supplement commences shortly after
birth and should continue through the time of
Chemical Measures of Caries Control eruption of the second permanent molars.
Substances which alter the tooth surface or tooth
Bis-biguanides
structure
Chlorhexidine and alexidine are potential
Fluorine anticaries agents as they are antiplaque agents.
It has been shown that chlorhexidine is adsorbed
The cariostatic activity of fluoride involves
onto tooth surface and salivary mucins then it is
several different mechanisms:
• Presence of fluoride during tooth develop- released slowly in an active form. But
disadvantage of chlorhexidine is that it has bitter
ment results in formation tooth with well
taste, produces brownish discoloration of hard
rounded cusps without any deep fissures and
pits. This makes the tooth caries resistant. and soft tissues and may produce painful
desquamation of mucosa.
• The ingestion of fluoride results in its
incorporation into the dentin and enamel of
Silver Nitrate
unerupted teeth. This makes the teeth more
resistant to acid attack after eruption into oral Silver nitrate impregnation of teeth was used for
cavity. many years to prevent or arrest caries. Silver
• Ingested fluoride is secreted into saliva; plugs the enamel by either the organic invasion
although present in low concentration in pathways such as the enamel lamellae or the
saliva, the fluoride is accumulated in plaque inorganic portion of enamel to form a less soluble
where it decreases microbial acid production combination.
and enhances the remineralization of the
Zinc Chloride and Potassium Ferrocyanide
underlying enamel.
• Fluoride from saliva is also incorporated into Use of solution of zinc chloride and potassium
the enamel of newly erupted teeth, thereby ferrocyanide would effectively impregnate the
enhancing the enamel calcification. enamel and seal off caries invasion pathways.
• Fluoride in saliva helps in remineralization But study shows that it is of little value in
of early carious lesion. reduction of caries.
 Dental Caries 419

Substances which interfere with carbohydrate bacterial growth. Although there are some studies
degradation through enzymatic alteration to indicate that ammoniated dentifrices are
capable of producing some reduction in dental
Vitamin K caries incidence, the magnitude of this reduction,
particularly in persons whose tooth-brushing
Synthetic vitamin K (2-methyl 1, 4 naphtho-
quinone) prevents acid formation in incubated habits are not controlled or supervised, is not so
great as to justify recommending them for
mixture of glucose and saliva. In study also it
widespread use as an anticariogenic agent.
has show to decrease incidence of caries forma-
tion in persons given chewing gums containing
Chlorophyll
vitamin K.
It is a green pigment of plants and has been
Sarcoside proposed as an anticariogenic agent on the basis
Persons who brushed teeth with dentifrices of a number of in vitro studies and animal
containing sodium N-lauroyl sarcosinate have studies. Water soluble form of chlorophyll,
been shown to have decreased incidence of sodium copper chlorophyllin, was capable of
caries. It has been stated that it has got ability to preventing or reducing the pH fall in
penetrate the dental plaque and prevent pH to carbohydrate-saliva mixtures in vitro.
fall below 5.5 after carbohydrate rinse.
Nitrofurans
Substances which interfere with bacterial
growth and metabolism They are derivatives of furfural which itself is
derived from pentoses. They have been found
Urea and Ammonium Compounds to exert bacteriostatic and bactericidal action on
many gram-positive and gram-negative bacteria
Reports suggest that a quinine urea mouthwash
and they can also inhibit acid formation. Studies
prevents acid formation in test in vitro on
show that nitrofurans compounds like
carbohydrate saliva mixtures. It may also be
furadroxyl (5-nitro-2-furaldehyde-2 hydro-
noted that oral bacteria count was decreased
xyethy semicarbazone) reduce dental caries.
after the use of quinine urea mouthwash and
salivary pH generally increased to value over 8
Penicillin
and remained high for approximately an hour.
The evidence indicated that urea upon It has got ability to inhibit the normal biologic
degradation by urease; releases ammonia which processes of lactobacilli which is one of the
acts to neutralize acids formed through etiological factors in the dental caries. Penicillin
carbohydrate digestion and also interferes with is given in dentifrices.
420 Textbook of Oral Pathology

CHAPTER
Sequel of Pulpal
21 Infection

The pulp is the formative organ of the tooth. It CLASSIFICATION

builds primary dentin during the development
of the tooth, secondary dentin after tooth 1st–Based on Severity of Inflammation
eruption and reparative dentin in response to Pulpitis (inflammation)
stimulation as long as the odontoblasts remain
Reversible
vital. The pulp has been described as both a
• Symptomatic (acute)
highly resistant organ and as an organ with little
resistance or recuperating ability. It is a delicate • Asymptomatic (chronic).
connective tissue liberally interspersed with tiny Irreversible
blood vessels, lymphatics, myelinated and • Acute
unmyelinated nerves, and undifferentiated – Abnormally responsive to cold
connective tissues cells. – Abnormally responsive to heat
• Chronic
CAUSES OF PULPAL INFLAMMATION – Asymptomatic with pulp exposure
– Hyperplastic
Mechanical cause—It includes traumatic accident, – Internal resorption.
iatrogenic damage for dental procedure, attrition, Pulp degeneration
abrasion. • Calcific
Thermal cause—It may cause through uninsulated • Others.
metallic restoration, during cavity preparation, Pulp Necrosis
polishing.
Chemical cause—It arises from erosion or 2nd–According to Involvement
inappropriate use of acidic dental material. According to involvement
Bacterial cause—It can damage the pulp through • Focal or subtotal or partial pulpitis—In it
the toxins secreted by bacteria from caries. inflammatory process is confined to a portion
 Sequel of Pulpal Infection 421

of the pulp, usually a portion of the coronal factors are removed. Repair involves the return
pulp. to normal tissue fluid dynamics, exit of
• Total or generalized pulpitis—In it most of polymorphonuclear leukocytes from the area and
the pulp is involved. the re-differentiation of odontoblasts if they have
According to severity damaged. The pulpal tissue after repair is usually
• Acute less vascular, more fibrous and less cellular than
• Chronic. before and may be less able to withstand a
According to presence or absence of direct subsequent insult.
communication between the dental pulp and oral Clinical Features
environment.
It is characterized by sharp pain lasting for a
• Pulpitis aperta (open pulpitis)—In it pulp is
communicated with the oral cavity. moment. It is more often brought on by cold than
• Pulpitis clausa (closed pulpitis)—No hot food or beverages and by cold air. It does
communication exists. not occur spontaneously and does not continue
when the cause has been removed. Tooth
TYPES OF PULPITIS responds to electric pulp testing at lower current.

Reversible Pulpitis Histopathological Features

It is a mild to moderate inflammatory condition It may range from hyperemia to mild to
of the pulp caused by noxious stimuli in which moderate inflammatory changes to the area of
the pulp is capable of returning to un-inflammed the involved dentinal tubules such as dentinal
state following removal of the stimuli. caries. Microscopically one sees involved
reparative dentin, disruption of the odontoblastic
Causes layer, dilated blood vessels, extravasation of fluid
It may be caused by any agent that is capable of (edema) and the presence of immunological
injuring the pulp like trauma, disturbed occlusal response. Chronic inflammatory cells
relationship, and thermal shock. predominate (Fig. 21.1). In some cases acute
inflammatory cells can also be seen.
Pathogenesis
Some stimuli of short duration, such as cutting
dentin may cause short term vasodilation and a
reversible increase in vessel wall permeability.
More severe stimuli and a greater degree of cell
damage cause more marked vasodilatation and
the movement of polymorphonuclear leukocytes
into the injured tissues. These acute inflammatory
reactions are usually limited to the odontoblast
and sub-odontoblastic regions adjacent to the
dentinal tubules involved. Odontoblastic nuclei
may be displaced into the tubules, due to either
increased local tissue pressure or dentinal fluid
during the injury. It is reversible, if the etiological Figure 21.1: Pulpitis showing inflammatory cell infiltration
422 Textbook of Oral Pathology

cells or some diapedesis of white blood cells.

Slowing of blood flow and hemoconcentration
due to transudation of fluid from the vessels
conceivable could cause thrombosis (Fig. 21.2).

Management
Removal of irritants before the pulp is severely
damaged.

Acute Pulpitis
Extensive acute inflammation of the pulp is a
frequent sequel of focal reversible pulpitis.

Figure 21.2: Pulp hyperemia showing cellular infiltrate and Causes

inflammatory cells and carious dentin
It usually occurs in a tooth with a large carious
Management lesion or a defective restoration where there has
Prevention is the best Management for it. It is been recurrent caries. Pulp exposure may occur
done by periodic care, early insertion of a filling due to faulty cavity preparation. Blow to tooth
if a cavity has developed. Removal of noxious with subsequent damage to pulp, cracked tooth
stimuli. syndrome can also be causative factors. It may
occur as acute exacerbation of chronic
Focal Reversible Pulpitis inflammatory process Recurrent caries in a tooth
It is the earliest form which is sometimes known around restoration.
as ‘pulp hyperemia’. There is excessive
accumulation of the blood within the pulp tissue Clinical Features
leading to vascular congestion. Severe pain is elicited by thermal changes and
pain persists even after thermal stimulus
Clinical Features disappears or been is removed. As greater
It is sensitive to thermal changes, particularly to proportion of the pulp becomes involved with
cold. The application of ice or cold fluids to the intrapulpal abscess formation, the pain may
tooth result in pain, but it disappears upon become even more severe and is often described
removal of the thermal irritant or restoration of as lancinating type. It may be continuous and its
normal temperature. It responds to electrical test intensity may be increased when the patient lies
stimulant at a lower level of current indicating down. The application of heat may cause acute
lower pain threshold than that of adjacent normal exacerbation of pain and the tooth reacts to the
teeth. Teeth usually show a deep carious lesion electric pulp vitality tester at a lower level of
or a large metallic a restoration or restoration current than adjacent normal teeth.
with defective margins. Severe pain is present when the entrance to
the diseased pulp is not wide open. Pressure
Histopathological Features increases because of lack of escape of infla-
There is dilatation of pulp vessels. Edema fluid mmatory exudate and there is rapid spread of
may collect because of damage to the capillary inflammation throughout the pulp with pain and
wall allowing acute extravasation of red blood necrosis. If a cavity is large then the inflammatory
 Sequel of Pulpal Infection 423

process does not tend to spread rapidly entrance of root canals. Root canal filing with
throughout the pulp. In this cases, the pain felt inert material like gutta percha should be done.
is dull, throbbing ache, but tooth is still sensitive
to thermal changes. The patient with acute Chronic Pulpitis
pulpitis is extremely uncomfortable and at least Chronic pulpitis may arise on occasion through
mildly ill. He is usually apprehensive and quiescence of previous acute pulpitis.
desirous of immediate attention.
Clinical Features
Histopathological Features
Pain is not prominent features of it but if present is
Continued vascular dilatation accompanied by of dull aching type which is more often intermittent
the accumulation of edema fluid in the than continuous. Because of degeneration of nerve
connective tissue surrounding the tiny blood tissue in the affected area the threshold from
vessels. The pavementing of polymorpho- stimulation by the electric pulp vitality tester is
nuclear leukocytes becomes apparent along the increased. The exposed pulp tissue may be
walls of the vascular channels, and these manipulated by a small instrument but bleeding
leukocytes rapidly migrate through the can occur.
endothelium lined structure in increasing
numbers. Collection of white blood cells are Histopathological Features
found beneath the area of carious penetration There is infiltration of the pulp tissue by varying
and odontoblasts in this area are destroyed numbers of mononuclear cells chiefly leukocytes
(Fig. 21.3). and plasma cells. Capillaries are usually
There may be localized destruction of pulp prominent and fibroblastic activity is evident and
tissue and formation of pus resulting in condition collagen fibers are seen.
known as ‘pulp abscess’. It contains pus arising Sometimes, there is an attempt to wall off the
from breakdown of leukocytes and bacteria as infection through deposition of collagen about
well as from digestion of tissue. In tissue section inflamed area with formation of granulation
it appears as a small void surrounded by a dense tissue. When this occurs on the surface of the
band of leukocytes. pulp tissue in a wide open exposure it is called
In some cases within a few days, the acute as ‘ulcerative pulpitis’.
inflammation spreads to involve most of the pulp
so that neutrophilic leukocytes fill the pulp. The
entire odontoblastic layer degenerates. As pulp
is closed, there is rise in pressure and the entire
pulp tissue undergoes rapid disintegration,
forming numerous small abscesses. Eventually
the entire pulp undergoes liquefaction and
necrosis which is called as ‘acute suppurative
pulpitis’.

Management
In early stages pulpotomy (removal of the
coronal pulp) and placing material that favors Figure 21.3: Pulp hyperemia showing many dilated blood
calcification such as calcium hydroxide over the vessels and necrotic area in the centre
424 Textbook of Oral Pathology

Management Histopathological Features

Root canal therapy followed by crown As caries penetrates the dentin it causes a chronic
restoration. inflammatory response. If the caries is not
removed, the inflammatory changes in the pulp
Irreversible Pulpitis will increase in severity as the decay approaches
the pulp. The postcapillary venules become
It is a persistent inflammatory condition of the congested and affect the circulation within the
pulp, which may be symptomatic or pulp, causing pathologic changes such as
asymptomatic and is caused by a noxious necrosis. These necrotic areas attract
stimulus. polymorphonuclear leukocytes by chemotaxis
and start an acute inflammatory reaction.
Causes After phagocytosis, the polymorphonuclear
It is by bacterial involvement of the pulp through leukocytes, which have a short life span, die and
caries, although many clinical factors, chemical, release lysosomal enzymes. The lysosomal
thermal or mechanical injury may also be the enzymes lysosome of the pulpal stroma and
cause. together with the cellular debris of the dead
polymorphonuclear leukocytes form purulent
Clinical Features exudates (pus). The inflammatory reaction
produces micro-abscess (acute pulpitis). The
In early stages of irreversible pulpitis, a paroxysm
pulp trying to protect itself walls off the areas of
of pain may be caused by the following: sudden
the microabscess with fibrous connective tissue.
temperature changes like cold, sweet and acid
The surrounding pulp tissue exhibits fibrosis
foodstuffs. The pain often continues when the
and mixture of plasma cells, lymphocytes and
cause has been removed and it may come and
histiocytes.
go spontaneously.
Pain is sharp, piercing or shooting and is Clinical Differences between Reversible and
generally severe. It may be intermittent or Irreversible Pulpitis
continuous, depending on the degree of pulpal
The pain of irreversible pulpitis is more severe
involvement and depending on whether it is
and lasts longer. In reversible pulpitis, the cause
related to an external stimulus. The patient may
of pain is generally traceable to a stimulus such
also state that bending over or lying down, i.e.
as cold water or air whereas in irreversible
change of position, exacerbates the pain which
pulpitis, the pain may come without any
is due to change in intrapulpal pressure. apparent stimulus.
Patient may complain of pain referred to adjacent
teeth to the temple or sinuses when an upper Management
posterior tooth is involved, or to the angle, when
lower posterior tooth is affected. Complete removal of the pulp or pulpectomy
In later stages, pain is more severe and is and placement of an intracanal medicament to
generally described as boring, gnawing or act as a disinfectant or obtundent such a cresatin,
throbbing or as if tooth is under constant eugenol or formacresol.
pressure. Patient is often awake at night due to
Chronic Hyperplastic Pulpitis
pain. Pain is increased by heat and is sometimes
relieved by cold, although continued application It is also called as ‘pulp polyp’ or ‘pulpitis aperta’.
of cold may intensify the pain. It is essentially an excessive, exuberant
 Sequel of Pulpal Infection 425

proliferation of chronically inflammed dental Histopathological Features

pulp tissue.
The surface of the pulp polyp is covered by
Definition stratified squamous epithelium. Such epithelium
may be derived from gingiva or freshly
It is a productive pulpal inflammation due to an desquamate epithelial cells of the oral mucosa
extensive carious exposure of a young pulp. It is
or tongue. The tissue in the pulp chamber is often
characterized by the development of granulation
transformed into granulation tissue which
tissue, covered at times by epithelium and
projects from the pulp into the carious lesion.
resulting from long standing low grade infection.
The granulation tissue is a young vascular
Causes tissue containing polymorphonuclear
neutrophils, lymphocytes and plasma cells. The
Slow progressive carious exposure of the pulp is
granulation tissue may become epithelized as a
the cause. For the development of a hyperplastic
result of implantation of epithelial cells on its
pulp, a large open cavity, a young resistant pulp
surface. Granulation tissue is made up of delicate
and a chronic low grade stimulus is necessary.
connective tissue fibers interspersed with a
Mechanical irritation from chewing and bacterial
variable number of small capillaries.
infection often provides the stimuli.
Inflammatory cell infiltration chiefly lympho-
Clinical Features cytes and plasma cells are also seen.

Teeth most commonly involved are deciduous Management

molars and first permanent molars as they have
an excellent blood supply because of a large root Elimination of polypoid tissue, followed by
opening, and this coupled with high tissue extirpation of the pulp is done. After removing
resistance and reactivity in young persons the hyperplastic tissue bleeding can be controlled
accounts for unusual proliferative properties of by pressure. Extraction of tooth can also be done.
the pulp tissue. It is asymptomatic and it is seen
only in teeth of children and young adults. Pulp Degeneration
Polypoid tissue appears as a fleshy, reddish
pulpal mass filling most of the pulp chamber or Degeneration is usually present in older aged
cavity or even extending beyond the confines of people.
the tooth. Some times, mass if large enough
interferes with comfortable closure of teeth. Causes
Polypoid tissue is less sensitive than normal pulp It is a result of persistent, mild irritation in teeth
tissue and more sensitive than the gingival tissue. of younger people.
It may cause discomfort during mastication, due
to pressure of food bolus. This tissue bleeds easily Clinical Features
because of rich network of blood vessels. The
tooth may respond feebly or not at all to the In early stages, there are no symptoms and no
thermal test. There is presence of squamous clinical findings. As degeneration progresses, the
covering as a result of grafting of exfoliated tooth may become discolored and the pulp
epithelial cells from adjacent oral mucosa. within does not respond to stimuli.
426 Textbook of Oral Pathology

Types Pulp Calcifications

Calcific degeneration—A part of pulp tissue is Various forms of pulp calcifications are found
replaced by calcific material that is pulp stones within the pulp which may be located in the pulp
and denticles are formed. The calcific material chamber or in the root canals. It can occur in any
has a laminated structure like the skin of an onion sex and in any teeth in the dental arch.
and lies unattached within the body of pulp. In
another type of calcification the calcified material Etiology
is attached to the wall of the pulp cavity and is There is no clear-cut etiology. There is no relation
an integral part of it which is called as diffuse between pulpal inflammation and irritation, as
calcification. that arising from caries or trauma, since pulp
Atrophic degeneration—It is observed in older calcification can be found in unerupted teeth.
people. The pulp tissue is less sensitive than Extremely high percentage of pulp stones yield
normal. pure growth of streptococci on culture but often
the affected teeth are normal.
Fibrous degeneration—It is characterized by
replacement of the cellular element by fibrous Sundell schematic presentation— Local metabolic
connective tissue. On removal from the root dysfunction → trauma → Hyalinization of
canal, the pulp has the characteristic appearance injured cell → vascular damage → thrombosis
of a leathery fiber (Fig. 21.4). vessel wall damage → fibrosis → mineralization
(nidus formation) → growth- pulp stones.

Classification
There are two main forms of pulp calcification:

Discrete Pulp Stones (denticles, pulp nodules)

It is of following types:
• True denticles—They are made of localized
masses of calcified tissue that resembles
dentin due to their tubular structure. Tubules
are irregular and few in number. More
common in pulp chamber than root canals.
Figure 21.4: Fibrosis of pulp seen as replacement of They are subdivided into
cellular element by fibrous connective tissue
• Free denticle—Denticle lying entirely within
Pulp artefacts—Vacuolization of the odontoblasts pulp tissue and is not attached to the dentinal
was once thought to be a type of pulp wall (Fig. 21.5).
degeneration characterized by empty spaces • Attached denticle—These are continuous with
formally occupied by odontoblasts. It is an the dentinal wall.
artefact caused by poor fixation of the tissue • False denticle—It is composed of localized mass
specimen. of calcified material and they do not exhibit
Tumor metastasis—It is rare except possibly in the dentinal tubules. Nodule appears to be made
terminal stages. It may occur due to direct local up of concentric layers or lamellae deposited
extension from the jaw. around a central nidus. It is composed of cells
 Sequel of Pulpal Infection 427

Figure 21.5: Pulp calcification Figure 21.7: Diffuse calcification presented as linear strands

around which laid down is a layer of reticular columns parallel with blood vessels and nerves
fibers that subsequently calcifies. They are of pulp (Fig. 21.7).
again classified as free or attached. They are
larger than true denticles and they may fill Clinical Significance of Pulp Calcification
nearly the entire pulp chamber.
As such they have very little clinical significance.
• Interstitial denticle—As the concentric
Usually it is discovered on the radiograph only
deposition of calcified material continues it
approximates and finally is in apposition with as radiopacity. Sometimes, it may cause pain
the dentinal wall where it may be surrounded from mild pulpal neuralgia to severe excruciating
by secondary dentin then it is called as an pain resembling that of tic douloureux as the
interstitial denticle. denticle can impinge on the nerve of the pulp.
Difficulty may be encountered in extirpating the
Diffuse Calcification pulp during root canal therapy. In some
It is also called as ‘calcific degeneration’. It is conditions like dentin dysplasia (type II), tumoral
amorphous unorganized linear strands or calcinosis, calcinosis universalis and Ehlers-
Danlos syndrome calcification can be seen.

Reticular Atrophy of Pulp

It is a degenerative or regressive change of pulp.

Etiology
Age changes in elderly person.

Clinical Features
There is vacuolization of the pulpal tissue and
Figure 21.6: Pulp calcification cells. A tooth is symptomless and responds
(Courtesy: Dr Sangamesh Halawar) normally to vitality test.
428 Textbook of Oral Pathology

Histopathological Features The periapical tissue may be normal or slight

evidence of inflammation of the apical
There is presence of large vacuolated spaces in
periodontal ligament may be seen.
the pulp with reduction in the number of cellular
elements. Degeneration and disappearance of
Management
odontoblasts.
Preparation and obturation of root canals.
Necrosis of Pulp
It is the death of pulp. It may be partial or total Cracked Tooth Syndrome
depending on whether a part or the entire pulp Patient complains of pain ranging from mild to
is involved. excruciating, at the initiation or release of the
biting pressure. It can mimic the condition as
Causes severe as trigeminal neuralgia. A crack may
It is sequelae of inflammation and can also occur involve enamel and dentine only or it may also
following trauma, in which the pulp is destroyed involve the pulp and symptoms will vary
before an inflammatory reaction. accordingly. Pain occurs due to fluid movement
within the dentinal tubules causing stimulation
Types of sub-odontoblastic nerve fibers. The fluid
movements are induced by pressure changes
• Coagulation necrosis—The soluble portion of
when biting with the offending cusp.
tissue is precipitated or is converted into a
If the crack involves dental pulp, direct
solid material. Caseation is a form of
bacterial invasion will occur with predictable
coagulation necrosis in which the tissue is
pulpal inflammation and resultant pulpitic pain.
converted into a cheesy mass consisting
Close examination of the crown of the tooth may
chiefly of coagulated proteins, fats, and water.
disclose a crack in enamel, which may be better
• Liquefaction necrosis—It results when
visualized by using a dye or by trans-
proteolytic enzymes convert the tissue into
illuminating the tooth with fiber-optic light.
softened mass, liquid or amorphous debris.
Crack can be confirmed by selective biting
pressure using a cotton roll or a small wooden
Clinical Features
stick to allow selective localization of such
It causes no painful symptoms. Discoloration of pressure. It is treated by splinting of the offending
the tooth is the first indication that the pulp is cusp with a cusp protecting restoration or by
dead. The tooth with partial necrosis can respond removing the split cusp and then restoring the
to thermal changes owing to presence of vital tooth.
nerve fibers passing through the adjacent
inflammed tissue. History of severe pain lasting Sequelae of Pulpitis (Pulpoperiapical
from a few minutes to a few hours, followed by abscess)
complete and sudden cessation of pain.
Cause for pulpitis is due to irritating
inflammatory products. Various factor like host
Histopathological Features
resistance, number and virulence of bacteria,
Necrotic pulp tissue, cellular debris and amount of antigenic material, degree of tooth
microorganisms may be seen in the pulp cavity. function and extent of other trauma also play a
 Sequel of Pulpal Infection 429

vital role. These products escape from pulp canal If granuloma has been subjected to repeated
and initiate local inflammatory response. The exacerbation due to periodic contact with the
pulp is non-vital in such cases. irritation from root canal, it may become fibrosed.
Periapical lesion in this group share some If root canal treatment is done successfully,
common clinical characteristics: remaining inflammation and granulomatous
• Periapical lesion is radiolucent. tissue will resolve leaving only fibrosed areas
• Radicular pulp is associated with apical lesion behind. Such entities are referred to periapical
is non vital. scar.
• Crown may often be discolored or show deep
caries and have a restoration which is close Acute Infections
to pulp.
Periapical Osteitis
• Crown may be partially or completely
missing due to traumatic injury. Microorganisms responsible for deep carious
• History of painful pulpitis. lesions penetrate the pulp and stimulate
Teeth with dens in dente have higher inflammation within soft pulpal tissue,
incidence of pulp death and pulpoperiapical ultimately leading to necrosis of pulp. The
lesion. Final diagnosis is by microscopic bacteria from necrotic pulp invade the marrow
examination. spaces of bone in the periapical region and trigger
the process of inflammation. This stage is called
Sequelae after Pulp Death as periapical osteitis. The later consequences are
edema, ischemia and necrosis.
If none of the irritating products of pulp necrosis The path of least resistance is along medullary
reach the periapical tissue, then no periapical spaces. The patient gets throbbing pain and there
pathos is induced. May arise at periapex but in is marked tenderness to vertical percussion.
such moderate amount that the host defense Clinician should prefer either endodontic
effectively combats and localizes the resultant treatment or extraction of tooth, under antibiotic
inflammation in circumscribed area. Teeth with cover. Antibiotics should be continued for three
contaminated gangrenous pulp have large to five days after extraction.
number of bacteria passing in the root canal
which may be sufficient to overwhelm the Abscess
defence of periapical tissues resulting in an acute
Definition
periapical abscess.
Resultant infection may be partially An abscess is a localized collection of pus,
controlled by body defence, by surgically surrounded by an area of inflamed tissue in
induced drainage or by antibiotic therapy, thus which hyperemia and infiltration of leucocytes
results in development of chronic periapical is marked.
periodontitis or chronic alveolar abscess. If
Bacteriology
odontogenic epithelial rests of Malassez (present
in PDL and periapical granuloma) proliferate, a Staphylococci are frequently associated with
radicular cyst may develop in response. If abscess formation. They produce the enzyme
adequate root canal treatment is done, there will called coagulase which causes fibrin deposition
be ultimate disappearance of granuloma and and thus helps in walling off the lesion.
complete resolution of radiolucency to normal Coagulase promotes virulence by inhibiting
bone. phagocytosis.
430 Textbook of Oral Pathology

Small pockets of necrotic tissue are formed Clinical Features

within cellulites, which coalesce and enlarge,
It develops in the periapical region of the tooth
compressing the surrounding fibrous connective
as sequelae to necrosis of pulp.
tissue. Thus an abscess is generated, which is a
Pain is severe and of throbbing type.
collection of pus surrounded by a wall of fibrous
Periapical abscess may confine to osseous
connective tissue.
structures and during the early period of abscess
formation, may cause excruciating pain without
Clinical Features
observable swelling. The patient may appear
When palpated clinically, a superficial abscess pale, irritable and weak from pain, loss of sleep
is fluctuant. Offending tooth is carious and as well as from absorption of septic products. He
mobile. Symptoms of acute inflammation, i.e. may have slight fever (99 to 100°F).
swelling and fever are evident. Patients experience sensitivity or pressure in
the affected area. Ice relives the pain and heat
Acute Periapical Abscess intensifies it aspiration yield yellowish pus. The
It is also called as ‘acute alveolar abscess’. tooth becomes more painful, appears elongated
and mobile. In acute periapical infection, tooth
Definition is sensitive to percussion and movement. There
is also painful lymphadenopathy. After some
It is a localized collection of pus in the alveolar period the affected pulp is necrotic and does not
bone, at the root apex of a tooth, following death respond to electric current or to application of
of the pulp. cold. Swelling is usually seen in adjacent tissues
adjacent to the affected tooth.
Etiopathogenesis The tissues at the surface of swelling appear
It may be result of trauma or chemical or taut and inflamed. The surface of tissue become
mechanical irritation. The immediate cause is the distended from the pressure of underlying pus
bacterial invasion of dead pulp. The most and finally ruptures due to pressure and lack of
common microorganism is streptococcus resistance caused by continued liquefaction.
viridans. When inflammatory response may When the maxillary anterior teeth are
extend into adjacent periapical alveolar bone, it involved, swelling of upper lip may extend to
will initiate necrosis of periapical tissue and one or both eyelids. When the maxillary teeth
diffuse rarefaction of bone, leading to formation are involved, the cheek may swell to an immense
of periapical abscess with symptoms of acute size, distorting the patient’s face. When the
inflammation. maxillary posterior teeth are involved, there is
Primary or necrotic abscess are pulpo- possibility of maxillary sinus to involvement.
periapical inflammatory conditions associated When the mandibular posterior teeth are
with teeth, which have not developed apparent involved, swelling of the cheek may extend to
periapical radiolucent lesion; usually described ear.
as acute apical periodontitis or acute periapical
Histopathological Features
abscess. Canal contains large number of virulent
bacteria that rapidly spread to periapically Central region of necrosis contain dense
tissues, causing acute periodontitis, tender tooth accumulation of polymorphonuclear leukocytes,
and alveolar swelling. surrounded by inflamed connective tissue wall
 Sequel of Pulpal Infection 431

of varying thickness. Acute abscess contain Clinical Features

necrotic and un-identical soft tissues. There is an
It starts at the gingival cervice and extends down
empty space, where suppuration has occurred.
on one or more surface of the root, frequently as
The root canal appears to be devoid of tissue and
far as apical region. Acute episode usually has
instead, cups of microorganism and debris may
sudden onset with extreme pain. There is also
be observed.
distension and discomfort. They are associated
Management with swelling of the soft tissues overlying the
surface of the involved root. Tooth is tender and
Establish drainage immediately, if possible — mobile. Pus usually exudes from the gingival
may be done by opening the pulp chamber and cervice.
passing file through the canal into the periapical
region. Trephination opening through mucosa
Histopathological Features
and bore to the abscess at apex. Through and
through drain is placed in the abscess and It consist of a central cavity filled with pus walled
irrigated with 1:1 mix of 3% H2O2 and normal off on one side by the root and on the other side
saline solution. by connective tissue, as the epithelial lining of
Antibiotics like Penicillin 500 mg, qID, for 5 the crevice has been destroyed by the
days and analgesics should be given. In 24 to 48 inflammatory process.
hours, it can be determined if the tooth can be
treated endodontically or extraction is necessary. Management
Warm saline mouth rinse often aid in
localizing the infection and maintaining Primary treatment for relief of acute symptoms
adequate drainage, before endodontic treatment is incision of the fluctuant abscess, from the depth
or extraction. of the abscess cavity to the gingiva. The incision
If there is need of retention of offending tooth, should extend into the soft tissues of the root
necrotic pulp should be opened and tooth should surface. If the surrounding tissue is normal, the
be treated endodontically. tooth may be retained and debridement of the
root surface by removal of granulation tissue
Periodontal Abscess should be done. Treatment for new attachment
and new tissue regeneration should be
It is usually culmination of a long period of
chronic periodontitis. performed. However, if the roots are denuded
beyond the apical thirds of the root, the tooth
Pathogenesis should be extracted and curettage should be
carried out to remove the granulation tissue from
It usually occurs in pre-existing periodontal the socket.
pocket. When such pocket reaches sufficient
depth of about 5 to 8 mm, the soft tissues, around Acute Exacerbation of a Chronic Lesion
the neck of the tooth may approximate the tooth
so tightly that orifice of the pocket is occluded. It is also called as ‘phoenix abscess.’
Bacteria multiply in the depth of pocket and
Definition
cause sufficient irritation to form an acute
abscess, with exudation of pus into this area. It It is an acute inflammatory reaction superim-
results in sufficient swelling to destroy the posed on an existing chronic lesion, such as on
cortical plates of bone. cyst or granuloma.
432 Textbook of Oral Pathology

Causes Management
The peri-radicular area may react to noxious Drainage, either via the root canal or by incision,
stimuli from a diseased pulp with chronic peri- if there is localized swelling.
radicular disease. At times, because of an influx
of necrotic product from a diseased pulp or Chronic Alveolar Abscess
because of bacteria and there toxins, this It is a long standing, low grade infection of the
apparently dormant lesion may react and cause periradicular tissues.
an acute inflammatory response.
Etiopathogenesis
Clinical Features
It results from direct extension of acute pulpitis
At the onset, tooth may be tender to touch.
or acute non-suppurative periodontitis or acute
Patients complain of intense pain, local swelling
exacerbation of periapical granuloma, cyst or
(Fig. 21.8) and possibly associated cellulitis.
chronic abscess. The surrounding tissue attempt
Mucosa over the radicular area may be sensitive
to localize the pyogenic infection by forming
to palpation and may appear red and swollen.
enclosure of granulation tissue; this in turn is
The patient has history of traumatic accident that
surrounded by fibrous connective tissue; this
turned the tooth dark after a period of time or of
results in well circumscribed lesion containing
post-operative pain in a tooth that had subsided necrotic tissue.
until the present episode of pain. Lack of Well-circumscribed lesion may form sinus
response to vitality test points to diagnosis due to:
necrotic pulp. • Inability of the body to completely contain
or localized the causative organisms.
• Increase in number of causative organisms.
• Lowering of patient’s general resistance.
• Trauma or surgical intervention.
Enlarging dentoalveolar abscess contains
purulent material that is under pressure due to
the production of pus—the purulent material
travels along path of least resistance, until it
reaches the surface, where due to limitation of
periosteal layer, it temporally forms subper-
iosteal abscess. Eventually, it erodes through the
Figure 21.8: Phoenix abscess seen as swelling below periosteum and penetrates the soft tissue, again,
the body of mandible following the path of least resistance. Path of least
resistance is determined by the location of
Histopathological Features breakthrough in the bone and the anatomy of
Area of liquefaction necrosis with disintegrating muscles and fascia plane in the area.
polymorphonuclear neutrophils and cellular
Clinical Features
debris. These are surrounded by infiltration of
macrophages and some lymphocytes. These It is characterized by less soreness and often, a
abscesses can maintain soft tissue component. better defined radiographic lesion. History of
 Sequel of Pulpal Infection 433

pain that started as dull ache and progressed to long standing then the resulting mild irritation
severe throbbing type. Sudden decrease in pain may lead to circumscribed proliferation of
signals the formation of sinus. Tooth is tender, periapical bone, appearing as condensing osteitis
vitality test is negative. or focal sclerosing osteomyelitis. There is
Draining fistulas are also commonly deposition of new bone along the existing
associated with chronic alveolar abscess. trabeculae, a process knows as appositional bone
Majority opens on labial and buccal aspect of deposition.
alveolus, as apices of both maxillary and
mandibular teeth are located nearer to the buccal Clinical Features
than the lingual cortical plate. In maxilla, roots
It occurs almost in young person before the age
of lateral incisors and molars are close to palatal
of 20 years. The tooth commonly affected is
cortical plate, so sinus appear there. Most root
mandibular first molar with a large carious
tips lie below the mylohyoid muscle, so pus
lesion. It is associated with non vital teeth or in
drains into the submandibular space.
teeth undergoing the process of degeneration.
The patients will demonstrate lymphade-
Tooth is usually asymptomatic. But in some
nopathy as well. Sinus opening appears as a
cases, patient may report pain or tenderness on
small ulcer. It is usually present opposite the root
percussion or palpation.
apex in the labial or buccal mucosa; it may be
located far away from the affected tooth. Histopathological Features
Occasionally, after temporary emphysema, sinus
heals and form slightly raised pale papule. As It appears as an area of dense bone with
the pus accumulates, another signs formation trabeculae borders lined by osteoblasts. Chronic
may take place eventually. inflammatory cells, plasma cells and
lymphocytes are seen in the scanty bone marrow.
Histopathological Features
Osteosclerosis
Macrophages and granulation tissue are present.
Lymphocytes and plasma cells are found at the Clinical Features
periphery of the abscessed area, with variable
Patient is usually over the age of 12 years. It is a
number of polymorphonuclear leukocytes at
mass of compact bone within the spongiosis,
center. Fibroblast may start to form capsule at
which does not cause expansion.
the periphery. Sinus tract are generally lined by
granulation tissue. In addition, chronic
Histopathological Features
inflammatory cells are also present.
It consists of thickened trabeculae and
Management concomitant decrease in the size and number of
Antibiotics are seldom used. If there is need of marrow spaces, vascularity and number of
retention of offending tooth, necrotic pulp should lacunae.
be opened and tooth should be treated
endodontically. The tooth which can not be Management
restored should be extracted. No treatment is required for the disease.

Condensing Osteitis Periapical Granuloma

It is also called as ‘chronic focal sclerosing It is the most common type of pathologic
osteomyelitis. If the exudate is of low toxicity and radiolucency encountered in dentistry. It is a
434 Textbook of Oral Pathology

growth of granulation tissue continuous with the of collagen and occasionally, nests of
periodontal ligament resulting from the death of odontogenic epithelium, Russell’s bodies
the pulp and diffusion of bacteria and bacterial (scattered eosinophilic globules of gamma
toxins from the root canals into the surrounding globulin), foam cells and cholesterol clefts. New
periradicular tissues through the apical and capillaries are lined by swollen endothelial cells.
lateral foramina. Types of granuloma according to histo-
pathology
Etiopathogenesis • Exudative
• Granulomatous
It occurs as a response to intense and prolonged
• Granulofibrosis
irritation from infected root canals producing
• Fibrous.
extension of chronic apical periodontitis beyond
There is more inflammation in the center with
the periodontal ligament. The expanding
fibrosis at the periphery. Connective tissue is
inflammation and increased vascular pressure
more prominent on the periphery and the
result in abscess formation and resorption of the
bundles of collagen become condensed there,
bone in the affected area, which is replaced by
apparently as a result of the slow expansion of
granulation tissue. It is the result of a successful
the soft tissue mass, resulting in formation of a
attempt by the periapical tissues to neutralize and
continuous capsule separating the granulation
confine the irritating toxic product that is
tissue from the bone. Epithelial rest of Malassez
escaping from the root canal. But continuous
may be seen with granulation tissue.
discharge into the periapical tissues induces a
Lymphocytes, plasma cells, macrophages and
vascular inflammatory response. Insult from
foreign body multinucleated giant cells giant cells
diseased pulp represents broad spectrum of
may also be present. Occasionally, cholesterol
inflammatory mediations like prostaglandins,
clefts may form the major portion; then it is called
kinin and endotoxins. Elevated level of IgG in
as cholesterol granuloma of the jaw.
pulpoperiapical lesion.

Clinical Features Management

Extraction of the involved tooth should be done.
The tooth is non vital, i.e. it does not respond to
Under certain conditions, root canal therapy,
thermal and electric pulp test. Tooth may be
darker in color, because of the blood pigments with or without subsequent apicoectomy are the
treatment options.
that diffuse into the dentinal tubules. Mild pain
can be occasionally experienced while biting or
Periapical Scar
chewing on solid foods. Sensitivity occurs due
to hyperemia, edema and inflammation of the It is a possible end point of healing. It is composed
apical periodontal ligament. There is, seldom, of dense fibrous tissue and is situated at the
swelling or expansion of the overlying cortical periapex of pulpless tooth, in which usually, the
bone. Tooth may feel to be slightly elongated in roots canal have been successfully filled.
the socket.
Formation of Scar
Histopathological Features
Irritant substance—Confined in the periapical
It consists of proliferating endothelial cells area, which leads to accumulation of chronic
capillaries, young fibroblasts minimum amount inflammatory cells. Young fibroblasts,
 Sequel of Pulpal Infection 435

endothelial cells and capillaries proliferate, jaw is affected, but most common site is molar
which lead to granuloma formation. After region, particularly first molar. There is a hard
endodontic treatment, the granuloma resolves, bony swelling in mandible. The hyperplasia is
but in some cases, granulation tissue gets slowly seen along the lower border of mandible.
organized with the production of more and more
collagen fibers, which in turn leads to scar Histopathological Features
formation.
Specimen shows parallel row of highly cellular
and reactive woven bone with perpendicular
Clinical Features
trabecular arrangement. uninflamed fibrous
It occurs usually after endodontic treatment and connective is seen in the trabecular pattern which
in patients treated by periapical curettage or root may form an interconnecting meshwork of bone.
resection. It is more common in anterior region
of maxilla. Tooth is nonvital and the patient is Management
asymptomatic. Extraction of offending tooth should be done.

Histopathological Features Osteomyelitis

It will show spindle shaped fibroblast scattered Definitions
throughout the dense collagen bundles, which
show advanced degree of hyalinization. It is the inflammation of the bone marrow that
produces clinically apparent pus and secondarily
Periostitis of the Jaws affects the calcified components.
It refers to inflammation of the periosteum of the It is infection of the bone that involves all the
jaws. three components viz. periosteum, cortex and
marrow.
Causes It may be defined as an inflammatory
Infected material from the apex of tooth, spreads condition of the bone that begins as an infection
to the surface of the bone where it meets the of medullary cavity and the haversian system
periosteum. Due to gravity, there is tendency of and extends to involve the periosteum of the
pus to track downwards to the lower margins of affected area.
the bone. Due to fluid nature of the material, the
swelling rises gradually. As a result of infection Predisposing Factors
of the deep surface of the periosteum, the Certain predisposing factors play an important
osteogenic layer gets stimulated to lay down the role in the onset and severity of the osteomyelitis,
new bone. It can also be caused by infected cysts, in addition to the virulence of microorganism.
infected tooth follicles and pericoronitis. The Conditions affecting host resistance-diabetes
other causes of periostitis are osteomyelitis, mellitus, tuberculosis, severe anemia, leukemia,
tuberculosis and syphilis. agranulocytosis, acute illness such as influenza,
scarlet fever, typhoid and exanthematous fever,
Clinical Features sickle cell anemia, malnutrition and chronic
It usually occurs in children and youths, with alcoholism.
equal distribution of sex. Mandible is affected Conditions affecting jaw vascularity—Metastasis
more commonly than maxilla. Any portion of the from remote area of infection such as another
436 Textbook of Oral Pathology

bony site, skin and kidneys, radiation, Disorganization of clot—However, disorganization

osteoporosis, osteopetrosis, fibrous dysplasia, of this pyogenic membrane occurs by virulent
bone malignancy and peripheral vascular microorganism or by chronic movement of the
disease. unreduced fractures of jaws.
Mechanical trauma—Mechanical trauma, due to
Etiology chronic movement of unreduced fractures,
Odontogenic infections which can be periapical burnishes the bone and causes ischemia, thereby
or periodontal infection, pericoronal infection introducing the microorganisms deep into the
and infection from infected dental cyst. underlying tissues.
Compound fractures of the jaws—Generally, these Accumulation of pus—When this protective barrier
fractures are compound through the tooth socket breaks, the pus accumulated in the medullary
into the mouth and rarely, to the skin. cavity gives rise to increased intra-medullary
pressure, which results in compression of
Traumatic injury—Local traumatic injury of the
vasculature, vascular collapse, venous stasis and
gingiva leads to periostitis, in patients with low
ischemia.
resistance to infection and later to osteomyelitis.
Middle ear infection and respiratory infection via Elevation of periosteum—Pus travels through the
Haversian and Volkmann’s canals and
hematogenous route, either from middle ear
accumulates beneath the periosteum, elevating
infection or from infection of the upper
it from the cortex, thereby further reducing the
respiratory tract.
blood supply.
Furunculosis of chin—Furunculosis of chin, i.e.
spread through lymphatic channel via infected Necrosis of bone—The reduced blood supply leads
to slow necrosis of the bone.
lymph nodes.
Peritonsillar abscess—Peritonsillar abscess has also Penetration of periosteum—If the pus continues to
been reported to cause osteomyelitis of the ramus accumulate, the periosteum is penetrated and
of mandible. mucosal and cutaneous fistulae develop and
thereby discharging the purulent pus.
Pathogenesis After therapy—As the therapy begins to be
effective and the host resistance increases, the
Compromised blood supply is a critical factor in
process become chronic. Inflammation regresses,
the establishment of osteomyelitis. The virulent
granulation tissue forms and new blood vessels
microorganisms get entry in the medullary cavity
are formed which cause lysis of bone; thus
via many routes like odontogenic infections,
causing fragments of necrotic bone from the
compound fractures, periostitis, hematogenous
viable bone.
route and lymphatic channel.
Inflammatory reaction—These microorganisms Involucrum—Small sections of necrotic bone may
be completely lysed, while large one get localized
cause intense inflammatory reaction within the
and get separated from the shell of the new bone
marrow of the bone. Pain is a feature of this stage.
Localization of infection—most of the by a bed of granulation tissue. This dead bone
surrounded by viable bone is called involucrum.
odontogenic infections, like periapical and
periodontal infections, are localized by pyogenic Sequestra—Small pieces of necrotic bone are
membrane or soft tissue abscess walls. called as sequestra, which are avascular and
 Sequel of Pulpal Infection 437

harbor microorganisms. These sequestra need to formation and release and hence, the
be removed, otherwise they continue to be infection gets directs into spongiosa and
chronically infected and infect the surrounding spreads.
granulation tissue and cause further • Removal of posterior mandibular teeth is
sequestration, which weakens the bone and may attended by more damage to the bone.
cause pathologic fracture. • Mandible is less vascular than maxilla.
• Thin cortical plates and relative paucity of
Cloacae—An involucrum contains one or more
holes on the surface which lead into channels, medullary tissue in the maxilla precludes
which can be traced to end in the depth of the confinement of infection within the bone and
permits dissipation of edema and pus into the
bone at the site of an area of bone destruction
soft tissues and paranasal sinuses.
around the sequestrum. These orifices are termed
Infantile osteomyelitis is more common in
‘cloacae’. Pus finds its way from the depth of the
maxilla than mandible, as it spreads by
bone to the surface, through the cloacae. The
presence of cloacae indicates that there is a dead hematogenous route and maxilla has more blood
bone or a foreign body at the deep end. supply than mandible.

Systemic spread of infection—Besides the Microbiology of Osteomyelitis

pathogenic activity of the virulent
microorganisms, these microorganisms Staphylococcus aureus and Staphylococcus albus,
hemolytic streptococci, gram-negative organisms
precipitate thrombi formation by virtue of their
like Klebsiella, Pseudomonas, Protease, E. coli.
destructive lysosomal packages. The coagulum
Anaerobic microorganisms like Pepto-
provides the host medium for further pathogenic
streptococci, bacteroides and fusobacteria. Some
proliferation as well as an isolating barrier from
specific forms like Mycobacterium tuberculosis,
the host immune response. It also allows systemic
Treponema palladium and A. israeli.
spread of infection.
Following parameters should be used for
Necrosis of bone—The necrosis of bone is brought recognition of pure anaerobic or mixed anaerobic
about by thrombosis of the vessels or infection:
compression of the vasculature. The necrosis of • Presence of foul smelling exudate.
bone, with superadded infection forms a base • Sloughing of necrotic tissues or gas in the
line pathogenesis of the osteomyelitis. necrotic tissue.
Compression of neurovascular bundles can • Gram stain revealing multiple organisms of
result in osteomyelitis mediated anesthesia. different morphological character.
The possibility of pre-existing predisposing • Presence of sequestra.
factors can further complicate this phenomenon.
After complete removal of sequestra and Classification
resolution of disease process, the jaw undergoes According to anatomic location of infectious
complete remodeling. process
• Intramedullary
Occurrence • Subperiosteal
It is more common in men, than women. It occurs • Periosteal.
in mandible in premolar area because: According to duration and severity:
• The cortical plate of the bone in mandible is • Acute
dense, so it takes longer time for sinus • Chronic.
438 Textbook of Oral Pathology

Depending upon the presence or absence of the medullary spaces, with subsequent necrosis
suppuration. of variable amount of bone.
Suppurative Clinical Features
• Acute suppurative osteomyelitis
In early acute suppurative osteomyelitis—It has
• Chronic suppurative osteomyelitis
rapid onset and course, severe pain, paresthesia
• Primary
or anesthesia of the mental nerve. At this stage
• Secondary
the process is truly intra-medullary, therefore
• Infantile osteomyelitis
swelling is absent, teeth are not mobile and
Nonsuppurative fistulae are not present.
• Chronic nonsuppurative
– Focal sclerosing In established suppurative osteomyelitis—In this
– Diffuse sclerosing type there is deep intense pain, anorexia, fetid
• Radiation osteomyelitis oral odor, malaise and fever, regional lympha-
• Garre’s sclerosing osteomyelitis denopathy. There is also soreness of involved
• Osteomyelitis due to specific infection: teeth which become loose within 10 to 14 days.
– Actinomycosis Pus exudes around the gingival sulcus or
– Tuberculosis through mucosal and cutaneous fistula. There is
– Syphilis firm cellulitis of cheek and abscess formation
with localized warmth and tenderness on
Clinical Staging of Osteomyelitis palpation. The patient feels toxic and
dehydrated.
• Initial stage—Spontaneous pain (localized).
• Acute stage (suppurative stage)—In this stage,
there is severe pain, soreness and looseness
of the involved teeth.
– Early acute stage—In reference to the
involved tooth, progressive sensitivity of
the adjacent teeth to percussion and pain
of the involved side.
– Late acute stage—Paraesthesia or anesthesia
of the lip region supplied by the mental
nerve. Other systemic symptoms can
occur.
• Osteonecrotic stage—Diminished spontaneous
pain, abscess formation and pus discharge.
• Sequestrum stage—Lack of symptoms
sequestrum formation visible on the
radiograph.
Figure 21.9: Necrotic bone seen in case of osteomyelitis

Acute Suppurative Osteomyelitis Histopathological Features

It is serious sequelae of periapical infection there It usually consists of necrotic bone. The
is often a diffuse spread of infection throughout medullary spaces are filled with inflammatory
 Sequel of Pulpal Infection 439

exudate that may or may not progress to the Acute Subperiosteal Osteomyelitis
actual formation of pus. The inflammatory cells
Pathogenesis
are chiefly neutrophilic polymorphonuclear
leukocytes, but may show occasional Mandibular periapical abscess develops close to
lymphocytes and plasma cells. cortex → rapidly ruptures through cortex →
invades subperiosteal space → elevation of
periosteum as pus pools below it → stripping of
more and more periosteum → local necrosis →
resorption of cortical plate → multiple sinus
formation.

Clinical Features
Patients usually complain of severe pain.
Intraoral and extraoral swelling and redness.
There is presence of regional lymphadenopathy.
Involved teeth are mildly sensitive to percussion.
Multiple extraoral and intraoral sinuses are
present.

Infantile Osteomyelitis
Figure 21.10: Suppurative osteomyelitis showing
inflammatory cells It is a rare type of osteomyelitis seen in infants
few weeks after the birth. It usually involves the
The osteoblastic rimming of the bony
maxilla.
trabeculae is generally destroyed. Depending
upon the duration or the process, the trabeculae Route of Infection
may loose their viability and begin to undergo
slow resorption. The periphery of the bone and • Hematogenous route—Infantile osteomyelitis is
haversian canals contains necrotic debris with usually transferred through the
acute inflammatory infiltrate. hematogenous route.
• Trauma—Prenatal trauma of oral mucosa
from obstetrician’s finger.
• Infection—Infection from mucous bulb used
to clear the airway immediately after birth.
• Infected nipple—Infected human or artificial
nipple.

Clinical Features
It is more common in maxilla due to hemato-
genous route. The infection is thought to arise in
maxillary antrum or lacrimal sac. It appears to
center in the region of first deciduous molars and
Figure 21.11: Acute suppurative osteomyelitis showing
the adjacent portion of maxilla, although it may
inflammatory infiltrate (Courtesy: Dr Sangamesh Halawar) involve the inferior aspect of the orbit.
440 Textbook of Oral Pathology

There is fever, anorexia and dehydration. In Intraorally and extraorally sinus develops
some cases, convulsions and vomiting may intermittentia and drains small amount of pus
occur. There is redness and edema of eyelids, and then gradually heals. Sinus extends from
alveolar bone and palate of the affected side. medullary bone, through cortical plate, to mucus
Intracanthal swelling, palpebral edema, membrane or skin. Sinus may be at a
conjunctivitis and proptosis may result. Maxilla considerable distance from the offending
on affected side is swollen both buccally and infection. Painless unless there is an acute or sub-
palatally, especially in the molar region. Sinus acute exacerbation.
develops and discharges pus intraorally and
extraorally. Histopathological Features
Complications may occur like TMJ infection There is significant soft tissue component which
and devitalization of adjacent tooth germs and consist of inflamed fibrous connective tissue in
may occur. areas of inter-trabecular bone. Scattered
Chronic Suppurative Osteomyelitis sequestra and pockets of abscess formation are
common.
Chronic osteomyelitis develops without initial
acute stage, if the virulence is of low grade.
Chronic osteomyelitis is persistent abscess of the
bone that is characterized by usual complex
inflammatory process including necrosis of
mineralized and marrow tissues, suppuration,
resorption sclerosis and hyperplasia.

Clinical Features
Virulence of the microorganism is low and the
host resistance is high. This type is not preceded
by an episode of acute symptoms. It has
insidious onset with slight pain, slow increase
in jaw size and a gradual development of
Figure 21.13: Chronic suppurative osteomyelitis seen as
sequestra without fistula. Local tenderness and
scattered sequestra and pockets of abscess formation
swelling develop over the bone in the area of
abscess. Sclerosing Osteomyelitis
Diffuse Sclerosing
Reactive proliferation of bone is the primary
response in diffuse sclerosing osteomyelitis due
to a balance between the virulence of infection
and resistance of host. It is analogous to focal
form of the disease. It occurs due to low grade
infection.

Clinical Features

It can occur at any age but most common in older

Figure 21.12: Exposed bone seen clinically as sequestra persons. Most cases reported in blacks and in
 Sequel of Pulpal Infection 441

female. It is common especially in edentulous

mandibular jaw.
Symptoms are very mild to absent. During
the period of growth the patient complains of
pain and tenderness. Pain persists for few weeks
to months to even years. Jaws may be slightly
enlarged on the affected side.

Laboratory Finding
Increased ESR

Histopathological Features Figure 21.14: Garre’s Osteomyelitis with mandibular

first molar
It shows dense irregular trabeculae of bone, some
of which are bordered by an active layer of called as osteomyelitis with proliferative
osteoblasts. The bone, in some lesions, shows a periostitis. It is characterized by formation of hard
pronounced ‘mosaic’ pattern, indicative of bony swelling at the periphery of the jaw. It is
repeated periods of resorption followed by essentially a periosteal osteosclerosis analogous
repair. The haversian canals are scattered with to the endosteal sclerosis of chronic, focal and
little marrow spaces. The soft tissue between the diffuse sclerosing osteomyelitis. For the lesion to
individual trabeculae is fibrous and show develop following conditions should be satisfied.
proliferative fibroblasts and occasional small • The periosteum must possess high potential
capillaries focal collections of lymphocytes and for osteoblastic activity.
plasma cells. Polymorphonuclear leukocytes • Mild infection serve as a stimulus.
may be present, if the lesion is undergoing an • Fine balance should be maintained between
acute phase. the resistance of host and number and
virulence of organisms.
Focal Sclerosing
Clinical Features
It is nonsuppurative inflammatory condition
It occurs mainly below 30 years; males are
often seen in dentulous jaw. When the resistance
affected more commonly than females. Most
of the alveolar bone to odontogenic infection is frequently involve the anterior surface of tibia
high or the virulence of the organisms is low, a
and femur. Mandible is affected more commonly
chronic condition characterized by the formation
than maxilla. It commonly occurs at the inferior
of focal areas of sclerosis around the roots of the border of mandible, in first molar region. It is
teeth can possibly result. It occurs around the
presented as hard non-tender swelling with
roots of molars with large carious lesion in young
medial and lateral expansion of jaw.
persons. Mass varies in size from 1or 2 cm to the
Histopathologically there is dense bony
involvement of the entire length on the affected
trabeculae with very little fibrous tissue seen.
side. Cortex may become 2 to 3 cm thick. It may
become secondarily infected and cause
Garre’s Osteomyelitis
considerable discomfort. Lymphadenopathy,
It is also called as ‘proliferative periostitis’. It was hyperpyrexia and leukocytosis are common
first described by Carl Garre in 1893. It is also findings.
442 Textbook of Oral Pathology

Histopathological Features Clinical Features

The supracortical and sub-periosteal mass is It occurs with a triad of radiation, trauma and
composed of much reactive new bone and infection. Effective response to infection becomes
osteoid tissue, with osteoblasts bordering many markedly diminished. It is common in mandible
of the trabeculae. These trabeculae often are than maxilla as irradiation is often directed to
oriented perpendicular to the cortex, with the mandible.
trabeculae arranged parallel to each other or It is common in males due to their probable
show a retiform pattern. The connective tissue involvement in traumatic events and increased
between the bony trabeculae is rather fibrous and risk of oral cancer. Intense pain and facial fistula
shows a diffuse or patchy sprinkling of develop from the subperiosteal tissues. Spread
lymphocytes and plasma cells. The periosteal is diffuse and throughout with signs of
reaction is the result of the infection from the inflammation and swelling.
carious tooth perforating its usually as attenuated
and stimulate the periosteum, rather than Management
producing the usual suppurative periostitis. It is directed at control of infection and penicillin
is the antibiotic of choice.
Radiation Osteomyelitis Investigations carried out in case of osteomyelitis
It is an infection of the irradiated bone. Gram staining, culture and sensitivity, WBC
count and complete hemogram, blood sugar,
Etiopathogenesis mantoux test, radiographs, scintigraphy and
computerized tomography.
After exposure to radiation (40 to 80 Gy), bone
undergoes marked decreased in vascularity.
Management of Osteomyelitis
Such bone has poor defensive process and
susceptible to traumatic injury. It draws infection Antibiotics Therapy
from extraction wound and infected pulp, severe
Empiric therapy
periodontitis and denture stomatitis. It will cause
• Regimen I
death of bone cells and result in progressive – Aqueous penicillin 2 million units, IV, 4
obliterative arteritis. This results in aseptic hourly.
necrosis of the portion of bone directly in beam – Oxacillin 1 gm, IV, 4 hourly.
of radiation, with compromised vascularity in the • Regimen II — if the patient is asymptomatic
adjacent bone. after 48 to 72 hours
Pathogenic organisms are introduced into – Penicillin V 500 mg, 6 hourly
this irradiated bone through odontogenic – Dicloxacillin 250 mg, 4 hourly, for an
infections, compound fractures of the jaws and additional 2 to 4 weeks.
mucosal lacerations. The organisms most
commonly found are Staphylococcus aureus, Definitive Therapy
Staphylococcus epidermidis. Higher incidences in Culture and sensitivity testing is performed and
jaw are recorded due to higher degree of antibiotic therapy is then modified accordingly,
infections and frequent trauma to these bones. particularly if the infection appears to be
 Sequel of Pulpal Infection 443

refractory after the treatment is instituted. If evacuated, drains are placed. The consistency,
favorable response occurs, no change is color and odor of the pus may provide important
indicated. clues to the diagnosis and initial treatment.
Initial therapy with gram stain result
Irrigation and debridement of the necrotic areas—
If the stain shows predominance of gram –ve
Thorough debridement of the affected areas
cocci in clumps suggestive of staphylococcus, should be carried out. Debride any foreign
penicillinase resistant penicillin alone is advised.
bodies, necrotic tissue or sequestra. These areas
• An initial course of oxacillin 1gm IV after
may be irrigated with hydrogen peroxide and
every 4 hours. saline.
• If improvement occurs then orally
dicloxacillin 250 mg after every 4 hours Extraction of offending tooth—Extraction of carious
should be administered. teeth with periapical infection, should be done.
If the stained slide shows predominately It should be carried out to remove the source of
gram +ve rods, then an established working infection from the oral cavity.
diagnosis of anaerobic infection (bacteroides) is Supportive therapy—Patients is suffering from
made. osteomyelitis required adequate rehydration in
• An aqueous penicillin 2 million units may be the form of fluids. Rich nutritional diet should
given IV, after every 4 hours. be given. Multivitamin supplements should be
• An oral antibiotic like penicillin V may be given.
started after 48 to 72 hours.
Sequestrectomy—It is the removal of sequestra
If patient is allergic to penicillin, which are small pieces of necrotic bone that are
• Clindamycin is recommended. In acute phase avascular and harbor microorganisms.
600 mg, after every 6 hours should be given.
Saucerization—It means excision of the margins
Once the acute phase is over orally 450 mg
may be given, after every 4 hours. of necrotic bone, overlying the focus of
osteomyelitis which allows better visualization
• Cephalosporin or cefazolin is the third drug
of sequestra and excision of margins of the
of choice in case of allergy to penicillin.
Cefazolin 500 mg, IV/ IM, after every 8 hours. affected bone.
Once acute symptoms have subsided, Decortication—Decortication of mandible refers
cephalexin 500 mg, after every 6 hours is to removal of the chronically infected and inferior
recommended. cortical plates, 1-2 cm beyond the area of
• As a fourth choice erythromycin is also useful involvement. Thus, access is provided to the
2 gm IV after every 6 hours. When acute stage medullary cavity.
is resolved then 500 mg is given orally, after
Hyperbaric oxygen therapy—In this patient is
every 6 hours.
placed in multiplace/monoplace chamber and
Incision and drainage—When early diagnosis is a concentration of 100% O2 is given. Duration of
made, drainage of the fluctuant areas should be treatment is of 1.5 to 2 hours for 5 to 6 days in a
carried out under antibiotic cover. After pus is week, for a total of 60 treatments.
444 Textbook of Oral Pathology

CHAPTER
Consequences of
22 Oral Infection

Facial infections are relatively a common through facial planes of head and neck there by
presentation to both, general medical and dental compromise the vital structures in these regions,
practice. Most originate in superficial structures e.g. intracranial odontogenic infection leading to
(skin, subcutaneous tissue, etc.) and are often necrotizing fascitis of head and neck. Most
easily diagnosed and treated. odontogenic infections can be managed
Infections originating in deeper structures can successfully with minimal complications. The
be severe, rapidly progressive and may cause key to successful management is sound surgical
prolonged morbidity, long term complications principles.
as well as potentially endanger life. Efficient
treatment requires accurate diagnosis, early Host Defense Mechanism
aggressive medical treatment and in most cases, The relationship between the host and microbes
urgent decisive surgical management. is a dynamic one. Usually, host resistance is the
Infection is a clinicopathological entity dominant factor. On the other hand, when the
involving invasion of the body by pathogenic host resistance is lowered, microbes predominate
microorganisms and reaction of the tissues to and clinical infection occurs. In establishing the
microorganisms and their toxins. Soft tissue presence of infection there is interaction between
infections of head and neck are commonly three viz. factors host, environment and
encountered in routine practice in dentistry. microbes.
These infections may be odontogenic or non- A compromised patient is more likely to have
odontogenic in origin. Once the infection extends infection and this infection can rapidly acquire
past the apex of the tooth the pathophysiology a serious form. Hence, patient history is more
of the infectious process can vary, depending important so as to recognize the patient ability
upon the number and virulence of the to defend himself against the infection. The
microorganisms, the host resistance and adverse relationship between the host and the
anatomic geography. infectious microorganism can be best understood
These odontogenic infections can become by imagining a balance on which the pathologic
severe, life threatening facial space infections. attribute of microbes are weighed against the
These infections have the potential to spread protective mechanisms of the host.
 Consequences of Oral Infection 445

Body defends against the microbial invasion synthesize IgA, some IgE and small amounts of
by three major defenses local defense, cellular IgG and IgM. The immunoglobulin in secretion
defense and humoral defense. The together with the local protective factor
microorganisms on the other hand use two constitutes mucosal immune system. IgA inhibits
weapons in this battle, i.e. virulence and number the colonization of mucosal epithelial cells by
of microbes. occupying potential sites of attachment. They
also neutralize the toxins and viruses.
Host Defense
Local Defence Humoral Factor

Epithelial lining—It is the first line of defense. It Immunoglobulin—The host synthesizes specific
physically hinders the penetration of surface protein molecules, with antibody activity, in
bacteria into deeper tissues. When there is a break response to antigenic stimulation. These proteins
in the continuity of the anatomic barriers, the are derived from B lymphocytes and plasma cells
microbes find an easy way into deeper tissues. and are called as IgA, IgM, IgD, IgG and IgE.
In the oral cavity, the bacterias find way through Secretory IgA and IgE antibodies directly
deep periodontal pockets and necrotic dental prevent the attachment of pathogenic bacteria
pulp. or parasites to epithelial receptor cells, which is
very crucial in preventing colonization and
Secretory and drainage system—This system assists
host defense by physical and chemical action. subsequent infection. By direct action, antibodies
can neutralize viruses and microbial toxins.
The mucociliary activity, peristaltic motion and
flushing action-all result in drainage and Complement system—This consists of group of
mechanical removal of bacteria. Obstruction and serum proteins, which by a series of reactions
impairment of drainage almost always result in produce and release byproducts whose functions
infection. Secretion of the oral cavity i.e. saliva are to initiate inflammatory reaction, regulate
and swallowing mechanism helps to remove the and enhance phagocyte function and attacks the
food particles and also prevents stagnation of bacterial cell membrane. The activation of
microbial flora at one site. Chemical constitution complement system by either pathway results
of saliva changes as the pH changes. This favors in generation of cytolytic activity, chemotatic
the growth of bacteria. Hence, the pH of saliva factor and mediator, anaphylactic toxin,
should be maintained to control the bacterial opsonising and phagocyte enhancing activity
growth. and immune adhered activity
Microbial floral interference—This refers to
Cellular Component
inhibitory effect exerted by one microorganism
on the growth and population of other. The Polymorphonuclear leukocytes—PMNs are the first
normal flora of oral cavity is able to discourage phagocytes to respond to the chemotatic factor
the growth of new species of bacteria. elaborated by complement system. Thus, they
Mucosal immune system—Beneath the mucosal predominate in acute infection, which persists
epithelium, lamina propria and connective tissue for about one week. Their function is to clear up
contain a large number of immunocompetent the battle field.
cells. The B lymphocytes and plasma cells with Lymphocyte—It consists of two types of cells- B
the help of certain T lymphocytes locally lymphocyte and T lymphocyte. B lymphocytes
446 Textbook of Oral Pathology

are responsible primarily for combating • To enter the host

extracellular pathogens, particularly those • To multiply in host tissues
bacteria that are not able to survive inside • To resist host defense
phagocyte or other cells which secrete specific • To damage the host.
antibodies against which, the host has previously
been exposed. T cells, on the other hand, are Virulence
responsible for combating intracellular It is the term applied to the properties in
pathogens which are primarily viruses and those particular strain microorganisms. Enhancement
bacteria that can survive within phagocyte cell. of virulence is known as exaltation and reduction
In addition, T cells are responsible for of virulence is known as attenuation. Virulence
surveillance against the proliferation of tumor is the sum total of several determinants such as
cells. These complex functions of T cells are adhesion, toxigenicity and communicability.
described in general as cell mediated immunity. Adhesion—The initial event is the attachment of
Both T cells and B cells produce lymphokinin bacteria to the body surface. This attachment is
hormone that plays several vital roles in not a chance event, but it is a specific reaction
resistance to infection. Primarily they regulate between the surface receptors on the epithelial
the action of phagocytic cells. cells and the adhesive structures on the surface
• Macrophage inhibiting factor—Inhibits the of the bacteria.
random movement of macrophages away Invasiveness—This refers to the ability of a
from the site of infection, keeping them where pathogen to spread in the host tissue after
they are needed. establishing infection. Highly invasive pathogens
• Interferon—Another lymphokine enhances cause generalized infections and less invasive
lysis of bacteria by phagocytes. ones causes localized infections.
• Mitogenic factor—Secreted by T cells;
Toxigenicity—Bacteria produce two types of
stimulates B cell proliferation enabling the
toxins, i.e. exotoxins and endotoxins exotoxins
body count to mount an antibody response
are heat labile proteins excreted by certain
to antigenic stimulation. bacteria and they diffuse readily in to the
Thus lymphocytes are responsible for surrounding tissues. Endotoxins, are heat stable
modulation and control of phagocyte cells. polysaccharide protein lipid complexes, which
form an integral part of the cell wall of gram-
Microbial Factors negative bacteria.
Pathogenicity
Effect of Infection on Host
Pathogenicity is the ability of a microbial species
Infectious agents initiate, in the host, a series of
to produce disease. It involves various
reactions that are collectively called as
mechanisms which can be of following types:
‘inflammatory reaction’. This response results in
• Subversion of host cell metabolism
generation and release of mediators,
• Production of host cell lytic factor
microvascular changes and mobilization and
• Production of potent toxins which
activation of leukocytes, all designed to eliminate
systemically act on the host. the infectious pathogens and repair tissue injury.
To set up an infectious process, a micro- Therefore, these reactions are protective in
organism must be able to do the following: nature.
 Consequences of Oral Infection 447

Direct injury to the host cells, enhancement alveolar bone consists of interconnecting marrow
of the parasite’s invasiveness and amplification spaces delimited by unyielding calcified tissue,
of these effects occur due to neutralization of host all of which are enclosed circumferentially by
defence. There is fever, endotoxic shock and layers of cortical bone of varying thickness. The
intravascular coagulation. The pathogen or its invasion of bacteria from marrow space triggers
products, in certain situations, may combine with the process of inflammation and cause some
antibodies or sensitized mononuclear leukocytes consequences of edema, ischemia, necrosis and
to produce harmful immunologic effects called isolation from systemic circulation and immune
as ‘hypersensitivity reaction’. system. This is the process by which bacteria
survive within the bone of jaw. The path of least
Compromised host—It is a person whose defense
resistance is along the medullary spaces. This
mechanisms have been lowered as a result of
explains the ability of odontogenic infection such
diabetes, tuberculosis, rheumatic fever,
as osteomyelitis, to spread for great distances
malignancy, radiation therapy, use of therapeutic
along the jaw before they erode through the
immunosuppressive drug or antibiotics,
cortical plate. Another factor in the spread of
extensive skin burns, genetic deficiency of
infection within the bone is the thickness of the
immune system and malnutrition.
cortical plate on either side. In maxilla, the buccal
cortical plate is thinner as compared to the
Oral Microflora
palatal; hence, the abscesses erode through the
The microbial flora that is characteristic of a buccal cortical plate. In mandible the buccal plate
particular site in majority of the population is of bone over the bicuspid and molar is thick,
referred as the indigenous flora or normal flora. while the lingual plate is thinner; hence, the
The oral microflora at birth is aerobic and after infection from lower molar teeth spread
eruption of teeth, anaerobes are also established. lingually, eroding through the lingual cortical
The intrinsic source of microorganisms in the oral plate and then spreads into the soft tissues of
cavity is gingival crevice material, pus cells and floor of mouth.
epithelial cells undergoing degradation and
Spread of infection within the soft tissues—Muscle,
salivary component.
fascia and bone function as anatomic barriers to
spread of infection within the soft tissues because
SPREAD OF INFECTION
muscles have tight dense capsule surrounding
Infection may spread by three routes: them and are well vascularized. They are more
susceptible to bacterial invasion than loose
Direct Invasion or Extension connective tissue surrounding them. Muscles
and fascia of head and neck are having potential
It is the propagation of an infection between the
space within which the invading bacteria can
layers of fascia; from one area to fascia of another
propagate for a while. Thus contain various
area. It can occur secondary to distension and
organs including nerves and blood vessels,
pressure, forcing the contents through facial
salivary glands, lymph nodes and fat with
layers by hydrostatic pressure- secondary to
surrounding loose fibrous connective tissue.
movement and function of the part.
These are the pathways around which infections
Spread of infection within the bone—The bone of can spread.
the alveolar process is quite similar to the dental There are various stages for spread of
structure, in terms of response to infection. The infection through the soft tissues:
448 Textbook of Oral Pathology

• Stage of inoculation—If the infection does not process of lymphocyte activation ceases and the
perforate the oral mucosa, it will enter the nodes will return to their previous size.
fascial spaces of head and neck region. For
several days after their initial entry into the Spread by Blood Vessels
soft tissues the bacteria will grow without
The hematogenous spread of head and neck soft
triggering an intense inflammatory response.
tissue infection may result in very serious
A mild edema may be present in the affected
complications such as bacteremia, resulting in
area and the patient may experience soreness.
distant infections involving the heart valve,
This minor swelling can later become soft and
cardiac, vascular and orthopedic prosthesis. The
doughy on palpation. This stage of infection
microorganisms entering the vascular system
responds quite well to either extraction or
may also result in thrombophlebitis, problems
endodontic treatment of the offending tooth.
particularly involving the intracranial sinus, e.g.
• Stage of cellulitis—As the bacteria that have cavernous sinus thrombosis. Another serious
inoculated the soft tissue space multiply, they consequence is generalized septicemia, which
elaborate toxins and metabolic byproducts can result in shock and death.
due to which, an intense inflammatory
response is triggered. PATHOPHYSIOLOGY OF INFECTION
• Stage of abscess—As the cellular phase of
The body’s response to infectious agent is
inflammation progresses, inflammatory cells
inflammatory, which is essentially a protective
consisting of many polymorphonuclear
phenomenon. Hence, the cardinal signs of
leukocytes are drawn to the area of infection
inflammation are present, to some degree, in
by various lymphokines, including
nearly all patients with infection.
leukotoxin. These phagocytes engulf the
• Redness (rubor) is seen when the infection is
bacteria and digest them. Other lymphokines
cause necrosis of the surrounding soft tissues. close to the tissue surface, which is secondary
to the intense hyperemia caused by increased
• Resolution—One or two days after the vasodilation of arterioles.
beginning of infection, specific immune • Calor or heat is due to inflow of relatively
system consisting of antibiotics and activated warm blood from deeper tissues, increased
T lymphocytes come into play. This is called velocity of blood flow and increased rate of
as ‘round cell infiltration’, consisting of metabolism.
lymphocyte macrophages and plasma cells. • Dolor or pain results from pressure on
Macrophages are efficient in phagocytosis of sensory nerve endings, from distension of
bacteria and necrotic debris and are able to tissues caused by edema or spread of
dispose off this material.
infection. Release of substance like kinins,
histamines or bradykinin is also responsible
Spread by Lymphatic System for pain. It is the most universal sign of
The face and neck infections result in regional infection.
lymph node enlargement, which results from • Swelling accompanies infection, unless the
proliferation of lymphocytes and histocytes in infection is confined to bone which cannot
response to antigenic stimulation. If the antigen swell. It is due to the accumulation of fluid,
is catabolized and becomes non antigenic, the exudate or pus.
 Consequences of Oral Infection 449

• Loss of function is another sign of infection. of tenderness on palpation. Tissues are grossly
A patient immobilizes the painful part in the edematous. There is marked induration, hence,
most comfortable position he can find. Hence, tissues are firm to hard an palpation. Tissues are
when the masticatory muscles are involved, often discolored; temperature is elevated with
there is limitation of jaw movement. malaise and lethargy. Usually massive cellulitis
• Fever occurs in some cases, which reflect a will ultimately suppurate, particularly if bacteria
non-specific physiologic response of host to are staphylococcal.
tissue injury. This injury results in increase Depending upon the location and proximity
of substance called pyrogen from endogenous to anatomic structures that guide the progress,
(injured tissue) and exogenous source the pus may evacuate into nose, maxillary sinus,
(infecting agent). In clinical fever, it appears oral vestibule, floor of mouth, infratemporal fossa
and into fascial spaces.
that the hypothalamic regulating center is
stimulated by endogenous pyrogen, which is
activated by bacterial endotoxin release from
granulocytes, monocytes and macrophages.

Cellulitis
It is also called ‘Phlegmon’. Occasionally, the
infectious process progresses out of the bone,
despite the use of supportive therapy and the
patient develops cellulites, either in vestibular
region or extraorally. It is a potential
complication of acute dental infection.

Definition Figure 22.1: Patient having cellulitis showing swelling on

left side face which involving submandibular space,
Cellulitis may be defined as a non-suppurative pterygomandibular space
inflammation of the subcutaneous tissues
extending along the connective tissue planes and Infections arising in maxilla perforate the
across the intercellular spaces. outer cortical layer of bone, above the buccinators
attachment and, cause swelling of upper half of
face. If infection in mandible perforates the outer
Bacteriology
cortical palate, below the buccinator attachment,
The alpha hemolytic streptococci are the classic there is diffuse swelling of the lower half of face,
etiologic agent. They produce enzymes like which then spreads superiorly as well as
streptokinase and hyaluronidase. These enzymes cervically. If maxillary tooth is involved, there
break down fibrin, connective tissue ground may be redness of eye.
substance and cellular debris, thus facilitating Laboratory Findings
rapid spread of bacterial invaders.
ESR and white cell count are raised.
Clinical Features Histopathological Features
There is widespread swelling, redness and pain Diffuse exudation of polymorphonuclear
without definite localization (Fig. 22.1). Presence leukocytes and occasional lymphocytes, with
450 Textbook of Oral Pathology

considerable serous fluid and fibrin, causing teeth into the floor of mouth. The 2nd and 3rd
separation of connective tissue or muscle fibers. molars are the teeth most commonly involved.
It presents only a nonspecific picture of diffuse Trauma—It may also be caused by oral soft tissue
acute inflammation. laceration and punctured wounds of the oral
floor.
Management
Sialadenitis—Submandibular gland sialadenitis
Surgical incision and drainage—It is performed and infected malignancy may be sometimes
when the presence of pus is diagnosed. In case contributory to Ludwig’s angina.
of large cellulites, a superficial erythematous spot
develops, which is pathognomonic of pus near Calculi—Salivary calculi or from intravenous
the superficial surface. These superficial fluctuant injection of the internal jugular vein, especially
areas can be incised and drained under local in drug abusers.
anesthesia. Usually ring block of peripheral skin, Osteomyelitis—Osteomyelitis in compound
as a wheel is made for skin anesthesia. Knife is mandibular fracture.
introduced in the most inferior portion of the
fluctuant area. A small sinus forcep is introduced Bacteriology
in the wound, opened in several directions and
Streptococci are the most commonly reported
pus is drained. A rubber drain is placed in the
organism from the culture. Other
deepest portion of the wound, so that just 12 cm
microorganisms are a hemolytic streptococci,
lie above the source of the skin, where it is
Bacteroides, Klebsiella, Fusiform bacilli and E.
sutured. A large dressing is applied. When no
coli.
superficial spot is present, fluctuance is more
difficult to determine, particularly if deep pus is
Clinical Features
suspected. Usually, extraction of the offending
tooth and specific antibiotic cover bring about There are three typical appearances of Ludwig’s
resolution of the process. angina.
First—It is characterized by brawny indurations.
Ludwig’s Angina
Tissues are board like and do not pit on pressure.
It is a condition which was first described by No fluctuance is present. The tissues may become
Ludwig in 1936. The word angina means gangrenous and when cut, they have a peculiar
sensation of choking or suffocation. It is the most lifeless appearance. A sharp limitation is present
commonly encountered neck space infection. between the infected tissues and surrounding
normal tissues.
Definition
Second—Three facial spaces are involved
This condition may be defined as an bilaterally, i.e. submandibular, submental and
overwhelming, rapidly spreading, septic sublingual. If the involvement is not bilateral, the
cellulitis involving submandibular, submental infection is not considered a typical Ludwig’s
and sublingual spaces bilaterally. angina (Fig. 22.2).
Third—The mouth is open and the tongue is lifted
Etiology
upwards and backwards, so that it is pushed
Odontogenic infection—It is usually an extension against the roof of the mouth and the posterior
of odontogenic infection from mandibular molar pharyngeal wall; when this occurs, acute
 Consequences of Oral Infection 451

respiratory obstruction is likely to occur. Two There is an intense pain on tongue movement
large potential spaces at the base of the tongue and the patient may be severely dehydrated,
are involved, i.e. submental and sublingual. owing to inability to take anything by mouth. If
the swelling has spread into the pterygoid region,
then there is difficulty in opening the mouth.

Fatal Complications
Respiratory obstruction—The infection of
Ludwig’s angina tends to spread through the
connective tissues which cover the small muscles
of the larynx and between the muscles of the floor
of mouth. The epiglottis and larynx become
edematous along with the posterior aspect of the
tongue. The tongue gets elevated and gets
pressed upward and backward, causing pressure
on the larynx. Therefore, dyspnea occurs in
paroxysm. Ultimately, death occurs due to
respiratory embarrassment.

Generalized Septicemia
Erosion of the carotid artery—Late spread of
infection to lateral pharyngeal space can also
cause erosion of the carotid artery.
Figure 22.2: Patient of Ludwig’s angina showing Cavernous sinus thrombosis with subsequent
typical open mouth appearance meningitis may be sequelae of it.
Swelling is firm, painful and diffused, with Others—Mediastinum extension, pharyn-
no sign of localization. Floor of mouth appears gomaxillary space extension, osteomyelitis and
erythematous and edematous. Stiffness in tongue airway obstruction.
movement generally develops. The patient
develops a toxic condition and speech becomes Prevention
impaired. Larynx and glottis become edematous.
As the disease continues the swelling starts Regular visits to the dentist and prompt
involving the neck. treatment of oral/dental infections can prevent
The patient always has fever and there is the conditions that increase the risk of developing
considerable salivation, as the patient is unable Ludwig's angina.
to swallow. There are chills accompanied with
Laboratory Findings
fever. There is an inability to swallow and to eat.
Respiratory distress is common. There is also A moderate leukocytosis is found. Fusiform
neck pain, redness of neck, fever, weakness, bacilli and spiral forms, various staphylococci,
fatigue, excessive tiredness, confusion or other diphtheria and may other microorganisms have
mental changes, difficult breathing and earache. been cultured on different occasions.
452 Textbook of Oral Pathology

Management Etiology
Intense and prolonged antibiotic therapy—Penicillin Dental Causes
is to be administered IM or IV, in high doses, • Periapical infection from the teeth—It may follow
because it is the empirical antibiotic of choice and dental infection particularly from upper
the oral flora, including most of anaerobes, are molar and premolar teeth. The anatomic
sensitive to it. Recently, combination of proximity of the roots of the maxillary
gentamicin and cloxacillin has been proved bicuspids and molar teeth to the floor of the
successful. sinus leads to potential infection of the sinus
Establishment and maintenance of an adequate airway by direct extension of an apical abscess.
are the essentials of therapy—Tracheostomy is a • Oroantral fistula—The accidental opening in
routine procedure, but it is often difficult to the floor of the maxillary sinus during dental
perform in the late stages. Attempt at blind extraction is called as oroantral opening.
intubations is often time consuming. Recently, • Periodontitis—It may spread from a deep
successful intubations with fibreoptic pocket of marginal periodontitis.
laryngoscope have been introduced as a • Traumatic—Injury of facial bones especially
worthwhile technique in the therapy of Ludwig’s nasal bones and malar bones.
angina. In the late stage, cricothyrotomy may be • Dental material in the antrum—Perforation of
endodontic filler substance. If root canal is
performed as an emergency procedure.
overfilled then there are more chances of
Incision and drainage—It is done for the release of gutta purcha point to be inserted into the
tissue tension. A horizontal incision, midway maxillary sinus.
between the chin and hyoid bone, is a classic • Implant—Implants are used in the upper
approach to surgical drainage of Ludwig’s edentulous jaw to aid the retention of
angina. It may be carried out under local dentures or bridges or replace missing teeth.
anesthesia. Implants are also used when there is
insufficiency of bone to support the denture.
Supportive therapy—Parenteral hydration, high
In these cases as bone is thin implant can
protein diet and vitamin supplements. penetrate the nose or sinus.
Extraction of offending tooth. • Infected dental cyst—Cyst which have become
infected and involve the maxillary sinus can
Maxillary Sinusitis also cause sinusitis.
Inflammation of the mucosa of the paranasal
sinuses is referred to as sinusitis. When maxillary Nondental Causes
sinus is involved it is called as maxillary sinusitis. Mechanical Obstruction of Ostium
When all the sinuses are involved it is called a
• Common cold—It is most common cause of
pansinusitis.
mechanical obstruction of ostium. It will
produce inflammatory edema of the nasal
Types
mucosa which obstruct the antronasal duct
• Acute and cause mucus to accumulate in the sinus.
• Subacute Trapped mucus becomes secondarily infected
• Chronic. by local commensal bacteria.
 Consequences of Oral Infection 453

• Allergic rhinitis—It may cause maxillary Nasal discharge is watery in the beginning
discomfort due to edema around the ostium but later becomes mucopurulent. The nasal
and retention of secretion. mucosa of the anterior nares may show
• Other conditions—Deviated nasal septum, reddening and inflammation and there may be
presence of nasal polyp and prolonged presence of pus. In cases of sinusitis from infected
nasotracheal intubation can cause stagnation teeth, the discharge has a foul odor. Tenderness
and relative anaerobiasis. to pressure or swelling over the involved sinus.
Since the anterolateral and posterolateral sinus
Direct bacterial contamination—Infected material walls are thinnest in the area above the tooth root,
may also be introduced directly by jumping or thumb pressure on the cheek here is the best way
hydrosliding feet first into contaminated water to elicit tenderness externally. Premolar and
without holding the nose or during diving, when molars on the affected side may be sensitive to
pressure changes in the nose force nasal secretion percussion. Intraorally, there may be a
into the sinus. mucobuccal swelling, reddening and pain near
Immune deficiency—Sinusitis can occur in the sinus region.
immune deficiency state, like leukemia, Subacute: It is an interim stage between acute and
lymphoma and AIDS. chronic sinusitis. It is devoid of symptoms
associated with acute congestion such as pain
Influenza—It can also occur in influenza when and generalized toxemia. Discharge is usually
bacteria invade as secondary microorganisms. purulent and associated with a nasal voice and
Blood borne infection—It can also occur in some stuffiness. Soreness of throat is common. Patient
cases of blood borne infection. can not sleep well due to cough that irritates him
constantly.
Disease of maxillary sinus—Benign mucosal cyst
Chronic: It develops as a result of neglected and
or tumors of the maxillary sinus can also lead to
overlooked dental focus of infection. The lining
maxillary sinusitis. becomes thicker and irregular. General
symptoms of chronic sinusitis include sense of
Clinical Features tiredness, low grade fever and feeling of being
Acute maxillary sinusitis: The main symptom is unwell. Stuffy sensation over the affected side
severe pain which is constant and localized. Pain of the face is present. Nasal obstruction, nasal
may be felt in the area of eyeball, cheek and discharge and headache are the related
frontal region. Pain may be exacerbated by symptoms.
stooping or lowering the head. Postnasal drip
Lab Findings
causing irritation, stuffiness and nasal discharge.
Pain may be referred to various areas including Elevated leukocyte count is seen.
the teeth, orbit, head and ear. Pain in the teeth
may be referred to the premolars and molars on Histopathological Features
the affected side. Pain is increased by biting on Acute sinusitis: The lining of the maxillary sinus
the affected side but unaffected by drinking hot, may show a typical acute inflammatory infiltrate
cold or sweet fluids. Teeth in the involved side with edema of the connective tissue and often
become sore and painful. Difficulty in breathing hemorrhage. Sometimes squamous metaplasia of
is common. Generalized toxemia develops, i.e. the specialized ciliated columnar epithelium
fever with chills, dizziness, malaise and nausea. occurs.
454 Textbook of Oral Pathology

Chronic sinusitis: The fundamental pathologic mandible. Posteriorly, it extends into the
changes are that of cellular proliferation. The retromandibular region and anteriorly, it ends
mucosal lining of the maxillary sinus shows at the end of the anterior border of ramus. The
remarkable thickening and the development of swelling tends to evert the lobule of ear. The
numerous sinus polyps. Polyps are simply patient complains of pain, which is referred to
hyperplastic granulation tissue with lympho- the ear and accentuated on eating. Diagnosis is
cytes and sometimes plasma cell infiltration. made eversion of lobule of ear, no evidence of
Tissues covered by ciliated columnar epithelium trismus. Possible escape of pus from parotid duct,
tend to fill the sinus and obliterate it and in some when the gland is milked. All signs of abscess
cases there is mild lymphocytic infiltration of the formation.
lining tissue with squamous metaplasia of the
epithelium. Infratemporal Space
Extraoral swelling over the region of sigmoid
Management notch and TMJ joint area and intraorally swelling
Removal of the cause of dental infection, is usually in the tuberosity area. The patient may
antibiotic, analgesic and anti-inflammatory exhibit trismus and sometimes swelling of
drugs, Nasal decongestant, stem inhalation, eyelids, if there is involvement of post-zygomatic
antral lavage, antrostomy and transnasal fossa. Entire cheek may be swollen, if the buccal
endoscopic surgery. space is also involved. The involvement of
pharynx may cause dysphagia and severe pain
Facial Space Infections or a feeling of pressure in the general area of
infection.
Facial spaces are potential spaces situated
between the planes of fascia, which form natural Space for the Body of Mandible
pathways along which the infection may spread.
When infection originates from incisors cuspid
Canine Space or bicuspid teeth, it involves the space of the body
of mandible. There is induration or fluctuation
In this swelling is present just lateral to the nose,
of the labial sulcus, if the outer cortical plate is
obliterating the nasolabial fold. Intraorally,
involved. When the inner cortical plate is
swelling is present in the labial sulcus. Rarely,
involved, the infection is restricted to the floor
palatal swelling is encountered.
of mouth. Infection originating from the molar
teeth and involving the outer cortical plate results
Buccal Space
in swelling in the oral vestibules, if the infection
Swelling is present over maxillary and perforates the bone above the external oblique.
mandibular molar or premolar, if apex is above If the perforation is below the mylohyoid line
the attachment. Facial swelling with little trismus the infection may point in the skin.
is present.
Submental Space
Parotid Space
Chin becomes grossly swollen, quiet firm and
The swelling extends from the level of the erythematous. Slight extraoral swelling just below
zygomatic arch to the lower border of the the chin is evident.
 Consequences of Oral Infection 455

Submandibular Space be marked by severe trismus because of irritation

of masseter and medial pterygoid. It can be so
Carious mandibular molar is usually present. It
severe that the mouth opening is restricted only
produces a swelling near the angle of the jaw,
to half a centimeter. Lateral pharyngeal wall,
which is brawny edematous in appearance
behind the palatine tonsil, is not swollen, which
(Fig. 22.3). After some days, swelling becomes
will help to differentiate between it and lateral
soft and fluctuant. Because of anatomic
pharyngeal space infection.
proximity, there is usually involvement of glands
and nodes, resulting in sialadenitis and Temporal Space
lymphadenitis.
In case of infection with superficial temporal
space, swelling is limited below by the zygomatic
arch and laterally, it is limited by the outline of
superficial and temporal line. When it is
associated with buccal space infection, the
swelling has a characteristic dumble shaped
appearance caused by the lack of swelling over
the zygomatic arch. Due to limited distentibility
of temporal facia, the pain is severe. Trismus is a
common finding. A deep temporal abscess
produces less swelling than one involving the
superficial temporal space. Since it lies deep to
Figure 22.3: Submandibular space infection presented as temporalis muscle, fluctuance is difficult to elicit.
swelling
Pterygomandibular Space
Sublingual Space
There is no external evidence of swelling.
Brawny erythematous tender swelling of the Intraoral examination reveals an anterior bulging
floor of mouth is present. Swelling is close to of half of the soft palate. Deviation of tongue to
mandible and spreads towards midline or the affected side. Severe trismus and difficulty
beyond. Elevation of the tongue may be noted in in swallowing. It must be distinguished from
late cases. Dysphasia and dyspnea may be the peritonsillar abscess. In latter, there is no
related complains.
offending tooth and trismus is less severe.
Masticator Space
Lateral Pharyngeal Space
The swelling may be either external or internal,
If the infection is confined to the anterior
or both. The external swelling consists of brawny
compartment external swelling occurs anterior
induration over the ramus and angle of
mandible. Internal swelling may predominate in to the sternocleidomas-toid muscle. The swelling
some cases. Such swelling involves the is first seen at the angle of mandible and in the
sublingual region and the pharyngeal wall. The submandibular region. There is severe pain on
pharyngeal swelling pushes palatine tonsils the affected side of throat. Pain is sometimes
towards midline. Pain is excruciating and often referred to the ears. Infection of space occurs with
radiates to the ear. Dysphagia may be present, abscess formation, which may impinge on
especially when the swelling is internal. pharynx and result in pain while swallowing and
Clinically masticatory space infection is likely to making it impossible. Constitutional symptoms
456 Textbook of Oral Pathology

are usually present along with signs of sepsis. Management

Trismus and pain are seen due to irritation of
It is a medical emergency and prompt
medial pterygoid muscle. Internally, the anterior
intravenous antibiotics should be started, after
part of lateral pharyngeal wall is swollen and
antibiotic sensitivity testing.
pushes the palatine tonsils together, with the soft
palate towards the mid line. With infection of
posterior compartment, the clinical picture is Brain Abscess
marked by septicemia. It can develop from bacteremia associated with
odontogenic infections.
Retropharyngeal Space
These infections are classically seen in children Pathogenesis
less than 5 years. These are generally due to otitis Once the bacterias reach the brain tissues → it
media and pharyngitis. Patient will have pain, results in local cerebritis → inflammatory
dysphagia and nuchal rigidity. There is bulging exudates collects along with degenerative
of posterior pharyngeal wall, which is often more
leucocytes → septic thrombosis of blood vessels
prominent on one side because of adherence of
occurs → cerebral edema develops around the
median raphe or pre-vertebral fascia. Lateral soft
infected area → as the abscess grows, its center
tissue radiographs can be helpful in establishing
becomes pus filled with a capsule of granulation
diagnosis by permitting visualization of widened
tissue at its periphery → as the abscess increases
retropharyngeal space.
in size, it may rupture into the ventricular system
FATAL COMPLICATIONS OF ORAL and the result is usually fatal → if it extends to
INFECTION subarachnoid space, meningitis may develop.

Bacterial Meningitis Clinical Features

It is the most common neurologic complication Headache due to rise in intracranial pressure can
resulting from oral and maxillofacial infections. occur. Nausea, convulsions and vomiting may
In this condition bacteria infect arachnoid pia occur. Dysphagia and visual defects, if it involves
mater and CSF. The infection quickly spreads the temporal lobe. Papilledema and convulsions
from its point of origin, via CSF, to the entire
are common. Hemiparesis, if abscess is on motor
subarachnoid space.
cortex. Confusion and stupor, if abscess is in
frontal lobe. Diagnosis is made by radionuclide
Clinical Features
scanning and CT scanning.
Headache, chills, fever and nausea can occur.
Pain in back and stiffness of neck. Kernig’s and Management
Brudzinski’s signs are positive, seizures,
confusion and stupor. Diagnosis is made by Drainage of the abscess via catheter through a
lumbar puncture and CSF examination. bur hole is the treatment of choice.
Microorganisms responsible—Staphylococcus, Once the size of the abscess is decreased the
Strepto-coccus, pneumococci, Proteus, Klebsiella complete capsule should be excised. Some
and H. influenza are common pathogens causing surgeons prefer to do craniotomy initially and
meningitis secondary to head and neck infection. then excise the entire abscess.
 Consequences of Oral Infection 457

Cavernous Sinus Thrombosis and elevated protein concentration. Blood culture

and sensitivity.
It is one of the most dreaded and life
threatening complication due to intracranial
Management
spread of infection from odontogenic source.
Massive doses of antibiotics, with proper surgical
Etiology intervention at the primary site of infection are
essential. The initial drug of choice is IV
The bacteria can reach cavernous sinus in septic
chloramphenicol 1 gm, after every 6 hours. An
emboli through venous and arterial systems.
attempt should be made to identify the causative
Septic thrombophlebitis of emissary veins can
organism by culture from the source of infection
directly lead to this phenomenon. It can also
or from blood culture, so that precise antibiotic
result from direct extension through skull, by an
sensitivity can be established. Use of anticoagulant
abscess located in deep spaces such as in
therapy to prevent further thrombosis and
infratemporal space. The direct antral infection
dissemination of septic emboli.
may also give rise to this disease.
Odontogenic Infection of Orbit
Bacteriology
Many organisms have been found in culture of Etiology
CSF including Pseudomonas, Corynebacterium, Odontogenic infection can spread to orbit through
Staphylococcus albus, Streptococcus, Diplococcus several routes. Infection of premolars and molars
pneumonia and Proteus. Most common organism can perforate the maxillary buccal plate and can
is Staphylococcus aureus. spread to pterygopalatine and infratemporal fossa
and reach the orbit, via inferior orbital fissure. The
Clinical Features maxillary anterior teeth can produce orbital
Proptosis or protrusion of eye is seen as a result cellulitis by retrograde spread through vessels like
of decreased venous drainage, chemosis and anterior facial, angular and ophthalmic vein.
edema of eyelid, which is secondary to venous
stasis. Limitation of extra-ocular movements Clinical Features
because of involvement of 3rd, 4th and 6th It can result in significant morbidity and
cranial nerves. Cranial nerve palsy of 3rd, 4th mortality. Progress of orbital infection posteriorly
and 6th nerve is usually evident due to irritation may involve the superior orbital fissure and
caused by pressure of venous congestion. Rapid spread to cavernous sinus via superior
progression of signs and symptoms from one eye ophthalmic vein. It may result in temporary loss
to other eye, due to spread of infection through of visual acuity. Long-term ophthalmologic
intercavernous sinus. There is high spiking fever. sequelae include permanent loss in visual acuity,
Signs of meningeal irritation including severe residual proptosis, diplopia and blindness. When
headache, stiffness of neck, ocular palsy and later CNS is involved, hemiparesis, seizures and
facial weakness. death have also been reported.

Investigations Management
Lumbar puncture should be made. CSF shows When orbital infection is suspected, early
neutrophils, decreased glucose concentration aggressive antibiotic therapy and surgical
458 Textbook of Oral Pathology

intervention, following appropriate consultation, Septicemia and Bacteremia

should be initiated to prevent serious ocular
Bacteremia
complications and CNS involvement.
It refers to the circulation of bacteria in blood.
Mediastinitis Transient bacteremia is frequent even in healthy
persons. In such cases, bacteria, are mopped up
It is usually a late complication of facial infection.
by circulating phagocytic cells and are unable to
The process occurs because it gives a pathway
initiate infection.
for spread of pus and infection from
submandibular region, floor of mouth and from Septicemia
all the related spaces in neck, beneath the
investing layer to deep cervical fascia. Following It implies overwhelming bacterial proliferation
this pathway, infection can come into close and release of toxins in blood. Source of infection,
relationship with trachea, larynx and great hypotension and pyrexia with rigors.
vessels and eventually reach the mediastinum.
Necrotizing Fascitis
The most common anatomic pathway is lateral
pharyngeal space, through visceral space This condition was first recognized in 1924 by
inferiorly, which may occur causing weakening Meleney. It is defined as rapidly progressing
and rupture of pleura. necrosis of subcutaneous tissue and facia, usually
sparing the muscles and accompanied by
Clinical Features toxicity, high fever and apathy.
Usually there is chest pain and severe dyspnea
Clinical Features
with unremitting fever and evidence of swelling
on the lateral aspect of neck of the affected side. A tender erythematous cellulitis with ill defined
Involvement of one or two facial spaces is usually margins presents the patient with high fever and
preceded by these signs and symptoms. apathy. Pain can be severe, but the affected area
Progressive septicemia, mediastinal abscess, of skin becomes anesthetic, secondary to
pleural effusion, empyema, compression of cutaneous nerve destruction, which can occur
mediastinum veins with decreased venous before clinical gangrene. The progression of
disease can be alarmingly rapid with skin color
return to heart and pericarditis may occur, with
changing from red blue to grey in as early as 36
death as the final step. Hemorrhage secondary
hours leading to frank cutaneous gangrene due
to erosion of internal carotid artery or one of its
to thrombosis of nutrient vessels, usually by 4th
branches is the most common cause of death in
or 5th day. Skin bullae may develop, but lymph
deep space infection.
adenitis is usually not seen. Skin death
subsequent to subcutaneous necrosis is common.
Management
The presence of diseases such as diabetes
Utmost priority is to be given for airway control. mellitus, malignancy and drug addiction, are
In such cases, either emergency tracheostomy or significant risk factors. Importance of
cricothyrio-dectomy may be performed. Specific Streptococcus pyogenes, a major cause of severe
antibiotic therapy —in high doses should be necrotizing and fulminating infection, must not
instituted. Surgical drainage of mediastinum. be forgotten.
 Consequences of Oral Infection 459

Management fibrous capsule, which effectively walls off or

Spectrum of antibiotics should include drugs separates the area of infection from the adjacent
active against anaerobic organisms such as tissues but do not prevent the absorption of
metronidazole. Continuous wound care is of bacteria or toxins. Periapical granuloma has been
utmost important. Irrigation with hydrogen described as a manifestation of vigorous body
peroxide, followed by dressing of charcoal lime defense and repair reaction, while cysts merely a
and boric acid solution soaked gauze should be progressive form of granuloma. Abscess occurs
applied. Hyperbaric oxygen therapy has been when the reparative and defensive phase is
used, but its value is not proven. Blood minimum. Majority of investigators indicate that
transfusion and general supportive measures an unusually high percentage of periapical
must be given. As soon as disease is controlled granuloma are the biologically sterile and for this
split skin graft should be applied, if necessary reason the possibility such lesions giving rise to
for reconstruction. focal infection is minimum.
Teeth with infected root canals—These are potential
Focal Infection sources of dissemination of microorganisms and
Focus of infection—It refers to circumscribed area toxins. Most commonly it shows occurrence of a
of tissue, which is infected with exogenous hemolytic streptococcus, which is the most
pathogenic microorganisms and which is usually important in etiology of rheumatoid arthritis and
located near a mucous or cutaneous surface. rheumatic fever.

Focal infection—It refers to metastasis from the Periodontal disease—It is equally significant as
focus of infection, of organisms or their products potential source of infection. The usual organism
that are capable of injuring tissue. recovered is Streptococcus viridians. Simple
massage of gingiva may result in transitory
Mechanism of Focal Infection bacteremia. The rocking of teeth in their socket
by forceps, before extraction, has been shown to
Metastasis of microorganism—It may spread by favor bacteremia in patients who have
hematogenous or lymphogenous route. They get periodontal disease. Due to pumping action
localized in tissues. Certain organisms have a during extraction microorganism may be forced
predilection for isolating themselves in specific from the gingival cervix into the capillary of
sites of the body. gingiva as well as into the pulp of tooth and thus,
Toxins or toxin products—It may spread by blood will results in bacteremia. Oral prophylaxis may
stream or lymphatic channels, from focus to a be followed by bacteremia. So, it is mandatory
distant site, where they may initiate to administer the antibiotics to the children who
hypersensitive reaction in the tissues. One are diagnosed rheumatic or congenital heart
example is scarlet fever, which is due to disease to prevent the positive consequences of
erythrocyte toxin liberated by the infective bacterial endocarditis.
streptococci.
Significance of Oral Foci of Infection
Oral Foci of Infections
There are reports that the oral foci of infection
Infected periapical lesion—particularly those of either cause, or aggravate many systemic
chronic nature an area usually surrounded by the disorders. Most common are as follows:
460 Textbook of Oral Pathology

Arthritis—It is of mainly rheumatoid and lead to variety of gastrointestinal diseases.

rheumatic fever type. Arthritis of rheumatoid type Gastric and duodenal ulcers are produced by
is of unknown etiology. These patients have high injection of streptococci.
antibody titer to group of hemolytic streptococci. Ocular diseases—Factor supporting the
It is tissue hypersensitive reaction. There are some hypothesis of Woods the role of foci of infection
points in favor of septic foci theory: streptococcal in ocular diseases. Many ocular diseases occur
infection of throat, tonsils or nasal sinus may in which no systemic cause, other than presence
precede the initial or recurrent attack, dramatic of remote foci of infection can be demonstrated.
improvement occurs sometimes after the removal Numerous instances of prompt and dramatic
of septic foci and temporary bacteremia may occur healing of ocular diseases are reported following
immediately after tonsillectomy, tooth extraction the removal of these foci. Occasionally, sudden
or after vigorous massage of gums. Against the transient exacerbation is observed, after the
theory, there are some points: often no infective removal of teeth and tonsils. Presence of blood
focus can be found, usually when focus is stream infection in early stages of ocular disease,
extirpated, no dramatic result is found and are evident. Iritis may be produced by
sulfonamide, antibiotics and vaccine have failed intravenous injection of microorganisms, e.g.
to produce desirable results. streptococci. There is some objection to these
points, i.e. many healthy people have focal
Valvular heart diseases—Subacute bacterial
infections, but do no have ocular diseases,
endocarditis is without doubt related to oral
spontaneous care may occur if nothing is done
infections. There is close similarity, in most and positive blood cultures are rare in acute iritis.
instances, between the etiologic agent of the
disease and microorganisms in the oral cavity, Skin diseases—Some forms of eczema and
dental pulp and in periapical lesions. Symptoms possibly urticaria, can be related to oral foci of
of subacute bacterial endocarditis have been infection. If the relationship does not exist, the
observed in some instances shortly after mechanism is probably sensitization, rather than
extraction of teeth. Transient bacteremia metastatic spread of the microorganisms.
frequently follows tooth extraction. Streptococci
Renal disease—Microorganism most commonly
of viridian type cause majority of subacute
involved in urinary infection are E.coli,
bacterial endocarditis. After tooth extraction, staphylococci and streptococci. Streptococci
there is streptococcal bacteremia, so there is
hemolyticus seems to be the most common.
occurrence of subacute bacterial endocarditis
Streptococci are uncommon inhabitants of dental
after dental operations, dental extractions. root canals or periapical and gingival areas. Since
Premedication of the patient should be done
the microorganisms commonly involved in renal
before extraction.
infection so it appear that there is little
Gastrointestinal disease—Some workers state that relationship between oral foci of infection and
constant swallowing of microorganisms might renal disease.
 CHAPTER
Bacterial
23 Infections

The literal meaning of the word ‘disease’ is ‘loss Predisposing Factors

of ease’. The oral cavity reflects the state of
Poor hygiene, crowded living conditions, pre-
systemic health more frequently than other parts
existing eczema and scabies and reduced
of the body. Even in ancient time, examination of
resistance due to preceding influenza or herpes
mouth and tongue was given great importance.
simplex infection can lead to this disorder.
The oral tissues are in direct physical continuity
with rest of the body and they are also related via Clinical Feature
blood, lymphatics and nerve pathways. The disease is mainly seen in preschool children
Furthermore, systemic influence such as and young adults. The face (angle of mouth, lips
endocrinological, immunological and psycho- and nose) is it the most common location. The
logical factors has an important role in the balance lesions frequently begin as red, itchy spots. The
between oral health and disease. close set, round or oval, flat, pustular vesicles
During both, development and maintenance,
with a characteristic stuck on appearance
local and systemic factor are concerned in disease
subsequently develop. The thin roofed vesicle
process of mouth. Oral health must be considered
then burst to form golden yellow crust and some
in relation to general health. The dentist’s role in
of the lesions may become confluent. The
general health is based on the fact that, he is the
surrounding skin is erythematous with
first person to see the oral lesion. In preventive
lymphadenopathy, which becomes tender.
dentistry, the utmost principle is of early
diagnosis. Dentist have an important note in Histopathological Features
preventive medicine as many systemic diseases
Pustules filled mainly with neutrophils
have primary oral manifestations.
leukocytes are seen directly beneath the horny
layer of the epithelium. The spinous layer below
Impetigo Contagiosa
show spongiosis and migration of leukocytes
It is also called as ‘nonbullous impetigo’ or from the connective tissue, where a slight to
‘impetigo vulgaris’. It is acute superficial, moderately, severe inflammatory infiltrate of
purulent infection of the skin. It is caused by neutrophils and lymphocytes is seen. At the later
Streptococcus pyogenes. stave when the bulla ruptures, the horny layer is
462 Textbook of Oral Pathology

absent and a crust composed of fibrin and red plaques develop and spread rapidly. Blisters,
neutrophils, leukocytes may be found resting on which break down to form large ulceration, may
the remaining epithelial layers. develop on the red lesion. The lesion has an
elevated, edematous, sharp border and an
Management irregular outline. Regional lymph nodes become
Single injection of long acting penicillin and 3-4 enlarged and tender. The untreated acute stage
gm of oral penicillin V, daily, for 10 days. of erysipelas resolves after 3-10 days, leaving dry,
desquamating and sometimes partially ulcerated
Erysipelas skin.
It is an acute, superficially spreading infection
of the dermis, usually of the face, with a well Oral Manifestations
demarcated, slightly indurated erythema and It is uncommon in oral, pharyngeal and nasal
progressive lymphangitis. mucosa. Erysipelas which develops in the oral,
pharyngeal or upper respiratory tract mucosa
Causes result in the same constitutional reaction, as
It is caused by group A streptococci. The described for skin lesion. There is severe local
microorganisms are thought to enter the tissues pain, redness and swelling.
through a small break in the mucosa or the skin, Edema may involve the tongue, uvula,
such as fissure, abrasion, erosion or excoriation. epiglottis and may lead to serious consequences.
Postsurgical erysipelas is caused by The submandibular lymph nodes become
transmission of streptococci from the nose, markedly tender and swollen.
throat, or hands of the patient or from, the
attendant or visitors. Histopathological Features
Nephrotic edema, lymphedema, dysga- Edema and dilatation of the lymphatics and
mmaglobulinemia, malnutrition and alcoholism capillaries. A marked and diffuse inflammatory
are known predisposing factors. infiltrate, predominately of neutrophils, is seen
throughout dermis, occasionally extending into
Clinical Features the subcutaneous fat.
The new born and infants are highly susceptible, Management
but elderly are also affected. It is more common in
Penicillin is the drug of choice and if sensitive to
women in 5th decade and in men, in 7th decade.
penicillin, erythromycin can be given.
The most common location is on abdomen, face,
scalp and legs. The incubation time it thought to Syphilis
be from few hours to several days. After short
prodormal phase, characterized by malaise, It is also called as ‘Lues’. It occurs most
vomiting, headache, pyrexia and chills. Erysipelas exclusively by venereal contact, in overcrowded
begins abruptly with a local sensation of burning living and primitive housing conditions.
and itching. The general condition of the patient
Classification
worsens with toxemia, high fever, insomnia and
restlessness. • Acquired syphilis—Contacted primarily as
A small area of the skin then becomes venereal disease due to sexual intercourse
intensely red and swollen. Subsequently, bright with infected partner
 Bacterial Infections 463

• Primary round, indurated and with rolled raised edges.

• Secondary It begins as a papule, before proceeding to
• Tertiary ulceration and varies in size from 5 mm to several
• Quaternary syphilis—The atypical malignant centimeters. It is painless, unless superinfected.
progression of tertiary neurosyphilis in It disappears without therapy after 10 days.
immuno-compromised HIV individuals is Regional lymph nodes become firm enlarged,
referred to as quaternary syphilis. rubbery in consistency and non tender. The
• Latent phase combination of indurated ulcer on appropriate
• Congenital mucosal surface and enlarged, non tender,
• Early syphilis—Primary syphilis, secondary regional lymph nodes which are painless,
syphilis and the early latent phase of the discrete and rubbery in consistency-constitute
disease are grouped as early syphilis. Early the classic signs of primary syphilis.
syphilis may last up to two years and is
infectious. Oral Manifestations
• Late syphilis—While late latent and tertiary are
grouped as late syphilis. Late syphilis is Oral lesions of primary syphilis are rare and
locally destructive and non-infectious. occur at the site of entry of treponema (Fig. 23.2).
Chancre has been described on lips, in males
Epidemiology (upper lip) and in females (lower lip), oral
There is decreased incidence after introduction mucosa, lateral surface of tongue, soft palate,
of penicillin in late 1940s. Patient infected with tonsillar area, pharyngeal lesion and gingiva.
Treponema pallidum and HIV may exhibit a Transmission can occur during kissing as a
malignant form of syphilis with slow consequence of sexual practice among
development of standard serologic response to homosexual and heterosexuals, or by contact
syphilis. The tertiary manifestations lead to with objects such as mouth piece of musical
considerable morbidity and mortality. instruments and medical or dental instruments.
It has narrow copper colored, slightly raised
Primary Syphilis
borders with reddish brown base in center. It
Clinical Features measures from 0.5 to 2 cm in diameter. Intraoral
Lesion develops at the site of inoculation chancres are slightly painful due to secondary
approximately 3 weeks after contact with infection and are covered with grayish white
infection. It occurs most frequently on penis in film. Occasionally, it retains white sloughy
males and vulva or cervix in females. Recently, material. They are contagious. Primary
occurrence on extragenital sites have increased involvement of tonsils is manifested by
as a result of increase in orogenital sex and considerable edema, redness, ulcerated and
increased contact among the infected eroded lesion. Regional lymphadenopathy
homosexuals. Extragenital sites of involvement occurs.
include fingers, perianal region, nipples and lips, Extraoral lip chancre may have more typical
tonsils and intraoral structures such as tongue brown crusted appearance which may be
and palate. multiple. Lower lip involved more frequently.
It is slightly raised, ulcerated, non-tender, Oral chancre heals spontaneously in 3 to 6 weeks
non-bleeding, firm plaque which is usually leaving small scars.
464 Textbook of Oral Pathology

Histopathological Features surrounding tissues, may be seen. Headache,

In the primary lesion, the chancre is anorexia, pain in joints and muscles.
microscopically characterized by presence of
dense infiltrate of plasma cells, lymphocyte,
macrophages and a proliferative granulation
tissue. Obliterative endarteritis, characterized by
perivascular infiltration of inflammatory cells is
seen. Deeper tissues show endothelial
proliferation, swelling and perivascular cuffing
by inflammatory cell infiltrate. Proliferative
granulation tissue is present at the margin of
ulcer. Plasma cells are numerous (Fig. 23.1).

Figure 23.2: Syphilitic ulcer on the lip which has

got ill defined border

Mucus patches are small, smooth, erythe-

matous areas or superficial grayish erosions
found on mucus membrane of vulva, penis, or
in oral cavity, on palate and tonsils. They are
described as snail track ulcers. Condyloma latum
are grayish, moist, flat topped, extralarge plaque
which sometimes coalesce into larger plaques,
found on moist mucocutaneous surfaces such as
vulva, anus, scrotum, thigh and axilla. Spilt
papule is a double papule which occurs at skin
Figure 23.1: Nonspecific granulation with inflammatory folds and angle of mouth. Generalized
cells (IC) and an ulcerated surface epithelium (U) symmetrical enlargement of the lymph nodes in
posterior cervical, suboccipital, supratrochlear
Secondary Syphilis
and inguinal regions. There is positive serological
Clinical Features test. Patient may enter the phase of latency
Organisms proliferate and spread by the way of without treatment.
blood stream to produce lesions else where. It
Oral Manifestations
usually appears within 3 to 6 weeks after primary
lesion. When appear on skin, they manifest as Mucus patches—Mucus membrane analogue of
fine macular or papular rash, sometimes papular or macular skin eruptions. It if found on
accompanied by alopecia. Circinate lesions on tongue, buccal mucosa, tonsillar and pharyngeal
face are characteristic of secondary syphilis. The region and lips. It appears as slightly raised
lesions either resolve completely or leave residual grayish white lesions surrounded by
areas of hypo- or hyperpigmentation. Fever and erythematous base. They are covered by grayish
generalized lymphadenopathy, which is white membrane. Trauma results in raw bleeding
painless, discrete and non adherent to the surface.
 Bacterial Infections 465

Snail track ulcers—Confluence and coalescence of vertical walls and dull red granulomatous base
these glistening mucous patches gives rise to the is the typical clinical feature of ulcerative
so called ‘snail track ulcers’. It is often painless gummatous lesion. Cutaneous lesions heal
but mild to moderately painful. slowly and leave behind tissue paper like scars.
Split papule—Split papule is a raised papular Single cerebral gumma may produce symptoms
lesion developed at the commissure of lip and suggestive of brain tumor.
with a fissure separating the upper lip portion Neurosyphilis—It occurs due to obliteration of
from lower lip portion. They are called as split small vessel artery involving vasa vasorum of
papules as they are cracked in the middle giving aorta ad other large vessels of the central nervous
a ‘split pea appearance’. They are highly system (neurosyphilis). There are saddle
infectious.
deformities of nose. Neurosyphilis is manifested
Condyloma latum—They are flat silver gray wart as tabes dorsalis and general paresis. Tabes
like papule, sometimes having ulcerated surface. dorsalis is the syphilitic involvement of dorsal
They are painless. Regional lymphadenopathy column of spinal cord and dorsal root ganglion.
is usually present. General paresis is syphilitic involvement of
cerebral tissue.
Histopathological Feature
The macular lesion shows inflammatory cell Tabes dorsalis—Patient looses the positional sense
infiltration and obliterative endarteritis. The of his lower extremities and walks with a
papular lesion exhibits endothelial proliferation, slapping step. Burning and pricking sensation of
swelling and perivascular chronic inflammatory the extremities, paresthesia, or at times, actual
cell infiltration. Condyloma latum reveals anesthesia of the part may accompany the
hyperplastic epithelium with hyperkeratosis and characteristic gait. Positive Romberg’s sign—
acanthosis. person is unable to stand erect unaided with his
eyes closed. Short, shooting, knife-like pains may
Tertiary Syphilis be experienced in the abdominal region called
Clinical Features ‘tabetic crises’, which results from involvement
It may occur at any age from the third year upto of the dorsal root ganglion. Charcoat joint—
the patient’s life. In tertiary syphilis, 1/3rd trophic changes consist of deep perforating ulcers
develop benign or gummatous form, 1/3rd and painless destruction of larger joints.
cardiovascular form and 1/3rd neurosyphilis, i.e. General paresis—Argyll Robertson pupil—Pupils
general paresis and tabes dorsalis. that react to accommodation but not to light.
Gumma—Gumma is due to a chronic destructive Increased irritability, fatigue, mental
granulomatous process which occurs anywhere sluggishness and carelessness in personal habits.
in the body. Gumma is the result of Loss of fine muscular coordination as indicated
hypersensitivity reaction between hyperergic by inability to enunciate clearly or to perform
host and treponema. There are two types of delicate tasks with the hands. Involvement of
gumma, i.e. central and cortical. The spinal cord is late manifestation characterized by
characteristic gumma is a chronic granulomatous paresthesia, burning and prickling sensation in
and usually localized lesion, which later ulcerates the extremities. Patient may get unrealistic ideas
which may be nodular. Punched out ulcer with of wealth or ability.
466 Textbook of Oral Pathology

Cardiovascular syphilis—It occurs in 10% cases of fibroblasts, which appear plump and often
late syphilis. Involvement of CVS in tertiary resemble epithelioid cells. Fibroblasts are plump
syphilis affects aorta and aortic valve and 80% and they often resemble epithelioid cells.
of deaths occur due to it. Medial necrosis and Occasional presence of giant cells and regular
destruction of elastic tissue occurs in the wall of presence of chronic inflammatory cells like
large blood vessels. Dilatation and aneurysm plasma cells, lymphocytes and histiocytes.
occurs.
Congenital Syphilis
Oral Manifestations It is infection of fetus established by the passage
They manifest any time during 3 to 10 years after of spirochetes from mother, through the placenta.
the primary infection. Gumma can occur
anywhere in the jaw but are more frequently on Clinical Features
palate, mandible and tongue. Transplacental infection after 18-week gestation
Gumma—Gumma may manifest as solitary, is related to development of immune comple-
deep, punched out mucosal ulcer. It usually starts ment rather than any toxic effect on organism.
as small, pale, raised, nodular mass in the midline It is manifested within the first 2 years of life
of the palate which ulcerates and rapidly (neonatal congenital syphilis) as rhinitis and
progresses to the zone of necrosis. It may cause chronic nasal discharge with maculopapular
perforation of palatal vault. Lesion is sharply eruptions, other than mucocutaneous lesion and
demarcated and the necrotic tissue at the base of loss of weight. The lesions can be seen in spleen,
the ulcer may slough away leaving punched-out kidney, bones and CNS. Bullae, vesicle and
defects. Breathing and swallowing difficulty may superficial desquamation with cracking and scaling
be encountered by the patient. of reddened soles and palms, petechiae, mucus
Numerous small healed gummata in tongue patches and condyloma latum.
results in series of nodules or scars in deeper After 2 years, interstitial keratitis, vasculari-
areas of the organ, giving the tongue an zation of cornea, 8th nerve deafness, arthropathy,
upholstered or tufted appearance. signs of congenital neurosyphilis, gummatous
Chronic superficial interstitial glossitis—Complete destruction of palate and nasal septum develop.
atrophy of papillary coating and firm fibrous Saber shins or anterior tibial bowing. Higou-
texture seen in leutic bald tongue is also referred menakis’s sign—irregular thickening of
to as chronic superficial interstitial glossitis. It is strenoclavicular portion of clavicle. Unexplained
exclusive found in males and is considered as nerve deafness, retinal and corneal damage can
pre-cancerous because of predictions to undergo also occurs.
carcinomatous transformation. Loss of papillae
is probably due to endarteritis leading to Oral Manifestations
circulatory deficiency of lingual vasculature. It Postrhagadic scarring and syphilitic rhagades—
has been labeled as precancerous because of its postrhagadic scars are linear lesions found
predilection to undergo carcinomatous around oral and anal orifices. They result from
transformation. diffuse leutic involvement of the skin in these
areas from 3rd to 7th week after birth. They
Histopathological Features
appear as red or copper colored linear areas
Gumma is characterized by central zone of covered with a soft crust. Rhagades are said to
coagulation necrosis and peripheral rim rich in be more frequent on the lower lip. Healed
 Bacterial Infections 467

syphilitic rhagades appear as ordinary cicatrices. Dark Field Examination Microscopy

The linear scars are radially arranged and
It is the most useful method of identifying
perpendicular to the mucocutaneous junction,
spirochete in primary acquired and occasionally,
which are more prominent on lower lip near
angle of mouth. Diminished coloring of lip is secondary syphilis. Not reliable for oral lesions,
evident and mucocutaneous border is indistinct. since the normal flora contains non-pathogenic
treponema which are difficult to distinguish from
Hutchinson’s triad—It consist of hypoplasia of
T. pallidum.
permanent incisors and 1st permanent molars,
eight nerve deafness and interstitial keratitis.
Lesion Biopsy
Changes in dentition—Retarded root resorption of
deciduous dentition. There may be ‘marring’ of Histopathological examination of suspected
permanent incisors present in congenital lesion, stained by silver impregnation technique,
syphilis. 6 to 28% of the incisors and 3 to 37% of is useful particularly when the lesion contains
the molars have hypoplasia. The crown of the few organisms, as may be in case of tertiary
first molar in congenital syphilis is irregular and lesion. For oral lesions this technique is of
enamel of the occlusal surface and occlusal third considerable value.
of the tooth appears to be arranged in
agglomerate mass of globules, rather than in well Management
formed cusp.
Benzathine penicillin—2.4 million units IM
Screw drive shaped incisors—Constriction of crown aqueous crystalline penicillin.
toward incisal edge screw results in driver or peg
shaped incisor. In addition, incisal edge is usually Gonorrhea
notch which may be due to the absence of central
tubercle or calcification center. It is primarily an infection of the genitourinary
Rounding of mesial and distal incisal line tract mucosa. Occasionally it can involve
angles occurs. There is spacing between cuspid extragenital sites. Local infection may occur at
and incisors. Moon molars—in molars positioning extragenital site (Rectal and pharyngeal
of the cusps toward the central portion of the gonorrhea). Rectal mucosa is affected in 30% to
crown, gives the tooth a bud shaped or a 50% of women with urogenital gonorrhea.
shrunken occlusal form called as mulberry Uncomplicated local infection at other
molars or Moon’s molars. Affected molars are extragenital sites is rare in adults. Initially,
dirty yellow in color due to hypocalcification. urogenital infection is symptomless and
Carabelli cusp—Prominent accessory mesio- associated with purulent urethral discharge,
lingual cusp of upper molar (Carabelli cusp). which responds to antibiotic therapy.
Malocclusion and open bite is present. In
congenital syphilis characteristic shape of tooth Etiology
crown can be identified on radiograph. It is caused by gram-negative intrabacillary
located diplococcus Neisseria gonorrhea. It is an
Diagnosis of Syphilis oval, paired, gram-negative microorganism.
The presence of clinical manifestations together Early sexual awakening, prostitution and varied
with history of a sexually active person should sexuality are believed to be responsible for the
give clue to the diagnosis of acquired syphilis. increase incidence of gonorrhea.
468 Textbook of Oral Pathology

Pathogenesis lesion may develop in gonococcal stomatitis, i.e.

isolated ulcers, gingivitis and membranous
Once the gonococci are directly deposited on the
gingivostomatitis.
genitourinary tract mucosa during sexual
Lips may develop acute painful ulcerations,
intercourse, they penetrate through the
limiting the motion. Gingiva may become
intercellular spaces of the epithelium and reach
erythematous, with or without necrosis. The
the subepithelial connective tissue. Within 2 to 3
tongue may present red, dry ulcerations or
days of infection, an intense inflammatory
become glazed and swollen with painful erosion
reaction occurs resulting in characteristic
with similar lesions on buccal mucosa and palate.
mucopurulent discharge through urethral
lumen. A chronic stage may be reached, if Temporomandibular joint involvement—Gonococcus
untreated and spread is either by direct extension infection involving articulating joint is most
through lymphatics or hematogenous route. common form of extragenital gonorrhea. Any joint
may be affected, the commonest being knee, ankle
Clinical Features and wrist. TMJ is affected in 14% of patients. It
It is primarily a disease of young adults between occurs as a result of hematogenous spread.
the age of 15 to 24 years and is more common in Difficulty in jaw movements due to pain and
males, as compared to females. Symptoms occur swelling of single or both joints is the presenting
in 1 day to 2 weeks, following contact with symptom. Cervical lymphadenopathy and fever
infected persons. There is profuse purulent may be present. Rarely, perforation to the
urethral discharge with frequent micturition, tympanic plate occurs. It may lead to fibrous
followed by dysuria. In some of the patients, ankylosis because articular cartilage is destroyed.
there may be fever and headache. In females,
acute gonorrhea includes urethral, cervical and Diagnosis
vaginal discharge.
In all forms of it, including those of oral cavity
and pharynx, the diagnosis rests on the
Oral Manifestations
identification of organism. Method used include
Pharyngeal gonorrhea—It is higher in pregnant gram stained smear, culture studies and direct
women (2 to 15%), sexually active homosexuals fluorescent antibody test.
(2 to 25%) and heterosexuals practicing oral sex.
History of fellatio is more associated with Management
pharyngeal gonorrhea. Sore throat and evidence
of pharyngitis. Pharyngeal gonorrhea is a term Single dose of the broad-spectrum cephalosporin
used for patients in whom neisseria gonorrhea antibiotic ceftriaxone 125 to 250 IM plus
is isolated from nasopharynx. doxycycline 100 mg orally, twice a day for 7 days
should be given.
Gonococcal stomatitis—Incidence is very rare and
often shows multiple, painful and round elevated
Leprosy (Hansen disease)
gray white eroded spots, with or without
pseudomembrane formation. Regional It is a chronic infectious disease which has
lymphadenopathy may be seen. Acute gingivitis predilection for the skin, nerves and mucous
develops around extraction site in patient who membrane. It probably originated in tropic and
practices fellatio repeatedly for days after spread to the east. Leprosy has always been
extensive dental extraction. The wide range of considered in superstitious dread and the person
 Bacterial Infections 469

suffering from leprosy was considered unclean Clinical Manifestations

and socially outcasted. General Features

Etiology Males are affected more commonly than females

with ratio of 3:1. It has got incubation period of 2
The leprae bacillus, mycobacterium leprae, first to 5 years during which patient passes through
observed by Hansen in 1868. It is not been silent or latent period. Toward the end of this
possible to grow the bacillus in culture media. It stage the symptoms are those of irritation of
is an acid fast, gram-positive, non motile bacterial nerve ending in the skin, persistent or recurrent
with affinity for Schwann cells and cells of paresthesia and numbness—localized to certain
reticuloendothelial system. area with no accompanying visible alteration in
the corresponding skin.
Types
Tuberculoid Type
• Tuberculoid Leprosy (TL)
• Lepromatous Leprosy (LL) It is a benign form of leprosy involving the skin
• Borderline tuberculoid (BT) nerves and regional lymph node. Lesions are
• Borderline lepromatous (BL) hypopigmented, erythematous and flat or raised
cutaneous lesions. Nerve involvement with loss
• Polyneuritic
of different types of sensation is also manifest.
• Maculoanesthetic
• Indeterminate Early tuberculoid leprosy—It is manifested by
• Erythema nodosum leprosum. hypopigmented macules which are sharply
demarcated and hypesthetic.
Pathogenesis
Intermediate tuberculoid lesion—Later the lesion are
After entry in the body the bacilli reach the larger with elevated and circinate margin. There
lymphatic and blood stream and are taken up is peripheral spread and central healing.
Schwann cells peripheral nervous system, where Fully developed lesion—In this lesions are densely
they start multiplying. If the host cell mediated anesthetic and loose normal skin organs (sweat
immunity is adequate bacilli are destroyed and glands and hair follicles). There may be severe
there is no disease. In the host immunity is neuritic pain.
unstable and suboptimal, there will be some Loss of eyebrows and eyelashes is prominent
restricted multiplication of bacilli and lesion will feature of later involvement. The sequelae of
develop. The variable status of host cell mediated peripheral nerve involvement may develop in
immunity is reflected in the different clinical some cases and this may give rise to muscle
types of leprosy. When there is relatively good atrophy, like contracture of hands and feet
immunity but not enough to eliminate the (Fig. 23.3), loss of phalanges, lagophthalamus,
infection, a localized type of disease called as exposure keratitis and corneal ulceration leading
tuberculoid type, is seen. When the host cell to blindness.
immunity is deficient a generalized form of the
disease lepromatous leprosy develops. In Borderline Type
between these two polar varieties of the disease It represent wide clinical picture and it is
there is a wide spectrum of manifestations, subdivided into borderline tuberculoid and
categorized as borderline leprosy. borderline lepromatous types.
470 Textbook of Oral Pathology

Skin and nerve involvement is characterized

by flat and raised asymmetrical areas of
hyperpigmentation with well defined or ill
defined margins. The raised lesions are rubbery
or soft in consistency and erythematous. The
edges of skin lesion are sharply demarcated, in
tuberculoid type and sloping in cases of
lepromatous type. The surface of lesion is dry
and rough in tuberculoid type, while it is smooth
and shiny in lepromatous form.

Lepromatous Leprosy
This form is more commonly seen in children and
female are affected more as compared to males.
This malignant form of the disease produced
widespread involvement of body skin,
peripheral nerves, mucous membrane, lymph
nodes, eyes, skeleton tastes and other internal Figure 23.4: Macular lesion seen on leg in leprosy
organs.
It develops early as erythematous macules
(Fig. 23.4) or papules without subsequently lead
to progressive thickening of skin and the
characteristic nodules. The borders of the lesion
are ill defined and centers of the lesion are
indurated and convex (Fig. 23.5).
Leonine facies—Loss of lateral portion of eye brow
is common. Much later, the skin of face and
forehead become thickened and corrugated

Figure 23.5: Leproma manifested as nodular

swelling in leprosy

(leonine facies) and earlobe becomes

pedunculous; nasal stiffness, epistaxis,
hoarseness and saddle nose also occur (Fig. 23.6).
Painless inguinal and axillary lymphadenopathy
is common along with sterility and
gynecomastia. Nerve involvement is a late
phenomenon. It runs chronic course and seldom
Figure 23.3: Claw hand seen in leprosy patient causes sudden death.
 Bacterial Infections 471

Erythema Nodosum Leprosum (ENL) Neural changes involving face—Peripheral nerves

such as facial and trigeminal nerve are frequently
They occur in lepromatous and borderline lep-
involved. Facial paralysis is observed in 3% of
romatous patients, most frequently in the latter
cases, complete or partial facial paralysis may
half of the initial year of treatment. Tender, in-
occur either unilaterally or bilaterally. Paralysis
flamed subcutaneous nodules develop, usually
of the orbicularis muscle results in facial
in crops. Each nodule lasts a week or two, but
disfigurement, difficulties in phonation and
new crop may appear. Low grade fever, lym-
mastication as well as drooling. Involvement of
phadenopathy and arthralgia can accompany
trigeminal nerve results in hypoesthesia and
sever, ENL.
anesthesia.
Skeletal changes involving the face—Rhinomaxillary
changes termed facies leprosa consist of atrophy
of anterior nasal spine, atrophy and recession of
the maxillary alveolar process confined to the
incisor region and endonasal inflammation.
Destruction of facial cartilage and bone is caused
by direct infiltration and secondary infection. From
nasal mucosa, granulomatous tissue invades
cartilage and bone resulting in nasal collapse and
a specific chronic osteomyelitis. Collapse of the nose
(saddle nose) is also observed. Loss of anterior nasal
spine and resorptive changes of pre-maxilla occur
only in later stage of the disease.
Oral and dental lesions—It is more commonly seen
in lepromatous leprosy although clinical changes
of oral mucosa have been described in borderline
leprosy. Involvement of oral mucosa, pre-
Figure 23.6: Facial appearance in leprosy
maxilla, soft palate, uvula, tongue, gingiva and
(note the saddle nose)
periodontium has been reported.
Orofacial Manifestations
Lepromas—Small tumor like masses called as
Depending on the type and duration of the lepormas develop on the tongue, lips or hard
disease, all patients with lepromatous leprosy palate. These nodules have a tendency to break
show facial and oral manifestations and it is rare down and ulcerate. Gingival hyperplasia with
in tuberculoid and borderline leprosy. loosening of teeth have been also reported. In
The lesions are macular or raised, well association with severe and granulomatous
defined, hypo-pigmented, unhydrotic and infiltration of pre-maxilla in childhood, the tooth
hyperaesthetic or anesthetic. Lepromatous diameter is suddenly and concentrically reduced,
infiltration of the helical and lobes of ear are while the less marked cases exhibit a well
characteristic of the rare stage of the disease. demarcated, tapering and shortening of root.
Advanced cases are characterized by nodular Transient interruption of odontogenesis results
involvement of facial, cutaneous and in slight, circumferential hypoplasia of enamel
subcutaneous structure. and cementum. In long standing lepromatous
472 Textbook of Oral Pathology

leprosy invasion of the pulp by granulomatous disease caused by acidfast bacilli Mycobacterium
tissue causes pulpal necrosis leading to a pinkish tuberculosis or rarely, mycobacterium bovis.
discoloration of crown. Anterior teeth are most
commonly affected. Transmission and Prevalence
Prevalence is more in the low income group with
Histopathological Features low socio-economic and unhygienic condition.
The typical granulomatous nodules show The common cause of entry of the bacillus in
collection of epithelioid cells and lymphocytes body is by inhalation. Infants and young children
in a fibrous stroma. Langerhans type giant cells are at risk in acquiring this disease. It can rarely
are present. Vacuolated macrophages called be transmitted through the placenta from the
lepra cells are scattered throughout the lesion and diseased mother to fetus. Microorganisms may
often contain the bacilli (Fig. 23.7). become disseminated by either bloodstream or
lymphatic spread. Infection can occur through
ingestion of unpasteurized infected cow milk.

Etiology
Causative organism—The first member identified
as tuberculous bacillus and designate as
Mycobacterium tuberculosis. Other micro-
organisms associated are Mycobacterium bovis,
Mycobacterium kanasasei, Mycobacterium xenopi
and Mycobacterium malmoense. The organism is
anaerobic, nonmotile, nonsporing, rod shaped
and is stained with special Ziehl-Neelsen nelson
stain.
Figure 23.7: Leprosy showing epithelioid cells (EC) Constitutional factor—Low income group are
and giant cells (GC)
affected. Low and unhygienic living conditions.
Diagnosis Malnutrition and overcrowding.
Skin smears should be examined as a routine Pathogenesis
with Ziehl-Neelsen stain. The percentage of solid
Initial tuberculosis usually occurs in lungs but
bacilli in a smear is known as morphological
occasionally occurs in tonsil or alimentary tract.
index. Skin, mucosal and nerve biopsy for
In most of the patients primary infection and the
histopathological examination are helpful in
associated lymph node lesions heal and calcify.
doubtful cases. Lepromin test is non specific test In some cases, caseous tuberculosis focus ruptures
to determine the hypersensitivity reaction and into vein and produces acute dissemination
is useful in determining the immunological throughout the body, a condition called as acute
status of patient for classification of leprosy. miliary tuberculosis. Meningitis often complicates
this condition.
Tuberculosis
Progressive pulmonary tuberculosis may
It is a systemic infectious disease of worldwide develop directly from a primary lesion or may
prevalence and of varying clinical mani- occur following reaction of an incompletely
festations. It is an infectious granulomatous healed primary focus. Post primary pulmonary
 Bacterial Infections 473

tuberculosis is a condition in which liquefied Oral Manifestations

center of tuberculosis pulmonary infection is
They are relatively uncommon and seen in
discharged into sinus. Extension of infection into
middle and older age groups, as cleansing action
pleura causes tuberculosis pleurisy.
of saliva and its antibacterial properties, in
Types general also provide protection against tubercle
bacilli. Tongue is most commonly affected
• Miliary tuberculosis—It spreads through followed by palate, lips, buccal mucosa and
bloodstream and there is wide involvement gingiva. Majority of oral lesions are secondary
of many organs like kidney, liver and is called to infection in some other parts of body.
as miliary tuberculosis.
• Pott’s disease—If tubercular involvement of Ulcer—The lesion may be preceded by an
spine occurs in children, then it is called as opalescent vesicle or nodule, a result of caseation
Pott’s disease. necrosis. It breaks down into an ulcer which is
• Scrofula—If it spreads by lymphatics to lymph usually superficial or deep and painful. It tends
nodes, it is called as scrofula. to increased slowly in size. In area of trauma may
be mistaken as traumatic ulcer or carcinoma.
Clinical Features Ulcers are non-specific in their clinical
Patient may suffer episodes of fever and chills, presentation and for this reason they are
easy fatigability and malaise. There may be overlooked by the clinician.
gradual loss of weight accompanied by persistent Two main types of oral tuberculosis infection
cough with or without hemoptysis. Local occur one is primary and other is secondary.
symptoms depend upon the tissue or organs Primary lesion—It develop when bacteria are
involved. directly inoculated in the oral tissue of a person
Tubercular lymphadenitis may progress to who has not acquired immunity to the disease.
acute abscess or remain as granulomatous lesion. It involves gingiva, tooth extraction socket and
In any case, swelling of neck is present which is buccal fold.
tender, painful and often show inflammation of
Secondary lesion—Infection is carried in by
the overlying skin. When abscess forms, it
hematogenous route or through break in the
perforates and discharges pus.
tissue surface, is deposited in the submucosa,
Pulmonary tuberculosis—A persistence cough, subsequently proliferates and ulcerates the
hemoptysis abundant sputum is usual features overlying mucosa. It occurs more frequently in
of pulmonary tuberculosis. There is also evening cases of extrapulmonary tuberculosis.
rise in temperature of 0.5° to 2°F, night sweats. The typical tuberculosis lesion is an irregular
lesion with ragged undermined edges, minimum
Scrofula—In glandular form of the disease, there
induration and often with yellowish granular
is marked enlargement of the cervical lymph
base. The mucosa surrounding the ulcer is
nodes with caseation and frequent breakdown
inflamed and edematous.
of the gland. Such tuberculosis infection is called
Sentinel tubercle—tiny, single and multiple
as ‘scrofula’.
nodules called ‘sentinel tubercle’ may also be
Cold abscess—The chronicity of the infection and seen surrounding the ulcer.
the lack of marked pain or acute inflammatory At the mucocutaneous junction, tubercular
symptoms have resulted in the term ‘cold ulcers are usually extremely shallow with
abscess’. granulating base. Crusting and oozing is seen
474 Textbook of Oral Pathology

when the lesion involves adjacent cutaneous

surface. They are usually painful. The nodular
form of tuberculosis presents as single or
multiple nodules of variable sizes, which initially
may appear a semitransparent lesion of pinhead
size. They are gray in size and of variable
consistency.
Miliary tubercle—Miliary tubercle of oral mucosa
are occasionally seen in miliary tuberculosis
which is a result of acute dissemination of the
infection through hematogenous and lymphatic
channel. The oral lesion appears as small, gray
tubercle with the tendency to break down and Figure 23.8: Tuberculosis showing giant cells and
ulcerate. lymphocyte with tubercle follicle
Tubercle involvement of the periapical tissue called Langhan’s giant cells having horse-shoe
and tooth socket has been reported. The socket shaped nuclear arrangement are seen (Fig. 23.8).
may be filled with so called tuberculosis Areas of infection demonstrate the formation
granulation tissue, consisting of many small, pink of granuloma, which are circumscribed
and red elevations. Tuberculosis gingivitis is an collection. This granuloma is called as tubercular
unusual form which may appear as diffuse, granuloma.
hyperemic or nodular papillary proliferation.
Diagnosis
Jaw involvement—it may involve maxilla or
mandible. Jaw bone involvement occur as a result Microscopy—A pulmonary TB suspect should
of ether deep extension of a gingival lesion, from submit 3 sputum samples for microscopy.
infected extraction socket, an extension of Morning sample is ideal.
tubercular granuloma at the apex of tooth or by Staining method—Ziehl-Neelsen, carbol fuschin
means of hematological spread. It may result in or kinjouncarbol fuschin have been use for
more diffuse form of osteomyelitis and is staining Mycobacterium.
normally more serous than infections from
periapical lesion. Tubercular involvement of jaw Polymerase chain reaction (PCR)—This technique
bone causes swelling and the symptoms include amplifies even very small proteins of
difficulty in eating, trismus, paresthesia of the predetermined target region of Mycobacterium
lower lip and enlargement of the regional lymph tuberculosis complex DNA.
nodes. Fistulae drain either intraorally or Tuberculosis skin test—The Mantoux test is the
extraorally often with blue margins. preferred skin test for detecting tuberculosis. It
In some case destructive involvement may involves the injection of 5 Tuberculin unit of
reach to the TMJ area. purified protein derivatives, usually 0.1 ml
intradermal. The skin test is read on the basis of
Histopathological Features millimeters of induration produced by PPD. 10-
Foci of caseous necrosis surrounded by epithelioid 15 mm induration is required for the test to be
cells, lymphocytes and multinucleated giant cells, positive.
 Bacterial Infections 475

Types
Central—Here, the infection is from the tooth or
its membrane and is accompanied by
radiographic changes.
Peripheral—The peripheral types originate in the
soft tissues and do not involve bone.

Predisposing Factors
• Trauma
• Presence of carious teeth
• Secondary bacterial invasion
Figure 23.9: Tubercular granuloma • Hypersensitivity reaction.
(Courtesy: Dr Sangamesh Halawar)

Clinical Features
Management
Cervicofacial Form: It is most common type of
Short term chemotherapy, isoniazid (5 mg/kg actinomycosis and is commonly seen in adult
with maximum of 300 mg daily or 15 mg/kg two males. Cervicofacial actinomycosis infections are
to three times weekly) and rifampicin (10 gm/ endogenous in origin and occur when dental
kg), ethambutol (25 gm/kg daily for no more plaque, calculus or gingival debris contaminate
than 2 months). the relatively deep wounds around the mouth.
The classical signs are chronic, low, grade
Actinomycosis persistence infection. Submandibular region is
the most frequent site of infection. It usually
It is a chronic granulomatous suppurative and spreads by direct tissue extension. Cheek and
fibrous type of disease caused by anaerobic, masseter region and parotid gland may also be
gram-positive, non-acid fast bacteria. Most involved. Trismus is a common feature, before
common are Actinomyces israelii, A. naeslundii, A. the formation of pus.
viscosus and A. odontolyticus. The organism is The first sign of infection is characterized by
considered to be transitional form between the presence of a palpable mass. Mass is painless
bacteria and fungi. The term Actinomyces was and indurated. There may associate changes
given by Harz to refer the ‘ray like appearance’ detectable at the portal of entry such as non-
of the organism in the granule. The breach in the healing tooth socket, exuberant granulation
continuity of mucosa caused either by trauma or tissue or periosteal thickening of the alveolus.
surgery, if the prerequisite for majority of Development of fistula is common. Skin
actinomycosis infections. surrounding the fistula is purplish. Adjacent
tissues have doughy consistency.
Classification
Sulphur granules—Several hard circumscribed
• Cervicofacial tumor like swelling may develop and undergo
• Abdominal breakdown, discharging a yellow fluid
• Pulmonary containing the characteristic submicroscopic
• Cutaneous. sulfur granules.
476 Textbook of Oral Pathology

Abdominal Form: Abdominal Actinomyces is There is formation of periapical granuloma.

extremely serious form of the disease. Trauma, On the tongue, the lesion is a painful nodule
due to surgery or other causes such as fish bone which eventually ulcerates. Untreated cases may
or chicken bone injury, usually precedes the onset reach to the point where the tongue may become
of the disease. fixed.
Generalized symptom of fever, chills,
vomiting, develop followed by symptoms of Histopathological Features
involvement of organs, such as liver and spleen.
There is palpable abdominal mass. Intestinal Central abscess formation, within which
manifestations develop later. characteristic colonies of microorganisms are
seen. Ray fungus appearance—round or
Pulmonary Form: It produces findings such as lobulated colony meshwork of filaments stain
fever and chills, accompanied by a productive with hematoxylin and peripheral club shaped
cough and pleural pain. Pleural invasion ends of filaments stain with eosin (Fig. 23.10).
resulting in empyema and there may be The typical lesion, whether in the soft tissue
formation of sinus. or in bone, is granulomatous one showing cen-
Subcutaneous Form: Infection result from tral abscess formation within which the charac-
traumatic transplantation of organism, usually teristic colonies of microorganisms are seen.
due to human bites. Lesion seen as subcutaneous Colonies consist club shaped filament that
swelling which enarlges slowly, softens and form radiating rosette pattern. Colonies appear
ruptures through the sinuses. Occasionally, these to be floating in a sea of polymorphonuclear
lesions burrow through deeper tissue and invade leukocytes, often associated with multinucleated
bones. giant cells and macrophages (Fig. 23.11).

Oral Manifestations Management

Organism may enter the tissue through oral Actinomyces infection produces a reactive
mucus membrane and may either remain inflammatory response which causes an area of
localized in the adjacent soft tissue or spread to necrosis and scar tissue around the abscess. This
involve salivary glands, bone or skin of face and results in decrease in the vascular supply to the
neck.
It produces swelling and induration of tissue.
It may develop into one or more abscesses, which
tend to discharge upon the skin surface liberating
pus, which contains typical sulfur granules.
There may be non healing tooth socket,
exuberant granulation tissue and periosteal
thickening of alveolus. Skin overlying abscess is
purple red and indurate or fluctuant. It is
common for sinus, through which the abscess has
drained, to heal but due to chronicity, new
abscesses are formed and perforate through skin
surface. There is disfigurement of face. Infection Figure 23.10: Actinomycosis colony( AC) in a sea of
may involve maxilla and mandible. lymphocytic infiltration (IC)
 Bacterial Infections 477

around fixed bridge or crown. The

commencement of gangrene is denoted by
blackening of skin. Small ulcers of gingival
mucosa spread rapidly and involve the
surrounding tissues of jaws, lips and cheeks by
gangrenous necrosis. Odor is foul. Patients have
high temperature during the course of the
disease, suffer secondary infection and may die
from toxemia or pneumonia. Overlying skin is
inflamed, edematous and finely necrotic which
results in formation of line of demarcation
between healthy and dead tissue.
In advanced stage, there is blue-black
discoloration of the skin. As gangrenous process
advances, slough appears and soon separated,
leaving a perforating wound in the involved area.
Figure 23.11: Actinomycosis showing central abscess
formation (sulphur granules) Large masses may be sloughed out leaving the
jaws exposed.
affected region and hence, makes penetration of
Noma neonatorum is arises in first month of
antibiotics difficult. Therefore, twofold therapy
life in low birth weight infants who also
including antibiotics and surgery is necessary.
demonstrate malnutrition and debilitating
illness.
Noma
It is also called as ‘Cancrum oris’, ‘gangrenous Management
stomatitis. It is rapidly spreading gangrene of
Parenteral fluid should be given urgently to
oral and facial tissues occurring usually in
correct dehydration and electrolyte balance.
debilitated or nutritionally deficient person.

Predisposing Factors Scarlet Fever

It occurs in persons who are undernourished. Predominately occurs in children during winter
Debilitated from infections such as diphtheria, months, caused by infection with group-A
dysentery, measles, and pneumonia, scarlet streptococci of beta hemolytic type that elaborate
fever, syphilis, tuberculosis and blood dyscrasias. erythrogenic toxins.
Excessive mechanical injury. It originates as a
specific infection by Vincent’s organisms. Clinical Features
Miscellaneous factors such as leukemia, sickle Incubation period is 3 to 5 days. The desqua-
cell trait, stress and chemotherapeutic agents can mation of skin begins around the middle of the
cause noma. second week after the onset. Patient exhibits
severe pharyngitis and tonsillitis, chills, fever and
Clinical Features
vomiting.
It is seen chiefly in children, but can be found in Throat becomes highly erythematous and
adults in certain conditions like in malnourished exudation is common. There may be enlargement
states. Common sites are areas of stagnation and tenderness of regional lymph nodes.
478 Textbook of Oral Pathology

Characteristic diffuse, bright scarlet to dusky red Management

skin rash which appear on the second or third
Antibiotics—Penicillin is the drug of choice, since
day of the illness. After 3 to 4 days, the rash fades.
group A streptococci are generally, highly
This rash is due to toxic injury to the vascular
sensitive to this antibiotics. The species are also
endothelium which produces dilation of the
sensitive to other antibiotics like erythromycin,
small blood vessels and consequent hyperemia.
tetracycline and chloramphenicol.
Oral Manifestations
Diphtheria
‘Stomatitis scarlatina’ accounts for the chief oral
It is an acute contagious disease caused by gram–
manifestation of scarlet fever. Mucosa of palate
positive bacillus Corynebacterium diphtheriae, also
may appear congested and throat is often fiery
red. The face, especially the temples and cheeks called as Klebs Loeffler’s bacillus.
are flushed and red, but pale area of circumoral
Transmission
pallor is often seen around the mouth. The tonsils
and faucial pillars are usually swollen and It is transmitted by droplet infection or direct
sometimes covered with grayish exudate. contact.
Strawberry tongue—Tongue exhibits white
Pathogenesis
coating and fungiform papillae are edematous
and hyperemic, projecting above the surface as The portal of entry is the upper respiratory tract
small red knobs and it is called as ‘strawberry and rarely skin, genitalia, eye and middle ear.
tongue’. The bacilli settle on the mucous membrane or
Raspberry tongue—Coating of tongue is soon lost, upper respiratory tract and lead to inflammation
beginning at the tip and lateral margins and the and necrosis of mucosal cells. The infection may
organ becomes deep red, glistening and smooth, spread to adjacent areas. In the primary invasive
except for swollen hyperemic papilla. The tongue region, it forms a thick, firm, leathery, blue white
in this phase is called as ‘raspberry tongue’. pseudomembrane composed of bacteria, necrotic
At the height of skin eruption oral mucosa is epithelium, macrophages and fibrin.
uniformly congested and the breath is fetid. In A narrow zone of inflammation surrounding
severe cases of scarlet fever, ulceration of buccal the area is seen. When the diphtheria bacteria
mucosa and palate have been reported. In some multiply in the local tissues, they produce
cases hypoplasia of teeth is seen in permanent powerful exotoxins. This exotoxin diffuses
teeth, if conditions occur at the time of tooth through the body through a hematogenous route.
developments. Heart, muscle, kidney, peripheral nerves and
adrenal glands are thus involved. Death may be
Histopathological Features caused by heart failure, airway obstruction which
is cause by edema or by the effect of toxin.
The pharyngeal mucosa may display superficial
necrosis, pseudomembranous exudate and Clinical Features
microbial colonies. The submucosa will evidence
an acute or subacute inflammatory cell It occurs most frequently in children, during the
infiltration. Tongue will show vasodilation with fall and winter months. Incubation period is two
inflammatory cell infiltration, being nonspecific days. Listlessness, malaise, headache, fever and
mucositis. occasional vomiting. Within a short time,
 Bacterial Infections 479

patient complains of sore throat. Mild redness Clinical Features

and edema of pharynx with cervical
Incubation period is up to seven days. Patient
lymphadenopathy.
usually suffers a sudden headache, nausea, bony
There may be swelling of the neck, called as
pain, profuse sweat, vomiting, chills and fever.
‘bull neck’. Nasal regurgitation of liquids during
A single cut or sore on the skin develops into a
drinking. Larynx is edematous and is covered
suppurative ulcer. Lymphatic vessels become
by pseudomembrane. It produces a mechanical
swollen and painful and the lymph nodes are
respiratory obstruction and typical croup. There
remarkably enlarged. The eyes also become
is generalized polyneuritis with weakness;
involved with conjunctivitis developing through
paresthesia may follow in the next 10 to 14 days.
localization of disease in the conjunctival sac.
Oral Manifestations Oral Manifestations
Formation of patchy ‘diphtheritic membrane’ Primary infection of the mouth usually occurs
which begins on tonsils and enlarges, becoming from eating infected meat. It is common on soft
confluent over the surface. It is thick and grayish palate, tongue, gingiva and angle of mouth. It is
in color. It tends to adhere and leave a raw usually accompanied by sever pain.
bleeding surface on removal. The ulcer is shallow with whitish fibrinous
Soft palate temporary paralyses usually pseudomembrane, but may extend more deeply
during 3rd and 5th week of the disease. The with superinfection of Vincent’s organism.
paralysis usually disappears in a few weeks or There may be generalized stomatitis
few months, at the most. develops. Single nodular masses eventually
develop into abscess. The tonsil, posterior
Prevention pharyngeal wall, soft palate, base of the tongue
The disease may be prevented by prophylactic and buccal mucosa may be covered by a grayish
active immunization with diphtheria toxoid. white membrane simulating the appearance of
diphtheria.
Management Regional lymphadenitis may arise in sub-
maxillary and the cervical groups of nodes.
The patient should be isolated and bed rest is Cervical lymph nodes are tender, enlarged and
very essential. may suppurate.

Tularemia Laboratory Diagnosis

It is caused by gram-negative bacillus Francisella • Serology—An agglutination test is used to
tularensis. It is contacted through infected demonstrate a rising titre of antibody in the
rabbits, muskrats, ground squirrels and other serum of patients of F. tularensis.
wild germ, particularly of rodent family. • Skin testing—Intradermal injection of an
extract of F. tularensis gives a positive delayed
Types hypersensitivity reaction in 1st or 2nd week
• Cutaneous of illness.
• Ophthalmic
Management
• Pleuropulmonary
• Oral Antibiotics of choice are streptomycin and
• Abdominal. tetracycline, either alone or in combination.
480 Textbook of Oral Pathology

Rhinoscleroma anogenital region and is caused by Donavan

granulomatosis and is a chronic slowly
It is unusual chronic infection caused by bacillus
progressing, mildly contagious disease.
Klebsiella rhinoscleromatis and it is common in
Europe and central and South America.
Clinical Features
Clinical Features It chiefly affects adult black of either sex, but may
occur in any race. Lesion begins as a small papule
Both sexes are equally affected. Common
that ulcerates, increases in size and eventually
between the ages of 20 to 40 years. Usually found
gives rise to velvety, beefy, granulating and
in upper respiratory tract, often originating from
spreading ulcerative lesion of inguinal and
nose, but involvement of lacrimal glands, orbit,
anogenital region. Inguinal ulceration is
skin, paranasal sinus and intracranial invasion
commonly secondary to the genital lesion and
have been describe.
arises initially as fluctuant swelling known as
Rhinitis stage—In this stage, there is nasal pseudobubo. Metastatic spread to bone and soft
obstruction, nasal deformity and epistaxis. There subcutaneous tissue has been reported.
is also swelling of upper lip and sore throat.
Oral Manifestations
Infiltrative stage—It is characterized by nasal
obstruction, due to the presence of exuberant Oral lesion appears to be the most common extra-
granulation tissue. Hoarseness results due to genital form of granuloma inguinale. Oral lesions
laryngeal involvement. Anesthesia of soft palate occur either as a result of autoinoculation
is common in this stage. through infected fingers or through oral coitus.
Nodular stage—The proliferation of nasal masses It is most commonly found on lips, buccal
may produce configuration known as ‘Hebra mucosa or palate or they may diffusely involve
nose’. Posterior extension of the lesion may the mucosal surface. Lesions of lip are
produce laryngeal and tracheal obstruction of characterized by extensive superficial ulceration
varying degrees. Complications include with well defined elevated, granulomatous
scleromatous infiltration of eustachian tube and margins.
unilateral scleroma of maxillary sinus. Maybe of three types, i.e. ulcerative,
exuberant and cicatrical.
Oral Manifestations • Ulcerative—It is painful sometimes bleeding
is associated with it.
Oral lesion appears as proliferative granu-
lomatous lesion of lip, soft palate and tonsil. • Exuberant—It appears as proliferative
There is also anesthesia soft palate and granular mass with an intact epithelial
enlargement of uvula. Taste sensation is also covering. The mucous membrane is inflamed
impaired. and edematous.
• Cicatrical—Fibrous scar formation may
Management become extensive.
Antibiotics should be given.
Histopathological Features
Granuloma Inguinale Donavan bodies present in smears which are
It is also called as ‘granuloma venereum’, recognized as large, gram-negative oval bacteria
‘donovanosis’. It is found in inguinal and with intense bipolar staining (safety pin
 Bacterial Infections 481

appearance). There is granulation tissue with Management

infiltration of polymorphonuclear leukocytes Antibiotic therapy of tetracycline and
and plasma cells. There is marked overlying erythromycin.
pseudoepitheliomatous hyperplasia.
Myiasis
Management
It is referred to the invasion of living tissues by
Streptomycin or tetracycline is given.
the larvae of certain species of flies.
Lymphogranuloma Venereum
It is a venereal disease caused by one of the three Types
strains of Chlamydia trachomatis. • Cutaneous or dermal myiasis—It affects skin.
• Myiasis of external orifices—It includes nasal,
Clinical Features oral, vaginal and anal myiasis.
There may be fever, chills, headache and malaise. • Myiasis of internal organs—It includes
It persists as firm, tender enlargement of inguinal intestinal and urinary myiasis.
lymph nodes. Nodes are tender and adherent to
the underlying tissues. Enlargement of lymph Clinical Feature
nodes both above and below the inguinal It is characterized by papular or migratory
ligament, is characteristic feature called as lesions. Popular lesions are itchy and
‘groove sign’. Overlying skin becomes reddened occasionally painful. Open lesion may produce
and dusky and multiple purulent fistulae serious discharge and larvae maybe detected
develop over enlarged glands. In females, through the opening. Migratory lesions are
placenta is frequently involved. Marked scarring superficial, red tunnel like lesions, which form
and local edema frequently develops secondary creeping eruptions.
to suppurative lymphadenitis. Diagnosed by
complement fixation test. Oral Manifestation
Oral Manifestations Oral myiasis is a rare condition. It presents as an
erythematous, edematous or granulomatous
It results due to orogenital contact or anti-
inoculation. Tongue is the most common site. lesion. Itching or pain may be present. These
lesions pulsate with movement of larvae. An
Lesion consists of small, slightly painful
opening is present from which larvae can come
superficial ulceration with non-indurated
borders which appear on lip. In long standing to surface of the lesion (Fig. 23.12).
infection, there is zone of cicatrical refraction,
Management
dark red area with loss of superficial epithelium,
or opaque lichenoid grayish papules. Surgical removal of larvae and irrigation of the
Dysphagia red soft palate and small red tissue with hydrogen peroxide is effective
granulomatous lesions accompanied by regional method of choice.
lymphadenopathy, are commonly associated
symptoms. Cervical lymph-adenopathy is Cat Scratch Disease
present. Skin covering the swollen nodes is It is also called as ‘cat scratch fever’. Disease is
violaceous and indurated usually with one or recognized in 1931 but the exact cause is
more draining sinuses. unknown. Viral as well as bacterial agents have
482 Textbook of Oral Pathology

Histopathological Features
Involved lymph nodes manifest
reticuloendothelial hyperplasia, focal
granuloma, suppuration and necrosis with
capsular thickening. Epitheloid cells and
multinucleated giant cells are occasionally seen.

Management
The disease has a benign course and treatment
is symptomatic including aspiration of the nodes,
if it becomes necessary.
Figure 23.12: Oral myiasis showing detachment of
palate and larva in the palate
Pyostomatitis Vegetans
been proposed as the cause of this disease. But It is an uncommon inflammatory disease of the
in 1988 the causative was named which initially oral cavity. The name is given as there is a
called as Rochalimaea henselae but was similarity between it and the skin lesions in a
reclassified as Bartonella henselae. dermatologic disease known as pyodermatitis
vegetans. It may occur due to intestinal
Clinical Features disturbances.
The incubation time of the disease ranges form
3-30 days. It occur at any age, but predominant Clinical Features
in children and young adults. It is thought to arise It occurs in any age with no sexual predilection.
after traumatic break in skin due to scratch or Oral lesions consist of large number of broad base
bite of household cat. Within few days of indolent papillary projections, tiny abscess or vegetations
primary lesion, often papules or vesicle develop developing in areas of intense erythema. This
at the site of injury. lesion may occur in any area of oral cavity, except
Within one to three weeks, lymphadenitis in tongue. It is multiple in numbers. it is painless.
develops. The nodes are painful and may be These small projections are red or pink in
several centimeter in diameter. The overlying color, but on careful examination may show tiny
skin may be inflamed. The lymph nodes pustules beneath the epithelium, which liberate
gradually become soft and fluctuant, owing to a purulent material when ruptured. When,
necrosis and suppuration. The lympha- condition is present, buccal and labial mucosal
denopathy may persist for one to six months. lesions have many folds and papillary projections
In early stage, low grade fever, headache, may develop on these folds. Yellow vesicles
chills, nausea, malaise or even abdominal pain discharged small amount of purulent material.
may occur. Other manifestations include These leave are as of ulceration, which may
nonpruritic macular or maculopapular rash, coalesce into larger areas of necrosis.
parotid swelling, conjunctivitis and grad Buccal mucosa show ‘cobblestone’
malseizures. In dental point of view there may appearance, while vestibular lesions appeared as
be involvement of preauricular, submaxillary or folds and ulcers, the lips are diffusely swollen
cervical chain of nodes. and indurated, gingival and alveolar mucosal
 Bacterial Infections 483

lesions are granular with erythematous swelling pattern of small abscess or spreads diffusely
and palatal lesions appear as multiple apthous throughout the tissue. Tiny areas of focal necrosis
ulcers. and micro abscess formation, either intraepithelial
or sub-epithelial, are common features of this
Histopathological Features lesion. Focal areas of degeneration and necrosis
of the overlying epithelium are present.
The papillary projections generally show an
intact stratified squamous epithelium with an
Management
underlying loose connective tissue, which is
generally densely infiltrated by large number of It is not specific. It is found that the oral lesions
plasma cells, lymphocytes and occasional are regressed when the intestinal disturbance is
polymorphonuclear leukocytes. It is arranged in brought under control.
484 Textbook of Oral Pathology

CHAPTER
Viral
24 Infections

HERPES SIMPLEX INFECTION Transmission

All herpes viruses contain DNA nucleus which It occurs during close personal contact. Primary
can remain latent in host neural cells, thereby infection of new born is believed to be caused
evading host immune response. HHV-6 is a newly by vaginal secretions during birth, which results
discovered virus and can infect T4-lymphocytes in viremia and disseminated infection of brain,
and is a possible factor in HIV infection. It is the liver, adrenals and lungs.
most common viral disease affecting men. Dentist may experience primary lesion of
fingers from contact with lesions of the mouth
Epidemiology or saliva of the patients who are asymptomatic
carriers of HSV called as 'herpetic whitlow'.
• HSV I — Infections above the waist Incidence varies according to socioeconomic
• HSV II — Infections below the waist group.
• Both HSV I and II can be transmitted sexually
• Occur in early childhood Clinical Features
• Preschool period is more prone due to frequent
Incubation period is 5 to 7 days, but may range
exchange of salivary and nasal secretions.
from 2 to 12 days. It develops in both, children
and young adults. Prodormal symptoms precede
Primary Herpes Simplex Infection
local lesion by 1 to 2 days and it includes fever,
It is also called as 'acute herpetic gingivostomatitis', headache, malaise, nausea, vomiting and within
'herpes labialis', 'fever blister', 'cold sore' and a few days, mouth becomes painful. There is also
'infectious stomatitis'. It occurs in patients with no irritability, pain upon swallowing and regional
prior infection with HSV-1. HSV reaches nerve lymphadenopathy.
ganglion supplying the affected area, After this, small vesicles, which are thin
presumably along nerve pathways and remains walled, surrounded by inflammatory base are
latent until reactivated. The usual ganglion formed. They quickly rupture leaving small,
involved is the trigeminal for HSV-1 and shallow, oval shaped discrete ulcers. The base
lumbosacral, for HSV-2. of the ulcer is covered with grayish white or

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 Viral Infections 485

yellow plaque. The margins of the sloughed

lesions are uneven and are accentuated by bright
red rimmed, well demarcated, inflammatory
halos.
The individual ulcer differs in size from 2-6
mm. As the disease progresses several lesions
may coalesce, forming larger, irregular lesions.
In severe cases, excoriation involving the lips
may become hemorrhagic and matted with
serosanguinous fibrin like exudate and parting
of the lips during mastication and speech, may
become extremely painful and difficult.
There is appearance of generalized marginal
acute gingivitis. Entire gingiva is edematous and
swollen and small gingival ulcers are seen.
Examination of posterior pharynx reveals
inflammation. Cervical and submandibular
lymphadenopathy can also occur. Lesions begin
healing in a week to 10 days and leave no scar.
HSV may confine to saliva for up to 1 month after
Figure 24.1: H. Simplex infection showing ballooning
onset of disease. degeneration (BD), vesicle formation (V), inflammatory cells (IC)

Histopathological Features patient with primary or recurrent herpes is

helpful in making the diagnosis.
Intraepithelial blisters filled with fluid.
Patient is easily diagnosed as having primary
Intranuclear inclusions: Lipschutz bodies which herpetic gingivostomatitis if he/she presents
are eosinophilic, ovoid, homogenous structure with typical clinical features of generalized
within the nucleus, tend to displace the nucleolus symptoms followed by eruption of oral vesicles
and nuclear chromatin peripherally. The and acute marginal gingivitis and does not have
displacement of chromatin often produces a peri- history of recurrent herpes.
inclusion halo.
HSV isolation—Isolation and neutralization of
When vesicle rupture, surface of the lesion is
virus in tissue culture is most positive method
covered by exudate made up of fibrin polymor-
of identification. Rabbit kidney and human
phonuclear leukocytes and degenerated cells.
amnion are sensitive to HSV.
Fresh vesicles can be opened and scraping made
from base of lesion and placed on microscopic Antibody titer—Antibodies to HSV appear in a
slide, which is stained with giemsa, PAP stain week and react peak in 3 weeks.
and searched for multinucleated giant cells, bal-
looning degeneration and synctium (Fig. 24.1). Management
It is treated by symptomatic like topical
Diagnosis
anesthetics, topical anti-infective agents or
Negative past history of recurrent herpes labialis specific antiviral drugs acyclovir, idoxiurdine,
and a positive history of close contact with a cytosine arabinoside and adenine arabinoside.

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Recurrent or Secondary Herpetic Infection palate and alveolar region. The lesions gradually
heal within 7-10 days and leave no scars.
Recurrent infections are limited to localized
portions of skin and mucus membrane.
Histopathological Features
Types Tzanck cells, ballooning degeneration and
typical Lipschutz bodies are present.
• Recurrent herpes labialis (RHL)
• Recurrent intraoral herpes simplex infection
Management
(RIH).
Oral acyclovir and topical use of carbon oxolone
Precipitating Factors is useful in herpetic gingivostomatitis.
It is precipitated by surgery involving trigeminal
Measles
ganglion as it remains latent in trigeminal
ganglion, trauma to lips, fever, emotional upset It is also called as 'Rubeola' or 'morbilli'. It is an
and upper respiratory tract infection. Sunburns, acute contagious dermatotropic viral infection,
fatigue, menstruation and pregnancy. Emotional primarily affecting children and occurs many
upset, allergy and dental extraction. times in epidemic form.

Clinical Features Transmission

If it occurs on lip, it is called as recurrent herpes Spread of disease occurs by direct contact with
labialis. If occurs intraorally it is called as recurrent a person or by droplet infection, the portal of
intraoral herpes infection. Recurrent herpes entry being the respiratory tract.
simplex infection may occur at widely varying
Clinical Features
intervals, from nearly every month in some
patients to only about once a year or even less in Incubation period is 8 to 10 days. Onset of fever,
others. Lesions may develop lips or intraorally. malaise, cough, conjunctivitis, photophobia,
In either location, lesion is preceded by lacrimation and eruptive lesions of skin and oral
tingling and burning sensation and feeling of mucosa occurs. Otitis media and sore throat can
tautness, swelling or slight soreness subsequent occur. Skin eruption begins on face, in the hair
development of vesicle. It is accompanied by line and behind the ear and spread to neck, chest,
edema at the site of the lesion, followed by back and extremities. It appears as tiny red
formation of clusters of small vesicles. It range macules or papules which enlarge and coalesce
from 1 to 3 mm in diameter, to 1 to 2 cm. But to form blotchy discolored irregular lesions,
sometimes it is large enough to cause which blanch on pressure. Fade away in 4 to
disfigurement. These gray or white vesicles 5 days with fine desquamation.
rupture quickly leaving small red ulcerations,
Oral Manifestations
sometimes with slightly erythematous halo on
lip covered by brownish crust on lips. In RIH Oral lesions precede 2 to 3 days before cutaneous
vesicles break rapidly to form small red rash and are pathognomonic of this disease. The
ulceration, sometimes with slight erythematous most common site is on buccal mucosa. Intraoral
halo. Cluster of small vesicles or ulcers 1 to 2 mm lesions are called as Koplik's spots and occur in
in diameter are commonly found on gingivae, 97% of cases. They are small, irregularly shaped

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 Viral Infections 487

flecks which appear as bluish white specks Chickenpox

surrounded by bright red margins. Generalized
It is also called as 'varicella'. It is an acute viral
inflammation, congestion, swelling and focal
disease occurring in children and most
ulceration of gingiva, palate, throat may occur.
commonly in winter and spring months.

Histopathological Features Clinical Features

Koplik spot represent areas of focal hyper- Incubation period is two weeks and mode of
parakeratosis which exhibits spongiosis, transmission is by air borne droplet or direct
intercellular edema, dyskeratosis, and epithelial contact with infected persons, with the probable
syncytial giant cells in epithelium. There is pink port of entry being respiratory tract. It is
staining inclusion in the nuclei or less commonly characterized by prodromal occurrence of
in the cytoplasm. This inclusion represents headache, nasopharyngitis and anorexia,
microtubular aggregates characterteristic of the followed by maculopapular or vesicular
paramyxovirus. There is also microabscess eruptions on skin and low grade fever. These
formation, epithelial necrosis and ulceration. eruptions usually begin on the trunk and spread
to involve the face and extremities. They occur
Management in successive crops so many vesicles in different
stages of formation or resorption may be found.
The patient should be isolated, if possible.
The skin eventually ruptures, forming a
Antiviral drug and vitamin A should be given.
superficial crust and heals by desquamation. The
disease runs its clinical course in a week to ten
Varicella Zoster Infection
days, seldom leaving any after effects.
It is an acute disease caused by varicella zoster Occasionally, secondary infection of vesicle
virus, which is a DNA virus similar to HSV and results in the formation of pustules which may
causes both, primary and recurrent infection. leave small pitting scar upon healing.
After primary disease is healed, VZV becomes
latent in the dorsal root ganglion of spinal nerve Oral Manifestation
or extramedullary ganglion of cranial nerve. Small blister like lesions occasionally involve the
VZV becomes reactivated causing lesions of oral mucosa chiefly buccal mucosa, tongue,
localized herpes zoster. Patients with HIV gingiva, palate as well as the mucosa of pharynx.
infection, leukemia and those on immuno- The mucosal lesion, initially a slightly raised
suppressive therapy have an increased vesicle with a surrounding erythema, ruptures
susceptibility to severe or potentially fatal herpes soon after formation and forms a small eroded
zoster. Herpes zoster infection can be deep seated ulcer with red margins, closely resembling
and disseminated causing pneumonia, aphthous lesion.
meningocephalitis and hepatitis.
Histopathological Features
Types
The cytological studies are identical to that of
• Chickenpox (varicella) herpes simplex infection. These viruses cause
• Shingles (herpes zoster) or zona. acanthylosis with formation of numerous free

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488 Textbook of Oral Pathology

floating Tzanck cells. There is also nuclear

margination of chromatin and occasional
multinucleation.

Management
No treatment is required in majority of the cases.

Herpes Zoster
It is also called as 'shingles' or 'Zona'. It is an acute
infectious viral disease of extremely painful and
incapacitating nature, characterized by
Figure 24.2: Intact vesicle seen in the case of herpes
inflammation of dorsal root ganglion, associated
zoster infection
with vesicular eruptions of skin and mucus
membrane of the area supplied by the affected
sensory nerve.

Predisposing Factors
It is caused by trauma. Development or
malignancy or tumor involvement of dorsal root
ganglion. Local X-ray radiation or immuno-
suppressive therapy.

Clinical Features
It affects males and females with same fre-
quency. Prodormal period of 2 to 4 days in which
Figure 24.3: There is corneal scarring and ulceration
shooting pain, paraesthesia, burning and tender- occur on the face in case of herpes zoster
ness appears along the course of affected nerve.
Unilateral vesicles on an erythematous base
Postherpetic neuralgia — Pain may continue for
appear in clusters, chiefly along the course of
nerve and giving picture of a single dermatome weeks to months. This unfortunate sequel called
as postherpetic neuralgia occurs in elderly
involvement. Vesicle turns into scab in 1 week
persons due to inflammation, fibrosis and
and healing takes place in 2 to 3 weeks (Fig. 24.2).
Nerves commonly affected are C3, T5, L1, L2 and scarring of nerve and may cause severe pain after
the skin lesions has healed (Fig. 24.4).
1st division of trigeminal nerve. It may affect
motor nerve. Involvement of 1st division leads to
Oral Manifestations
corneal scarring and blindness which are
presumably related to viral spread, neural It results from involvement of 2nd and 3rd
damage, vasculitis and inflammatory immune divisions of trigeminal nerve. It may be found
response (Fig. 24.3). on buccal mucosa, tongue, uvula, pharynx and
If Hutchison's sign (cutaneous zoster of the larynx. Lesions of oral mucosa are extremely
side of tip of nose) is present, then the probability painful. The lesions rupture to leave areas of
of ocular involvement is more. erosion (Figs 24.5 and 24.6).

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 Viral Infections 489

Figure 24.6: Ulcer occurring on dorsal surface of tongue

Histopathological Features
These are similar to primary infection.

James-Ramsay Hunt Syndrome

It is zoster infection of geniculate ganglion with
involvement of the external ear and oral mucosa.
The clinical manifestation of it is facial paralysis
Figure 24.4: Healed vesicle of herpes zoster causing as well as pain of the external auditory meatus
discoloration
and pinna of the ear. In addition, vesicular
eruption occurs in the oral cavity and
oropharynx with hoarseness of voice, tinnitus,
vertigo and occasional other disturbances.

Lab Findings
• Cytology
• Viral isolation
• Antibody titer increased
• Fluorescent antibody stained smear or
fluorescent conjugated monoclonal antibody.

Management
Figure 24.5: Vesicle seen on to the lip and buccal
mucosa Acyclovir 800 mg five times daily which is
associated with significantly accelerated healing
within 48 hours of the onset of rash.
Trigeminal herpes zoster occurring during
tooth formation causes pulpal necrosis and Postherpetic neuralgia — To control postherpetic
internal root resorption. Findings are similar to neuralgia, prednisone 40-60 mg daily for 1 to 2
herpes infection, but it is associated with weeks. Steroid injection can be given in a patient
neurogenic pain of unilateral nature and with age more than 60 years, for the treatment
segmental distribution of the lesion. of postherpetic neuralgia.

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Coxsackievirus Infection Laboratory Diagnosis

They are RNA retroviruses and are named after No ballooning degeneration seen which is
town in upper New York where they were first helpful to distinguish herpangina from herpes
discovered. They are divided into 2 groups: simplex and herpes zoster.
• Type A - 24 types
• Type B - 6 types. Management
These viruses can cause hepatitis, meningitis,
myocarditis, pericarditis and respiratory disease. Self-limiting and supportive treatment by proper
Three clinical types are observed. hydration and topical anesthetic, when eating
• Herpangina or swallowing is difficult.
• Acute lymphonodular pharyngitis
• Hand-foot mouth disease. Acute Lymphonodular Pharyngitis
It is caused by A10 and is same as herpangina.
Herpangina
Yellow-white nodules appear that do not
It is also called as 'aphthous pharyngitis', progress to vesicles or ulcers. It is self-limiting
'vesicular pharyngitis'. A4 causes majority of the and only supportive care is indicated.
cases. A4 to A10 and A16 to A22 have also been
implicated. Frequently occurs in epidemic, with Clinical Features
highest frequency from June to October. It
appears to be transmitted from one person to The disease affects predominately children and
another through contact. young adults, occasionally older adults can also
be affected. The lesion appears on uvula, soft
Clinical Features palate, anterior pillars and posterior oropharynx.
Majority affected are young children aged 3 to It has got 5 days incubation period and course
10 years. Incubation period is of 2 to 10 days. may run for 4 to 14 days, with local oral lesions
Initially, generalized symptoms of fever, chills, resolving within 6 to 10 days. The chief complain
headache, anorexia, prostration, abdominal pain is of sore throat, 41 degree Celsius temperature,
and sometimes vomiting. Sore throat, dysphagia mild headache, anorexia and loss of appetite. It
and occasionally, sore mouth can occur. consists of raised, discrete, whitish to yellowish
It occurs on posterior pharynx, tonsil, faucial solid papules of 3 to 6 mm in diameter,
pillars and soft palate. Lesion starts as punctuate surrounded by narrow well defined zone of
macule which evolves into papules and vesicles. erythema. Lesion is nonvascular, nonulcerated,
Within 24 to 48 hours, vesicles get ruptured tender, superficial and bilateral.
forming small 1 to 2 mm ulcers. Ulcers show a
grey base and inflamed periphery. They Histopathological Features
generally heal without treatment in 1 week.
The papular lesion consists of densely packed
Histopathological Features nodules of lymphocytes. In some cases, overlying
epithelium has shown inclusion bodies which
In these areas of affected epithelium exhibits
in some instance were intra-nuclear, but in other
intracellular and intercellular edema, that leads
cytoplasmic.
to spongiosis and formation of intraepithelial
vesicle. There is rupture of vesicle with formation
Hand-foot Mouth Disease
of subepithelial vesicle. There is also epithelial
necrosis and ulceration. It is cause by A16, A5, A7, A9, A10, B2 and B5.

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 Viral Infections 491

Clinical Features headache. The patient is extremely ill and may

It primarily affects children between the age of become comatose during this period. The skin
6 months and 5 years. It is characterized by lesions begin as small macules and papules
appearance of maculo-papular, exanthematous which first appear on face but rapidly spread to
and vesicular lesions of skin, particularly cover much of the body surface, within few days.
involving the hands, feet, legs, arms and The papules develop into vesicles. The pustules
occasionally buttocks. There is anorexia, low are small elevated and yellowish green, with
grade fever and sometimes lymphadenopathy, inflamed border. They are secondarily infected
diarrhea, nausea and vomiting. and occasionally become hemorrhagic.
Desquamation marks the beginning of the
Oral Manifestations healing phase of disease.
The most common sites for oral lesions are hard Oral Manifestations
palate, tongue and buccal mucosa. A sore mouth
Ulceration of oral mucosa and pharynx is
with refusal to eat is one of the most common
common. Multiple vesicles appear and rupture
findings in this disease. The tongue may become
to form ulcers of non- specific nature. In some
red and edematous. Oral lesions are more
cases, the tongue is swollen and painful, making
extensive than herpangina. Clinical
swallowing difficult.
manifestations last for 3 to 7days.
Management
Laboratory Findings
Once the disease is present, treatment is
Intracytoplasmic viral inclusion can be seen in
symptomatic and immunization is essential.
vesicular scrapping of the lesions. There is rise
in acute or convalescent serum antibody titer to Foot and Mouth Disease
coxsackie A16.
It is a viral infection which rarely affects man,
Management but does affect hogs, sheep as well as cattle.
Transmission of this disease occurs through
No specific treatment is necessary since the
infected animals; in human beings, it is usually
disease is self- limiting and generally regresses
within one to two weeks. through milk from infected animals.

Smallpox Clinical Features

It is also called as 'variola'. On December 9, 1979, It is manifested by fever, nausea, vomiting,
the WHO global commission for the certification malaise and appearance of ulcerative lesions of
of smallpox declared that smallpox eradication oral mucosa and pharynx. Development of
has been achieved throughout the world. Thus, vesicle on skin also occurs in some cases, usually
there has been for the first time in history that a on the palms of hands and soles of feet.
disease was totally eliminated from this planet.
Oral Manifestations
Clinical Features
It can occur at any site, but lips, tongue, palate
Incubation period is of seven to ten days. Disease and oro-pharynx appear to be affected. These
manifests itself clinically by the occurrence of lesions being as small vesicles which rapidly
high fever, nausea, vomiting, chills and rupture, but heal within two weeks.

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Condyloma Acuminatum Management

It is also called as 'genital wart', 'venereal wart' Genital warts are treated by excision, electro-or
or 'verruca acuminate'. It is caused by human cryosurgery, CO2 laser therapy application of
papillomavirus (HPV). It can be transmitted chemical agents such as podophyllin,
from mother to infant, at birth and resulting cantharidin and 5-fluorouracil. Immuno-
syndrome is called as juvenile onset respiratory modulating agent such as interferons can also
papillomatosis. be used.

Clinical Features Molluscum Contagiosum Infection

They are pink, fleshy papillomatous lesions It is caused by virus of pox group.
developing on external genitals, perianal
mucosae, adjacent skin as well as in vaginal and Transmission
anal canal. Wart growth is favored by moist It occurs due to intimate skin contact. There is
warm environment of perianal skin and mucosal
marked increase at the time of onset of sexual
surface. It proliferates and coalesces rapidly to
activity, poverty, overcrowding and poor
form diffuse papillomatous clusters of varying hygiene.
size. Recurrence is common.
Clinical Features
Oral Manifestations
Incubation period is 14 to 50 days. It is more
It may involve gingiva, cheek, lip, hard palate, common in children and young adults. It is more
tongue and floor of mouth. Small keratotic warts
common on skin or inner thigh lower abdomen
occurring alone or in clusters on oral mucosa can
or external genitals. It manifests as multiple or
be seen. The enlargement may be larger than 1 isolated discrete elevated nodules, or sometimes
cm in diameter. The lesion is sharply delineated
papules, with depressed centers, which may be
and may appear sessile and pedunculated. It
keratinized and are normal or slightly red in
presents as discrete papillomatous growth. color.
These lesions are hemisphere in shape,
Laboratory Investigation
usually about 5 mm in diameter. Multiple lesions
Virus isolation can be done by staining of viral sometimes numbering 100 can also occur. It is
antigen DNA by hybridization restriction, self-limiting and regresses spontaneously within
endonuclease analysis and polymerase chain 1 to 2 months.
reaction.
Oral Manifestations
Histopathological Features
It usually occurs in children, on the face, due to
The papillomatous projection making up the shared sleeping accommodation. Most
verrucoid lesion generally shows a parakeratotic commonly involved sites are lips, tongue and
surface with marked underlying acanthosis. buccal mucosa; lesions are similar to skin lesions.
Vacuolated cells in the spinous layer are
common. The supporting connective tissue is Histopathological Features
usually edematous, with dilated capillaries and It shows thickening and down growth of
a chronic inflammatory cell infiltrate. epithelium with the formation of large

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 Viral Infections 493

eosinophilic intracytoplasmic inclusion bodies Histopathological Features

known as Henderson-Paterson inclusion or
There are changes within the vascular
simply Molluscum bodies, measuring
endothelial cells. Scattered infected cells are
approximately 25 microns in diameter. These
swollen showing intracytoplasmic and
bodies, characteristically are accumulated in the
intranuclear inclusion and prominent nucleoli.
crater formed by the distinctive central
Salivary ductal cells also affected and form owl
umblicated or the dome shaped lesion.
eye cells.
Management
Management
It includes curettage, followed by local cautery,
Prevention by passive immunization with
cryotherapy. Topical application of caustic acid
hyperimmune gamma globulin can be success-
and irritants such as phenol, TCA, podophyllin
ful.
and cantharidin.
AIDS
Cytomegalovirus Infection
It may be clinically expressed or latent. When It is a devastating fatal disease which is in
expressed, it results in characteristic enlargement epidemic form throughout the world. It is an
of cells with prominent and pathognomonic, incurable viral STD which is cause by human
intranuclear and intracytoplasmic inclusions. immune deficiency virus. It stands for:
• A—Acquired, i.e. contagious not inherited
Epidemiology • I—Immune, i.e. power to receive disease
• D—Deficiency.
It is wide spread with prevalence rates based on • S—Syndrome, i.e. number of signs and
the presence of CMV antibodies, ranging from complains indicative of particular disease.
80 to 100%, in most adult population. Blood and Four identified etiological agent are of
transplanted tissues are also potential means of substantially lenti virus (HIV-I an HIV-II) that
transmission of virus to susceptible individuals, causes slow infection in which sign and
rather than saliva, urine, serum, vaginal symptoms only appear many months or years
secretion. Infants can acquire it in utero from after infection and two member of oncovirus
maternal virus reactivation during pregnancy. (HTLV-I and HTLV-II) that are capable of
oncogenic transformation and are usually
Clinical Feature
associated with leukemia or lymphoma. The case
It may be accompanied by mononucleosis like of AIDS was detected in June 1981 when 5 young
illness or severe illness with neurologic homosexuals men came with the suffering from
abnormalities. Hepatosplenomegaly, jaundice, rare lung infection due to microorganism called
petecheal hemorrhages can also occur. Pneumocystis carinii. In India the first description
Pneumonia, microcephaly, cerebral calcification of AIDS came in Madras where 6 women out of
and hearing defect can also occur. 125 who were screened were HIV positive in
high risk group of prostitutes.
Oral Manifestations
AIDS appear to be endemic in central and
Patient may suffer from gingivitis and gingival equatorial Africa and it may be old disease of
hyperplasia. Oral ulcers may occur sometimes. Africa that has gone unrecognized. HIV-1

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494 Textbook of Oral Pathology

infection has also become the primary emphasis Category B — Condition is attributed to HIV
of effort at controlling STDs. Moreover, the infection or indicative of defect in the cell
knowledge gain about sexual and other behavior mediated immunity.
associated with transmission of HIV, as well as • Bacillary angiomatosis
strategies that have been effective in modifying • Oropharyngeal and vulvovaginal candidiasis
those behaviors, is transferable to other sexually • Cervical carcinoma in situ
transmittable and bloodborne infections and has • Constitutional symptoms like fever (38.5°C)
revolutionized standard approaches to the and diarrhea
control of these infections. • Oral hairy leukoplakia and herpes zoster
Oral and perioral lesions are common in patient • Idiopathic thrombocytopenic purpura.
with human immunodeficiency virus, are often
Category C— AIDS indicative condition
the presenting feature and may have deterioration
• Candidiasis of bronchi, trachea or lung and
of general health and a poor prognosis.
esophageal candidiasis
Definition • Invasive cervical cancer
• Disseminated or extrapulmonary coccidio-
WHO has given following definition of AIDS: idomycosis, extrapulmonary cryptococcosis
One or more opportunistic infections listed • Chronic intestinal cryptosporidiosis more
in clinical features that are atleast moderately than 1 month duration
indicative of underlying cellular immune • Cytomegalovirus retinitis with loss of vision
deficiency. • HIV-related encephalopathy
Absence of all known underlying causes of • Herpes simplex bronchitis, pneumonitis and
cellular immune deficiency (other than HIV esophagitis
infection) and absence of all other causes of • Kaposi sarcoma, Burkitt's lymphoma and
reduced resistance reported to be associated immunoblastic lymphoma
with atleast one of those opportunistic diseases. • Mycobacterium tuberculosis infection at any
pulmonary or extrapulmonary sites
Classification (see box below)
• Pneumocystis carinii pneumonia and recurrent
Category A—In adolescent less than 13 years with pneumonia
documented HIV infection • Progressive multifocal leukoencephalopathy
• Persistence generalized lymphadenopathy. • Toxoplasmosis of brain and wasting syndrome
• Active condition. • Recurrent Salmonella septicemia.

1st classification (given in 1993) by CDC

CD4 + T cell categories A B C
Asymptomatic, acute HIV Symptomatic, not A AIDS indicator condition
and PGL or C conditions
More 500/μL A1 B1 C1
200 to 499/μL A2 B2 C2
Less than 200/μL
AIDS indicator T cell count A3 B3 C3

https://t.me/RoyalDentistryLibrary/
 Viral Infections 495

2nd classification by USPHS-CDC immune complex. Cutaneous anergy to multiple

• Group I — Acute infection skin test antigens. Anergy is impaired or inability
• Group II — Asymptomatic infection to react to skin antigens.
• Group III — Persistence generalized
Prevalence
lymphadenopathy.
• Group IV — Other disease It is more common in western countries
– Subgroup A — Constitutional diseases particularly in United State. Largest population
– Subgroup B — Neurological diseases of AIDS is in homosexual, intravenous drug
– Subgroup C — Secondary infectious users, heterosexuals with sexual contact with
diseases AIDS patient. Patients who received transfusion
- C1—Specified secondary infectious of blood or blood pigments donated by the
diseases listed in CDC surveillance person with risk factors. Ninety two percent of
victims are males, 6.5% female with 1% children.
definition for AIDS.
It is common at the age of 25 to 49 years.
- C 2 —Other specified secondary
infectious stages Six Group at Risk of Developing AIDS
– Subgroup D—Secondary cancer
• Homosexuals or bisexuals—71.4%
– Subgroup E—Other conditions.
• Intravenous drug users—18.4%
AIDS-Related Complex • Hemophilia
• Recipient of multiple blood transfusion
For clinical and research studies, persons • Infant born of parents belonging to first three
exhibiting complex clinical problems and high risk groups
immunological or hematological abnormalities • Heterosexual contacts of high risk group.
on the laboratory tests, have been classified as
having AIDS related complex. ARC requires any Etiology
two or more symptoms and two or more T lymphocytes—There is quantitative and
abnormal laboratory findings. It must be present qualitative defiency of T4 helper cells in AIDS
for at- least 3 months. patients. This lead to certain investigators to
focus their efforts on determining if etiologic
Symptoms
agent was a virus that manifested a particular
There is lymphadenopathy, weight loss of 15 lbs tropism for T4 helper lymphocytes.
or 10 % of body weight. Fever of 38.5°C which is HTLV-III virus — Dr Robent C Galleo
intermittent or continuous, diarrhea, fatigue and determined that type C retrovirus was tropic for
malaise and night sweats. T4 lymphocytes in adult T cell leukemia/
lymphoma. He named the virus, humans T cell
Lab Findings
leukemia/lymphoma virus (HTLV-I). So, it is
Decreased number of T helper cell. Decreased considered to be etiological agent for AIDS. But
ratio of T helper cells to T suppressor cells. as it causes lymphoproliferation in T cell
Anemia or leucopenia or thrombocytopenia or leukemia, where as AIDS is a disease of
lymphopenia. Increased serum globulin level. lymphodepletion. The answer came in the
Decreased blastogenic response of lymphocytes discovery of type D retrovirus of HTLV family
to mitogen. Increased level of circulating that has been termed as HTLV-III.
496 Textbook of Oral Pathology

LAV virus—On the other hand, virus called as lymphocytes → it get differentiated into effecter
lymphoadenopathy associated virus (LAV), was cell like T helper cell or T4 and T suppresser cell
being isolated from the AIDS patient in Europe. or T8 → T4 cells secrete various lymphokines
HTLV-III and LAV is closely related which induce lymphocyte to differentiated into
members of same class of virus. Finally it is plasma cell → it secrete specific antibodies
proved that HTLV and LAV are cytopathic against viral antigen → it destroy the virus.
human T-lymphocytotropic viruses that
Mechanism in AIDS — HIV virus is lymphotropic
manifested selective infectivity for the helper/
virus → its primary target is T4 cell → when the
inducer subset of T cells that as phenotypically virus enters the blood stream, it integrates its
designated reactivity with monoclonal antibody
gene into DNA of some primary T4 lymphocyte
T4 or Leu3.
→ this viral DNA then becomes integrated into
HIV — In order to avoid different nomenclatures the host chromosomes → the chromosomal
retrovirus responsible for the AIDS are named integration is prerequisite for replication of
'Human immunodeficiency virus' which belong retroviruses, but also for the latency → once the
to family of retroviruses. viral genes are integrated into cells of own DNA,
they can apparently remain dormant for an
Characteristic of the HIV Virus indefinite period of time, without causing its
affects. This is called as 'incubation period' →
HIV is a spherical enveloped virus, about 90-120
once the viral gene is activated, virus particles
nm in size. The nucleocapsid has an outer
convert T4 lymphocytes into AIDS virus factory
icosahedral shell and inner cone shaped core,
→ when the number of T4 lymphocyte is
enclosing the ribonucleoproteins. The genome
severely depleted, the immune system collapses
is diploid, composed of two identical single
and variety of infections occur at this stage
stranded, positive sense RNA copies. Inside the
patient is said to have AIDS.
envelops is a protein core, which contain
enzymes reverse transcriptase, intregrase, Transmission
protease, etc. all essential for viral replication and
maturation. When the virus infects a cell, the Repeated intimate contact — It is in 90% of cases.
viral RNA is transcribed by the enzymes, first It depends upon number of sexual partners,
into single stranded DNA and then to double receptive anal intercourse and presence of other
stranded DNA (provirus) which is integrated STDs. All these are in high risk group.
into the host cell chromosomes. The virus is Prostitution is a major heterosexual factor
extremely sensitive to heat, thus boiling and associated with AIDS.
autoclaving are very effective measure of Use of contaminated blood products—Intravenous
inactivating the virus. drug users, HIV contaminated blood transfusion,
blood clotting concentrate and organ
Mechanism of Action transplantation.
HIV attacks the immune system of the body. Due Perinatal transmission—It occurs in 13% among
to that an individual is not able to protect himself children born to HIV seropositive mother.
from potentially harmful organism.
Other nosocomal routes—Transmission from
Normal mechanism—Pathogenic viruses → patient to patient due to reuse of contaminated
identified by macrophage → it activate T and shared needles.
 Viral Infections 497

Professional hazards—The risk of transmission the stage of HIV infection and is a predictor of
from HIV infected patient to health care workers the progression of HIV disease. Ninety-five
is more than health care workers to patient. percent of AIDS patients have head and neck
lesion and about 55% have important oral
Clinical Features manifestation. They are as follows:
Protozoan and helminthes infection—
Oral Disorders in HIV Disease
Cryptosporidiosis (intestinal) causing diarrhea
for over one month. The most common A. Fungal
opportunistic infection is by Pneumocystis carinii More common
which causes pneumonia. CNS infection or other • Candidiasis.
disseminated infections and toxoplasmosis. Less common
• Aspergillosis
Fungal infection—Candidiasis causing
• Histoplasmosis
esophagitis, cryptococcosis causing CNS
• Cryptococcus neoformans
infection, disseminated histoplasmosis and
• Geotrichosis.
bronchial or pulmonary candidiasis.
B. Bacterial
Bacterial infections—Mycobacterium avium More common
intracellulare causing infection disseminated • HIV gingivitis
beyond lung and lymph node. Mycobacterium • HIV periodontitis
tuberculosis causing tuberculosis. • Necrotizing gingivitis.
Less common
Viral infections—Cytomegalovirus, causing
infection in the internal organs other than liver, • Mycobacterium avium intracellulare
• Klebsiella pneumoniae
spleen and lymph nodes. Herpes simplex virus,
• Enterobacterium cloacae
causing chronic mucocutenous infection with
ulcers persisting more than one month. • E. coli
• Salmonella enteritidis
Malignancy—Kaposi’s sarcoma and squamous • Sinusitis
cell carcinoma. Lymphoma limited to bronchi • Exacerbation of apical periodontitis
and non-Hodgkin lymphoma. • Submandibular cellulitis.
C. Viral
Oral Manifestations
More common
Oral manifestations of HIV disease are common • Herpes simplex
and include oral lesions and novel presentations • Varicella zoster
of previously known opportunistic diseases. • Epstein-Barr including hairy leukoplakia
Careful history taking and detailed examination Less common
of the patient's oral cavity are important parts of • HPV virus
the physical examination and diagnosis requires • CMV virus.
appropriate investigative techniques. Early D. Neoplasm
recognition, diagnosis and treatment of HIV- More common
associated oral lesions may reduce morbidity. • Kaposi's sarcoma.
The presence of these lesions may be an early Less common
diagnostic indicator of immunodeficiency and • Non-Hodgkin lymphoma
HIV infection, may change the classification of • Squamous cell carcinoma.
498 Textbook of Oral Pathology

E. Lymphadenopathy Clinical Features

F. Neurologic disorders Patient with HIV usually has lesion of hard
Less common palate and soft palate. The clinical appearances
• Paraesthesia of oral candidiasis vary. The most common
• Facial palsy presentations include pseudomembranous and
• Hyperesthesia erythematous candidiasis, which are equally
• Dysphagia. predictive of the development of AIDS and
G. Miscellaneous angular cheilitis. These lesions may be associated
Less common with a variety of symptoms, including a burning
• Recurrent aphthous ulceration mouth, problems in eating spicy food and
• Progressive necrotizing ulceration changes in taste. All three of these common
• Toxic epidermolysis forms may appear in one individual.
• Delayed wound healing Pseudomembranous candidiasis (Thrush)—
• Thrombocytopenia characteristic creamy white, removable plaques
• Xerostomia and sicca type syndrome on the oral mucosa are caused by overgrowth of
• HIV embryopathy fungal hyphae mixed with desquamated
• Hyperpigmentation epithelium and inflammatory cells. The mucosa
• Granuloma annulare may appear red when the plaque is removed.
• Exfoliative cheilitis This type of candidiasis may involve any part of
• Lichenoid and other drug reaction. the mouth or pharynx.
Erythematous candidiasis—Erythematous
Candidiasis candidiasis appears as flat, red patches of varying
size. It commonly occurs on the palate and the
Oral candidiasis is most commonly associated
dorsal surface of the tongue. Erythematous
with Candida albicans, although other species,
candidiasis is frequently subtle in appearance and
such as C. glabrata and C. tropicalis, are frequently
clinicians may easily overlook lesions, which may
part of the normal oral flora. A number of factors
persist for several weeks if untreated.
predispose patients to develop candidiasis:
infancy, old age, antibiotic therapy, steroid and Angular cheilitis—Angular cheilitis appears
other immunosuppressive drugs, xerostomia, clinically as redness, ulceration and fissuring,
anemia, endocrine disorders and primary and either unilaterally or bilaterally at the corners of
acquired immunodeficiency. Candidiasis is a the mouth. It can appear alone or in conjunction
common finding in people with HIV infection. with another form of candidiasis.
Reports describe oral candidiasis during the
acute stage of HIV infection, but it occurs most Diagnosis
commonly with falling CD4+ T cell count in Candida is a commensal organism in the oral
middle and late stages of HIV disease. Several cavity. Candidiasis is diagnosed by its clinical
reports indicate that most persons with HIV appearance and by detection of organisms on
infection carry a single strain of Candida during smears. Smears taken from clinical lesions are
clinically apparent candidiasis and when examined using potassium hydroxide (KOH),
candidiasis is quiescent. PAS, or Gram's stain.
 Viral Infections 499

Management It can be found on any mucosal surface and

are contagious to both host and sex partner.
It is discussed in Chapter of Oral Keratotic and
Nonkeratotic Lesions.
Management
Recurrent Herpes Labialis Simultaneous irradiation of all lesions and care
of infected partner. Local excision and
It mainly appear as herpes labialis and recurrent
cauterization of base.
intraoral herpes. Herpes labialis occurs as
characteristic lip lesion consisting of vesicles on
Herpes Zoster
an erythematous base that heals within 7 to 10
days. RHL are small, shallow, irregular and It occurs more frequently in HIV infected
erosive like lesion that may coalesce and seen to patients and carries poor prognosis. The
occur only on keratinized epithelium like that occurrence of unilateral vesicles that break and
of gingiva, hard palate or dorsal surface of scab is characteristic of this infection. They are
tongue. Diagnosis is made by isolation of virus self-limiting. Main complication is neuropathy
from ulcer. after inflammation. Diagnosis is made by
Antiviral drugs and symptomatic, systemic cytological smear and finding of multinucleated
acyclovir 30 mg/kg/day. Acyclovir resistance giant cells.
foscarnet.
Treatment—Systemic acyclovir 800 mg orally or
15-30 mg/kg/day IV 8 hrly for 10 to 15 days.
Oral Human Papilloma Virus Lesions
It is cause by human papilloma virus. Oral warts, Cytomegalovirus Infection
papillomas, skin warts and genital warts are
It has got predilection for salivary glands because
associated with the human papilloma virus many HIV infected patients have xerostomia. It
lesions (HPV). Lesions caused by HPV are
is a hypothesis that in such patient's salivary
common on the skin and mucous membranes of
glands, cytomegalovirus infection produce
persons with HIV disease. Anal warts have inflammation causing reduced salivary
frequently been reported among homosexual
production.
men. Because the HPV types found in oral
lesions in HIV-infected persons are different Treatment—Systemic ganciclovir or foscarnet.
from the HPV types associated with anogenital
warts, clinicians should probably not use the Vesicular-erosive Lesions
term condyloma acuminate to describe oral HPV Other conditions like recurrent aphthous
lesions. stomatitis, erythema multiforme and lichenoid
reactions may occur in HIV patients. They are
Clinical Features reflection of immune dysfunction. Report says
that pituitary suppresses the reduced host
HPV lesions in the oral cavity may appear as
cortisone production, might account for this
solitary or multiple nodules. They may be sessile
lesion.
or pedunculated and appear as multiple,
smooth-surfaced raised masses resembling focal Treatment—Short-term dosage of corticosteroids,
epithelial hyperplasia or as multiple, small topical corticosteroid cream or gel dexama-
papilliferous or cauliflowerlike projections. thasone rinse, chlorhexidine rinse, short-term
500 Textbook of Oral Pathology

topical tetracycline, rarely systemic antibody or Hairy Leukoplakia

metronidazole and thalidomide for chronic
Oral hairy leukoplakia, which presents as a non-
resistance ulcer.
movable, corrugated or "hairy" white lesion on
the lateral margins of the tongue occurs in all risk
Salivary Gland Disease and Xerostomia
groups for HIV infections, although less
Salivary gland disease associated with HIV commonly in children than in adults. It occurs in
infection (HIV-SGD) can present as xerostomia about 20% of persons with asymptomatic HIV
with or without salivary gland enlargement. infection and becomes more common as the CD4+
Reports describe salivary gland enlargement in T cell count falls.
children and adults with HIV infection usually
involving the parotid gland. Etiology
The enlarged salivary glands are soft but not
Exact etiology is not known but Epstein-barr
fluctuant. In some cases, enlarged salivary
glands may be due to lymphoepithelial cysts. The virus has identified in these lesions. One
hypothesis is that basal epithelial cells of lateral
etiology of HIV-SGD is as yet unknown but the
margin of tongue normally harbours EBV in
enlarged parotid glands can be a source of
annoyance and discomfort. Xerostomia is majority of adult population, who are EBV
seropositive and carrier of that disease. It is
sometimes seen in individuals with HIV-SGD.
found primarily in homosexual male. Direct
HIV-infected patients may also experience dry
mouth in association with taking certain infection of Langerhans cell due to HIV induced
loss of factor essential for their integrity and
medications that can hamper salivary secretion,
function, permit reactivation of EBL with
such as antidepressants, antihistamines and anti-
anxiety drugs. frequent epithelial hyperplasia.

Management Clinical Features

Removal of the enlarged parotid glands is rarely It is unique and significant lesion which
recommended. For individuals with xerostomia, primarily occurs unilaterally or bilaterally on the
the use of salivary stimulants such as sugarless lateral border of tongue. It can also occur on
gum or sugarless candies may provide relief. dorsum of the tongue, buccal mucosa, floor of
Candies that are acidic should be avoided as mouth, retromolar area and soft palate. There is
frequent use may lead to loss of tooth enamel. characteristic corrugated and white appearance.
The use of salivary substitutes may also be It does not rub off and may resemble the keratotic
helpful. An increase in caries can occur, so lesion. The surface is irregular and may have
fluoride rinses (that can be bought over the prominent folds or projections, sometimes mark-
counter) should be used daily and visits to the edly resembling hairs (Fig.24.7). Occasionally,
dentist should occur two to three times per year. however, some areas may be smooth and flat.
Lesions occur most commonly on the lateral
margins of the tongue and may spread to cover
Exfoliative Cheilitis
the entire dorsal surface. They may also spread
There is chronic exfoliation of superficial layer downward onto the ventral surface of the
of epithelium on the vermillion border of lip. tongue, where they usually appear flat.
 Viral Infections 501

Elimination or almost complete clinical

resolution of the lesion has occurred in patients
treated with agents such as desciclovir (an analog
of acyclovir), phosphonoformate, Retin A and
podophyllin resin, although lesions tend to recur
within few months. Occasionally, Candida
albicans may be found in HL lesions.
Treatment consists of antifungal medications
like topical agent — clotrimazole 10 mg 5 times
a day, nystatin 10000 units/gm 5 times a day.
Systemically : ketoconazole 200 mg bd a day,
Figure 24.7: Hairy leukoplakia showing characteristic acyclovir, azidothymidine and retinoid acid
corrugated appearance podophyllin resin.
Sometimes white lesion satisfies many
Kaposi's Sarcoma
criteria for diagnosis of hairy leukoplakia, but if
EBV not present this is called as 'pseudo-hairy It is also called as 'angioreticuloendothelioma'.
leukoplakia'. Presence of hairy leukoplakia is It is the most common tumor associated with
fairly indicator of HIV prosensitivity and is AIDS and occurs in 1/3rd of AIDS patients.
predictor of deficiency immunocompetence.
Etiology
Histopathological Features
There is higher incidence of Kaposi's sarcoma is in
Histologically lesion shows hyperkeratosis, homosexual men with AIDS as compared to
acanthosis, ballooning cells, epithelial cells heterosexuals with AIDS. It has been suggested that
contain Epstein-barr virus and no or minimum there is transmissible agent prevalence in
inflammation. Immunochemistry tissue in situ homosexual population, which stimulate certain
hybridization, noninvasive tissue in situ factor such as angiogenesis protein that may be
hybridization, or electron microscopy does critical in the pathogenesis of neoplasm. The patient
demonstrate of Epstein-barr virus. The lesion of with AIDS often shows clustered lesion in the oral
leukoplakia consists of Langerhan’s cells. cavity which suggests direct inoculation of mucosa
with sexually transmitted agent.
Management
Some theories suggest role of cytomegalovi-
Hairy leukoplakia usually is asymptomatic and rus in the pathogenesis of Kaposi's sarcoma, but
does not require treatment. HL is almost always studies on prevalence of antibodies to cytome-
a manifestation of HIV infection and clinicians galovirus in patient with classic and epidemic
should arrange evaluation of HIV disease and Kaposi's sarcoma have failed to demonstrate role
appropriate treatment for patients with HL. HL of cytomegalovirus.
has disappeared in patients receiving high-dose
acyclovir for herpes zoster, presumably because Epidemiology and Form
of the anti-EBV activity of acyclovir. Doses of Kaposi's sarcoma appear in various forms like
acyclovir (2.5 to 3 mg per day for 2 to 3 weeks) classic, African (cutaneous variant), African
usually eliminate HL, but the lesion usually (lymphadenopathy variant ) and Kaposi's
recurs with cessation of treatment. sarcoma associated with AIDS.
502 Textbook of Oral Pathology

Classic type is a rare neoplasm and occurs in in diameter and are usually tender on palpation.
older man. Usually it appears as blue-black It is slow growing but can behave as a very
macule on the lower extremities. It is slow aggressive lesion with rapid visceral
growing and rarely involves the lymph nodes involvement.
and visceral organs.
African Kaposi's sarcoma is considered an Oral Manifestations
endemic disease and affects children, 10-year-
old or younger patients, more common in men It has tendency to involve the oral cavity, with
than women. It appears as exophytic growth hard palate as the most common site. But lesions
located in legs and arms. This form is locally may occur on any part of the oral mucosa
aggressive and lymph nodes involvement is rare. including the gingiva, soft palate, buccal mucosa
The lymphadenopathic form occurs in children and in the oropharynx. It can involve either alone
of 10 years age and younger with same frequency or in association with skin and disseminated
in men and women. The visceral and massive lesions. It may be the first symptom of AIDS.
nodal involvement is common. It can appear as a red, blue, or purplish lesion. It
Kaposi's sarcoma is observed in patients with may be flat or raised solitary or multiple.
kidney transplantation and in patients who re- Occasionally, yellowish mucosa surrounds the
ceive the immunosuppressive drugs for variety lesion. The lesions may enlarge, ulcerate and
of diseases. Drugs such as prednisolone, become infected. Good oral hygiene is essential
cyclosporine and cyclophosphamide have been to minimize these complications.
associated with development of Kaposi's sar- It may vary in size from few mm to a
coma. It usually affects legs, arms, lymph nodes centimeter or more in diameter and are tender
and visceral organs. and painful.
Kaposi's sarcoma with AIDS is common in
Histopathological Features
homosexual but can occur in all risk groups.
Male to female ratio is 20:1. Generally affects It consists of interweaving band of spindle
skin, oral and visceral organs. shaped and or plump endothelial cell and
atypical vascular channels, enmeshed in reticular
Clinical Features or collagen fibers. It consists of numerous, small
capillary type blood vessels which may or may
It occurs commonly in head and neck region. Tip not contain blood. Inflammatory cell infiltration
of nose is peculiar and frequent location of it. It
is common. In late stage, lesion consists of well
can involve lymph nodes, soft tissue, extremities,
defined nodules or lesions with diffuse
GIT, lung, liver, pancreas, spleen and adrenal involvement of the lamina propria.
gland. It can occur at any age but most common
in 5th, 6th, 7th decade except in Africa where it
Management
occur in children. It occurs most commonly in
men but also has been observed in women. Treatment is determined on the basis of the
It begins as multinucleated neoplastic process number, size and location of the oral lesions. The
that manifests as multiple red or purple macules choice of therapy depends on the effect of
and in more advanced stage, a nodule occurring treatment on the adjacent mucosa, pain
on the skin or mucosal surface. Size of it ranges associated with treatment, interference with
from a few millimeters to a centimeter or more eating and speaking and the patient's preference.
 Viral Infections 503

It is important to perform thorough dental neously with no formation of gingival pockets,

prophylaxis before initiating therapy for lesions sometimes involving only isolated areas of the
involving the gingiva. Response to therapy is mouth. Teeth may loosen and eventually fall out,
improved if all local plaque and calculus are but uninvolved sites can appear healthy.
removed. Local application of sclerosing agents Necrotizing stomatitis may develop and
may reduce the size of oral lesions. areas of necrotic bone may appear along the
Local treatment is appropriate for large oral gingival margin. The bone may eventually
lesions that interfere with eating and talking. sequestrate. Patients with necrotizing ulcerative
Oral lesion can be treated surgically or with periodontitis and necrotizing stomatitis
localized intralesional chemotherapy. frequently complain of extreme pain and
Intralesional vinblastine, radiation therapy, spontaneous bleeding. It will produce severe pain.
intravenously interferon alpha and
sclerotherapy with 3% sodium tetradecyl sulfate. Management
Clinicians should refer patients to a periodontist
Atypical Periodontal Disease
or dentist for management. The following
Periodontal disease is a fairly common problem protocol has achieved reasonable success: plaque
in both asymptomatic and symptomatic HIV- removal, local debridement, irrigation with
infected patients. It can take two forms: the rapid povidine-iodine, scaling and root planning and
and severe condition called necrotizing maintenance with a chlorhexidine mouth rinse
ulcerative periodontitis and it's associated and once or twice daily.
possibly precursor condition called linear
gingival erythema. The presenting clinical Idiopathic Thrombocytopenic Purpura
features of these diseases often differ from those Reports have described idiopathic thrombocy-
in non-HIV-infected persons. topenic purpura (ITP) in HIV-infected patients.
Oral lesions may be the first manifestation of this
Clinical Features condition. Petechiae, ecchymosis and hematoma
It often occurs in clean mouths where there is can occur anywhere on the oral mucosa. Spon-
very little plaque or calculus to account for the taneous bleeding from the gingiva can occur and
gingivitis. The onset is often sudden, with rapid patients may report finding blood in their mouth
loss of bone and soft tissue. In linear gingival on waking.
erythema, the gingiva may be reddened and
Diagnostic Tests
edematous.
Patients sometimes complain of spontaneous Diagnosis of AIDS can be made by history and
bleeding. In acute-onset ulcerative gingivitis, medical examination for symptoms and signs.
ulcers occur at the tips of the interdental papilla Individual is generally young and in high risk
and along the gingival margins and often elicit group. There are several circumstances in which,
complaints of severe pain. The ulcers heal, it is important for dentist to know HIV antibody
leaving the gingival papillae with a characteristic status.
cratered appearance. To ensure accurate differential diagnosis and
Necrotizing ulcerative periodontitis may appropriate treatment of the oral lesions those
present as rapid loss of supporting bone and soft are associated with development of AIDS. To
tissue. Typically, these losses occur simulta- assess the risk to dental HCW after needle stick
504 Textbook of Oral Pathology

or other injury from contaminated once by The Western Blot Method

contact with patients blood and saliva. To convey
The prepared HTLV-III virus particles consists
with CDC guideline that all HCW engaging in
of specific HTLV-III specific proteins separated
invasive dental practice should be aware of HIV
antibody status. by a process called electrophoresis and subsequent
transfer of nine protein bands to nitrocellulose
membrane strip, which are reacted with patient's
Laboratory Studies
serum followed by an enzyme linked antihuman
Body fluid and material for infection and antibody and enzyme substance.
causes. Blood studies for impaired body immu- Positive test is indicated when the treated,
nity will show anemia, leukopenia, lymphope- i.e. HTLV-III specific nitrocellulose strip are
nia (low T helper cell), reversal of T4/T8 ratio, exposed to infected human serum and a goat
thrombocytopenia, nonspecific and mild eleva- antihuman antibody strip display the
tion of LFT, immunoglobulin levels like IGG characteristic band detected for each of three
and IGA are elevated and B cell abnormalities (env, pol and gag) group of viral protein on
are seen. exposure to X-ray film. Those that react with only
one of these three groups of antigens are termed
Screening Test for AIDS indeterminate and those react with only non
viral proteins are negative. It is more specific for
ELISA (Enzyme-linked immunosorbent assay) HTLV-III antibodies and is used to eliminate
It is a color reaction test in which prepared false-positive result.
whole HTLV-III virus particle which acts as
antigen, will bind with antibodies to HTLV- Indirect Immunofluorescence and Microfiltration
III in an infected human serum. Then this Enzyme Assay System
complex is added to goat antihuman antibody
labeled with chemical enzymes, which gives It is rapid and relatively inexpensive.
color reaction with particular chemicals, e.g.
orange color with peroxides labeled goat Test from HIV Antigen in Serum or Plasma
antihuman antibody. ELISA test is simple and
It has greatest utility during early phase of HIV
convenient to perform; it is very sensitive for
small amount of HTLV-III antibodies and in infection.
initial screening tests. It is not 100% reliable
Viral Culture and Polymerase Chain Reaction
because it is not completely specific only for
HTLV-II antibodies and reacts with other Cultivation of HIV from blood and other body
related viral antibodies and other nonspecific fluids and tissues with detection of the increased
chemicals giving false-positive result of about filter by either HIV antigen or reverse
5 to 25 per 1000 patients. In case of positive transcriptase assay provide method that
test, there is strong indication of past exposure specifically demonstrates the presence of virus
and infection with virus but this does not show in given patient or specimen. The variable
whether the patient is infectious because it amount of virus present in peripheral blood
does not show whether virus is still active or monocyte at different stage of infection and
has been destroyed. degree of immunosuppression both influence the
 Viral Infections 505

extent HIV-I viremia. Polymerase chain reaction functional defects of T cell lymphocytes, it will
technique have provided an opportunity for correct the immune defect. Transplant of fetal
detection of very small amount of an infectious thymus of cultural thymic epithelium and
agent like HIV-I based on reported cycle of injection of thymic hormone have been
enzymatic duplication of number of copies of successfully utilized in treatment of AIDS.
either DNA or RNA specific for microorganism.
Lymphokines and cytokines—Lymphokines are
materials produced by lymphocyte. Interleukin-
Surrogate Marker for Progression of HIV-I
I is macrophage product. In 'in vitro' system
infection
interleukin-I enhance plague forming cells
The absolute CD4 +T cell lymphocyte count responses and the generation of cytotoxic T cell
correlate best with progression of HIV-I related alloantigen. In the presence of macrophage,
immune dysfunction. Other serum neoprotein interleuckin-1 stimulates the production of
beta-2-microreceptor HIV P24 antigen inter- interleukin-2, which stimulates and maintains
leukin-2 receptor IgA and impaired delayed type the growth of T cell activated by antigens.
of sensitivity are also used. Various studies have conformed that purified
interleukin-2 (which stimulate and maintain
Management growth of T cell activated by antigen),
Various drugs are used for immunotherapy are preparation in in vitro system can normalize
as follows: lymphocyte reaction in high percentage of
individuals with unexplained lymphadenopathy
Interferon — It is a useful therapeutic agent in
and immunologic abnormalities, but the result
this syndrome of infection and neoplasms in
are not significant in patients with AIDS.
view of their antiviral antiproliferative and
immunomodulater activity. The interferon is a Bone marrow transplantation — Syngeneic
glycoprotein produced by a number of different (identical twin) allogenic (HLA/NHC matched)
types cells. Type I interferon (alpha and beta) bone marrow transplantation has been
are produced by leukocytes and fibroblasts. Type successful in reconstituting immune function in
II interferon (gamma) is produced by the patients with severe congenital immune
lymphocytes and monocytes. Low doses of defects. If this could be therapeutic in patient
interferon enhance the antibody formation and with AIDS that have appropriate marrow donor.
lymphocyte blastogenesis. They also prolong cell
Monoclonal antibodies therapy—In this, antibodies
cycle and cause inhibition of intracellular are directed against T cell differentiation
enzyme system (anti- neoplastic effect). The
antigens as a result of that number of circulating
gamma interferon stimulate macrophage
leukemic cells are decreased in patients with
oxidative metabolism and have antimicrobial adult T cell active lymphoblastic leukemia.
effect.
Pharmacological immunomodulation—Amitidine,
Thymic replacement therapy — The thymic
isoprinosine and retinoid are also used but
epithelium plays an important role in
results are insignificant.
transformation of blood borne precursor cell into
mature T cells. Thymic hormones or factor Intravenous immunoglobulin therapy—It reduces
mediates this effect, since the immune system incidence of bacterial and viral infection.
in AIDS is characterized by numerical and Infusion of hyperimmune gamma globulin
506 Textbook of Oral Pathology

enriched for neutralizing antibodies for LAV/ Prevention

HTLV III could prove beneficial for individuals
• Educational counseling of general public
with AIDS or ARC who have inadequate specific
• Avoid sexual contact with suspect and in high-
antibodies.
risk group
Antiviral drug- HPA-23—It is an oraganometallic • Use of disposable syringes and needles
compound of tungsten and antimony, • Blood donor should be properly screened
azidothymidine—it is analogues of thymidine. It • Avoid multiple sex partners, intimate kissing
appears to inhibit multiplication of HTLV-III and oral contact
virus and cyclosporine—it shows marked increase • Educate healthcare workers on safety
in T lymphocyte population. measures.
 CHAPTER
Fungal and
25 Parasitic Infections

CANDIDIASIS Contd...

2nd
It is also called as ‘candidosis’.
A. Candidiasis of nails and skin
Candidiasis is the disease caused by infection
• Candidal onychia
with yeast like fungus Candida albicans. • Candidal paronychia
B. Candidiasis confined to skin
Classification • Interdigital candidiasis
• Intertriginous candidiasis
• Candidiasis (moniliasis)
1st C. Candidiasis confined to mucosae
A. Oral candidiasis Oral mucosa
Acute • Acute oral candidiasis
• Acute pseudomembranous candidiasis (thrush). Acute pseudomembranous candidiasis (thrush)
• Acute atrophic candidiasis (antibiotics sore mouth) Acute atrophic candidiasis (antibiotics sore mouth)
Chronic • Chronic oral candidiasis
• Chronic atrophic candidiasis. Chronic atrophic candidiasis (denture sore mouth)
Denture stomatitis Chronic hyperplastic candidiasis
Median rhomboid glossitis. Gastrointestinal mucosa
Angular cheilitis • Pharyngeal candidiasis
• Id reaction. • Esophageal candidiasis
• Chronic hyperplastic candidiasis. • Intestinal candidiasis
B. Chronic mucocutaneous candidiasis Respiratory mucosa
• Familial CMC • Bronchial candidiasis
• Localized CMC Genitourinary mucosae
• Diffuse CMC • Candidal vulvovaginitis
• Candidiasis endocrinopathy syndrome D. Mucocutaneous candidiasis
C. Extraoral candidiasis Confined to mucocutaneous surface
• Oral candidiasis associated with extraoral lesions • In condition with major immunologic defect
orofacial and intertriginous sites (candidal Swiss-type a gammaglobulinemia
vulvovaginitis, intertriginous candidiasis) Hereditary thymic dysplasia
• Gastrointestinal candidiasis Di George syndrome
• Candida hypersensitivity syndrome AIDS
D. Systemic candidiasis • In condition with minor immunological or other
• Mainly affect the eye, kidney and skin systemic defect
Contd... Contd...
508 Textbook of Oral Pathology

Contd... Conditions associated with increased vulnerability of

• Chronic mucocutaneous candidiasis (CMC) oral candidiasis and their mechanism
syndromes Category Condition Mechanism
Familial mucocutaneous candidiasis
Candidiasis endocrinopathy syndrome Altered local Poor oral hygiene Promotes organism
Localized chronic mucocutaneous candidiasis resistance to adherence and colonization
Diffuse chronic mucocutaneous candidiasis infection
Chronic mucocutaneous candidiasis in association Xerostomia Absence of antimicrobial
and flushing effect of saliva
with thymoma
Recent antibiotics Inhibits competitive oral
Confined to mucocutaneous junctions
treatment bacteria
• Candidal angular cheilitis Dental appliance Isolated mucosa from saliva
• Perianal candidiasis and functional cleansing
E. Systemic candidiasis serve as organism reservoir
• Candidal endocarditis
Compromised Early infancy Immune competence has not
• Candidal septicemia
immune system completely developed
• Candidal meningitis function
Genetic immune Specific humoral or cellular
deficiency immune defects
Causative Organisms AIDS Deficient cellular immune
response
It is most commonly cause by Candida albicans Corticosteroids Inhibition of immune
therapy function
(the yeast like fungus occur in yeast and mycelial Pancytopenia Depletion of circulating leu-
forms). Other organism which are responsible for kocytes cause by chemo-
therapy, aplastic anemia and
candidiasis are Candida stellatoidea, Candida similar hemopoietic disor-
tropicalis, Candida parapsillosis, Candida ders
pseudotropicalis, Candida famata, Candida rugosa, Generalized Anemia, Epithelial thinning and
patient malnutrition, altered maturation, poor
Candida krusei and Candida guilliermondi. debilitation malabsorption tissue oxygenation
Morphologically Candida can exist in three Diabetes mellitus Recurring hyperglycemia
forms, i.e. yeast cells, pseudohypha and and mild ketoacidosis
chlamydospore forms. Candida albicans is the Advanced Metabolic toxicity or limited
systemic disease blood perfusion of tissue
commonest pathogen of all candida species and
it appears as moist creamy colonies and on blood Malnutrition states such as low serum vitamin
agar as dull grey colonies. A, pyridoxine and iron levels, age (infancy,
pregnancy, old age), hospitalization and oral
Predisposing Factors epithelial dysplasia, endocrinopathies such as
hypoparathyroidism, hypothyroidism and
Marked changes in oral microbial flora owing to
Addison’s disease also leads to candidiasis.
administration of antibiotics (broad spectrum),
Primary and acquired immunodeficiency
excessive use of antibacterial mouth rinses,
states such as hypogammaglobulinemia also act
xerostomia secondary to anticholinergic agent or
as predisposing factors. Tight and close fitting
salivary gland disease.
garments encourage the growth of Candida.
Chronic local irritant (denture, orthodontic
Areas around the indwelling catheter are also
appliance and heavy smoke), administration of
involved.
corticosteroids, cytotoxic drugs, immuno-
suppressive agents and radiation to head and
Oral Candidiasis
neck also leads to candidiasis.
Acute and chronic disease such as leukemia, Oral involvement is probably the most common
lymphoma, diabetes and tuberculosis. manifestation of human candidal infection. It can
 Fungal and Parasitic Infections 509

occur either solely confined to the oral mucosa or white area may develop beneath the complete
or as a part of any of the several mucocutaneous denture or partial denture. It is occasionally
candidiasis syndromes. associated with (coexist with) dysplastic or
carcinomatous change (Fig. 25.1).
Thrush
Histopathological Features
It is the prototype of oral infection caused by
yeast like fungus. It is the superficial infection of Fragments of the plaque material may smear on
upper layer of oral mucus membrane and results a microscopic slide, macerated with 20%
in formation of patchy white plaque or flecks on potassium hydroxide and examined for hyphae.
mucosal surface. There is presence of yeast cells and hyphae or
mycelia in the superficial and deeper layer of
Clinical Features involved epithelium. The submucosa may be free
from any infection or may contain a chronic
In infants: In neonates, oral lesions start between
inflammatory cell infiltrate.
the 6th and 10th day after birth. Infection is
On staining with PAS organism was
contracted from the maternal vaginal canal
identified by bright magenta color. Hyphae are
where Candida albicans flourishes during the
pregnancy. The lesions in infants are described
as soft white or bluish white, adherent patches
on oral mucosa which may extend to circumoral
tissue. They are painless and noticed on careful
examinations. They may be removed with little
difficulty.
In adult: Common sites are roof of the mouth,
retromolar area, and mucobuccal fold. But it is
common on any other mucosal surface and it is Figure 25.1: Candidiasis presented as white
plaque in the palate
common in women as compared to male.
Prodormal symptom like rapid onset of bad taste
may be there. Spicy food will cause discomfort.
Patient may complain of burning sensation and
there may be history of dryness of the mouth.
Inflammation, erythema, and painful eroded
areas may be associated with this disease.
Sometimes typical, pearly white or bluish white
plaque. It is multiple, curdy, loosely adherent
patches on any part of oral mucosa. Mucosa
adjacent to it appears red and moderately
swollen. Lesions are relatively inconspicuous.
White patches of it are easily wiped out with wet
gauze which leaves either a normal or
erythematous area or atrophic area. Deeper
invasion by the organism leaves an ulcerative Figure 25.2: Candidiasis showing organism in the
lesion upon the removal of patch. Erythematous corneum layer taking up magenta red color (arrow)
510 Textbook of Oral Pathology

varying in length showing branching. Hyphae vascularity. Occasionally, microabscesses may be

are accompanied by yeasts, squamous epithelial seen in superficial epithelial areas.
cells (Fig. 25.2).
Chronic Hyperplastic Candidiasis
Acute Atrophic Candidiasis It also called as ‘candidal leukoplakia’ because
It is also called as ‘antibiotics sore mouth’. When of its firm presentation as firm and adherent
the white plaque of pseudomembranous white patches occurring in the oral mucosa.
candidiasis is removed, often red atrophic and
painful mucosa remains. Clinical Features
It predominantly occurs in men of middle age
Clinical Features or over. The majority of these patients are heavy
In this type, lesion appears as red or smokers. It occurs on cheek, lip and tongue.
erythematous rather than white, thus Candidal leukoplakia is extremely chronic form
resembling the pseudomembranous type in of oral candidiasis in which firm, and white
which white membrane has been wiped off. It leathery plaques are found.
can occur at any site but it usually involves the Lesions may persist without any symptoms
tongue and tissue underlying an appliance. for years. Many women with oral candidiasis
Patient usually described vague pain or a also report symptoms of vaginal itching and
burning sensation. If the discomfort is not discharge indicative of vaginal candidiasis. It
spontaneous, pain can be elicited by mild does not rub off with lateral pressure. Lesion
abrasive pressure with cotton gauze. Careful range from slightly white to dense white with
examination reveals a few white thickened foci cracks and fissures occasionally present
that rub off leaving a painful surface (Fig. 25.3). (Fig. 25.4). The borders are often vague, which
Lesion closely resembles erosive lichen planus mimic the appearance of epithelial dysplasia. It
and erythroplakia. may occur as a part of chronic mucocutaneous
candidiasis.

Histopathological Features
Epithelial dysplasia is seen but it is reversible.
There is mycelial invasion of the deeper layers of
mucosa and skin occurs. Epithelium is usually

Figure 25.3: Atrophic candidiasis showing red lesion

in the center of tongue

Histopathological Feature
Atrophic epithelium containing few hyphae in
the superficial layer. Lamina propria shows mild Figure 25.4: Chronic hyperplastic candidiasis
acute inflammatory infiltrate and increased showing leathery plaque
 Fungal and Parasitic Infections 511

parakeratinized. There is acanthosis of spinous Management

layer and bulbous elongated retepegs. There is
Troches containing clotrimazole and Nystatin 4
also pseudoepitheliomatous hyperplasia and
to 5 times after meal and bed time.
microabscesses formation. It contains inflamm-
atory exudates and chronic inflammatory cell
infiltration of polymorphonuclear neutrophils in Median Rhomboidal Glossitis
the corneum. There is debate that it is form of chronic atrophic
candidiasis but the area of tongue affected by
Id Reaction
median rhomboid glossitis is frequently followed
A person with chronic candida infection may by candida infection. It is described in detail in
develop secondary response characterized by chapter of tongue disorders.
localized or generalized sterile vesicueopapular
rash that is believed to be allergic response to Treatment of Oral Candidiasis
candida antigen (also called as monolids). Removal of the causes—Replacement of denture
or relining or adding mycostatin suspension
Candida Associated Lesion below it while insertion in mouth in case of
Denture Stomatitis angular cheilitis and denture sore mouth. The
denture must be cleaned thoroughly and
It is also called as ‘chronic atrophic candidiasis. It
regularly and should be left out of the mouth at
is common clinical manifestation of
night in hypochlorite solution. Withdrawal or
erythematous candidiasis. Candida albicans is
change of antibiotics use if feasible.
always found in this lesion but the typical white
Oral candidiasis may be treated either
patch of thrush does not usually develop in it. It
occurs due to tissue invasion but organism effect topically or systemically. Treatment should be
of fungal toxin hypersensitivity to fungus. maintained for 7 days. Response to treatment is
often good; oral lesions and symptoms may
Clinical Features disappear in a fairly short period (ranging from
2 to 5 days), but relapses are common because of
It is usually found under complete denture and
the underlying immunodeficiency. As with other
partial denture and found mostly in women and
causes of oral candidiasis, recurrences are
always include palate. It exhibits patchy
common if the underlying problem persists.
distribution often associated with speckled curd
Topical treatments are preferred because they
like white lesion. There is soreness and dryness
of mouth. Palatal tissue is bright red somewhat limit systemic absorption, but the effectiveness
edematous and granular. Red patches may be depends entirely on patient compliance.
erythematous or speckled. The redness of Following are most commonly used topical
mucosa is rather sharply outlined and restricted treatment:
to the tissue actually in contact with the denture. Clotrimazole—It is an effective topical treatment
The multiple pinpoint foci of hyperemia usually (one oral troche [10-mg tablet]) when dissolved
involving the maxilla frequently occur. in the mouth five times daily. Used less
frequently, one vaginal troche can be dissolved
Histopathological Features in the mouth daily. 1% gentian violet can be use
Epithelium is atrophic and there are superficially but it is not ideal because of the superficial
oriented mycelia. Intense chronic inflammatory necrosis of mucosa it may produce and also
infiltrate in lamina propria. because of the unsightly staining.
512 Textbook of Oral Pathology

Nystatin preparations—It includes a suspension, • Fluconazole is a triazole antifungal agent

a vaginal tablet, and an oral pastille. Nystatin effective in treating candidiasis (100 mg tablet
vaginal tablets (one tablet, 100,000 units, taken once daily for 2 weeks). Several studies
dissolved in the mouth three times a day). suggest fluconazole is effective as a
Nystatin oral pastille (available as a 200,000-unit prophylactic agent, although the most
oral pastille, one or two pastilles dissolved slowly effective prophylaxis dosing regimen is still
in the mouth five times a day). Nystatin oral unclear. Numerous reports, however,
suspension 100,000 units/cc, 1 teaspoon of which describe oral and esophageal candidiasis
is mixed with 1/4 cup of water and used as oral failing to respond to treatment with
rinse. fluconazole, and in some of these cases
Topical creams and ointments—Containing investigators isolated resistant strains.
nystatin, ketoconazole, or clotrimazole may be • Itraconazole (100 mg capsules) may be used
useful in treating angular cheilitis. Another for the treatment of oral candidiasis (200 mg
therapeutic choice is amphotericin B (0.1 mg/ml). daily orally for 14 days). Itraconazole oral
Five to 10 ml of oral solution is used as a rinse suspension is now available (200 mg daily for
and then expectorated three to four times daily. 2 weeks. Salivary levels of itraconazole are
maintained for several hours after
Mycostatin cream — One lack unit or lactose administration.
containing vaginal tablet keeps under the tongue. • Ketoconazole, fluconazole, and itraconazole
The addition of absorbable corticosteroids and
may interact with other medications
antibiotic agents to mycostatin cream and
including rifampin, phenytoin, cyclosporin
ointment accelerate the symptomatic effect.
A, terfenadine, digoxin, coumarin-like
Amphotericin B — 0.08 Mg/kg 2 to 3 hourly in medications, and oral hypoglycemic
ointment or cream base, suspension. Elixir medications.
containing both tetracycline and amphotericin B
may also prove to be beneficial in acute atrophic Candidal Onychia and Paronychia
candidiasis.
Candidal infection of nail and of the soft tissue
Rinses—Mycostatin rinses for 7 to 10 days 3 to 4 on the sides and at the base of the nail is common
times a day. 0.2% chlorhexidine solution, use of in housewife, nurses, and dishwasher,
1 % chlorhexidine gel in denture stomatitis. bartenders, and fruit pickers. Involved nail show
Systemic Treatment—several agents are effective green black discoloration and transverse ridges.
for systemic treatment. Soft tissues are inflamed and tender. Treatment
• Nystatin 250 mg DS for 2 weeks followed by is by daily topical application of amphotericin B
1 troche per day for third week lotion or nystatin ointment.
• Ketoconazole is a 200 mg tablet taken with food
once daily. Patient compliance is usually Interdigital Candidiasis
good. Careful monitoring of liver function is It is also called as erosion interdigitalis. It is
necessary for long-term use because of commonly involved 3rd and 4th fingers. It is
reported side effects, including hepato- characterized by pruritic, inflamed, and scaling
toxicity. Lack of efficacy of ketoconazole may lesions between fingers. Treatment includes
occur because of poor absorption in those topical application of amphotericin B lotion or
with an abnormally high gastric pH. nystatin ointment.
 Fungal and Parasitic Infections 513

Intertriginous Candidiasis Management

Candida infection of skin surface such as waist, The use of topical and systemic antifungal agents
groin, armpit, elbows, submammary area, gluteal should be done.
fold, axilla, scrotum. Intertriginous candidiasis
causes colonization in skin like axilla or Bronchial Candidiasis
submaxillary fold. Opposing skin surface in these
sites, particularly in obese person prevent It is a chronic infection of bronchial mucosa
adequate ventilation and remain moist favoring which may last for years without producing
candidal growth. severe discomfort. It mimics chronic bacterial
Candida has characteristic way of causing dry infection of the bronchus and chronic cough with
pustulation with desiccation in lower level of mucoid sputum is common symptoms
stratum corneum of skin. Lesions are very tender. experience by the patients. Diagnosis is
Lesion spread from affected skin as an area of established by the demonstration of Candida in
glaze red skin or an easily detached overlying
sputum and through culture studies. It includes
epidermis, i.e. often invaded leaving paper like
systemic use of antifungal agents.
fungal lesion along the margin. Satellite lesion
may develop around deeper denuded tissue.
Candidal Vulvovaginitis and Balanitis
Gastrointestinal Candidiasis Candida vulvovaginitis is a common form of
An extension of oral infection may occasionally candidiasis, which increases during pregnancy,
lead to either pharyngeal or esophageal diabetes, and uses of antibiotics. Candidal
involvement. inflammation of glans penis may occur due to
Causes sexual contact. Diabetic women and those on long-
term drug therapy are prone to develop vaginal
Long-term broad-spectrum antibiotics, cortico-
steroid therapy and immunosuppressive drugs. infection. It is characterized by erythematous
Leukemia, diabetes and acquired immuno- plaques on the glans, penis and around the
deficiency syndrome. prepuce. Symptoms usually clear up on topical
application of antifungal agents.
Clinical Features
It presents as acute enterocolitis, diarrhea, as Chronic Mucocutaneous Candidiasis
proctitis with anal pruritis of perineal
Classification
eczemation. Dysphagia, chest pain, and GIT
bleeding may be presenting symptoms. • Chronic familial mucocutaneous candidiasis
Esophageal candidiasis—Esophageal candidiasis • Chronic localized mucocutaneous candidiasis
with or without gastric ulceration are common • Endocrinopathy candidiasis syndrome
forms of GIT candidiasis. It is diagnosed by • Chronic diffuse mucocutaneous candidiasis
characteristic appearance of edematous ulcerated • Chronic mucocutaneous candidiasis in
mucosa on barium swallow. association with thymoma.
Histopathological Features
Etiology
There is pseudomembrane formation, infla-
mmation along with localized ulceration that It occur due to defect in cellular immunity, defect
exhibits tuberculoid granuloma enclosing yeast in structure of epidermis, diabetes, pregnancy
and hyphae. and steroids.
514 Textbook of Oral Pathology

Clinical Features Chronic mucocutaneous candidiasis in association

with thymoma—Rarely patient with thymoma
Chronic familial mucocutaneous candidiasis—It is an
manifest chronic mucocutaneous candidiasis.
inherited disorder, probably an autosomal Oral and skin surface are usually involved.
recessive disorder and affects both sexes. It is
characterized by candidiasis of mouth, nails, and Management
skin. Children under the age of 10 years show
For resistance form of CMC and for systemic
superficial candidal infection. Oral candidiasis
candidiasis miconazole (250 mg 6 hourly), 5-
is chronic hyperplastic exhibiting firm white flucytosine (200 mg daily) in addition to
patches involving either buccal or lingual amphotericin B is available. Transfusion of
mucosa. transfer factor and transplantation of culture of
Chronic localized mucocutaneous candidiasis—This thymic fragments have been used to correct T
form occurs early in life with oral mucosal, skin cell deficiency associated with CMC.
and nail involvement. There is also presence of
chronic oral candidiasis and hypoplastic Candidiasis with Major Immunological Defect
infection of nail fold in infancy. Oral lesion Swiss-type agammaglobulinemia—It is
includes white patches characterized by horny characterized by severely impaired resistance to
masses mainly found on face and scalp. Patient infections of all types as a result of failure of
shows increase tendency to fungal and bacterial thymic development and antibody production.
infection in the absence of immunological or Infants die in the first year of life. Oral candidiasis
genetic defect. is a consistent feature of this syndrome.
Candidiasis endocrinopathy syndrome—It is also Hereditary thymic dysplasia—Impairment of cell-
genetically transmitted characterized by early mediated immunity due to thymic dysplasia
onset and candida lesion of skin, scalp, nails and favors severe viral and candidal infection. Oral
mucous membrane. There is subsequent candidiasis is predominant feature of this
appearance of hypoparathyroidism, disease.
hypoadrenocortism and other endocrine Di George syndrome—It is characterized by
abnormalities. There is also occurrence of dental underdeveloped thymus and parathyroid glands
hypoplasia and severe caries. There may be due to a defective development of the 3rd and
balanitis and vulvovaginitis, keratoconjunctivitis, 4th branchial pouches. Oral candidiasis is a
alopecia and juvenile cirrhosis can also occurs. consistent feature of this disease.
Chronic diffuse mucocutaneous candidiasis—It is of AIDS—Oral manifestation of AIDS among other
late onset and exhibits extensive raised crusty features include candidiasis of the oral mucosa,
sheets involving the limbs, groin, face, scalp and where the candidal infection is opportunistic in
shoulders. Mucocutaneous candidiasis is nature.
characterized by extensive oral, pharyngeal and
often laryngeal involvement. Cutaneous Systemic Candidiasis
involvement is characterized by raised, crusty This term is reserved for that situation in which
proliferative lesions predominately on the skin there is presence of both necrotizing
of face and scalp. These lesions are also called as inflammation and candida granuloma formation
‘candidal granuloma’. Oral lesions are widespread, in one or more visceral organ usually as result
proliferative and raised white lesions. of hematogenous dissipation of organism.
 Fungal and Parasitic Infections 515

Etiology Transmission
It occurs in immunosuppressive patient as a Infection results from inhaling the dust
result of leukemia, lymphoma, steroids, cytotoxic contaminated with droppings particularly from
therapy and extensive therapy. Damaged heart infected birds.
wall, contaminated IV instruments. Indwelling
catheter, used of contaminated drug, Types
paraphernalia by drug abuses.
• Acute primary histoplasmosis
• Progressive disseminated histoplasmosis
Clinical Features • Chronic cavitary histoplasmosis.
Candidal endocarditis—It is characterized by fever,
dyspnoea and edema of congestive cardiac Clinical Features
failure. Vascular vegetations of candidal growth Acute primary histoplasmosis: There is chronic
often result in embolization to major vessels. The low grade fever, malaise, headache and
disease is fetal in majority of cases. productive cough. There may be pleuritic pain.
Candidal meningitis—It is predominately disease Primary infection is mild, manifesting as self-
of male children characterized by variable limited pulmonary disease that heals to leave
features ranging form stiffness of the neck, fibrosis and calcification. Chest radiograph may
hemiplegia and other neurological signs. The show patchy infiltrate which may exhibit signs
finding of Candida in spinal fluid is of diagnostic of calcification.
importance. The condition is fatal in half of the Progressive disseminated histoplasmosis: It is
cases. common in children and elderly. It is manifested
Candidal septicemia—It occurs in those with severe by hepato-splenomegaly and lymphadenopathy.
oral and esophageal thrush. Features include Patients with disseminated form show evidence
fever, chills, shock and coma. Condition can be of bone marrow involvement by anemia and
fatal if not treated in time. leucopenia. There is also kidney involvement
with gastrointestinal and oropharyngeal
Management ulcerative lesions.

Systemic candidiasis requires systemic Chronic cavitary histoplasmosis: It closely

administration of high doses of amphotericin-B 10 mimics chronic cavitary tuberculosis. The
mg qID, miconazole and 5-flucytosine. Mycostatin cavitary lesions are bilateral and are found in the
50,000 unit 8 hourly. Daily use of 200 mg upper lung fields. Symptoms include cough,
ketoconazole for 2 weeks. Side effects are increased dyspnea and weight loss.
liver enzyme, abdominal pain.
Oral Manifestations
Histoplasmosis Oral lesions are common in the progressive
It is also called as ‘Darling’s disease’. It is caused disseminated form. It is seen on buccal mucosa,
by Histoplasma capsulatum, a dimorphic fungus gingiva, tongue, palate or lip. Patient may
that grows in the yeast form in infected tissue. complain of sore throat, painful chewing,
Infection results from inhalation of dust hoarseness, difficulty in swallowing. Oral lesions
contaminated with dropping, particularly from are nodular, ulcerative or vegetative. If left
infected birds. untreated, it will progress to form firm papule
516 Textbook of Oral Pathology

or nodules which ulcerate and slowly enlarge. Clinical Features

Ulcerated area covered by nonspecific gray
Primary pulmonary blastomycosis: It is more
membrane and is indurated.
common in men than women and typically
occurs in middle age. It follows a chronic course
Laboratory Diagnosis
with malaise, low grade fever and mild cough.
The organisms are found in large numbers in If untreated, shortness of breath, weight loss and
phagocytic cells and appear as tiny intracellular blood tinged sputum are encountered.
structures measuring little more than 1 micron
Cutaneous blastomycosis: Infection of skin,
in diameter.
mucosa and bone may also occur, resulting from
Biopsy shows small oval yeasts within
metastatic spread of organism through
macrophages and reticuloendothelial cells as
lymphatic system. Skin and mucosal lesion starts
well as chronic granuloma, epithelial cells, giant
as subcutaneous nodule and progresses to well
cell with casseation necrosis.
circumscribed indurated ulcer. Elevated,
Histopathological Features verrucous, crusted and single or multiple lesions
developing on exposed part of the body,
It is a granulomatous infection which chiefly particularly on hands and face. Lesion leaves a
affects the reticuloendothelial system. The slanted serpiginous border with a tendency
mucosal epithelium shows ulceration, in majority towards healing in the center. When the crust is
of the cases. In nonulcerated areas, pseudo- lifted, pus exudes.
epitheliomatous hyperplasia is often seen. The
submucosa shows a dense infiltrate of Disseminated or systemic blastomycosis: It
granulocytes, lymphocytes, plasma cells and results from the spread of infection from
histiocytes. Multinucleated giant cells and pulmonary form. Bones are involved in larger
casseation necrosis are often seen. Causative number of cases. Liver, kidney, spleen and
organism is identified with PAS stain. gastrointestinal tract may be involved. Cerebral
abscess are rare, but may occur as a result of brain
Management involvement.
Ketoconazole—Six to 12 months is given for 6 to
Oral Manifestations
12 months.
It may be primary or secondary to some infection
Blastomycosis elsewhere in the body. Oropharyngeal pain,
It is cause by ‘blastomyces dermatidis’. Organism accompanied by the enlargement of cervical
is a normal inhabitant of soil and that is the lymph nodes, may be a presenting sign of oral
reason for it to be common in agricultural worker. disease.
It is transmitted through the respiratory tract. There is nonspecific, painless verrucous ulcer
Infection with blastomycosis begins in a vast with indurated borders often mistaken for
majority of cases by inhalation, as primary squamous cell carcinoma. Other lesions are hard
pulmonary infection. nodules and appear as sessile projection,
granulomatous appearing plaque.
Types
Histopathological Features
• Primary pulmonary blastomycosis
• Cutaneous blastomycosis The inflamed connective tissue shows occasional
• Disseminated or systemic blastomycosis. giant cells, macrophages and the typical round
 Fungal and Parasitic Infections 517

organisms, often budding, which appear to have usually arise in lateral wall of nose and maxillary
a doubly refractile capsule. Microabscesses are sinus; may rapidly spread by arterial invasion
frequently found and if the lesion is not ulcerated to involve the orbit, palate, maxillary alveolus
overlying pseudoepitheliomatous hyperplasia and ultimately the cavernous sinus and brain
may be prominent. through hematogenous spread and may cause
death. Ptosis, proptosis, fever, swelling of cheek
Diagnosis and paresthesia of face can occur.
Intracranial involvement maybe heralded by
The index of suspicion should increase when
cranial neuropathy, especially of the trigeminal
chronic, painless, oral ulcer appears in an
and facial nerve. There is increased lethargy,
agricultural worker or when review of system
progressive neurologic deficit and ultimately
reveals pulmonary symptom. Diagnosis is made
death. Fungus invades artery to cause fibrosis
on the basis of biopsy and on culturing the
and ischemia. There is appearance of a reddish
organism from tissue.
black nasal turbinate and septum. Nasal
discharge caused by necrosis of nasal turbinate.
Management
Antifungal medication. Intravenous ampho- Oral Manifestations
tericin B for 8 to 10 weeks causes resolution of
Ulceration of palate, due to necrosis and invasion
the disease.
of palatal vessels. Ulcer may be seen on gingivae,
lip and alveolar bone. It is large and deep,
Mucormycosis
causing denudation of underlying bone.
It is also called as ‘phycomycosis, zygomycosis’.
Histopathological Findings
Etiology and Predisposing Factors
The tissue involved by mucormycosis shows
It is caused by saprophyte fungus. More common necrosis and chronic inflammatory infiltrate. The
in patients with decreased resistance, due to vessels in the area may be thrombosed with
diseases like diabetes, tuberculosis, renal failure, organisms in the lumen. Culture studies are
leukemia, cirrhosis and in severe burn cases. essential to identify the order family and genus
of fungus. The organism appears as large,
Types nonseptate hyphae with branching at obtuse
• Superficial—It involves external ear, angle. Round and ovoid sporangia are also seen.
fingernails and skin There is extensive tissue destruction which
• Visceral disturb normal blood flow resulting in infarction.
• Pulmonary
Management
• Gastrointestinal
• Rhinocerebral or rhinomaxillary form—it is Surgical debridment is the treatment of choice.
important with dental point of view. Systemic amphotericin can be given.

Clinical Features Cryptococcosis

Rhinomaxillary form begins with inhalation of It is also called as ‘torulosis’. It is a chronic fungal
fungus by susceptible individual. Infection infection caused by Cryptococcus neoformans and
518 Textbook of Oral Pathology

Cryptococcus bacillispora. The causative organism Management

is gram-positive, budding, yeast-like cell with an
Mild to moderate cases can be treated with
extremely thick, gelatinous capsule, measuring
5 to 20 microns in diameter. Infection occurs due ketoconazole for 6 to 12 weeks. The severe form
to inhalation of airborne microorganism. It has requires amphotericin-B, intravenously for up to
increased incidence in immunosuppressive 10 weeks.
patients.
Coccidioidomycosis
Clinical Features It is also called as ‘valley fever’, ‘desert fever’ or
There is slight predilection for middle aged ‘coccidial granuloma’. The disease appears to be
males. The infection usually occurs in lungs. It transmitted to man and animals by inhalation
may be asymptomatic and in some cases, patient of dust contaminated by the spores of the
may complain of cough with mucoid causative organism, Coccidioides immitis.
expectoration. Occasionally, pleuritic pain and
hemoptysis can also occur. Types
The skin lesion appears as multiple brown
• Primary non-disseminated coccidioidomy-
papules which ultimately ulcerate, the clinical
cosis.
picture is nonspecific. Meningoencephalic lesions
• Progressive disseminated coccidioidomy-
produce a variety of neurologic signs and
cosis.
symptoms, generally associated with increased
intracranial pressure. The radiograph of chest
Clinical Features
shows infiltrates and occasionally ‘coin’ lesion.
General: It is common in all age groups and
Oral Manifestations predominately seen in males. Symptoms occur
Lesion of hard palate, soft palate, gingiva, usually 14 days after the inhalation of fungus.
extraction socket, tongue and tonsillar pillar are Infection is common in summer months,
common. They appear as simple nonspecific, especially after periods of dust storm. It is self-
single or multiple ulcers. They are nodular and limiting and runs its course within 10 to 14 days.
granulomatous, which may ulcerate during the Primary non-disseminated coccidioidomycosis:
course of disease. Primary pulmonary coccidioidomycosis—The
patient generally develops manifestations
Histopathological Feature suggestive of respiratory disease such as cough,
In tissue section it appears as a small organism pleural pain, headache and anorexia. Patient may
with round or ovoid structure with a large clear also complaint of low grade fever and joint pain.
halo, sometimes described as ‘tissue microcyst’. Primary cutaneous coccidioidomycosis: Skin lesions
The tissue reaction is generally granulomatous are also present, like erythema nodosum of
type; epitheloid cell proliferation is minimal. erythema multiforme. Primary lesions, when
Multinucleated giant cells as well as they occur, are associated with regional
inflammatory cell infiltrate are common. lymphadenopathy. Granulomatous, verrucous
or necrotic ulcers exuding thick pus are seen on
Diagnosis involved skin surface.
The organisms can be cultured on Sabouraud’s Progressive disseminated coccidioidomycosis: The
glucose agar. disease usually runs rapid course and the
 Fungal and Parasitic Infections 519

dissemination extends from the lungs to various Oral Manifestations

viscera, bones, joints, skin and central nervous
They are similar to candidiasis or thrush, being
system, where meningitis is the most frequent
white, velvety, patch like covering of the oral
cause of death.
mucosa, isolated or diffuse in distribution.
Oral Manifestations Tonsillar lesions are common in association with
oral lesions.
Lesions of head and neck, including the oral
cavity, occur with some frequency. The lesions Histopathological Features
of oral mucosa and skin are proliferative,
granulomatous and ulcerated lesions that are The organism is small, rectangular shaped;
nonspecific in their clinical appearance. These spores measuring approximately 4 to 8 microns,
lesions tend to heal by hyalinization and scar often with rounded ends. The tissue reaction is
formation. Lytic lesions of bones of jaw may non-specific and of acute inflammatory type.
develop.
Management
Histopathological Features
It includes topical and systemic application of
The tissue reaction is similar to any specific nystatin and amphotericin B.
granuloma. There is accumulation of large
mononuclear cells, lymphocytes and plasma Sporotrichosis
cells. Foci of coagulation necrosis are often found
in the center of small granulomas and It is a fungal infection caused by Sporotrichum
multinucleated giant cells are scattered schenckii. The disease predominately affects skin.
throughout the lesion. The organism is found
within the cytoplasm of giant cells, as well as is Causes
lying free in the tissue. It is caused by exposure to a wide variety of
animals, both domestic and wild. Accidental
Management
injury from the thorns of some plants or bushes.
Amphotericin B has been found to be an effective Accidental laboratory or clinical inoculation in
chemotherapeutic agent for the disease. Long- hospital workers.
term therapy is required for complete cure.
Clinical Features
Geotrichosis
It is caused by organism Geotrichum candidum. It The incubation period is from week to 3 weeks.
is found in patients with debilitating diseases. It involves the skin, subcutaneous tissues and
oral nasal and pharyngeal mucosa. The skin
Clinical Features lesion often described as sporotrichotic ‘chancre’
These are more commonly found in lungs and appears at the site of inoculation as firm, red to
oral mucosa. Lung involvement produces purple nodule which soon ulcerates. Regional
symptoms of pneumonitis or bronchitis. The lymphadenopathy is generally developed and
expectoration is often tinted with blood. it may ulcerate and drain.
520 Textbook of Oral Pathology

Oral Manifestations mucoid discharge and appears as soft, reddish

pink, polypoid growth of tumor like nature
Nonspecific ulceration of the oral, nasal and
which spreads to the pharynx and larynx. The
pharyngeal mucosa also occurs. Long standing
lesions are vascular and bleed readily.
lesions become granulomatous, vegetative or
papillomatous. Pain is present and the cervical
Histopathological Features
lymph nodes are always enlarged.
The organisms appear as sporangia containing
Histopathological Features large number of round or ovoid endospores with
size of 5 to 7 microns in diameter. The
The fungus is small, ovoid branching organism
surrounding tissue reaction itself is a nonspecific,
with septate hyphae showing budding form. It
consisting of a vascular granulation tissue with
is only 3 to 5 microns in diameter and can be
focal abscess formation and occasional
cultured on Sabouraud’s medium. The tissue
multinucleated giant cells. Both, acute and
reaction is a granulomatous one with epitheloid
chronic inflammatory cells are present in
cells, multinucleated giant cells of Langhans type
variable number.
and lymphocytes, often surrounding a central
area of purulent or caseous necrosis.
Management
Polymorphonuclear leukocytes are prominent
with pseudoepitheliomatous hyperplasia of the Surgical removal of the growths and application
overlying epithelium of skin or mucosal lesion. of cautery is the treatment recommended for
rhinosporidiosis.
Management
Aspergillosis
It includes oral administration of potassium
iodide in suitable doses. It is a fungal infection caused either by
sensitization to, parasitic colonization of, or
Rhinosporidiosis tissue invasion by species of genus Aspergillus.
It is caused by Rhinosporidium seeberi. It is second to candidiasis, as an opportunistic
infection, in immunocompromised patients.
Clinical Features
Clinical Features
It is more common on oropharynx, nasopharynx,
larynx, skin, eyes and genital mucosa. Initial The respiratory tract, external auditory canal,
symptoms include nasal irritation, accompanied nasopharynx, cornea, gastrointestinal tract and
by mucoid discharge. The skin lesion appears as occasionally the skin may be the primary sites of
small verrucae or warts, which ultimately infection. In some cases there may be asthmatic
become pedunculated. Posteriorly, these episode in sensitive persons. It may present
polypoid masses may extend into the pharynx. swelling, ulceration, crusting and necrosis of
The lesions are soft, friable and highly vascular. anterior turbinates, nasal septum and nasal wall.
Genital lesion resembles condyolomas.
Oral Manifestation
Oral Manifestations
It is rare but in some cases involvement of palate
The soft palate appears to be the most frequent and tongue can occur. Soft palate involvement
site of oral involvement. It is accompanied by a seems to be more common in upper respiratory
 Fungal and Parasitic Infections 521

tract involvement. A lesion on the palate is diarrhea can also develop. The spleen becomes
manifested as a painful ulcer, surrounded by a enlarged, often massively. If not treated, patient
ring of black necrotic tissue. Oropharyngeal will become anemic and wasted.
aspergillosis, in patients with hematological Mucocutaneous leishmaniasis: Incubation period
malignancies, presents as yellowish-black is 1 week to 1 month. It is usually seen in young
ulceration of soft palate and posterior part of men. There is past history of superficial ulcer of
tongue. These patients complain of intense local skin, caused by bite of an infected sandfly, which
pain, oral bleeding and dysphagia. heals with depressed scar. Nasal mucosa
becomes congested and ulcerates. Later, all the
Histopathological Features soft tissues of nose may be destroyed.
Granulomatous reactions are seen in majority of
the lesions and consist of central necrosis Oral Manifestation
surrounded by epitheloid cells and fibrosis. Giant Visceral leishmaniasis: There may be increase
cells and chronic inflammatory infiltrates are pigmentation of face. There may be spontaneous
seen in connective tissue. bleeding, edematous gingiva and loose teeth.
Mucocutaneous leishmaniasis: Mucosal lesion
Management usually occurs 1-2 years after skin lesion. Lips,
Amphotericin B is the treatment of choice. soft palate and larynx may be involved. The
Disseminated aspergillosis in immuno- mucosal lesions are long standing, destructive,
compromised patients should be treated on an granulating ulcers which in many instance cause
severe mutilation of structure involved. Regional
individual basis with intravenous antifungal
lymphadenopathy is common.
agents and surgical debridment.
Management
Leishmaniasis
Amphotericin is the drug of choice for visceral
It is caused by the genus Leishmania which leishmaniasis. In mucocutaneous lesion, small
consists of three flagellate protozoa, which cause lesion may be treated by freezing with liquid
variety of distinct infections in man and are carbon dioxide, curettage or infiltration with 1-
transmitted by sandfly bites. 2 ml sodium stibgluconate. If the lesions are
multiple parenteral injection of amphotericin B
Types should be given.

• Visceral leishmaniasis—It is also called as kala Trichinosis

azar. It is caused by Leishmania donovani. It is caused by Trichinella spiralis, which is small,
• Cutaneous leishmaniasis—It is caused by spiral, thread like organism. Human infection
Leishmania braziliensis. occurs as a result of eating parasitized food,
usually pork, which has not been completly
Clinical Features cooked.
Visceral leishmaniasis: Incubation period is 2
Clinical Features
weeks to 2 years. Onset may be insidious with a
low grade fever or it may be abrupt with Most common site involved are striated muscle,
sweating and high intermittent fever. Cough and masseter, neck muscle and diaphragm. There is
522 Textbook of Oral Pathology

fever, facial and periorbital edema, muscle pain muscle, jaw and tongue can occur. There is also
and eosinophilia. monotony of speech.
There may be petechiae of buccal mucosa,
Oral Manifestations palate and floor of mouth. There is also bleeding
from gingiva, lips and nose.
Tongue is the most common site involved. It also
occurs in muscles attached to mandible, in Management
mandibular alveolar process and in gingival There is no specific treatment for trichinosis and
tissues. Trismus, muscular cramps of the facial in severe cases, prognosis is poor.
 CHAPTER
Periodontal
26 Pathology

Diseases of the periodontium are commonly Periodontitis Associated with Endodontic

encountered in daily practice. It is one of the Lesions
implicated causes of loss of teeth in adults. In • Endodontic-periodontal lesion
nearly every case, the condition begins as a • Periodontal-endodontic lesion
minor localized disturbance which unless • Combined lesion.
adequately treated, may gradually progress to
Developmental or Acquired Deformities and
the resorption of alveolar bone and exfoliation
Conditions
of tooth.
• Localized tooth-related factors that
Classification of Gingival Diseases predispose to induced gingival disease or
periodontitis
Gingival Diseases
• Mucogingival deformities and conditions
• Plaque induced gingival diseases
around teeth
• Nonplaque induced gingival lesions.
• Mucogingival deformities and conditions on
Chronic periodontitis edentulous ridges
• Localized • Occlusal trauma.
• Generalized.
Aggressive periodontitis GINGIVAL INFLAMMATION/GINGIVITIS
• Localized
• Generalized. It may occur in an acute, subacute, or chronic form.
Periodontitis as manifestation of systemic
disease Etiology
• Necrotising periodontal diseases Local Factors
• Necrotising ulcerative gingivitis • Microorganisms—The plaque associated with
• Necrotising ulcerative periodontitis. gingivitis is complex and heterogeneous. In
Abscesses of periodontium early stages of gingivitis, the Actinomyces
• Gingival abscess group of organisms are the dominant genus
• Periodontal abscess in the supragingival plaque. In children flora
• Combined lesion. associated with gingivitis is different as
526 Textbook of Oral Pathology

compared to adults. In children it is associated pirin, if applied to gingiva will provoke an

with Leptotrichia, Selenomonas and some inflammatory reaction.
Bacteroides. In patient with persistent Systemic Factors
gingivitis there is appearance of gram- • Nutritional disturbances—Nutritional imba-
negative rods and filamentous microorga- lance is frequently manifested as changes in
nism which are followed by spirochetes. the gingiva and deeper underlying periodon-
There is increase of P. intermedia in pregnancy tium.
gingivitis. • Pregnancy—Gingiva undergoes changes
• Calculus—either supragingival or subgin- during pregnancy which are termed as 'preg-
givae or both cause irritation of the contacting nancy gingivitis'.
gingival tissue. This irritation is produced by • Diabetes mellitus—It is reported in association
the by-products of the microorganisms or by with severe periodontal diseases and gingival
the mechanical friction resulting from the inflammation.
hard, rough surface of the calculus. • Psychiatric phenomena—It appears to have a
• Food impaction—The impaction of food and definite influence upon the severity of perio-
accumulation of debris on the teeth due to dontal disease.
oral negligence result in gingivitis. The • Hormonal changes—Puberty, menstruation
degradation products of food debris may also may also result in gingival inflammation.
prove irritating to the gingival tissues. • Others—Allergy and hereditary factor can
• Faulty or irritating restorations or appliances— also play important role in gingivitis.
Faulty restoration may act as an irritant to
gingival tissues and thereby induce gin- Types of Gingivitis
givitis. Overhanging margins of proximal According to course and duration
restorations may directly irritate the gingiva • Acute gingivitis—It is a painful condition that
and in addition allow the collection of food comes on suddenly and is of short duration.
debris and organisms which add further • Subacute gingivitis—It is a less severe phase
insult to these tissues. Prosthetic and ortho- of the acute condition.
dontic appliances impinging on the gingival • Recurrent gingivitis—It reappears after having
tissues produce gingivitis as a result of pre- been eliminated by treatment or disappears
ssure due to and of the trapping of food and spontaneously and then reappears.
microorganisms. • Chronic gingivitis—It comes slowly, is of long
• Mouth breathing—Drying of the oral mucous duration and is painless unless complicated
membrane, because of mouth breathing, due by acute and subacute exacerbations.
to environment of excessive heat or from According to distribution
excessive smoking, will result in gingival irri- • Localized gingivitis—It is confined to the
tation with accompanying inflammation. gingiva in relation to a single tooth or a group
• Tooth malposition—Teeth which have erupted to teeth.
or which have been moved out of physiologic • Generalized gingivitis—It involves the entire
occlusion, due to repeated subjected to abnor- mouth.
mal forces during mastication. • Marginal gingivitis—It involves gingival
• Chemical and drug application—Many drugs margins but may include a portion of the
like phenol, silver nitrates, volatile oils or as- contiguous attached gingiva.
 Periodontal Pathology 527

• Papillary gingivitis—It involves the Histopathological Features

interdental papillae and often extends into It will reveal infiltration of the connective tissue
the adjacent portion of the gingival margin. by varying numbers of lymphocytes, monocytes
• Diffuse gingivitis—It affects the gingival and plasma cells. Polymorphonuclear leukocytes
margins, attached gingiva and interdental are occasionally noted, particularly beneath the
papillae. crevicular epithelium which is nonkeratinized
and irregular. It is infiltrated by inflammatory
Clinical Features cells and is frequently ulcerated. The capillaries
of the connective tissue may be engorged and
Symptoms—The earliest symptoms of gingival sometimes may increase in number. Areas of
inflammation are increased gingival fluid fibrosis, hyperemia, edema and hemorrhage
production and bleeding from the gingival may be present.
sulcus on gentle probing.

Signs—Gingiva become redder when there is an Histopathological Types

increase in vascularization or when the degree of • Early lesion—It follows when an infiltrate of
epithelial keratinization is reduced. The color lymphocytes, plasma cells, neutrophils, mast
becomes paler when vascularity is reduced and cells and macrophages appear in addition to
keratinization is increased. The color may be the polymorphonuclear leucocytes. There is
sometimes reddish blue or deep blue, if venous destruction of collagen. Initially there is deve-
stasis has occurred. lopmental of rete pegs with increases number
The color change starts in the interdental of neutrophils in junctional epithelium.
papillae, gingival margins and spreads to the • Established lesion—In it gingival pocket is for-
attached gingiva. (Fig. 26.1). The consistency of med and the infiltrate contains a predomi-
gingiva may be spongy that pits on pressure and nance of plasma cells and lymphocytes;
there may be marked softness of the gingiva. polymorphs continued to predominate in the
Sometimes in inflammation there may be gingi- junctional epithelium. The junctional epithe-
val enlargement and it may lead to changes in lium shows elongated rete pegs in connective
the contour of gingiva. tissue with destruction of basement mem-
brane. There is also cellular debris seen with-
in the widened intercellular spaces of junc-
tional epithelium.

Management
The local irritants should be removed at this stage.
Thorough plaque control should be done with
scaling and polishing. Use of chlorhexidine, on a
short term basis.

Acute Necrotizing Ulcerative Gingivitis

It is an endogenous oral infection that is
Figure 26.1: Gingival inflammation presented as characterized by necrosis of gingiva. It is also
redness of gingiva called as 'trench mouth' due to its prevalence in
528 Textbook of Oral Pathology

combat trenches. Other synonyms for this are socioeconomic group, in underdeveloped
'Vincent's infection', 'acute ulceromembranous countries.
gingivitis', 'fusospirochetal gingivitis' and 'acute
Symptoms—Onset is sudden with pain,
ulcerative gingivitis'. Tissue destruction is caused
tenderness, profuse salivation and peculiar
by endogenous organisms that act either on the
metallic taste. Spontaneous bleeding from
tissue or, indirectly by triggering an inflamma-
gingival tissue. There is also a loss of sense of
tory reaction.
taste and diminished pleasure from smoking.
Etiology The typical fetid odor ultimately develops, which
may be extremely unpleasant. Teeth seem
• Role of bacteria — It is caused by fusiform
slightly to be extruded and are sensitive to
bacilli and spirochetes. In addition to it bac-
pressure or have a woody sensation. They are
teroides intermedius is also responsible for
slightly movable and the patient is unable to eat
ANUG.
properly. Gingiva may become superficially
• Local predisposing factors
stained with brown color. There is blunting of
– Poor oral hygiene, pre-existing marginal
interdental papillae.
gingivitis and faulty dental restorations.
– Deep periodontal pockets offered favo- Signs—A typical lesion consists of necrotic
rable environment for occurrence of the punched out, crater like ulcerations developing
disease. most commonly on the interdental papillae and
– Area of gingiva traumatized by opposing marginal gingiva. Removal of the lesion leaves
in malocclude teeth. raw surface. The surface of gingival crater is
– As tobacco smoke has a direct toxic effect covered by a gray, pseudomembranous slough,
on the gingiva, smoking and emotional demarcated from the reminder of the gingival
stress can predispose for ANUG. mucosa by pronounced linear erythema. In some
• Systemic predisposing factors cases, ulceration may develop on cheek, lip,
– Nutritional deficiency—Nutritional defici- tongue, palate and pharyngeal area. If untreated,
ency like vitamin C, vitamin B2 accentuate it may result in progressive destruction of the
the severity of the pathologic changes periodontium and denudation of the roots,
induced by the fusospirochetal bacterial accompanied by increase in the severity of
complex. complications. Regional lymph nodes are
– Debilitating disease—Chronic diseases like enlarged. There may be a slight elevation of
leukemia, aplastic anemia, syphilis, severe temperature.
gastrointestinal disturbances and AIDS
Histopathological Features
can act as predisposing factors.
– Marked malnutrition. It appears as a nonspecific acute necrotizing
– Psychosomatic factors—The disease often inflammation of the gingival margin involving,
occurs in association with a stress situation both, stratified squamous epithelium and the
as well as with increase in adrenocortical underlying connective tissue. The surface epi-
secretion. thelium is destroyed and is replaced by a pseudo-
membranous meshwork of fibrin, necrotic
Clinical Features epithelial cells, polymorphonuclear neutrophils
It is most commonly seen in the age group of 16 and various types of microorganisms. The
to 30 years, but can be seen in children from a low underlying connective tissue is markedly
 Periodontal Pathology 529

hyperemic with numerous engorged capillaries Clinical Features

and a dense infiltration of polymorphonuclear
nuetrophils. Numerous plasma cells may appear Both sexes are affected but it is more prevalent in
in the periphery of infiltrate. Necrotic material is women and occurs after age of 40. Gingiva
a prominent feature. becomes bright red, edematous and desquama-
tion of the surface epithelium of the attached
Management gingiva is also seen. It is usually manifested in
The involved areas are isolated with cotton rolls three forms, i.e. mild, moderate and severe.
and dried. A topical anesthetic is applied and Mild—This may be diffuse and extends through-
after 2 to 3 minutes, the areas are gently swabbed out the gingiva. It is painless.
with a cotton pellet to remove the pseudo-
Moderate—There is a patchy distribution of
membrane and nonattached surface. After the
area is cleansed with warm water, the superficial bright red and gray areas involving the marginal
calculus is removed. The patient is told to rinse and attached gingival (Fig 26.2). The surface is
the mouth every 2 hours, with a glassful of an smooth, shiny and normally resilient. Patient
equal mixture of warm water and 3% hydrogen may complain of a burning sensation and
peroxide. Twice daily rinse with 0.12% chlor- sensitivity to thermal changes. There is slight
hexidine are also effective. pitting on pressure and epithelium is not firmly
Patients with severe ANUG and lymphade- adherent to the underlying tissues.
nopathy are treated with antibiotics Penicillin Severe form—It is characterized by scattered
V 250 or 500 mg, 6 hourly or erythromycin 250 irregularly shaped areas, in which gingiva is
or 500 mg, 6 hourly with metronidazole 400 mg, 8 denuded and strikingly red in appearance. The
hourly, for 7 days are the drugs of choice. Scaling overall appearance of gingiva is speckled.
is performed, if sensitivity permits. After the disease Epithelium is friable and can be easily removed
process is diminished, complete gingival curettage from the underlying connective tissue leaving a
and root planing is done. Supportive treatment red surface that bleeds readily to minimum
consists of copious fluid consumption and trauma. A blast of air directed at the gingiva
administration of nutritional supplements. causes elevation of the epithelium and conse-
quent formation of bubbles.
Desquamative Gingivitis
It is a clinical manifestation of several different
diseases.
Etiology
• Dermatological diseases—like lichen planus,
mucous membrane pemphigoid, bullous
pemphigoid or pemphigus.
• Infections—Tuberculosis, chronic candidiasis,
histoplasmosis.
• Endocrine imbalance, chronic infection and
drug reaction.
• Abnormal response to irritation and idiopathic
causes. Figure 26.2: Denudation of gingiva seen in desquamative
• It can be caused by chemical burns. gingivitis
530 Textbook of Oral Pathology

Histopathological Features fluctuant and pointed with a surface orifice, from

Histopathological features may mimic either which an exudate may be expressed. The adja-
bullous lesions of mucous membrane pemphi- cent teeth are sensitive to percussion.
goid or lichenoid reaction. Sometimes, epithe-
lium may be thin and atrophic with little or no
keratin at the surface and dense diffuse
inflammatory cells in the underlying connective
tissue. Retepegs are blunted and there may be
clefting below the basement membrane with
edema and a mild chronic inflammatory
infiltrate.

Management
A complete history should be taken to uncover
a possible coexistent extraoral cause. All possible Figure 26.3: Gingival abscess presented as red swelling
local irritants should be removed. The patient Histopathological Features
must be carefully instructed in plaque control
It consists of a purulent focus in the connective
and instructed for using a soft toothbrush,
oxidizing mouth wash (hydrogen peroxide 3%, tissue, surrounded by a diffuse infiltration of
polymorphonuclear leukocytes, edematous tissue
diluted to one part peroxide and two parts of
and vascular engorgement. The surface
warm water) should be use twice daily. Improve-
ment has been noticed with tetracycline therapy. epithelium has a varying degree of intra-and
extracellular edema, with invasion of leukocytes
Doxycycline monohydrate 100 gm daily for 4 to
and ulceration.
11 weeks. Topical corticosteroid ointment and
creams such as triamcinolone 0.1% fluocinolone
Management
0.05% is applied and gently rubbed into the
gingiva several times daily. After topical anesthetic is applied, the fluctuant
area of the lesion is incised with a blade and the
Gingival Abscess incision is gently widened to permit drainage.
Etiology The area is cleansed with warm water and
It results from bacteria which are carried deep covered with a gauze pad. After the bleeding
into the tissue, when a foreign substance core stops, patient is instructed to rinse every 2 hours
food (e.g. lobster shell fragment) is forcefully with a glassful of water.
embedded into the gingiva.
Pericoronal Abscess
Clinical Features
It is also called as 'pericoronitis'. It is the infection
It is limited to the marginal gingiva or the inter- of soft tissues surrounding the crown of a
dental papilla. It is localized, painful, rapidly partially erupted tooth.
expanding lesion that is usually of sudden onset.
Clinical Features
Signs—In early stages, it appears as a red swelling
with a smooth, shiny surface (Fig. 26.3). Within The most common type of pericoronal infection is
24 to 48 hours, the lesion usually becomes found around the mandibular 3rd molar.
 Periodontal Pathology 531

Pericoronal infection of infancy—Pericoronal Drainage—Careful probing should be done

infection of infancy is often associated with the around the 3rd molar, which permits entry into
supradental tissues, involving the superior the expanded follicle and allows evacuation of
portion of the follicle and the overlying pus and other septic material.
mucoperiosteum, which may become inflamed. Extraction—When the symptoms become
It ultimately develops into small fluctuant subacute, the impacted 3rd molar should be
abscess. When this fluctuance is digitally extracted.
ascertained, incision and drainage should be
Operculectomy—Sometimes when the retention of
carried out, followed by warm saline rinses at
3rd molar is necessary, the inflamed tissue
frequent intervals.
surrounding the occlusal portion of the tooth
Symptoms—Pain, malaise and swelling of should be excided.
peritonsillar region. Extraction of maxillary 3rd molar—Maxillary 3rd
Signs—It may result in cellulitis and muscular molar can be a contributing factor to the
trismus. There is also regional lymphadeno- pericoronal infection of the mandibular 3rd molar.
pathy, submaxillary and pharyngeal abscess. In such cases, especially when the mandibular
Operculum may get traumatized by opposing 3rd molar is fully erupted in proper place, the
maxillary 3rd molar should be extracted prior to
teeth during mastication. Edema, visible in both
the retention of the mandibular 3rd molar,
submandibular area and peritonsillar region
considering the recurrent nature of the
occurs. There is extreme tenderness on palpation
inflammatory episode.
of the abscess.
GINGIVAL ENLARGEMENTS/SWELLINGS
Histopathological Features
Chronic Inflammatory Enlargement
Connective tissue stroma is hyperemic which
Etiology
shows acute inflammatory cells.
It can be caused by prolonged exposure to dental
Complications plaque, which may occur due to poor oral
hygiene, abnormal relationship of adjacent and
The involvement may become localized in the
opposing teeth, lack of tooth function,
form of a pericoronal abscess. It may spread
overhanging margins of dental restoration and
posteriorly into the oro-pharyngeal area and
improperly contoured dental restoration or
medially, to the base of the tongue. Peritonsillar
pontics. Food impaction, irritation from clasps
abscess, cellulitis and Ludwig's angina are
or saddle areas of removable prosthesis and
common.
nasal obstruction. Habits—such as mouth
breathing can cause gingival enlargement, which
Management
is more common in the anterior region.
Antibiotics—Immediately, antibiotics should be
started. Most commonly use antibiotics are Clinical Features
phenoxylmethyl penicillin 250 mg four times The enlargement is generally papillary or
daily. In pericoronitis due to ulceromembranous marginal and localized or generalized. It origi-
gingivitis metronidazole 200 gm three times daily nates as a slight ballooning of the interdental
for 7 days is given. papilla or the marginal gingiva.
532 Textbook of Oral Pathology

Signs—In early stages, it produces a life preserver-

like bulge around the involved teeth (Fig. 26.4). It
increases in size, until it covers a part of the crown.
Lesions may be deep red or bluish red. They are
soft and friable, with a smooth shiny surface and
tendency to bleed. It progresses slowly and
painlessly, unless complicated by acute infection
or trauma. Sometimes, enlargement can occur as
a discrete, sessile or pedunculated mass,
resembling a tumor. They may undergo
spontaneous reduction in size, followed by
exacerbation and continued enlargement. Painful Figure 26.5: Dense nodular overgrowth seen in patient of
fibromatosis gingivae
ulceration, sometimes, occurs between the mass
and the adjacent gingiva.
Clinical Features
Histopathological Features It may be present at birth or may become apparent
It shows exudative and proliferative features of with eruption of deciduous or permanent teeth. It
chronic inflammation. Lesions may contain is manifested as dense smooth diffuse or nodular
preponderance of inflammatory cells and fluid overgrowth of gingival tissue of one or both arches
with vascular engorgement, new capillary for- that usually occurs at the time of eruption of teeth
mation, associated with degenerative changes. (Fig. 26.5). It has a characteristic pebbled surface.
In some cases surface has a nodular appearance.
Fibromatosis Gingiva Tissue is of normal or pale color. In some cases, it
may appear pink. It is often so firm, leathery and
It is also called as 'elephantiasis gingivae'. It is a
dense that it prevents normal eruption of teeth
diffuse overgrowth of gingival tissues. It is
(Figs 26.6A and B).
hereditary. It may be autosomal dominant as well
It is not painful and shows no tendency for
as of recessive type.
hemorrhage. Extent may be such that the crown
of fully erupted teeth may be hidden. The dense
firm gingival swelling results in varying spacing
between the teeth and change in profile and facial
appearance

Histopathological Features

Epithelium is thickened with elongation of rete

pegs, although the bulk of the tissue is composed
of dense fibrous connective tissue. Bundles of
collagen fibers are coarse and show few
interspersed fibroblasts or blood vessels. Mucoid
Figure 26.4: Inflammatory enlargement of gingiva changes in fibrous tissue are frequent and
showing ballooning of the interdental papilla occasional presence of giant cells has been noted.
 Periodontal Pathology 533

Clinical Features
It occurs in newborn infants and females are ten
times more affected than males. The maxilla is
more commonly affected than mandible and
anterior region is more commonly affected than
posterior region. Occasionally more than one
growth may be present.
Signs—Patient complains of smooth swelling.
The swelling is usually round or oval, showing
irregular lobulation. Base is usually peduncu-
lated, but it may be sessile. Usually size is less
than 1 to 1.5 cm in diameter, but sometime it may
be larger. Larger lesion may be large enough to
A protrude from the mouth.

Histopathological Feature
It is similar to granular cell myoblastoma except
that, it does not exhibit pseudoepitheliomatous
hyperplasia. The cells consist of large cells closely
packed beneath the surface epithelium, from
which they are separated by a narrow zone of
connective tissue. The cells are round or
polyhedral, occasionally oval or elongated. The
cytoplasm of the cells contains abundant fine
eosinophilic granules (Fig. 26.7) . The nucleus is
small, vesicular and eccentrically situated with
B
well defined central nucleus. Nucleus is
Figures 26.6A and B: Fibromatosis gingivae preventing basophilic.
normal eruption of teeth

The covering epithelium is usually mildly

acanthotic and some hyperkeratosis may be
present. Electron microscopic study show both
fibroblasts and myofibroblast like cells.

Management
Surgical correction should be done.

Congenital Epulis
This is the growth of gingiva in infants. It is
described in 1871. It is believed to diverse cells
origin, i.e. it consist of fibroblast, histiocytes, Figure 26.7: Congenital epulis of newborn showing
undifferentiated mesenchymal cells etc. granular cells
534 Textbook of Oral Pathology

Management Signs—The first change noted is a painless bead

It should be surgically excised. Recurrence rate is like enlargement in the size of the gingiva,
rare. starting with one or two interdental papillae
(Fig. 26.8). The surface of gingiva shows an
Retrocuspid Papilla increase in stippling and finally, a cauliflower,
It is a small elevated nodule located on the lingual warty or pebbled surface. As the enlargement
mucosa of the mandibular cuspids. increases, the gingival tissue becomes lobulated
and clefts are seen between each enlarged gingiva
Clinical Features (Fig. 26.9). Palpation reveals that the tissue is
It is extremely common in children usually dense, resilient and insensitive. It shows little
between the ages of 8-16 years. There is no sex tendency to bleed.
predilection. They may develop massively, covering a
considerable portion of the crown. They may
Signs—It is a soft, well circumscribed, sessile, interfere with occlusion. The presence of an
mucosal nodule, commonly bilateral; it is located enlargement makes plaque control difficult,
lingual to the mandibular cuspids, between the resulting in a secondary inflammatory process
free gingival margin and the mucogingival that complicates the gingival hyperplasia.
junction. It is usually of dimension of 2-4 mm.

Histopathological Features
The structure appears as an elevated mucosal tag,
often showing mild hyperorthokeratosis or
hyperparakeratosis, with or without acanthosis.
The underlying connective tissue is sometimes
highly vascularized and may exhibit large
stellate fibroblasts as well as occasional epithelial
rests.

Management
Figure 26.8: Drug-induced gingival enlargement showing
It is benign lesion that is easily removed and does lobulated appearance
not recur.

Gingival enlargement due to drugs

Dilantin Sodium
It can be caused by Dilantin sodium, cyclosporin
or nifedipine.

Clinical Features
Gingival hyperplasia may begin as early as two
weeks after Dilantin therapy. The hyperplasia is
generalized throughout the mouth, but it is most
severe in maxillary and mandibular anterior Figure 26.9: Drug-induced enlargement showing ‘bead
region. like’ enlargement
 Periodontal Pathology 535

Histopathological Features intracellular or extracellular edema with

The stratified squamous epithelium covering the prominent intercellular bridges and leukocytic
tissue is thick and has a thin keratinized layer. infiltration. Between the capillaries, there is
The retepegs are extremely long and thin, moderately fibrous stroma, with a varying degree
sometimes called as 'test tube' pegs, with of edema and leukocytic infiltration.
considerable confluence. Mitotic figures are
Management
seldom seen. The bulk of tissue is made up of
large bundles of fibers, interspersed with Most gingival enlargement during pregnancy can
fibroblasts and fibrocytes. There may be a pronoun- be prevented by the removal of local irritants and
ced hyperplasia of the connective tissue and the institution of a fastidious oral hygiene.
epithelium. There is acanthosis of epithelium and
the elongated rete pegs extend deep into the Granuloma Pyogenicum
connective tissue. It is a nonspecific, tumor-like, conditional
enlargement of the gingiva that is considered as
Pregnancy Tumor
an exaggerated conditional response to minor
It is an inflammatory reaction to the local trauma.
irritants. It usually appears after 3rd month of
pregnancy.
The lesions appear as discrete, mushroom
like, flattened spherical masses, that protrude
from the gingival margins or more frequently
from the interproximal space and are attached
by sessile or pedunculated base (Fig. 26.10). It
tends to expand laterally. The pressure from the
tongue and the cheek perpetuates its flattened
appearance. It is generally dusky red or magenta;
it has a smooth glistening surface that frequently
exhibits numerous deep red, pinpoint markings
(Fig. 26.11). The consistency varies from semi- Figure 26.10: Pregnancy tumor showing ‘mushroom’ like
firm, but may have a varying degree of softness enlargement
and friability. It is usually painless, unless its size
and shape foster the accumulation of debris
under its margin or interfere with occlusion, in
which cases painful ulceration may occur.

Histopathological Features
It consists of a central mass of connective tissue,
the periphery of which is outlined by thick
stratified squamous epithelium with prominent
rete pegs. The connective tissue consists of
numerous, diffusely arranged, newly formed and
engorged capillaries, lined by endothelial cells.
The epithelium exhibits some degree of Figure 26.11: Pregnancy tumor
536 Textbook of Oral Pathology

Clinical Features
It varies from a discrete spherical, tumor-like mass
with a pedunculated attachment to a flattened,
keloid like enlargement with a broad base
(Fig. 26.12). It is bright red or purple color and
either friable or firm, depending on its duration.
In majority of the cases, it presents with surface
ulceration and purulent exudation.

Histopathological Features
It appears as a mass of granulation tissue with Figure 26.12: Granuloma pyogenicum presented as
discrete spherical tumor like mass
chronic inflammatory cell infiltration. Endothe-
lial proliferation and formation of numerous
vascular spaces are the prominent features. The Pathogenesis
surface epithelium is atrophic in some areas and Periodontal pockets are caused by micro-
hyperplastic in others. organisms and their products, which produce
pathologic tissue changes that lead to the
Management deepening of the gingival sulcus. Pocket formation
Removal of lesion, along with elimination of starts as an inflammatory change in the
irritating factors. connective tissue wall of the gingival sulcus. The
cellular and fluid inflammatory exudates cause
PERIODONTAL POCKETS degeneration of the surrounding connective
tissues, including the gingival fibers. Just apical
Classification to the junctional epithelium, an area of destroyed
collagen fibers develops and becomes occupied
• Gingival pocket (relative or false)—It is formed
by inflammatory cells and edema. Collagen loss
by gingival enlargement, without destruction
may occur due to enzymes, like collagenase and
of the underlying periodontal tissues. The
other lysosomal enzymes from polymor-
sulcus is deepened because of the increased
phonuclear leukocytes and macrophages, which
bulk of the gingiva.
become extracellular and destroy the collagen.
• Periodontal pocket (absolute or true)—There is
As a consequence of loss of collagen, the apical
destruction of the supporting periodontal
portion of the junctional epithelium proliferates
tissue; progressive pocket deepening leads to along the root, extending in finger like projections.
destruction of the supporting periodontal As the apical portion migrates the coronal portion
tissues and loosening and exfoliation of the of the junctional epithelium detaches from the root.
teeth. As a result of inflammation, polymorphonuclear
• Suprabony pocket (supracrestal or supra- neutrophils invade the coronal end of junctional
alveolar)—In it, bottom of the pocket is coronal epithelium. An increase in number of these cells,
to the underlying alveolar bone. results in loss of tissue cohesiveness and tissue
• Infrabony (intrabony, subcrestal or intra-alveolar) detachment from the tooth surface. Thus, the
— In it, bottom of the pocket is apical to the bottom of the sulcus shifts apically, resulting in
level of the adjacent alveolar bone. deepening of the periodontal pocket.
 Periodontal Pathology 537

Clinical Features Types

Symptoms—Gingival bleeding or/and suppura- • Mild periodontitis—It is characterized by an
tion, tooth mobility and diastema formation may attachment loss of 2 to 4 mm, minimum
be present. Patient may complain of localized furcation invasion and little tooth mobility.
pain or deep pain in the bone. • Moderate periodontitis—It exhibits 4 to 7 mm
Signs—In some cases, pus may be expressed by of probing attachment loss, early to moderate
applying digital pressure. When explored with furcation invasion and slight to moderate
a probe, the inner aspect of the periodontal tooth mobility.
pocket is generally painful. There may be bluish • Severe periodontitis—Probing attachment loss
red, thickened marginal gingiva and a bluish red of 7 mm or more, with significant furcation
vertical zone from the gingival margin to the invasion often through and through, with
alveolar mucosa. excessive tooth mobility.

Histopathological Features Clinical Features

There may be circulatory stagnation, destruction It generally affects both the sexes equally and is
of the gingival fibers and surrounding tissues, seen in older age groups more frequently. It is
which results in discoloration. There is atrophy usually generalized, although some areas may
of the epithelium and edema, which results in a be involved more deeply than others.
shiny surface of lesion. There is increased
Symptoms—It is usually painless, but sometimes
vascularity, thinning and degeneration of the
exposed root may be sensitive to heat and cold,
epithelium with close proximity to the engorged
in absence of caries. Areas of localized deep pain,
vessels.
sometimes radiating deep into the jaws are
Management established.

Pocket irrigation — Devices like squeeze bottles Signs—The characteristic finding in it is gingival
and blunt hypodermic needles can be use to inflammation, which results from accumulation
irrigate the pocket with chemotherapeutic agents. of plaque and loss of periodontal attachment. The
Flap surgery to eliminate pockets. gingiva is slightly or moderately swollen and
exhibits alteration in color from pale to magenta.
Adult Periodontitis There is also loss of stippling and gingival
bleeding, which may be either spontaneous or
It is also called as 'slowly progressive periodontitis', easily provoked. There is presence of pocket with
'chronic adult periodontitis' and 'chronic variable pocket depth. Both, horizontal and
inflammatory periodontitis'. angular bone loss can be found.
Tooth mobility is found in advanced cases,
Etiology
where bone loss has been considerable. Teeth
• Local factors—It occurs in association with give off a rather dull sound when tapped with a
plaque, calculus and poor oral hygiene. metal instrument. The embrasures may be open
• Systemic disease—Development of systemic because the interdental papillae are deficient.
disease like diabetes mellitus, hormonal Gingival recession is a common phenomenon in
alteration or immunologic defect can later stages of disease, which may expose the
accelerate periodontal diseases. cementum (Fig. 26.13).
538 Textbook of Oral Pathology

The lesions are more generalized and all or most

of the teeth are affected, without any definite
pattern of distribution.
The age of onset of the disease ranges from
the middle to late teens and till 30 years of age.
Signs—Gingiva is acutely inflamed, often
proliferated, ulcerated and fiery red. Bleeding
may occur spontaneously, or on slight provo-
cation. In some cases, gingiva appears pink and
free of inflammation; but in spite of this, deep
pockets can be revealed on probing (Fig. 26.14).
Some patients may have systemic manifesta-
Figure 26.13: Gingival recession of lower anterior region tions like weight loss, mental depression and
in periodontal disease
general malaise.
Histopathological Features
Histopathological Features
The enlarged free marginal gingiva is densely
infiltrated with lymphocytes and plasma cells. It is as not different from chronic periodontitis
The apical borders of the inflamed area approach
the crest of the alveolar bone and the crestal Management
fibers of the periodontal ligament. The crevicular
If microflora contains gram-positive microorga-
epithelium shows various areas of proliferation nisms, then it should be treated with 250 mg
and often, tiny ulcerations. There is appearance
amoxicillin and 125 mg potassium clavulanate
of giant cells and osteoclasts on the surface of
three times daily, for 14 days, along with scaling
the bony crest. Principal periodontal ligament and root planning. If flora is gram-negative, then
fibers become disorganized and are detached from
clindamycin should be given with dose of
the tooth.
150 mg, four times a day, for 7 days, along with
scaling and root planning.
Management
Scaling and curettage can be done.

Rapidly Progressive Periodontitis

Etiology
Microorganisms responsible are Actinobacillus
actinomycetemcomitans, Bacteroides gingivalis etc.
Altered chemotactic response to neutrophils has
been reported in some cases.

Clinical Features
Rate of destruction is rapid over a period of time, Figure 26.14: Rapidly progressing periodontitis presented
as compared to slowly progressive periodontitis. as destruction of periodontal ligament
 Periodontal Pathology 539

Juvenile Periodontitis noglobulins. Actinobacillus actinomycetemcomitans

It is also called as 'periodontosis'. It is an autosomal and Capnocytophaga can produce fibroblast
recessive and X-linked character. It is an aggre- inhibiting factors, which impair the defence
ssive, but uncommon form of periodontitis mechanism. Polyclonal B-lymphocyte activation
found in children and young adults. by periodontal bacteria may result in production
of antibodies which are unrelated to the activating
Definition agent.

It is a disease of the periodontium, occurring in

Clinical Features
an otherwise healthy adolescent and is
characterized by a rapid loss of alveolar bone Juvenile periodontitis affects both, males and
about one or more teeth of the permanent females and is seen most frequently in the periods
dentition. between puberty and 20 years of age. It can be
localized or generalized and maxillary teeth are
Causes more frequently affected than mandibular, with
Inherited defect in neutrophilic chemotactic strong left-right symmetry. Teeth more commonly
function, which affects the ability of polymor- affected are 1st molars and incisors. There is an
phonuclear leukocytes to phagocytose and increase in the number of affected teeth with
degranulate, thus impairing the host resistance advancing age, which leads to widely accepted
is responsible for juvenile periodontitis. Several assumption that the disease starts with a localized
authors describe it as a hereditary and familial lesion and at later stages becomes generalized.
pattern. Microorganisms responsible are usually
Signs—The most striking feature is the lack of
gram-negative anaerobic rods, along with
clinical inflammation, despite the presence of
minimum amount of attached plaque.
deep periodontal pocket. Presence of deep pockets
Mechanism of Bone Loss with secondary inflammation may occur. Deep,
dull radiating pain may occur with mastication,
Actinobacillus actinomycetemcomitans can produce due to irritation of the supporting structures by
substances that can kill PMNs and monocytes, mobile teeth and impacted food.
thereby compromising the patient's ability to fight
the invading bacteria or their products. These Symptoms—Initial complain is mobility and
leukotoxins may be counteracted by the pathological drifting of first molars and incisors.
development of serum antibodies. Inhibition of Classically, the clinician sees a distolabial
PMNs may be induced by some gram-negative migration of the maxillary incisors, with a
bacteria. Endotoxins from Actinobacillus actino- diastema formation. Subgingival calculus is
mycetem-comitans can induce Schwartzman uncommon. As the disease process continues,
reaction, macrophage toxicity, platelet aggre- denuded root surfaces become sensitive to thermal
gation, complement activation and bone resorp- and tactile stimuli. Periodontal abscess may form
tion. at this stage and regional lymph node enlargement
Actinobacillus actinomycetemcomitans, may occur. The rate of bone loss is three to four
Capnocytophaga and Bacteroides can produce times faster than in typical periodontitis. Rapid
proteolytic enzymes that can destroy collagen, and typically angular loss of alveolar bone occurs,
activate complement system or degrade immu- which may progress to tooth loss.
540 Textbook of Oral Pathology

Histopathological Features Clinical Features

A thin, frequently ulcerated pocket epithelium, There is reddened, scaly, rough palms and soles,
infiltrated by numerous leukocytes covers large inflamed gingivae and horizontal bone
area of inflammatory cell accumulation, com- destruction. It usually begins after eruption of the
posed mainly of plasma cells and blast cells with primary second molars. Loss of entire primary
lymphocytes and macrophages present in small dentition by the age of 5 years and loss of
numbers. There is a proliferation of the epithelial secondary dentition by the age of 20 years.
attachment along the root surface. There is also Gingival swelling and severe halitosis can also
slight cellular infiltration in the connective tissue. occur.

Management
Histopathological Features
Standard periodontal therapy—It includes scaling
and root planning, curettage, flap surgery with It includes marked chronic inflammation of the
and without bone grafts, root amputation, lateral walls of the pocket, with predominantly
hemisection, occlusal adjustment and strict plasma cell infiltrate, considerable osteoclastic
plaque control. activity and apparent lack of osteoblastic activity.
An extremely thin cementum is present. The
Antibiotic therapy—Tetracycline 250 mg, four underlying connective tissue exhibits increased
times for daily 7 days is given. vascularity and mixed inflammatory cellular
infiltrates, mixed infiltrates consist of polymor-
Papillion Lefevre Syndrome
phonuclear leukocytes, lymphocytes, histiocytes
It is an autosomal recessive and inherited and plasma cells.
disorder. It is a triad of hyperkeratosis of palms
of the hand and soles of feet. Extensive prepu- Management
bertal destruction of the periodontal bone
supporting the dentition, usually extensive Management should be carried out with the help
generalized horizontal bone loss. Calcification of of antibiotics, mechanical plaque control and
dura is seen. management of skin lesion.
 CHAPTER
Temporomandibular
27 Joint Pathology

It is a unique joint in which translatory as well D. Neoplastic disturbances of the temporomandibular

as rotational movements are possible and where joint
both the ends of bone articulate, in the same E. Extraarticular disturbances of TMJ
plane, with that of other bone. It is also called as • Costen sysdrome
ginglymodiarthrodial type of joint, meaning that • Myofacial pain-dysfunction syndrome.
it has a relatively sliding type of movement
between bony surfaces, in addition to hinge Osteoarthritis
movement, common to diarthrodial joint.
It is also called as ‘osteoarthrosis’ or ‘degenera-
tive arthritis’. It is primarily a disorder of
TEMPOROMANDIBULAR JOINT DISORDERS
movable joints characterized by deterioration
Classifications and abrasion of the articular cartilage with
formation of new bone at the joint surface. There
Shafer’s
is destruction of the soft tissue component of the
A. Developmental disturbances
joint and subsequent erosion with hypertrophic
• Aplasia of mandibular condyle
changes in bone. There is breakdown of the
• Hypoplasia of mandibular condyle
connective tissue covering of the condyle, arti-
• Hyperplasia of mandibular condyle.
cular eminence and the disk. Articular eminence
B. Traumatic disturbances
shows resorption and the underlying bone
• Luxation and subluxation (complete and
becomes sclerotic.
incomplete dislocation)
• Ankylosis
Etiopathogenesis
• Injuries of articular disk
• Fractures of condyle. The lesion is brought about either by an increase
C. Inflammatory disturbances of TMJ in the functional demands of the healthy tissue
• Arthritis due to specific infection or by deterioration in the functional capacity of
• Rheumatoid arthritis the tissue. Breakdown of the joint may occur
• Osteoarthritis when the tissues are subjected to repetitive
• Traumatic arthritis. overload in excess of their functional capacity
542 Textbook of Oral Pathology

or with normal load when the capacity is reduced tend to clump together and become unevenly
as a part of aging process. distributed throughout the fibrous matrix. At this
By another theory, bone growth does not cease stage, the sub-articular bony end plate may still
completely after puberty and remodeling of the be intact, but later osteoclastic resorption of the
joint progresses under functional demands. bone takes place. Vertical splitting of the articular
Degenerative joint disease may develop when the layer takes place and this is followed by frag-
remodeling rate of bone exceeds that of the mentation and loss of articular surface, with
cartilaginous repair. The gross evidence of these exposure of the underlying bone. Attempted re-
changes is the formation of marginal osteophytes pair by proliferation of overlying articular tissue
with development of new bone in the area downward into the resorbing areas of sub-
adjacent to the cartilage. articular bone is sometimes seen and this leads
to uneven thickening of the articular surface.
Clinical Features Osteophytic lipping was found only on the
anterior surface of the condyle. Destruction of the
It is common in many joints, but it is not frequently
articular cartilage, in turn, causes erosion and
found in TMJ. It occurs in patients older than 40
perforation with ultimate destruction of the inner
years of age and 85% of them are older than 70,
articular disk. Secondary arthritic changes in the
with a mean age of 53 years. Females are affected
articular disk may result in flattening of the disk.
6 times as frequently as males.
Initial destruction of the soft tissue component of
Symptoms: Unilateral pain over the joint, which the joint occurs with subsequent erosion and
may be sensitive to palpation, occurs. Pain on hypertrophic changes in the bone. The connective
movements or biting occurs, which may limit tissue covering the condyle eminence and disk
mandibular function. Pain is usually located to breaks down. The bony surface of the condyle and
the immediate preauricular region. articular eminence shows resorption with the
underlying bone becomes sclerotic.
Signs: There is deviation of the jaw towards the
affected side. Stiffness of the joint is present. Management
There is presence of crepitation of the joint; the
Elimination of the cause: It includes occlusal adjust-
sound indicates degeneration within the arti-
ment or replacement of the missing teeth and ill
culating surfaces of the joint or disk. There is limi-
fitting prosthesis, grinding, treatment of caries
tation of jaw movements, which becomes increa-
and periodontal disease. We can also give phy-
singly apparent with function.
siotherapy, myotherapy, medical therapy, cor-
Early signs may progress to spasm of the
ticosteroids and occlusal splints.
masticatory muscles resulting in stiffness and
locking of the jaw. If not treated at this point it Rheumatoid Arthritis
may lead to irreversible changes in the TMJ.
It is a debilitating systemic disease of unknown
Normal course is between 1 to 3 years. Severe
origin, characterized by progressive involvement
symptoms last for about nine months, but gra-
of the joint, particularly bilateral involvement of
dually burn out leaving little or no disability.
large joints. Bony components of the TMJ are
affected secondary to the granulomatous invol-
Histopathological Features
vement of the synovial membrane that subse-
The earliest changes occur in the articular quently spreads to the articular surface of the
covering of the condyle. It shows that the cells condyle.
 Temporomandibular Joint Pathology 543

Etiopathogenesis Clinical Features

Its manifestations are probably due to a two phase General: It more commonly occurs in temperate
process. Phase one result from some systemic climate and has its highest incidence in women
infection, which evokes an inflammatory from 20 to 50 years of age. In typical cases, small
response within the joint. Phase two as an auto- joints of fingers and toes are the first to be affec-
immune reaction to the antigen generated by the ted. Swelling of the proximal but not the distal,
initial inflammation itself or it may be associated interphalangeal joints gives the finger a spindle
with derangement of the immune response to appearance and swelling of the metatar-
the exogenous antigen. sophalangeal joints results in broadening of feet.
In the active phase, TMJ may get involved
Symptoms: It includes bilateral stiffness, crepitus,
bilaterally. The joint space enlarges with synovial
tenderness and swelling over the joint. Fever,
effusion which attacks the fibrocartilage and
malaise, fatigue, weight loss, pain and stiffness
ultimately produces erosion of the underlying
in the limb are also evident. Polyarthritis deve-
bone. This causes pain, stiffness and limitation
lops subsequently. Large and weight bearing
of movement. The process then enters the hea-
joints are frequently affected.
ling phase, where the symptoms subside and
remodeling of the articular surface occurs. Bony Subcutenous nodules: There is formation of sub-
components of the TMJ are affected secondary cutaneous nodules on the pressure points, sites
to the granulomatous involvement of its synovial of friction and various vascular lesions, both
membrane that subsequently spreads to the necrotizing and obliterative types. Severe defor-
articular surface of the condyle. mities of extremity can occur as a result of joint
However, chronic phase may follow before collapse, tendon rupture and muscle involve-
healing occurs. Here, there is proliferation of the ment.
synovial membrane due to inflammation this is Signs: The joint may become red, swollen and
called as pannus formation. This pannus then
warm to touch. Muscle atrophy around the joint
encroaches the joint space and causes destruction
is common. In hands, it may produce an ulnar
of the articular cartilage. In this lipping of the drift. Bursitis can also occur.
condyle and marginal proliferation is seen, this
result in narrowing of joint space. Here, predo- TMJ Involvement
minant clinical findings are crepitus, pain on
biting and tenderness. The granulomatous tissue It can be acute or chronic and usually, it is bila-
replaces the articular surface and small adhe- terally involved.
sions develop between the articular surface and Acute case: In acute cases, there is bilateral stiff-
disk. ness, deep seated pain, tenderness on palpation
Psychosomatic: Emotional trauma, anxiety and and swelling over the joint. There is limitation
in opening of mouth.
environmental strain can lead to the onset of
rheumatoid arthritis. Immunological—The pre- Chronic cases: In chronic cases, crepitus is the most
sence of rheumatoid factor in the serum and frequent finding. Functional disturbances like
synovial fluid of affected patients suggests deviation on opening and inability to perform
immunological etiology. Also present are plasma lateral excursions are common. Anterior open
cells and lymphocytes on histological exami- bite is present due to bilateral destruction and
nation. anteroposterior positioning of the condyle.
544 Textbook of Oral Pathology

Fibrous ankylosis of the joint which may be partial Classification

or complex occurs in long-term. Pain on biting is
referred to the temporal region, ear and angle of • True (intra-articular): It is any condition that
mandible. produces fibrous or bony adhesion between
the articular surfaces of the TMJ.
Histopathological Features • False (extra-articular): It is the one which
results from pathologic conditions outside
Proliferation of synovial lining cells with intense
the joint, that result in limited mandibular
infiltration of lymphocytes, plasma cells and
mobility.
polymorphs. As the inflammation becomes chro-
• Bony: If bone is present between the arti-
nic, granulation tissue spreads across the cartila-
culating surfaces and prevents movements,
genous articular surface undermining the mar-
gins and eventually eroding the adjacent bone. it is called as bony ankylosis.
Later on, the fibrous tissue becomes dense and it • Fibrous: If the medium which prevent, the
may unite the head of the condyle to the articular movements is fibrous, it is called as fibrous
fossa. ankylosis.
• Partial: If there is incomplete union between
Investigations the articulating surfaces, it is called as partial
ankylosis.
Rose Waller test is positive in 70% of the patients
• Complete: If there is complete union between
with rheumatoid arthritis.
the articulating surfaces, it is called as comp-
Antinuclear antibodies are detected by indi-
rect immunofluorescence. lete ankylosis.
Analysis of synovial fluid is essential for the
immediate diagnosis of joint infection, infla- Etiology
mmation and degene-rative disease. False
Management Myogenic: The most common problem associated
Adequate rest to the joint, soft diet is advocated. with muscle origin is fibrosis, which may result
Treatment should be given for suppression of from chronic infection of the elevator muscles
the active process, preservation of function and of mastication. Myositis ossificans can also
prevention of deformities. produce limitation of opening.
Intra-articular corticosteroid injections, Neurogenic: They include epilepsy, brain tumor,
nonsteroidal anti-inflammatory drugs, immuno- bulbar paralysis and cerberovascular accidents.
modulator, slow acting antirheumatic drug can
be given. Psychogenic: Here the affected persons exhibit no
Local treatment is done with heat, diathermy, pain, but cannot get the jaws separated also
jaw exercise or a mouth stretcher. Muscle streng- called as hysterical trismus and is apparently
thening exercise and hydrotherapy. produced due to fright.
Bone impingement: The most common is coronoid
Ankylosis
impingement. Malformation of coronoid such as
Ankylosis, a Greek word which means ‘stiff exostosis or elongation can cause the mandible
joint’. It is an abnormal immobility and consoli- to impinge on the posterior aspect of the zygoma,
dation of the joint. when opening is attempted.
 Temporomandibular Joint Pathology 545

The formation of fibrous adhesions or duce several millimeters of interincisal opening

cicatrical bands of scar tissues usually occurs after (Fig. 27.1). Asymmetry of the face with fullness
a traumatic accident or burns. on the affected side and relative flattening on the
Neoplastic disease like osteochondroma. unaffected side (Fig. 27.2).
In unilateral ankylosis, patient’s face is
True deviated towards the affected side (Fig. 27.3). The
Congenital: Abnormal intrauterine development, chin is retracted on the affected side and slightly
birth injuries and congenital syphilis. bypasses the midline. Slight gliding movement
Trauma: Trauma to the chin forces the condyle towards the affected side. Cross bite is present.
against the glenoid fossa, particularly with bleed- Bilateral: Face is symmetrical with micrognathia.
ing in the joint. There is bird face appearance (Fig. 27.4). No glid-
Malunion of condylar fractures: Injuries may be ing movement. With bilateral ankylosis, neither
associated with fracture of the malar-zygomatic protrusive nor lateral movements are possible. An
compound. In the case of injury to the joint, there attempt at forced opening in bony ankylosis, there
is hemorrhage within and outside the joint is no pain but in case of fibrous ankylosis, there is
capsule. If Injury occurs during the period of pain.
active bone formation, it may result into haem- Due to long-standing ankylosis, atrophy or
arthrosis, immobility of jaw movements which fibrosis of muscles of mastication may result. In
subsequently leads to ankylosis. case of congenital ankylosis, there is difficulty
of introducing the nipple into the mouth of new-
Inflammatory: Primary inflammation of the joint.
born infants. Class II malocclusion, protrusive
Inflammation of the joint secondary to a local
incisors and anterior open bite are also the fea-
inflammatory process (otitis media, osteomyelitis,
tures. It is due to the patient’s attempt at forcing
etc). Inflammation of the joint secondary to a blood
food through the anterior teeth for a period of years.
stream infection (septicemia, scarlet fever,
gonorrhea). Rheumatoid arthritis is the co- Histopathological Features
mmonest cause of bilateral ankylosis. Gonococcal
arthritis can also cause ankylosis of TMJ. Atrophic or destructive changes in the carti-
Inflammation secondary to radiation therapy. lagenous component of the joint with loss of
meniscus are a constant finding. There is pro-
Others: Loss of tissue with scarring and metas- gressive destruction of the joint tissue with
tatic malignancy. narrowing of the joint space. Normal soft tissues
Clinical Features are replaced by thick fibrous bands, which blend
in with the periarticular tissue. An overall flatte-
General: It is seen primarily in a young age or ning of articulation, with glenoid fossa and arti-
between 1 to 10 years. Pain and trismus is present cular eminence becoming less pronounced and
which is directly related to the duration of condylar process become enlarged. The enlarged
ankylosis. Depending upon the duration, there
condyle is composed of dense sclerotic bone.
may poor oral hygiene, carious teeth and perio-
Apparent union of injured parts is by gra-
dontal problems malocclusion.
nulation tissue → it is replaced by dense fibrous
Unilateral: Unilateral ankylosis is more common connective tissue → formation of fibrous car-
than bilateral ankylosis. Mouth opening is tilage → metaplasia of fibrous cartilage → direct
impossible, but the patient may be able to pro- transformation into bone → ankylosis.
546 Textbook of Oral Pathology

Figure 27.1: Intraoral view of patient who is having Figure 27.3: Unilateral ankylosis showing deviation of
ankylosis showing trismus mandible on affected side

Figure 27.2: Extraoral view of unilateral ankylosis showing Figure 27.4: Bilateral ankylosis showing bird face
fullness on affected side appearance

Management psychiatric problem, use of phenothiazine deri-

Brisement force, condylectomy, gap arthroplasty vatives.
is performed.
Clinical Features
Subluxation (Hypermobility) It may be unilateral or bilateral.
It is the unilateral or bilateral positioning of the Symptoms: Cracking noise, temporary locking of
condyle anterior to the articular eminence, with
the condyle and immobilization of the jaw.
repositioning to normal accomplished physio-
Patient describes weakness of the joint while yaw-
logic activity. It is self- reducing incomplete dis-
ning. Pain is associated with last few millimeters
location, which generally follows stretching of
of mouth opening.
the capsule and ligaments.
Signs: The condyle may get locked when the
Etiology mouth is opened widely and upon closing, it will
It is caused by long continuous opening of return with a jumping motion, accompanied by
mouth, oral surgical procedures, osteoarthritis, a sound caused by movement of the condyle over
 Temporomandibular Joint Pathology 547

the articular eminence. On palpation ‘click’ on dency. Sudden excruciating pain in the TMJ,
opening and sliding of condyle over the articular followed by rapidly developing swelling.
eminence, are common. Tenderness of the affected area with limitation
of movements occurs. Deviation to the affected
Histopathological Features side while opening the jaws.
Due to long-standing opening of the mouth, the
ligament and capsule are stretched. As the Histopathological Features
ligament does not contain elastic fibers, once it Gross examination of the articular cartilage reveals
is stretched beyond its capacity, it does not come white chalky deposits. This deposit is also often
back to its normal length. The lax capsule and found in the synovial and adjacent tissues. The
over stretched ligament are responsible for cartilage may be fragmented.
subluxation. Early findings in gout are crystalline depos-
its of uric acid which are diffuse in nature. This
Gout is followed by small accumulations (microtophi)
It is a chronic metabolic disorder characterized with surrounding granulomatous foreign body
by acute exacerbations of joint pain and swelling reaction, which contains pleomorphic, nonnucle-
associated with an elevated blood uric acid and ated and foreign body giant cells.
deposition of crystals of monosodium urate.
Management
Predisposing Factors Diet should be low in uric acid and fat, i.e. sweet-
Drugs such as thiazide diuretics, operations, bread, meat, extract peas, beans. Increased elimi-
trauma, alcohol and rapid weight loss can lead nation of uric acid by uricosuric agents like
to gout. colchicine 0.5 mg every 2 hourly, to a maximums
of 6 mg in 24 hours.
Clinical Features
Synovial Chondromatosis
Acute gouty arthritis: Initially, metacarpopha-
It is a benign chronic progressive metaplasia that
lyngeal joints are commonly involved. Later foot,
will not resolve spontaneously. Although it is
ankles, hand, wrist and elbow may be affected. nonneoplastic, it may resemble a malignant
There is excruciating pain, which is worse at
condition histologically.
night. Sometimes, it is associated with anorexia,
fever and general malaise. Joint returns to nor-
Pathogenesis
mal after few days, with desquamation of the
overlying skin. It denotes the condition whereby a cartilaginous
focus develops within the synovial membrane
Chronic tophaceous gout: As the disease becomes
of the joint. This is generally believed to occur
chronic, pain and stiffness persist, with irregular
through metaplasia of mesenchymal cell rests in
swelling. Tophi are found in cartilage of the ear,
the underlying connective tissue of the mem-
nose or eyelids. In half the cases, palms of hands
brane.
may show white streaks along the creases.
There is formation of metastatic foci.
TMJ involvement: It is seen in middle age with Eventually, they are detached from the affected
equal sex distribution. It has a hereditary ten- membrane and become cartilaginous mobile
548 Textbook of Oral Pathology

bodies within the joint cavity. Many of these Signs: Crepitus, preauricular swelling, enlarged
cartilaginous foci then undergo calcification. joint with effusion and local tenderness.
These joint bodies acquire a perichondrium,
which enables them to grow by proliferation of Histopathological Features
chondrocytes.
Trauma may be a predisposing factor, others There is nodule of loose cartilage in the joint space.
factors are malocclusion, occlusal habits, and There is also presence of inflamed synovial
subluxation or tension sites. membrane. Cartilage is consist of hyperchromatic
cartilage cells.
Clinical Features
Female to male ratio is 3:1 with greatest incidence Management
at 40 to 60 years of age. These bodies, if symptomatic, should be removed.
Symptoms: Facial pain, limitation of motion and Removal of metaplastic foci and synovectomy
deviation towards the affected side. are the preferred treatment
 CHAPTER

28 Diseases of Tongue

The tongue makes up a large part of the oral foramen cecum, from which the thyroid glands
cavity and can be affected in numerous lesions. develop as an endodermal outgrowth. Anterior
The tongue may be affected as a part of oral 2/3rd is formed by fusion of tuberculum impar
disease or as a signs of a systemic disease and two lateral lingual swelling. The posterior
(Table 28.1). The word ‘tongue’ is derived from 1/3rd of the tongue has a more complicated
the Latin word ‘lingua’ and Greek word ‘glossa’. developmental origin. It first exists as a central
It is partly oral (anterior 2/3rd of tongue) and mound called the copula, which is the result of
partly pharyngeal (posterior 1/3rd of tongue). fusion of the 3rd branchial arches.
The endodermally derived mucosa of the 2nd
EMBRYOLOGY OF TONGUE to 4th branchial arches and the copula, provide
The tongue develops from the ventral wall of the covering for the posterior thirds of the tongue.
primitive oropharynx and may be conceived as A V-shaped terminal sulcus, whose apex is the
a mucosal pocket filled with striated muscle. It foramen cecum, signifies the mobile body of the
is derived principally from the primordia of the tongue from its fixed root.
branchial arches in such a way that the body of
ANATOMY OF TONGUE
the tongue comes from the 2nd arch, whereas
the root comes from the 3rd, 4th and possibly Surface
the 5th arch.
During the 4th week of development, paired The tongue is a muscular organ situated in the
lateral thickening of mesenchymal appears on floor of mouth, associated with the function of
the internal aspect of 1st branchial arch, to form deglutition, taste and speech. It lies partly in the
the lingual swelling. Between and behind the mouth (oral part), which compromises the
swelling there appears median eminence called anterior 2/3rd and in the pharynx (pharyngeal
as tuberculum impar. But soon, elevations of the part), which comprises the posterior 1/3rd. Both
ventromedial portion of the 1st arch arise on each the parts are separated by the inverted ‘V’ shaped
side of tubercle and merges with each other. sulcus called the sulcus terminalis.
The tuberculum impar is a transient elevation Tongue has a base, body and a tip. It has two
with its caudal border marked by a blind pit, the surfaces, a dorsal and a ventral surface. The
550 Textbook of Oral Pathology

dorsal surface is divided into an oral and pharyn- ing the foliate papillae. They are more numerous
geal part and the ventral surface is confined to in infants than in adults. With age, they undergo
the oral cavity only. At the apex of sulcus termi- atrophy.
nalis, there is a depression, called the foramen
cecum. Papillae
In the anterior part of the tongue the mucous
There are four types of papillae: circumvallate,
membrane is thin with reduced lamina propria
fungiform, filiform and foliate papillae.
and is closely attached to the underlying mus-
cular tissue. The color of the anterior part of the Circumvallate papillae: They are usually 8 to 12 in
mucous membrane is pink and is marked by a number and are the largest of the papillae. They
variety of papillae that gives the tongue a charac- are situated in a row parallel to and close to the
teristic roughness. sulcus terminalis. Papillae are 1 to 3 mm in dia-
The anterior part of the tongue is divided in meter and are flattened with a circular depre-
to half by the median lingual sulcus. The post- ssion. They are surrounded by a moatlike trough.
erior part also called as pharyngeal part or base The fungiform papillae: They are smaller than the
of the tongue is located posterior to the palato- vallate papillae and are distributed over the
glossal arch. The surface without papillae shows dorsal surface of the tongue, being most
a slightly corrugated appearance, due to the numerous on the anterior part. They are round
underlying lymphoid tissue called the lingual and mushroom shaped and is distinguished
tonsil. from the filiform papillae by their larger size and
The root of the tongue is attached to the epi- bright red color. Their number is about 100/cm2
glottis by a medial fold (the glossoepiglottic fold). on the tip and 50/cm2 in the middle. They carry
Laterally, pharyngo-epiglottic (glossopharyn- taste buds.
geal) folds pass from the sides of the tongue and
pharyngeal wall to the epiglottis. The root of ton- The filiform papillae: These are smallest, but most
gue is attached to the hyoid bone, below and the numerous and are evenly distributed over the
mandible above. dorsum and are often arranged in rows parallel
The ventral surface is smooth and purplish to the sulcus terminalis, except for the tip where
with no papillae. The tongue is connected to the they run transversely. The papillae are conical,
floor of the mouth by a sickle shaped fold of broadest at the base and whitish due to marked
mucous membrane called as lingual frenulum. degree of keratinization. The concentration of
On the ventral surface, lingual veins are often papillae in man is calculated about 500/cm2.
visible as bluish streaks. At the lateral side of the They are more heavily concentrated in center of
vein is a fringed fold of mucous membrane called dorsum of tongue.
as the plica fimbriata or fimbriated fold. The foliate papillae: They are vertical folds of the
Anteriorly, on either side of the frenulum, the mucosa located at the margins of the tongue, just
caruncles opening for the submandibular ducts anteriorly to the palatoglossal arch.
are visible.
Papillae simplices: They are connective tissue
Taste buds: These are peripheral gustatory organs papillae which are similar to the papillae of
which are composed of modified epithelial cells. dermis of skin. They are present beneath the
They are most numerous on the sides of circum- entire tongue surface, including the mucosal
vallate papillae and less on the walls surround- papillae described above.
 Diseases of Tongue 551

Muscles fibers being connected with the body of hyoid

bone. In front, it blends with the fibers of
The muscles of tongue are grouped into two sets:
styloglossus. It shortens the tongue and makes
an extrinsic set and an intrinsic set. The extrinsic
its dorsum convex by pulling the tip of tongue
muscles include genioglossus, hyoglossus,
downwards.
styloglossus and palatoglossus.
Genioglossus: It is the largest and arises from the Transverse muscle: It originates from the median
upper mental spine and spread in a fanlike fibrous septum and runs in horizontal course,
fashion and is inserted into the tongue from its laterally to be inserted into submucous fibrous
tip to the root. It draws the tongue forward and tissue. By their action, intrinsic muscles alter the
acting together this muscle is able to flatten the shape of the tongue making it narrow and
tongue, making a concavity from side to side. elongated.

Hyoglossus: It is a flat, quadrilateral muscle Vertical muscle: It is found at the borders of

arising from the hyoid bone. It runs as thin plate anterior part of tongue. Its fibers extend from
upward, connect with fibers from the stylo- dorsal to ventral surface, mainly near its lateral
glossus and enter the tongue lateral to the genio- borders, but fibers are interspersed through the
glossus. It depresses the tongue. tongue. It makes the tongue broad and flattened.
Styloglossus: It originates from the styloid process,
Arterial Supply
passes downwards and forwards and inserts into
the side of the tongue, connecting with fibers The lingual artery, a branch of the external
from the hyoglossus. The styloglossus draws the carotid, is the main vessel supplying the tongue.
tongue upwards and backwards. Before the artery reaches the posterior edge of
the hyoglossus muscle, it gives off a branch to
Palatoglossus: It originates from the palate and
the hyoid bone area. In its course below the
runs in the palatoglossus arch, continuing into
hyoglossal muscle, it gives off a lingual dorsal
the side and dorsum of the tongue.
artery, which runs steeply upward dividing into
Intrinsic muscles: These are situated inside the many branches supplying the base of tongue and
tongue and constitute a greater part of the organ. posterior part of the dorsum. The root of the
They are divided into the superior longitudinal, tongue is also supplied by the ascending
inferior longitudinal, transverse and the vertical pharyngeal artery and tonsillar artery. At the
muscles. lower border of the anterior part of the
Superior longitudinal: It arise from submucous hyoglossal muscle, the lingual artery gives off
fibrous layer close to the epiglottis and from the the sublingual artery, which supplies the
median fibrous septum. If runs forward to the sublingual region medial to the sublingual gland.
edges of tongue, some its fibers being inserted
into mucous membrane. It shortens the tongue Venous Drainage
and makes the dorsum concave, by the turning Deep lingual vein is the largest and the principal
the tip and side of the tongue upward. vein of the tongue. Deep lingual vein originates
Inferior longitudinal: It is a narrow band lying near the tip of the tongue and runs backward,
close to the inferior surface of tongue, between close to the mucous membrane on the ventral
genioglossus and hyoglossus. It extends from the surface of the tongue. It joins the sublingual vein,
root apex of the tongue, some of its posterior at the posterior border of the hypoglossal muscle
552 Textbook of Oral Pathology

to form the vena comitans of the hypoglossal in the oral cavity, which interrupts the air pass-
nerve, which drains to the facial or internal age through mouth or pharynx thereby produ-
jugular vein. The dorsal lingual vein drains the cing consonants. Certain consonants like c, d, j,
dorsum and sides of the tongue. It joins the i, n, t, z, l, g, etc. requires movement of tongue.
sublingual veins, which follow the artery deep
Mastication: The tongue has a direct crushing
to the hypoglossal muscle and enter the internal effect on food by pressing it against the hard
jugular vein, near the hyoid bone.
palate. The tongue pushes the food onto the
occluding surfaces and helps to mix in the saliva.
Nerve Supply The sensory ending on the tongue enable to select
All the muscles of the tongue, except the those parts of the food mass that are sufficiently
palatoglossus are supplied by the hypoglossal well masticated to be ready for swallowing.
nerve. The palatoglossus is supplied by the Deglutition: When the food bolus is placed on
pharyngeal plexus. Lingual branch of dorsum of tongue, it is pressed lightly against
mandibular nerve is nerve for general sensation the hard palate just behind the incisors. It is a
for anterior 2/3rd of tongue. Glossopharyngeal coordinated muscular activity involving the
nerve is the nerve for general sensation for tongue and constrictor muscle of the pharynx,
posterior 1/3rd of the tongue. Posterior most part to close the palatal velum and the epiglottis. It
of tongue is supplied by vagus nerve, through allows the passage of the bolus into the eso-
internal laryngeal nerve. Taste sensation is phagus, without regurgitation into the nose or
carried out by chorda tympani branch of facial lower respiratory tract. The process is initiated
nerve for anterior 2/3rd and glossopharyngeal by the voluntary action of collecting food onto
nerve for posterior 1/3rd. the tongue and propelling it backwards into the
pharynx. The muscles involved in this process
Lymphatic Drainage are the mylohyoid and the pharyngeal cons-
The tip of the tongue drains bilaterally into the trictors. Bolus is pushed backwards by raising
submental nodes. Right and left halves of rest of the back of tongue. Food bolus is sucked from
the anterior 2/3rd of the tongue, drain mouth into pharynx, by creating a negative
unilaterally to submandibular lymph nodes. A pressure, while airways are still closed by rapid
few central lymphatic, drains laterally into the relaxation of muscles of tongue and pharynx.
same nodes. Some of the lymph vessels, from Digestion: Tongue has a slight digestive function
the lateral margins of the tongue, drain to the by virtue of salivary lipase, present in serous
jugulodigastric nodes. The posterior 1/3rd of the lingual salivary glands.
tongue drain bilaterally to the juguloomohyoid
Taste: It acts as a special sense organ of taste, by
nodes, in which most of the lymph drains from
virtue of presence of numerous taste buds. The
the tongue.
tip of the tongue is most sensitive to substances
eliciting a sweet sensation. The lateral margins
Functions of Tongue
are most sensitive to substances causing sour
Speech: It is the result of interaction between sensation. The base of the tongue is most sensi-
different organs. Even small changes in the tive to substances eliciting a bitter sensation. The
position or shape of the tongue may cause dis- salty quality is more widespread, but is greatest
turbance in speech. Tongue is one of the organs at the tip.
 Diseases of Tongue 553

Barrier function: Mucosa covering the tongue acts Table 28.1: Classification of Tongue Disorders
as a barrier protecting the deeper tissues from A. Congenital and developmental disorders
• Aglossia and microglossia
mechanical damage. It also prevents entry of
• Macroglossia
microorganisms and toxic substances. • Ankyloglossia
• Cleft tongue
Jaw development: Muscular pressure from the • Ankyloglossum superius syndrome
• Lingual varices
tongue is an important factor in determining the
• Lingual thyroid nodule
shape of the mandibular arch. • Variations in tongue movement
• Patent thyroglossal duct cyst
Thermal regulation: It is more pronounced in dogs, • Tongue thrusting
where there is a considerable loss of heat from • Lingual polyp
• Reactive lymphoid aggregate
the tongue. • Lingual cyst
B. Local tongue disorders
Secretion: Major secretion of tongue is provided • Fissured tongue
by salivary glands activity which maintains the • Median rhomboidal glossitis
• Benign migratory glossitis
moist surface of oral mucosa. • Hairy tongue
• Crenated tongue
Defense mechanism: Secretary immunoglobulin • Foliate papillitis
system of tongue plays an important role in body • Leukokeratosis nicotine glossi
C. Depapillation of tongue
defence. Local disease
• Eosinophilic granuloma
Maintenance of oral hygiene: By virtue of its move- • Traumatic injuries
ment it can reach all parts of the oral cavity remo- • Lesions due to automutilation
• Allergic stomatitis
ving food debris from the gums, vestibule and • Facial hemiatrophy
floor of mouth. Thus it helps in maintenance of • Cranial arteritis
oral hygiene. • Chronic candidiasis
Systemic disease
• Iron deficiency anemia
Sucking: Tongue also plays an important role in • Plummer-Vinson syndrome
sucking in both bottle feeding and breast feeding. • Pernicious anemia
• Niacin deficiency
General sensitivity: Due to extreme sensory inner- • Folic acid deficiency
• Peripheral vascular disease
vations terminating in both simple and organi- • Dermatomyositis
zed nerve endings, there is perception of heat, • Diabetes
cold, pressure and chemical discrimination. • Syphilis
• Zoster infection
• Tuberculosis
Symbolic function: Functions that are traditionally D. Neurological disease
associated with the tongue, but that have no • Glossodynia
anatomic and physiologic basis should be men- • Dyskinesia
• Paralysis
tioned because images of this type are well estab- • Oropharyngeal dysphagia
lished cultural and literary tradition. It must fre- E. Cyst
quently influences a patient perception of a lin- • Anterior median lingual cyst
• Bronchogenic cyst
gual abnormality. Expressions such as ‘speaking • Epidermoid and dermoid cyst
with a forked tongue’ or ‘speaking in different • Gastric mucosal cyst
• Parasitic cyst
tongue’ all describe the mental attitude and • Thyroglossal duct cyst
behaviors, by which they are expressed. Contd...
554 Textbook of Oral Pathology

Contd... intubation can be carried out to prevent airway

F. Benign tumor obstruction.
• Fibroma
• Glomus tumor
• Granular cell tumor
Macroglossia
• Leiomyoma
Macroglossia is tongue enlargement, which leads
• Rhabdomyoma
• Plasmacytoma to functional and cosmetic problems. Although
F. Premalignant lesion and conditions this is a relatively uncommon disorder, it may
• Leukoplakia
• Lichen planus
cause significant morbidity. Normal speech and
• Oral submucous fibrosis swallowing reflexes require normal tongue
G. Malignant tumor anatomy and function. Swallowing begins as the
• Squamous cell carcinoma
• Malignant lymphoma
tongue mixes food with saliva to form a food
• Malignant melanoma bolus, which is then propelled into the pharynx
• Metastatic tumor by the tongue. Articulation also depends on the
• Sarcoma
H. Miscellaneous tongue's ability to alter the impedance of air and
• Pigmentation of tongue change the resonant characteristics of the upper
• Phlebectasia airway. In macroglossia, increased tongue bulk
may impair these functions.
Aglossia and Microglossia
Aglossia: It is the complete absent of tongue at birth. Classification
Microglossia: It is the presence of small rudimen- • Congenital
tary tongue. • Acquired
– Hypertrophic: In it, muscles of the tongue
Causes are hypertrophic. It usually occurs in men-
It usually occurs in syndromes such as hypo- tally retarded patients.
glossia-hypodactylia syndrome, Pierre Robin – Inflammatory: It may involve the tongue
syndrome. It is also associated with cleft lip and partially or completely. It is due to various
palate. causes like syphilitic, Ludwig’s angina, etc.
– Neoplastic: It can be based on benign and
Clinical Features malignant tumors.
– Relative macroglossia: It is a condition, in
Symptoms: Patient encounters difficulty in eating
which a normal sized tongue appears ab-
and speaking.
normally large, if it is particularly enclosed
Signs: Patient may have high arched palate and within a small oral cavity.
a narrow constricted mandible. It is usually – Apparent macroglossia: It is a condition
associated with other oral or generalized malfor- where the tongue appears large due to poor
mations. There may be an airway obstruction, muscular control of the tongue, although
due to negative pressure generated by degluti- there is no increase in the bulk of tongue
tion and inspiration. tissue.

Management Etiology
Nonsurgical technique such as positioning, naso- • Congenital: It includes hemangioma,
gastric intubation and temporary endotracheal lymphangioma and lingual thyroid.
 Diseases of Tongue 555

• Inflammatory: Inflammatory causes include It is associated with syndromes like Beck-

tuberculosis, actinomycosis, dental infection, with’s hypoglycemic syndrome which includes
syphilitic gumma, Riga disease, ranula and neonatal hypoglycemia, mild microcephaly, um-
sublingual calculus. bilical hernia, fetal visceromegaly and postnatal
• Traumatic: Traumatic causes include dental somatic gigantism.
irritation, hematoma and postoperative
edema. Histopathological Features
• Neoplastic: The neoplastic causes can be divi- It shows hypertrophy and hyperplasia of muscle
ded into malignant and benign lesions; with cells.
the malignant lesions including carcinoma
and sarcoma. The benign lesions include gra- Management
nular cell tumor, neurofibroma, leiomyoma Orofacial therapy uses a palatal device to sti-
and lipoma. mulate muscular tone and proper tongue posi-
• Metabolic: Metabolic causes are myxodema, tion. Majority of the cases of macroglossia are
amylodosis, lipoid proteinosis, chronic ste- treated surgically. Indications for surgery include
roid therapy and acromegaly. airway obstruction, speech difficulties, dys-
• Muscular hypertrophy: Over development of phagia and cosmetics. The procedure of choice
musculature, this may or may not be associa- is partial glossectomy. Surgical goal is to reduce
ted with generalized muscular hypertrophy the tongue size and thus improve the condition.
or hemi-hypertrophy. Removal of primary cause and correction of
the dental arch deformity and malocclusion by
Clinical Features
orthodontic treatment is done. Correction of
Macroglossia is most prominent in infants, but defective articulation by speech therapy is
tongue size may remain above normal in child- carried out.
hood and adolescence. As hyoid descends with
age, macroglossia may improve. Macroglossia Ankyloglossia
causes a variety of signs and symptoms. It is also called as ‘tongue-tie’. It is a condition in
Symptoms: This includes tongue protrusion, which the lingual frenulum is either too short or
which exposes the tongue to trauma. This expo- anteriorly placed limiting the mobility of the
sure also leads to mucosal drying and recurrent tongue. Reports of partial ankyloglossia affecting
upper respiratory tract infections. Other symp- several generations, suggest a possible genetic
toms include swallowing difficulties, airway basis for the minor variation in the attachment
obstruction, drooling and failure to thrive. The of the genioglossus muscle. It may be traumatic
enlargement is generalized and may cause or congenital.
variety of difficulties with speech, feeding and
airway problems. Types
Signs: It may produce displacement of teeth and • Complete: Fusion of tongue and the floor of
malocclusion, due to the strength of muscles mouth.
involved and pressure exerted by the tongue on • Partial: Short lingual frenum.
teeth. Crenation or scalloping of the lateral
borders of the tongue; the tips of scalloping fit Clinical Features
into the interproximal spaces between the teeth Symptoms: It may limit the movement of the
occurs. tongue. In extreme cases of ankyloglossia, nur-
556 Textbook of Oral Pathology

sing and feeding problems can occur. Recurrent Cleft Tongue

tongue biting, poor sucking and inability to chew It is also called as bifid tongue.
some food. It was felt that tongue-tie was asso- It is the condition in which there is cleavage
ciated with speech abnormalities, especially lis- of the tongue due to lack of fusion of the lateral
ping and inability to pronounce certain sounds halves. It results due to incomplete merging and
and words, viz. t, d, n, l, as, ta, te, time, etc. failure of groove obliteration by underlying
Signs: When there is an attempt to stick the ton- mesenchymal proliferation. Completely bifid or
gue out, there may be a V shaped notch at the cleft tongue is rare. It is due to lack of merging
tip. Physical examination will easily demonstrate of lateral swellings of the organ. Partially cleft
the short or anteriorly placed lingual frenulum. tongue is manifested as deep grooves in the
Patients have midline mandibular diastema and midline of dorsal surface. Food debris and
inability to clean the teeth and lick the lips with microorganisms may collect in the base of cleft
tongue. and cause irritation.
Syndromes associated are ankyloglossum Syndromes associated: Seen with oral-facial-digital
superious syndrome, Rainbow’ syndrome, Fra- syndrome, with thick fibrous bands in lower
ser’s syndrome and orofacial digital syndrome. anterior mucobuccal fold, which eliminate the
sulcus and is associated with clefting of
Management hypoplastic mandibular alveolar process.

Physician education, parental education and Ankyloglossum Superius Syndrome

reassurance should be given to the patient. It is characterized by the attachment of the
Surgery—indication for surgery, i.e. frenectomy are tongue to the hard palate and by limb malfor-
as follows: mation.
• If complete fusion of tongue is present then
it requires surgery. Etiology
• When nursing and feeding become a prob- A genetic disorder is the most important cause
lem, surgery is indicated. of this syndrome. Intrauterine environmental
• Children between 2 to 4 years, with poor factors can also cause this syndrome.
development of speech and anxious parents
desire for the necessary treatment. Clinical Features
• In cases where tongue tie has recurred after It is a very rare disorder and is associated with
snipping. jejunal and ileal atresia. Patient has difficulty in
speech and mastication. Patient may have diffi-
Complications of Surgery culty in swallowing.
• Injudicious cutting of the frenum in a new-
born can cause hemorrhage and the tongue Management
may become too mobile and may be swallo- Surgical separation of the tongue from the palate.
wed, causing asphyxia.
• There is also possibility of a subsequent infec- Lingual Varicosities
tion at the base of the tongue, with formation A varix is a dilated, tortuous vessel, most comm-
of large ulcer and spreading stomatitis. only a vein, which is subjected to increased
 Diseases of Tongue 557

hydrostatic pressure and is poorly supported by Clinical Features

the surrounding tissues. Varicosities involving Females are more affected as compared to males
the lingual veins are relatively common, and are in a ratio of 4:1 due to hormonal influence. The
seen on ventral or lateral surface of tongue. These age of onset ranges from birth to the 6th decade,
may occur due to loss tonicity of supporting with a peak in the 2nd decade. There is no racial
tissue. and geographical distribution.
Clinical Features Signs: It is manifested as a nodular mass in or
It is seen after the age of 50 years. near the base of the tongue, in general vicinity
of the foramen caecum. It is often but not always,
Signs: Patient is symptom free with lesion appea-
in the midline.
ring as red or purple shotlike clusters of vessels
on the ventral surface and lateral borders of the Symptoms: There may be complains of dyspha-
tongue as well as in the floor of the mouth. There gia, dysphonia, dyspnea, hemorrhage with pain
appears to be no significant relationship between or feeling of tightness or fullness in the throat.
the presence of leg varicosities and sublingual
varices. Histopathological Features
They resemble either normal thyroid tissue or
Histopathological Features thyroid tissue of an embryonal or fetal type. It
Vessels are dilated with presence of smooth characteristically has an incomplete or poorly
muscle and elastic fibers. Secondary thrombosis defined capsule. Follicular cells sometime atro-
may lead to occluded lumen with platelets and phic in nature.
erythrocytes.
Management
Lingual Thyroid Nodule Thyroxin should be given to reduce the size of
The thyroid gland develops in the embryo from swelling. When it causing difficulty to the pati-
the ventral floor of the pharynx, by means of an ent, inspite of thyroxin therapy, excision or abla-
ectodermal invagination of diverticulum. The tion with radioiodine is indicated.
tongue forms at the same time from this pharyn-
geal floor and is anatomically associated with Variations in Tongue Movement
thyroid gland by connection through the thyro- Ability to curl up the lateral borders of the tongue
glossal tract, the lingual remnant of which is into a tube is noted in 65% of Caucasians and is
known as the foramen caecum. inherited as an autosomal dominant trait. It is
The lingual thyroid is an anomalous condition the ability to fold back the tip of the extended
in which follicles of thyroid tissue are found in tongue, without the aid of the teeth.
the substance of the tongue, possibly arising from
Trefoil tongue: Clover leaf pattern.
a thyroid anlage that failed to migrate to its pre-
destined position or from anlage remnants that Gorlin sign: Extensibility of the tongue, both,
became detached and were left behind. forward to touch the tip of nose and backwards
into the pharynx.
Etiology
Functional insufficiency of the chief thyroid Tongue Thrusting
gland in neck and failure of the primitive thyroid Positioning of tongue between the anterior teeth
anlage to descend are the cause of the condition. during swallowing, speaking or at rest. It is an
558 Textbook of Oral Pathology

infantile swallowing pattern. It may be associa- Reactive Lymphoid Aggregate

ted with macroglossia. In these cases, anterior The lingual tonsils, one of the largest oral lym-
open bite is present. phoid aggregates, are located on the posterior
portion of the tongue on the dorsolateral aspect.
Patent Thyroglossal Duct Cyst
They are reported to occur on the gingiva, buccal
Development mucosa, tongue and floor of mouth.

Thyroid gland develops from an anlage of Histopathological features: It is typically surroun-

endothelial cells in the midline of the floor of ded by a crypt lined by stratified squamous
pharynx, between the first and second branchial epithelium. Hyperplastic lymphoid polyps have
arches, just posterior to tubercular impair. These also been described as polypoid structures
cells sink into the base of developing tongue, composed entirely of lymphoid tissue.
descend into the neck and proliferate below the
larynx to form thyroid gland. Remnants of this Lingual Cyst
part are referred to as thyroglossal duct. Cystic It is a rare lesion, located in the anterior midline
degeneration of it is called as duct cyst. of the tongue. It is movable and compressible
(Fig. 28.1).
Clinical Features It arises as a result of epithelial entrapment
It usually occurs in young persons with no sex during fissural closure of the lateral lingual
predilection. It is seen above the thyroid, in vici- processes.
nity of the hyoid bone, in midline of the neck. In Histopathological features: It is lined by aberrant
some cases dysphagia may occur.
gastric epithelium and is referred to as gastric
Sign: It is a firm cystic mass in which formation cysts.
of fistula may take place. It is compressible. It Management: Surgical excision is recommended.
yields yellow fluid on aspiration.

Histopathological Features
The cyst is lined by columnar, respiratory or stra-
tified squamous epithelium. Follicle of thyroid
is frequently located in juxtaposition to the cyst
lining.

Management
It should be surgically excised or enucleated.

Lingual Polyp
It can occur at any age, with no sex predilection.
It is a circumscribed, sessile or pedunculated
lesion. It may sometimes cause asphyxia in neo-
nates.
Management: It consists of surgical removal. Figure 28.1: Lingual cyst of newborn seen as growth
 Diseases of Tongue 559

Fissured Tongue nulomatosa, facial paralysis, fissured tongue and

non-pitting, non-inflammatory painless edema
It is also called as ‘scrotal tongue’, ‘plicated ton-
of face.
gue’ and ‘lingua dissecta’.
Histopathological Features
Definitions
It shows hyperplasia of the rete pegs and loss of
A tongue with or without a central fissure shows
keratin on the surface of filiform papillae. Pap-
parallel fissures lateral to the midline or fissures
illae are separated by grooves. There is formation
at right angle to the long axis of the tongue. A
of microabscess in the upper epithelial layer as
tongue being characterized by furrows, one
polymorphonuclear leukocytes are migrating
extending anteroposteriorly and others laterally
into the epithelial.
over the entire anterior surface.
Management
Types
Clean the debris with brush.
• Foliaceus
• Cerebriform
Median Rhomboid Glossitis
• Plicated.
It is also called as ‘central papillary atrophy of
Etiology tongue’. It is a developmental defect resulting
from an incomplete desent of tuberculum impar
It is genetically determined and seen in mentally
and entrapment of a portion between fusing late-
retarded and psychotic individuals. Extrinsic
ral halves of the tongue.
factors like chronic trauma or vitamin deficiency.
Definition
Clinical Features
It is a benign lesion of the tongue, characterized
Well-marked fissuring increases with age, as
by rhomboid or oval shaped changes of the
does the number, width and depth of the fissures
tongue mucosa in the midline, just anterior to
in affected individuals. It is manifested as small
the foramen caecum.
furrows or grooves on the dorsal surface, often
radiating out from the central groove along the
Pathogenesis
midline of the tongue.
It is usually painless, except in some occasio- It is a congenital abnormality of the tongue which
nal cases in which food debris tends to collect in is due to failure of tuberculum impar to retract
the groove and produce irritation. or withdraw before fusion of lateral halves of
the tongue, so that a structure devoid of papilla
Pattern: Six different patterns of tongue fissuring
is interposed between it.
were observed, i.e. plication, central longitudinal
fissuring, double fissures, transverse fissuring Etiology
arising from a central fissure, transverse fissuring
The persistent tuberculum impar suggests its
with a central fissure and lateral longitudinal
developmental origin. Fungal infection with
fissuring.
candida albicans can be causative factors.
Syndrome: It is associated with Melkerson- Median rhomboid glossitis is more common
Rosenthal syndrome which includes cheilitis gra- in a diabetic, than in non-diabetic subjects.
560 Textbook of Oral Pathology

Clinical Features Benign Migratory Glossitis

Males predominate over females (3:1). It is common It is also called as ‘geographic tongue’, ‘wande-
in adults with age ranging from 15 to 84 years. It ring rash’, ‘glossitis areata exfoliativa’ and ‘ery-
is located just anterior to the foramen caecum on thema migrans’.
dorsal surface of the tongue, in midline and
anterior to the circumvallate papillae.
Definition
Signs: It appears as an ovoid, diamond or rhom-
boidal shaped reddish patch or plaque on the It refers to irregularly shaped reddish areas of
dorsal surface of the tongue, immediately anterior depapillation and thinning of dorsal tongue
to the circumvallate papillae. In some cases flat epithelium that is surrounded by a narrow zone
or slightly raised area stands out from rest of the of regenerating papillae that which are whiter
tongue because it has no filiform papillae. than the surrounding tongue surface.
The surface is dusky red and completely de-
void of filiform papillae and usually smooth. The Etiology
texture may be varied from a reddish, smooth,
Immunological reaction, allergic, emotional
granular surface to a lobulated and indurated
stress, hereditary factors, infections and nutri-
surface.
tional deficiencies can be etiological factors.
Symptoms: It is generally asymptomatic, but pain In patients with geographic tongue, there is
and ulceration are reported. Rest of the tongue a high frequency of history of asthma, eczema
may be coated or matted. and hay fever.

Histopathological Features
Classification
There is loss of papillae with varying degrees of
hyperparakeratosis. There is proliferation of spi- • Type I: Lesion confined to the tongue, with
nous layer with elongation of rete pegs, which both active and remission phases. No other
may branch and anastomose. Lymphocytic infil- lesion elsewhere in the oral cavity.
tration within connective tissue and numerous • Type II: As type one with similar lesions else-
blood vessels are seen. Connective tissue also where in the mouth.
shows increased vascularity and chronic • Type III: Lesions on the tongue that are not
inflammatory infiltrate. typical and that may be accompanied by lesi-
Degeneration and hyaline formation, within ons elsewhere in the mouth. It consists of two
underlying muscles is seen. When present fungal forms:
hyphae are usually found in the parakeratin or in • Fixed form: A few areas of the tongue are affec-
the very superficial spinous layer of epithelium ted, but no movement is observed. They may
or in both. Epithelium is devoid of filiform pap- disappear only to recur at the same area.
illae and is slightly thickened. There is also elon- • Abortive forms: This form starts as yellow-
gation and branching of rete pegs. white patches, but disappear before acqui-
ring the typical appearance of geographic
Management tongue.
If candidal organism is found, it is treated with • Type IV: No tongue lesions are present, but
an antifungal agent. Only in long-standing cases, geographic areas present elsewhere in the
cryosurgery or an excisional biopsy is indicated. mouth.
 Diseases of Tongue 561

Clinical Features Condition may persist for weeks or months

and then regress spontaneously only to recur at
It is common in young and middle aged adults,
a later date.
with an age range of 5 to 84 years with a predilec-
tion for females. Ectopic geographic tongue: Lesion is not always res-
Lesion confines to dorsal surface and lateral tricted to tongue and similar irregular or circinate
border of the tongue, but may occur on the ven- lesions occur elsewhere in oral cavity and are
tral surface. It is extremely variable in size and called as ectopic geographic tongue or erythema
diameter and it may be single or multiple. circinate migrans or annulus migrans.
Symptoms: It is asymptomatic, but the patient
may complain of burning sensation that is made Histopathological Features
worse by spicy or citrous food.
Filiform papillae are lost and at the margins of
Signs: Initially appears as a small erythematous, the lesion, there is usually hyperparakeratosis
non- indurated, atrophic lesion, bordered by a and some acanthosis. Parakeratin is desqua-
slightly elevated distinct rim that varies from mated with marked migration of polymor-
gray to white to light yellow. Loss of filiform phonuclear leukocytes and lymphocytes into
papillae produces pink to red smooth shiny epithelium. This produce degeneration of epi-
surface except the residual fungiform papillae
thelial cells and microabscess formation, called
(Fig. 28.2).
as Munro’s abscess, near the surface. There is also
Multiple areas of desquamation of filiform
an inflammatory cell infiltration in the under-
papillae, in an irregular circinate fashion are
lying connective tissue, chiefly neutrophils, lym-
seen. Central portion or lesion, sometimes appe-
phocytes and plasma cells. Due to neutrophil
ars inflamed, while the border is outlined by thin
infiltrate there is destruction epithelium which
yellowish white line or band. Fungiform papillae
produces atrophic mucosa.
persist in the desquamated areas as small
elevated red rods. Area of desquamation remains
for a short time in one location and then heals Management
and appears in another location thus giving rise Topical local anesthetic agents like lidocaine,
to the term ‘migration’. dyclonine hydrochloride or diphenhydramine
can be given.
Bland diet, elimination of irritants and psy-
chological reassurance is useful.
Topical corticosteroids and topical applica-
tion of salicylic acid and tretinoin (retinoic acid
or vitamin A) for external use.

Hairy Tongue

It is also called as ‘lingua villosa’. It designates

an overgrowth of the filiform papillae on the dor-
Figure 28.2: Geographic tongue presented as sum of the tongue, giving the tongue a superficial
erythematous area resemblance as that of hairiness.
562 Textbook of Oral Pathology

Etiology Earthy or encrusted tongue: Extreme degree of

coating occurring in dehydrated, debilitated and
There may be delayed shedding of the horny layer
terminally ill patient can lead to a very thick,
of the filiform papillae or there may be an increase
leathery coating on the tongue which is referred
in the rate of formation of keratin. Fungal
to as ‘earthy’ or ‘encrusted’ tongue. It is heavily
organisms like candida albicans and systemic
coated with bacteria and fungi and forms a
disturbances (anemia, gastric upset). Oral use of
thickened malted layer.
certain drugs (sodium perborate, sodium peroxide
Color may be varied from yellowish white
and antibiotics like penicillin and aureomycin).
to brown or even black depending upon their
Extensive X-ray radiation around head and
staining by extrinsic factors such as tobacco,
neck for the treatment of tumors. It also occurs
certain foods, medicines or chromogenic orga-
in patient with inter-maxillary fixation and with
nisms of the oral cavity.
disturbed orophysiology, due to recent surgery
in the oral cavity. A lowered pH of oral secretion, Histopathological Features
which blocks the normal desquamation of
The filiform papillae are extremely elongated and
epithelial cells covering the filiform papillae.
hyperplastic with keratosis. External colonization
Debilitating diseases in which the tongue
movement is limited by some illness. Various of the papillae by basophilic microbial colonies
is a prominent feature.
foods, particularly coffee and tea, probably con-
tribute to the diffuse coloration.
Management
Clinical Features Brushing of the tongue twice daily for two minu-
The lesion involves the dorsum, particularly the tes, making gentle movements over the involved
middle and posterior one-third. In heavily area towards the tip of the tongue. The topical
keratinized surface layers of filiform papillae, application of podophyllum in acetone and alco-
continuous desquamation through friction of hol suspension seems to be quite effective. Appli-
tongue with food, palate and upper anterior teeth cation of topical keratolytic agents and prescrip-
occurs and is replaced by new epithelial cells. tion of yogurt or other Lactobacillus acidophilus
cultures have been used in the treatment of this
Symptoms: Papillae which are of considerable disease.
length will occasionally brush the palate and
may produce gagging. There is hypertrophy of Crenated Tongue
filiform papillae. The papillae may reach a length
Definition
of 2 cm.
Signs: The papillae are elongated, sometimes The term is applied to a condition in which
markedly so and have the appearance of hair. indentations of teeth are observed at the lateral
The hyperplastic papillae then become pigmen- margins of the tongue.
ted by the colonization of chromogenic bacteria,
Etiology
which can impart a variety of colors ranging
from green to brown to black. This gives it a coa- It may occur due to abnormal tongue pressure
ted or hairy appearance and retains debris and habits and tongue thrusting habits. Any enlarge-
pigments from substances such as food, tobacco, ment of the tongue may cause indentations on
smoke and candy. the teeth.
 Diseases of Tongue 563

Clinical Features Clinical Features

Often, impression of teeth is seen on the tongue. It As a result of heavy smoking, there is loss of
is usually an asymptomatic and harmless con- papillae. No other clinical features are found in
dition. these patients.

Foliate Papillitis Depapillation of the Tongue

One foliate papilla is present in newborn at each Local Disease
side of the tongue, consisting of 4 to 8 leaves and
located at the junction of the anterior two-thirds Eosinophilic granuloma: It is not related with
of the tongue and the base. The folds are separated eosinophilic granuloma of bone. It is also called
by grooves of different depths, perpendicular to as ‘ulcerated granuloma eosinophilicum diuti-
the longitudinal axis of the tongue. In adults, they num’, ‘traumatic granuloma’ or ‘reparative
are barely noticeable, but sometimes they swell. lesion’. The cause of the lesion is unknown. It is
Foliate papillae frequently become inflamed characterized by a well demarcated proliferative
and enlarged, so that it is clinically evident. Such ulceration covered by thick masses of fibrin and
enlargement is usually bilateral. This reactive detritus. It may occur at any age and does not
lingual tonsil has often been called as “foliate show a sex predilection. It is located on the dor-
papillitis”. Hypertrophy of lymphoid tissue may sum, margin or inferior surface of the tongue. Some
be followed by secondary traumatization resul- of the cases are also found on labial mucosa, floor
ting in a so called foliate papillitis. of the mouth, alveolar ridge and gingiva. The
lesions are ulcerative, not indurated and rather
Clinical Features well circumscribed. There is putrid odor. The
ulceration is probably due to moist environment
Symptoms may be partly due to upper respira-
and frequent traumatization. It can be confused
tory tract infection and partly due to irritation.
with squamous cell carcinoma.
It is more common in women, usually in the
Histopathologically it, will show masses of
second half of life. Soreness, tenderness, pain and
eosinophilic granulocytes as well as neutrophils,
occasionally, a sour or metallic taste. The condi-
plasma cells and histiocytes. The presence of mast
tion may be bilateral with a duration varying
cells also has been reported. Proliferation of
from few weeks to many years.
capillaries and myoblasts are other common
Management findings. When one is dealing with an eosino-
philic granuloma, spontaneous healing can be
It consists of elimination of irritating factors such
expected in a matter of weeks.
as sharp edges of teeth and dentures. If necess-
ary, surgical removal may be performed. Traumatic injuries: The tongue may be repeatedly
traumatized, either mechanically or chemically.
Leukokeratosis Nicotina Glossi It is associated with jagged teeth, rough margins
It is also called as ‘smoker’s tongue’. of restorations and inadvertent contact of tongue
with dental medicaments such as phenol and
Definition eugenol. Localized area of depapillation is often
It is homogenous, like leukoplakia with evenly noted with papillary regeneration around such
distributed, pinpoint, hemispherical depressions, areas. A sharp edge of the tooth may cause a
showing a so called golf ball appearance. yellowish, not indurated and well circumscribed
564 Textbook of Oral Pathology

ulcer at the borders of the tongue. Severe damage dal glossitis. It is diagnosed by scraping and cyto-
of the tongue may occur during epileptic seizures. logical examination.
Prolonged oral intubation may cause a large
permanent cleft of the tongue. Cotton roll ulcers Systemic Conditions Affecting Tongue
are rare, but may occur on the borders of the
Iron deficiency anemia: There is inhibition of
tongue. Such ulcers are not indurated and can be epithelial reproduction, secondary candidiasis
extremely painful.
and chronic xerostomia. There are atrophic chan-
Lesions due to automutilation: Injuries to the tongue ges on the dorsum of the tongue. It first appears
can occur due to self inflicted bites. It usually at the tip and lateral borders with loss of filiform
occurs in mentally handicapped persons. papilla. In extreme cases, the entire dorsum beco-
mes smooth and glazed. The tongue may be very
Allergic stomatitis: It refers to edematous changes
painful and is either pale or fiery red (Fig. 28.3).
in part or all of the oral and lingual mucosa, due
to hypersensitivity reaction. It can occur due to Plummer-Vinson syndrome: Sideropenic anemia
certain drugs like antibiotics, cancer chemo- shares atrophic glossitis, angular cheilitis, gen-
therapeutic agent and anticholinergic agents. It eralized atrophic oral mucosa, oral ulceration and
can also occur due to variety of allergens such as secondary candidiasis. The tongue may be red or
monomer of the denture, mouthwashes, chewing pale, painful and fissured. There is also
gum and lipstick. There is edematous swelling of dysphagia and dystrophy of nails.
the tongue. There is depapillation of the tongue.
Pernicious anemia: The patient suffer from general
Facial hemiatrophy: It is characterized by unilateral weakness, burning or itching sensation from the
atrophy of the skin, subcutaneous tissues and oral mucous membrane with disturbance of taste
muscle of the face. There is atrophy of half of the and occasional dryness of mouth. There may be
tongue. paresthesia, atrophy of filiform and fungiform
papillae. In advanced cases, dorsum of the tongue
Cranial arteritis: Rheumatic polymyalgia and
becomes completely atrophic, smooth and fiery
temporal arteritis is an inflammatory condition
red surface. Tongue appears flabby because the
of large and medium sized arteries in elderly
normal muscle tonus is reduced.
persons. There is no sex predilection. Symptoms
include headache, fever, sweating, malaise,
fatigue, anorexia and weight loss. Blindness is
the most severe complication. Several cases of
painful ulceration and gangrene of the tongue, as
a result of arteritis, have been reported. There is
also lingual pain and intermittent blanching of
the tongue also has been described. Early use of
adequate corticosteroid therapy at the level of 40
to 60 mg daily is required for the treatment of
suspected cranial arteritis.
Chronic candidiasis: Chronic atrophic candidiasis
can be present on the dorsum of the tongue. It is Figure 28.3: Depapillation of tongue seen in
difficult to distinguish it from median rhomboi- nutritional deficiency
 Diseases of Tongue 565

Niacin deficiency: Deficiency of niacin results in a Syphilis: Depapillation of the tongue usually
disease called as ‘pellagra’. The tongue become occurs in secondary and tertiary syphilis. In
fiery red and devoid of papillae. The filiform secondary syphilis mucous patch occur, which
papillae are most sensitive and disappear first. may be single or multiple on the tongue. Tongue
The fungiform papilla may become enlarged. In in tertiary syphilis may show gumma formation.
advanced cases all the papillae are lost and A more diffuse, chronic, non-ulcerating, irregular
reddening become intense. In this tongue may induration, with an asymmetrical pattern of
become so swollen that indentation from teeth are grooves and smooth atrophic field covering the
found along borders of the tongue. entire dorsum is seen. Gumma is often developed
Folic acid deficiency: There is marked glossitis. The in chronic interstitial glossitis. There is atrophy
tongue is fiery red and atrophy filiform and of filiform and fungiform papillae.
fungiform papillae. The tongue is often swollen Zoster infection: It is a viral infection caused by
and small cracks may appear on the dorsum of herpes zoster virus. Numerous vesicles occur on
the tongue. the ventral surface of the tongue.
Peripheral vascular disease: It includes scleroderma Tuberculosis: The most frequent involved area is
and lupus erythematous. Fibrosis of submucosal dorsum of the tongue. There is ulceration with
tissue secondary to the obliteration of small
irregular outline and undermined borders,
vessels by an autoimmune process is responsible
covered by yellowish gray fibrinous layer. There
for a scarred, shrunken and atrophic appearance
is usually pain associated with ulceration.
of the tongue in scleroderma. Isolated irregular
areas of lingual mucosa, atrophy and ulceration Glossodynia
caused by arteritis, are seen in lupus erythematous.
In scleroderma the tongue shrinks, losing its Terminology
mobility and papillary pattern. The color of tongue • Glossalgia: The term glossalgia is used to
changes to a vivid appearance due to circulatory describe painful tongue.
disturbances. In the end stages, the tongue lies as
• Glossopyrosis: The term glossopyrosis is used
a stiff, reduced body in the floor of mouth.
to describe burning sensation in the tongue.
Dermatomyositis: It is a clinical syndrome consis- • Lingual paresthesia or dysesthesia: When just
ting of polymyositis associated with skin lesions. discomfort is felt, it is called as lingual pares-
The oral mucosa may show dark red or bluish thesia or dysesthesia.
erythema. In the early stages, tongue is markedly • Stomatodynia: When the entire oral cavity is
swollen and later becomes harder. In the late involved the terms stomatodynia, stomoto-
phase, tongue is atrophic. pyrosis and oral dysesthesia are used.
Diabetes: Decreased-nutritional status of the
Etiology
lingual papillae, as a result of vascular changes
affecting sub-papillary dorsal capillary plexus Local factors: Oral habits such as excessive use of
supplying it, causes atrophic glossitis. Central tobacco, excessive drinking, frequent uncontrolled
papillary atrophy of the dorsum in which low movements of the tongue or bruxism. Local dental
flat papillae are noticed just anterior to the row of causes such as dentures, irritating clasps or a
circumvallate papillae, is associated with recently fixed bridge. Referred pain from infected
diabetes. teeth or tonsils, Moeller’s glossitis and periodontal
566 Textbook of Oral Pathology

disease. It may be caused by peppermint oil in Management

sweets (since it is a volatile oil) and primary and
Removal of local cause, construction of plastic
secondary sensitizers. Malposed teeth, faulty
retainers. Treatment of muscular problems by
dentures, malocclusion and allergy to dental
correction of malocclusion or by muscle relaxants
material. Geographic tongue, plicated tongue,
such as diazepam. Treatment of the systemic
lichen planus and median rhomboidal glossitis.
cause. Surgical exploration of glossodynia with
TMJ disturbances, electro-galvanic discharge due
neuropathy. Use of topical analgesics 0.5% aq.
to dissimilar metallic restoration. Atherosclerotic
Diphenhydramine alone or mixed with 0.5% of
changes in the lingual arteries have also been a
dyclonine or lidocaine.
cause for glossodynia. Allergy to denture base
material, metallic restoration and particular food,
Dyskinesia
medication, mouthwashes and dentifrices.
It is defined as an impairment of voluntary
Systemic factors: Multiple myeloma, amyloidosis,
motion, causing movements that are incomplete
muscular tension and hypoestrogenism. Defici-
or only partial.
ency states such as pernicious anemia and
pellagra, diabetes, vitamin B deficiency. Gastric
Generalized Neurological Disease
disturbances such as hyperacidity or hypoacidity,
angioneurotic edema, mercurialism. Xerostomia, Huntington’s chorea: It is a hereditary disease
prolonged antibiotic therapy, psychological manifesting progressive degeneration of nerve
problems and hypothyroidism. Glossodynia may elements and characterized by choreiform
occur in diabetic patients either as a result of hyperkinesia including grimacing of face. The
candidiasis or from peripheral neuropathy. speech may be affected early, due to the involun-
tary movements of the tongue.
Neurological factors: Trigeminal neuralgia, damage
of lingual nerve after surgery, glossopharyngeal Gilles de la Tourette’s syndrome: It is characterized
neuralgia. Cerebrosclerosis and subclinical by motor incordination and tics, with echolalia
cerebrovascular accidents can possibly cause and coprolalia. The patient exhibits bizarré facial
pain in the tongue. tics, protrusion of the tongue and the tendency
for lip licking.
Idiopathic: When no local, systemic or neurological
causes can be detected, the term essential or Chorea minor: It is a disease of the childhood,
idiopathic can be used. In these circumstances, characterized by the occurrence of rapid
depression, cancerophobia and neurosis are irregular, aimless involuntary movements of the
regarded as the possible causes. muscles of the extremities, face and trunk. The
tongue is protruded quickly and rapidly with-
Clinical Features drawn to prevent biting of the tongue by invo-
luntary movements of the jaw muscle.
The description varies from patient to patient
some refer it as pain and others as burning,
Amyotrophic Lateral Sclerosis
tingling or numbness in the tongue. It may occur
as an isolated features or it may be one from It is a chronic progressive disease of an unknown
group of oral symptoms. It may occur with clini- cause characterized by atrophy and fasciculations
cally observable changes in tongue or without of the wasted muscle. It involves demyelination
observable changes. of the corticobulbar tracts and degeneration of
 Diseases of Tongue 567

cranial nerve motor nuclei. The muscles of palate, Causes

pharynx and tongue are commonly affected. It may be caused by acute anterior poliomyelitis,
Speech may be slurred eating solid food and infectious polyneuritis, neurofibromatosis,
drinking can cause choking.
syringobulbia, irradiation of head and neck and
Early stage: Hypoglossal nerve involvement compression by a tortuous internal carotid artery
results in flaccidity, symmetric weakness, and in the neck.
slowness of movement and atrophy of tongue.
Clinical Features
Middle stage: In the middle stage a gradual and
generalized weakening of the tongue occurs. This There is paralysis and atrophy of the muscles of
is accompanied by spasticity, which results in one-half of the tongue. The affected tongue
reduced rate, range and force of articulatory deviates towards the paralyzed side when pro-
tongue movements. truded. The movements towards the normal side
are weak or absent. When the tongue lies on the
Late stage: In the late stage, there is virtually unin- floor of the mouth, it may deviate or curl slightly
telligible articulation. toward the healthy side and movements of the
tongue towards the back of the mouth on the
Buccolingual Masticatory Syndrome healthy side are impaired.
If the paralysis is not accompanied by atrophy,
This syndrome consists of repetitive, non-rhythmic
the tongue may appear to bulge slightly and to be
abnormal movements that occur at the speed of
higher and somewhat more voluminous on the
normal voluntary movements.
paralyzed side. When atrophy supervenes,
Spontaneous dyskinesia: It consists of movement of paralyzed side becomes smaller and the tongue
mandible and protrusion of the tongue or the lips. may become curved towards the paralyzed side
with sickle shaped deformities. In some cases,
Tardive Dyskinesia hypoglossal nerve may be affected bilaterally,
causing impairment of the tongue, mobility in
• Vermicular movements: Fine tremors and lateral direction and atrophy of sides of the tongue.
fasciculations of the tongue are described as It can be seen in syndromes like Dejerine
‘vermicular movements’. (anterior bulbar syndrome), Jackson-Mackenzie
• Fly-Catcher’s tongue or bon-bon sign: Rapid (vagoaccessory-hypoglossal syndrome), Collet-
darting movements of the tongue is ‘fly- Sicard, Duchenne, Mobius and Tapia.
catcher’s tongue or ‘bon-bon’ sign.
• Rabbit syndrome: When bon-bon sign is Oropharyngeal Dysphagia
associated with smacking movement of the
lips with constant lip tremor, it is referred as It is dysphagia caused by weakness of the tongue.
‘rabbit syndrome’. Symptoms are aspiration while swallowing,
regurgitation of food into nose, pharyngeal pain
Paralysis on swallowing and inability of the tongue to move
the bolus of food into the pharynx. Dilatation or
It is also called as ‘glossoplegia’. It usually occurs atony of the pyriform sinuses and pharynx with
due to unilateral injury of the nucleus in the retention of contrast media in the valleculae is
medulla or the peripheral hypoglossal nerve. characteristic radiographic findings.
568 Textbook of Oral Pathology

Leukoplakia Etiology
It can occur any where in the oral cavity but tongue Physical trauma, alcohol, tobacco smoke and
is the one of the commonst site. If it occurs on the candidiasis. Syphilis, sepsis, chronic dental
tongue it is called as ‘chronic superficial glossitis’. trauma. Chronic superficial glossitis.
Etiology
Clinical Features
Etiological factors are classically known as 6 ‘S’.
They are smoking, syphilis, sharp tooth, sepsis, Carcinoma of the tongue is disease of middle and
sprits and spices. later decades of life, with mean age at presen-
tation being about 60 years. Males are more
Clinical Features commonly affected than females. The majority
It is confined to the anterior 2/3rd of the tongue. of tongue carcinoma occurs on lateral border of
It gradually spread to the on the dorsum. The anterior 2/3rd of the tongue and undersurface
surface may become fissure and cracked, due to of the tongue. The lesions on the posterior border
contraction of the underlying scarred tissue by of the tongue are usually of higher grade
chronic inflammation. The affected area of the malignancy, metastasize earlier and often have
tongue shows milk-white patches with cracks a poor prognosis. Cancers located in the anterior
and fissures. In course of time atrophy tends to 2/3rd of the tongue are detected in early stages,
succeed hypertrophy, the thickened papillae as compared to those in the posterior 1/3rd of
disappear and the white membrane is worn off. the tongue.
The surface becomes smooth and red. The most common presenting signs of car-
Clinical Staging of Leukoplakia on the Tongue cinoma of tongue is a painless mass or ulcer,
although in most patients the lesion ultimately
• Stage I: Appearance of thin grey transparent
becomes painful, especially when it becomes
film on the affected part of the tongue. The
secondarily infected. Excessive salivation
thin milky film may be wide spread.
gradually appears along with the growth. In late
• Stage II: The thin film turns opaque and
stages, saliva becomes blood stained. As the
white. In the beginning it looks soft, but later
patient is unable to swallow saliva, offensive
on cracks and fissures appear.
• Stage III: Hyperplasia causes small nodules smell in the mouth occurs due to bacterial
and warty outgrowth. Desquamation also stomatitis. There is complained of sore throat and
appears simultaneously which leaves areas of pain in case of lesions on posterior border of the
smooth red and shiny surface. tongue.
• Stage IV: It is this stage of appearance of clini- Patient may complaint of immobility of the
cally detectable carcinoma. The carcino- tongue which occurs due to extensive carcino-
matous changes usually occur with the matous infiltration of the lingual musculature.
fissure. It should be suspected if there is local It becomes worse when floor of mouth is invol-
thickening, bleeding and pain. ved and ultimately, it causes difficulty in speech.
Hoarseness of voice and dysphagia is present
Squamous Cell Carcinoma when the carcinoma involves posterior 3rd with
It is the most common oral carcinoma with 60% involvement of pharynx and larynx.
cases arising from the anterior 2/3rd of the tongue Carcinoma of the tongue may be seen in four
and remainder from the base. varieties.
 Diseases of Tongue 569

• Ulcerative variety: Is usually seen near the edge • Posterior 3rd: It drains into jugulodigastric
of the tongue. The ulcer looks irregular and group of the upper deep cervical nodes on both
the edges are raised and everted. The floor is sides of the neck.
covered by yellowish grey slough. Base is • Blood: It is rare and extremely late in occu-
indurated. rrence. It is only seen when the growth is in
• Warty growth: It usually possesses a broad and extreme posterior part of the tongue.
indurated base. It is developed on excess pro-
liferating growth of filiform papillae. Rarely, Management
does it take cauliflower type look.
Surgery: If the growth is less than 1 cm in dia-
• An indurated plaque or mass: In this case a typi-
meter, it should be removed along with a wide
cal indurated submucous plaque, can be felt.
margin of mucosa, not less than 1 cm. If it is
• A fissure: it is usually presented as a chronic
localized to anterior 2/3rd of the tongue, partial
fissure which does not to heal.
glossectomy or subtotal glossectomy should
The tumor may begin as a superficially
be carried out. When the growth reaches within
indurated ulcer with a slightly raised border and
2 cm of jaw, hemimandibulectomy may be re-
may proceed either to develop a fumigating, quired with excision of the growth.
exophytic mass or to infiltrate the deep layers of
the tongue, producing fixation and induration Radiotherapy: When the growth is more than
without much surface changes. At an early stage 1 cm in diameter in anterior 2/3rd, the preliminary
tongue cancer may appear as thickened, leuko- treatment is radiotherapy in the form of interstitial
plakic patches, or as a nodule. radiotherapy.
Prognosis: The 5 years survival rate of cancer
Spread of Carcinoma tongue is not more than 25%.

• Local spread: It spreads by infiltration and Complications

invasion.
Patient usually dies due to cancerous cachexia,
• Carcinoma of anterior 2/3rd of the tongue: It starvation, inhalation bronchopneumonia,
usually starts on the lateral margins of the asphyxias and hemorrhage from involved cer-
tongue and invades the floor of the mouth vical lymph nodes.
early, but it does not extend to the other side
across the midline. Pigmentation of Tongue
• Carcinoma of posterior 3rd of the tongue: It tends The tongue may exhibit various patterns of racial
to spread to the corresponding tonsils, melanin pigmentation. Endogenous pigmen-
epiglottis and soft palate. tation is rarely identifiable on the dorsum because
• Lymphatic spread. of the thickness of the epithelium, but jaundice
may be apparent under the thinner ventral mu-
• Tip of the tongue: Carcinoma of tip of the tongue
cosa. Exogenous pigmentation of filiform papillae
may drain to the submental nodes, but may
of the normal and coated or hairy tongue is very
also drain to the juguloomohyoid nodes.
common and result from microbial growth,
• Anterior 2/3rd: It drains into the submandi- metabolic products, food debris and dyes from
bular lymph nodes. candy, beverages and mouth rinses.
570 Textbook of Oral Pathology

Pigmentation of the tongue has been described Clinical Features

by a commonly used antichemotherapeutic agent
doxorubicin hydrochloride, which also discolor It is common in 40 to 60 years or elder age groups.
patient’s urine, nail beds and skin folds. It occurs on the ventral surface of the tongue,
Extravasation of red cells around lingual sometimes resembling a caviar tongue. It is sin-
varicosities may give patchy, bluish red dis- gle, bluish and rather well demarcated lesions
coloration. The thin tissue overlying a ranula is may also occur on the dorsum or borders of the
said to have a greenish blue appearance. tongue.

Phlebectasia
Managemment
It is also called as varicosity. It is the local wide-
ning of veins due to loss of elasticity of the blood No treatment is necessary, unless there is ulcera-
vessel walls and results from aging of the tissues. tion or thrombosis.
 CHAPTER

29 Diseases of Lip

ANATOMY Contd...
• Plasma cell cheilitis
Lips are fleshy folds lined by skin externally and • Cheilitis due to drugs
C. Carcinoma of lip
mucous membrane internally. The upper and
D. Miscellaneous
lower lips close along the red margin which • Chapping of lips
represents the mucocutaneous junction. The lips • Actinic elastosis
• Lip ulcers due to caliber persistent artery
are covered with skin on the external surface and
mucus membrane on the inner surface, which Oral commissural is the angle where the
has profuse salivary glands. The lip extends from upper and lower lip meet. The upper lip includes
the lower end of the nose to the upper end of the the philtrum, a midline depression, which can be
chin. The upper lip borders onto the nose and is followed to the nose. Each lip mainly consists of
separated from the cheek by a variably deep bundles of striated muscle, orbicularis oris,
groove called as nasolabial groove. The lower superficial fascia and submucosa. The skin of the
lip is separated from the chin proper by a more lip contains sweat glands, hair and sebaceous
or less sharp and deep groove that is convex gland. The dermal papillae are numerous, with
superiorly called as labiomental groove. rich capillary supply, which produce reddish pink
Table 29.1: Classification of lip disorders color of the lips.
A. Developmental
• Congenital lip pits Vermilion zone—It is the transitional zone between
• Commissural lip pits the skin and the mucus membrane. The vermilion
• Double lip
• Cleft lip and cleft palate
zone contains no hair or sweat glands and
B. Cheilitis contains a few sebaceous glands. In some people,
• Glandular cheilitis sebaceous glands may be seen as creamy yellow
• Granulomatous cheilitis
• Angular cheilitis dots. Fordyce’s spots along the border between
• Contact cheilitis vermilion border and the oral mucosa can also
• Eczematous cheilitis
• Actinic cheilitis
occur.
• Exfoliative cheilitis Submental artery to the lower lip and inferior
Contd... and superior labial arteries to the upper lip
572 Textbook of Oral Pathology

supply the lip. Anterior facial vein and its bran- Histopathological Feature
ches corresponding to the facial artery provide
It shows the tract lined by stratified squamous
the venous drainage of lips. Lymphatic drainage
epithelial. Chronic inflammatory cell infiltrate is
is from central part of lower lip lymphatics drain
noted in the surrounding connective tissue.
to the submental node and rest of the lip to sub-
mandibular nodes. Mental nerve and superior
Management
labial nerve are the nerve supply of the lip.
Surgical excision for cosmetic purpose should be
DEVELOPMENTAL DISTURBANCES OF LIP carried out.

Congenital Lip Pits Commissural Pits

It is also called as paramedian lip pits, congenital Commissural lip pit occurs due to failure in
fistulas of the lower lip. They arise from persis-
fusion of embryonic process. These are invagi-
tent lateral sulci on the embryonic mandibular
nation occur on lip.
arch.
Clinical Features
Etiology
It is more common amongst males and black
It is inherited as autosomal dominant trait. There people are affected more than white people.
is notching of lips at an early stage of develop- Commissural pit appears as a unilateral or
ment with fixation of tissues at the base of the bilateral pit at the corner of the mouth on the
notch. It may occur due to failure of complete vermilion surface. If it is unilateral, it occurs on
union of embryonic sulci of the lip. the right side of the lip.
In some cases small amount of fluid can be
Clinical Features seen from the pit when the pit is squeezed which
It is more commonly seen in females. It is may occur from minor salivary gland.
common on vermilion border of either lips and It is localized at angle of mouth with the tract
most commonly on lower lip. It may be upto diverging dorsolaterally into the cheek. Size
3-4 mm in diameter and may extend as deep as ranges from a shallow dimple to a tract measu-
2 cm and communicated with underlying minor ring 4 mm in length and tissue slightly raised
salivary glands. Lip pits or fistula is unilateral above the opening.
or bilateral depression. In some cases, sparse
mucus secretion may be visible from the base of Histopathological Features
the pit.
There is narrow invagination lined by stratified
Lips, sometimes appear swollen, accentua- squamous epithelium. Ducts from minor sali-
ting the appearance of the pit. Congenital lip pits
vary gland may drain into the invagination.
may occur in association with Van der Woude’s
syndrome (cleft lip, cleft palate and congenital
Management
lip pits) popliteal pterygium syndrome (popliteal
webbing, cleft lip/palate, genital abnormalities, No treatment is necessary as it is asymptomatic.
and congenital bands connecting the upper and In cases of secondary infection surgical excision
lower jaw). should be carried out.
 Diseases of Lip 573

Double Lip ectodermal groove separating that mesodermal

mass which actually constitutes the facial
It is an anomaly characterized by a fold of excess
process.
tissue on the inner mucosal surface of the lip. It
Either absence or deficiency of these meso-
may be congenital or acquired because of trauma
dermal masses or their failure to penetrate the
to the lip. The congenital variety occurs as a result
ectodermal grooves lead to breakdown of the
of persistence of horizontal sulcus which is
ectoderm, causing cleft formation.
situated between pars glabrosa and pars villosa
Cleft palate occurs due to disturbances in
of the lip. The acquired variety occurs due suck-
normal fusion of palatal shelves; failure to unite
ing of lip inside the oral cavity.
due to lack of force, interference by the tongue
or a disparity in the size of parts involved. Cleft
Clinical Features
of soft palate and uvula do not appear to be
Upper lip is more commonly affected than a formed as a result of non-fusion of parts but
lower lip. It usually occurs on inner aspect of rather as posterior extension of palatal process;
upper lip. When upper lip is tensed double lip thus cleft of these parts is basically extension of
resembles ‘cupid bow’. a cleft of hard palate.
It is associated with Ascher’s syndrome
which consists of double lip, blepharochalasis (it Definition
is drooping of the tissue between eyebrow and • Cleft lip—It is a birth defect that results in a
edge of the upper eyelid so that it hangs loosely unilateral or bilateral opening in the upper
over the margin of the lid) and nontoxic thyroid lip between the mouth and the nose. It is also
enlargement. called as harelip. It is wedge shaped defect
resulting from failure of two parts of the lip
Histopathological Features to fuse into a single structure.
There is abundance of minor salivary gland. • Cleft palate—Cleft palate is a birth defect
Ascher’s syndrome shows hyperplasia of the characterized by an opening in the roof of
lacrimal gland or prolapse of orbital fat. the mouth caused by a lack of tissue develop-
ment.
Management
Etiology
Surgical excision of excess tissue can be per-
• Hereditary—It is one of most important factors
formed for esthetics purpose.
to be considered in the etiology.
• Genetic—The main possible mode of trans-
Cleft Lip and Cleft Palate
mission is by a single mutant gene; producing
It occurs along many planes as a result of fault a large effect or by number of genes (poly-
or defect in the development. genic inheritance), each producing small
Mandibular cleft lip occurs due to failure of effects which together create this condition.
copula to give rise to mandibular arch or per- • Nutritional disturbances—Riboflavin deficient
sistence of the central groove of mandibular diet can produce cleft palate and cleft lip.
process. • Developmental—Physiological, emotional and
Maxillary cleft lip occurs due to failure of traumatic stress during developmental
mesodermal penetration and obliteration of the stages.
574 Textbook of Oral Pathology

• Defective vascular supply—Defective vascular cleft lip, the central portion of the alveolar arch is
supply to the area may lead to ischemia rotated anteriorly and superiorly. The medial or
which in turn may lead to cleft formation. prolabial segment of skin contains no muscle or
• Mechanical disturbances—Here, the size of vermilion. In palatal clefts, the muscles of soft
tongue may prevent union of the parts. palate are hypoplastic and insert in the posterior
• Infection—Infection and lack of inherent margin of the remaining hard palate rather than
developmental force. the midline raphe.
• Miscellaneous—Steroid therapy during preg-
nancy, alcohol, toxins in the circulation. Classification
1st
Cleft Formation
• Unilateral incomplete
In general, patients with clefts have a deficiency • Unilateral complete
of tissue and not merely a displacement of • Bilateral incomplete
normal tissue. A cleft lip occurs when an epi- • Bilateral complete.
thelial bridge fails, due to lack of mesodermal
delivery and proliferation from the maxillary 2nd by Veau’s
and nasal processes. • Cleft lip
Clefts of the primary palate occur anterior to – Class I — A unilateral notching of ver-
the incisive foramen. Clefts of the secondary milion not extending into the lip.
palate are due to lack of fusion of the palatal – Class II — A unilateral notching of
shelves and always occurs posterior to the inci- vermilion with cleft extending into lip but
sive foramen. The secondary palate closes 1 week not including the floor of the nose.
later in females, which may explain why isolated – Class III — A unilateral cleft of vermilion
clefts of the secondary palate are more common extending into the floor of the nose.
in females. A cleft lip increases the probability – Class IV — Any bilateral cleft of the lip,
of development of cleft palate developing. whether this is complete or incomplete.
The cleft of lip occurs earlier and inhibits • Cleft palate
tongue migration, which may then prevent – Class I — Involving only soft palate.
horizontal alignment and fusion of the palatal – Class II — Involving soft and hard palate
shelves. In unilateral cleft lip, the floor of the nose but not alveolus (Fig. 29.1)
communicates freely with the oral cavity, maxilla
on the cleft side is hypoplastic, columella is
displaced to the normal side and the nasal ala
on the cleft side is laterally, posteriorly and
inferiorly displaced. The lower lateral cartilage
of the nose is lower on the cleft side, its lateral
cruz is longer and the angle between the medial
and lateral cruz is more obtuse.
The muscles of the orbicularis oris do not
form a complete sphincter but instead are
directed superiorly to the ala nasi, laterally and
the base of the columella. medially. In bilateral Figure 29.1: Cleft involving lip as well as palate in a child
 Diseases of Lip 575

– Class III — Involving soft and hard palate affected side. Sucking become difficult to some
and alveolus of one side. extent but not greatly. There is defective speech
– Class IV — Involving both the soft and particularly with the labial letters B, F, M, P and
hard palate and alveolus on both sides of V. Cleft of mandibular lip or jaws are rare. There
the premaxilla. is soft tissue mass between the ends of the bone,
uniting the tongue to the lip so that tongue is
3rd by Kernahan and Stark bound down.
• Unilateral incomplete cleft of the primary Cleft palate—There may be cleft of the hard and
palate. soft palate or in some cases, cleft of soft palate
• Complete cleft of primary palate ending at alone. Entire pre-maxillary portion of bone may
the incisive foramen. be missing and in such instances, the cleft
• Bilateral complete cleft of the primary palate. appears to be entirely a midline defect (Fig. 29.3).
• Incomplete isolated cleft of the secondary Cross bite due to medial collapse of premaxilla.
palate. Eating and drinking is difficult due to regurgi-
• Complete cleft of the secondary palate—soft tation of food and liquid through the nose.
and hard palate. Speech problem is serious and tends to increase
• Unilateral complete cleft of the primary and due to mental trauma. There is defect in smelling
secondary palate.
• Bilateral complete cleft of the primary and
secondary palate.
• Incomplete cleft of the primary palate and
incomplete cleft of the secondary palate.

Clinical Features
General—It is more common in boys than in girls.
It is more frequently seen on the left side than
on the right side. Left side is involved in 70% of
the cases. A typical patient with cleft palate, cleft
lip and ridge exhibits a large defect with a direct
opening in the nasal cavity. Disturbances in the Figure 29.2: Cleft of lip without involvement of palate
dental structures are seen in this region so that
teeth may be missing, deformed, displaced or
divided, thus producing supernumerary teeth.
Cleft lip—A unilateral cleft involves only one side
of the lip; a bilateral one involves both sides and
later gives rise to ‘hair lip’. Incomplete cleft lip
extends for varying distances forward to the
nostril, but not upto the nostril. The upper part
of lip has fused normally (Fig. 29.2). Complete
cleft lip extends into nostril and palate is
commonly involved. It is often associated with
flattening and widening of the nostril of the Figure 29.3: Cleft involving palate seen as defect
576 Textbook of Oral Pathology

due to contamination of the nasal mucus mem- in two stages, the soft palate first followed by
brane with the oral organism through the cleft the hard palate.
palate. The alveolar cleft interferes with the • Bone grafting—Sometimes closure of palatal
dental lamina and the upper lateral incisors may cleft may be done by bone grafting.
be small, absent or even duplicate. • Orthodontic therapy—Orthodontic therapy is
Cleft of palate may also vary in severity, done to correct malocclusion.
involving uvula or soft palate or extending all • Cleft rhinoplasty—To improve nasal function
the way through the palate and indirectly to the and correct the distortion.
alveolar ridge on one or both sides. Isolated cleft • Speech therapy—Speech therapy is given to
palate is associated with other developmental improve pronunciation of the words.
abnormalities like congenital heart disease, poly- • Psychotherapy—Psychological management is
dactyly and syndactyly, hydrocephalus, micro- necessary.
cephalus, clubfoot, supernumerary ear, spina • Feeding plate—To overcome initial feeding
bifida, hypertelorism and mental deficiency. problems, feeding plate is used which acts
Airway problems may arise in children with cleft as an obturator to prevent nasal reflux.
palates, especially those with concomitant struc-
tural or functional anomalies. For example, Syndromes Associated with Cleft Palate
Pierre Robin syndrome is the combination of
micrognathia, cleft palate and glossoptosis. • Pierre-Robin syndrome
Affected patients may develop airway distress • Goldenhar syndrome
from their tongue becoming lodged in the palatal • Median cleft face syndrome
defect. Ear infection and respiratory tract • Oral facial digital syndrome
infection. • Apert’s syndrome
• Nagar syndrome
Management • Otopalatodigital syndrome
• Down’s syndrome
The complete rehabilitation of the condition
• Marfan syndrome.
requires a multidisciplinary approach.
• Cheiloplasty—It is surgical closure of the lip.
Glandular Cheilitis
A general ‘rule of tens’ is used in determining
optimal timing of lip closure, i.e. 10 weeks of It is also called as ‘cheilitis glandularis’. It is an
age, 10 pounds of body weight and 10 gm of uncommon condition in which lower lip
Hb. At the time of lip closure, when an infant becomes enlarged, firm and finally everted.
is under general anesthesia, an impression is
made for the new obturator. Etiology
• Obturator—Between 3rd and 9th months of
age, an obturator is used to provide cross- It occurs due to chronic exposure to sun, wind
arch stability, support and to prevent collapse and dust as well as use of tobacco. In several
of maxillary arch. cases, emotional disturbances, as well as familial
• Palatoplasty—It is performed to close an open- occurrence, suggesting a hereditary pattern is
ing in the palate. Surgeons may close the observed. Inflammation of enlarged heterotopic
palate in one surgery, when the child is about salivary glands may also lead to glandular
one year of age or the palate may be closed cheilitis.
 Diseases of Lip 577

Types Malignant transformation—It is apparently pre-

malignant and epidermoid carcinoma can be
• Simple— Multiple, painless, pinhead sized
associated with it in 18 to 35% of cases.
lesions with central depression and dilated
canals present.
Histopathological Features
• Superficial suppurative type (Baelz’s disease)—
It is characterized by painless swelling, indu- There is presence of stratified squamous epithe-
ration, crusting and superficial ulceration of lium which covers inflammatory connective
lip. tissue. In a milder form, there is some fibrosis
• Deep suppurative type (cheilitis glandularis surrounding the salivary glands and in the more
apostematosa, myxadenitis labialis)—Deep severe forms, there may be a dense, inflamma-
seated infection with abscess and fistula tract tory infiltrate. The underlying salivary gland
that eventually forms a scar. tissue shows hypertrophy and inflammation. In
some cases there is dysplastic changes can be
Clinical Features seen in the epithelium.
It is more common in adults but some times it
can also occur in children. Lower lip is involved Management
more often than the upper lip. Labial salivary Vermilionectomy—Due to high incidence of
glands become enlarged and sometimes nodular. associated malignancy, a vermilionectomy or
Orifices of secretory ducts are inflamed and surgical stripping of lips has been recommended.
dilated appearing as small red macules over the If the lips are grossly enlarged, excision of an
mucosa. Viscid mucus secretion may seep from elongated ellipse of tissue may be required.
these openings of everted hypertrophic lips.
Volkmann’s cheilitis—It is more severe suppura- Granulomatous Cheilitis
tive form of glandular cheilitis. The lip is It is also called as ‘Miescher’s syndrome’ or ‘cheilitis
considerably and permanently enlarged and is granulomatosa’. It is a condition of unknown
subjected to episodes of pain, tenderness and etiology that is not related to cheilitis glandularis
increased enlargement. The surface is covered except by the similarity in the clinical appearance
by crust and scales beneath which the salivary of the two diseases.
duct orifice may be discovered (Fig. 29.4).
Clinical Features
It is seen in adults as well as in children and there
is female predilection. There is diffuse swelling
of the lips, especially the lower lip. In some cases,
an attack is accompanied by fever and mild
constitutional symptoms including headache
and even visual disturbances. Enlarged lip can
create cosmetic problems, difficulty during eat-
ing, drinking or speaking.
In some cases, scaling, fissuring and vesicles
or pustules have been reported. The swelling is
Figure 29. 4: Volkmann’s cheilitis seen as crusted area usually soft and exhibits no pitting on pressure.
578 Textbook of Oral Pathology

Swelling eventually becomes firmer and acquires General protein deficiency can also cause
the consistency of that of hard rubber. The cheilitis.
regional lymph nodes are enlarged in some • Diseases of skin—Atopic dermatitis involving
cases, but not always. The skin and adjacent the face is often associated with angular
mucosa may be of normal color or erythematous. cheilitis. The incidence also appears to be
It is associated with Melkersson-Rosenthal increased in seborrhoeic dermatitis.
syndrome which consists of fissured tongue and • Other factors—Hypersalivation, Down’s syn-
facial paralysis. drome, large tongue and constant dribbling
being the contributory factors. A rare cause
Histopathological Features is the presence of a sinus of developmental
It consists of chronic inflammatory cell infiltrate origin at the angles of the mouth.
particularly peri and paravascular aggregation
of lymphocytes, plasma cells and histiocytes. Clinical Features
There is focal noncaseating granuloma formation It occurs in young children as well as in adults.
with epithelioid cells and Langhans type of giant It is characterized by feeling of dryness and a
cells. There is generalized edema and dilated burning sensation at the corners of the mouth. It
blood vessels present in the connective tissue. is usually a roughly triangular area of erythema
and edema at one or more, commonly both the
Management angles of mouth (Fig. 29.5).
Corticosteroid injection—Repeated injection of Epithelium at the commissures appears
triamcinolone into the lips every few weeks may wrinkled and somewhat macerated. In time,
be effective. Cheiloplasty – surgical stripping of wrinkling becomes more pronounced to form
lip can be done. one or more deep fissures or cracks which appear
ulcerated but which do not tend to bleed,
Angular Cheilitis although a superficial exudative crust may form.
It is also called as ‘perleche’, ‘angular cheilosis’. Linear furrow or fissures radiating from the
angle of mouth (rhagades) are seen in more
Causes severe forms, especially in denture wearers. If
• Microorganisms—Particularly Candida albi- the lesion is not treated, they often show a ten-
cans, but also staphylococci and streptococci. dency for spontaneous remission.
• Mechanical factors—Overclosure of jaws such
as in edentulous patients or in patients with
artificial dentures which lack proper vertical
dimensions. In it, folds are produced at the
corners of the mouth in which saliva tends
to collect and the skin becomes macerated,
fissured and secondarily infected. Progna-
thism may give rise to a similar state of affair
in young. The recurrent trauma from dental
flossing may occasionally be also implicated.
• Nutritional deficiency—It can also occur due
to riboflavin, folate and iron deficiency with Figure 29.5: Angular cheilitis presented as fissuring and
a superimposed fungal or bacterial infection. crack at the corner of mouth
 Diseases of Lip 579

Management Mouthwashes and dentifrices—Essential oils such

as peppermint, cinnamon, clove, spearmint and
Underlying primary cause should be identified
bactericidal agents can cause cheilitis. Propolis,
and treated. A course of vitamin B and iron
supplements is useful in these cases. derived from resin and collected by bees, is a well
known sensitizer which has been used in
Fusidic acid ointment—It is used in staphylococcal toothpastes.
infection. The lesions should be swabbed first and
then fusidic acid ointment or cream should be Dental preparations—Mercury and eugenol may
applied at least four times a day. cause cheilitis in the absence of stomatitis.
Miconazole may be preferred, if angular chei- Allergy to epimine containing materials used for
litis is due to candidiasis (cream applied locally crowns and bridges can cause cheilitis.
together with an oral gel).
Foods—Oranges, mangoes and artichokes are
Gentian violet application—In some cases it is
among the food plants which occasionally cause
useful.
allergic cheilitis and dermatitis of the skin
around the lips.
Eczematous Cheilitis
The lips are involved secondary to atopic eczema Miscellaneous objects—Metal hair clips, metal
but possibility of contact dermatitis must also be pencils, cobalt paint on blue pencil can also cause
considered. The management of atopic eczema cheilitis.
of the lips is with an emollient and topical
steroids. Clinical Features

Lipstick cheilitis is usually confined to the vermi-

Contact Cheilitis
lion borders but more often extends beyond that.
Contact cheilitis is an inflammatory reaction of There may be persistent irritation and scaling or
the lips provoked by the irritants or sensitizing a more acute reaction with edema and vesi-
action of chemical agents in direct contact with culation
them.
Management
Causes
Topical steroids will give symptomatic relief but
Lipsticks—They are composed of mineral oils and
the offending substance must be traced and
waxes which form the stick; castor oil as a solvent
avoided.
for the dyes, lanolin as an emollient preservative,
perfumes and color. The color includes azo dyes
Actinic Cheilitis
and eosin, which is a bromofluorescein deriva-
tive. Sunscreen applied in the form of lipstick It is also called as ‘actinic keratosis’ or ‘solar
can also cause contact cheilitis. cheilosis’.
Lipsalves and other medicaments—Lipsalves con- It is a premalignant squamous cell lesion
taining lanolin are frequently applied for dryness resulting from long term exposure to solar
or chapping. Phenyl salicylates and antibiotics radiation and may be found at the vermilion
have also been incriminated as a cause of border of lip as well as other sun exposed
cheilitis. surfaces.
580 Textbook of Oral Pathology

Etiology Management
Chronic sun exposure is the main cause so it Topical fluorouracil—For mild cases, application
usually occurs in hot, dry regions, in outdoor of 5% fluorouracil three times daily for 10 days
workers and in fair skinned people. is suitable. It produces brisk erosion but lips heal
within 3 weeks. Application of 5-fluorouracil to
Clinical Features the lip will produce erythema, vesiculation,
erosion ulceration, necrosis and epithelialization.
The lower lip is more commonly affected than the
In some cases, podophyllin is also used.
upper lip as it receives more solar radiation than
Rapid freezing with CO2 snow or liquid nitro-
the upper lip. It is less common in females due to
gen on swab stick is used to remove superficial
sunscreen effect of lipstick and less common in
lesions.
blacks due to protective effect of melanin.
In the early stages, there may be redness and Vermilionectomy (lip shaves)—Under local anes-
edema but later on, the lips become dry and thesia, the vermilion border is excised by a scal-
scaly. If scales are removed at this stage, tiny pel and closure is then achieved by advancing
bleeding points are revealed. With the passage the labial mucosa to the skin. Postoperative
of time, these scales become thick and horny with complications include paresthesia, lip pruritis
distinct edges. Epithelium becomes palpably and labial scar tension.
thickened with small grayish white plaques. Ver- Laser ablation—Carbon dioxide laser therapy has
tical fissuring and crusting occurs, particularly been used to vaporize the vermilion. Good
in the cold weather. At times, vesicle may appear results with no postoperative paresthesia or
which rupture to form superficial erosions. significant scarring have been reported.
Secondary infection may occur. Eventually Following management, prevention of recu-
warty nodules may form which tend to vary in rrence by regular use of sunscreen lip salves is
size with fluctuation in the degree of edema and advisable. Liquid or gel waterproof preparation
inflammation. containing para-aminobenzoic acid probably
The possibility of malignancy must always gives the best protection.
be considered if following features are present;
Ulceration in actinic cheilitis, a red and white Exfoliative Cheilitis
blotchy appearance with an indistinct vermilion
It is a chronic superficial inflammatory disorder
border, generalized atrophy or focal areas of
of the vermilion border of lips characterized by
whitish thickening, persistent flaking and crus-
ting and indurations at the base of keratotic persistent scaling.
lesion.
Causes
Histopathological Features These cases may occur due to repeated lip suc-
It shows flattened or atrophic epithelium ben- king, chewing or other manipulation of the lips.
eath which is a band of inflammatory infiltrate
Clinical Features
in which plasma cells may predominate. Nuclear
atypia and abnormal mitoses can be seen in more Most cases occur in girls and young women and
severe cases and some develop into invasive majority have personality disorders. The process
squamous cell carcinoma. The collagen generally starts in the middle of the lower lip and spreads
shows basophilic degeneration. to involve the whole of the lower lip or both the
 Diseases of Lip 581

lips. It consists of scaling and crusting, more or to females. It is most common on the lower lips
less confined to the vermilion borders and of fair skinned people and persons who work in
persisting in varying severity for months or outer climate. It usually begins on vermilion
years. The patient complains of irritation or bur- border of the lip to one side of the midline and it
ning and can be observed frequently biting or may be covered with a crust due to absence of
sucking the lips. saliva. It is preceded by actinic cheilitis which is
characterized by innocuous looking white
Management plaque on the lip.
Reassurance and topical steroids are helpful in Patient may complain of difficulty in speech,
difficulty in taking food and inability to close the
some cases but in some, psychotherapy or
mouth. There is also pain, bleeding and pares-
tranquilizers are used.
thesia.
Plasma Cell Cheilitis It often commences as a small area of thicke-
ning, induration and ulceration or irregularity
It is an idiopathic benign inflammatory condition of the surface. In some cases, it commences as a
characterized by dense plasma cell infiltrate in small warty growth or fissure on the vermilion
the mucosa close to the body orifice. It can affect border of the lip. Crater like lesion having a
penis, vulva, lips, buccal mucosa, palate, gingiva, velvety red base and rolled indurated borders
tongue, epiglottis and larynx. are present (Fig. 29.6). As the lesion enlarged it
It presents as circumscribed patches of ery- takes papillary or an ulcerative form. In untrea-
thema, usually on the lower lip in elderly per- ted cases there is total destruction of lip and
sons. It responds to topical application of power- invasion of cheek, the gums and the mandible.
ful steroids or to intradermal injection of triamci- Papillary lesion grows slowly and infiltrated
nolone. the deeper tissue relatively late whereas
ulcerative growths invade early (Fig. 29.7). It may
Drug-induced Cheilitis metastasize and it is usually ipsilateral. Car-
Hemorrhagic crusting of the lips is a feature of cinoma of the upper lip metastasizes earlier and
Steven Johnson syndrome which is commonly more frequently than carcinoma of the lower lip.
caused by drugs but, cheilitis can occur as an
isolated feature of a drug reaction—either as a
result of allergy or a pharmacological effect. The
aromatic retinoids, etretinate and isotretinoin
cause dryness and cracking of lips in most
patients.

Carcinoma of Lip
Squamous cell carcinoma is the commonest
malignancy to affect the vermilion zone.

Clinical Feature
There is peak appearance in 6th and 7th decades Figure 29.6: Malignancy of lip presented as ulcerative
of life. It is more common in males as compared growth on lower lip
582 Textbook of Oral Pathology

avoidance of the causative environmental

conditions.

Actinic Elastosis
It is also called as ‘solar elastosis’ or ‘senile
elastosis’.

Causes
It is caused by prolonged exposure to UV light.
UV radiation can produce collagen degeneration
in the dermis and extent of this effect is
Figure 29.7: Granulomatous ulcerative growth seen in dependent upon factors such as the thickness of
malignancy
stratum corneum, melanin pigment, clothing or
It involves submaxillary and submental nodes chemical sunscreens.
first and then deep cervical nodes. Spread by
direct extension into surrounding structures and Clinical Features
by metastasis which is through lymphatic On the labial mucosa exposed to sun, white area
channels. of atrophic epithelium develops with underlying
scarring of the lamina propria. In outdoor elderly
Histopathological Features population, lips may show actinic elastosis in
They are mostly well differentiated malig- which vermilion border blends with the skin
nancies. surface. Clinical features include leathery
appearance, laxity with wrinkling and various
Management pigmentary changes.
Clinically, it is manifested in three forms:
Surgical— Prognosis is good if the treatment is • Cutis rhomdoidalis—Thickened skin with
done before metastasis. The best results are seen furrow giving an appearance of rhomboidal
when being obtained when the entire lip muco- network.
sal field is removed for early lesion. • Dubreuilh’s elastoma—Diffuse plaque like
lesions.
Chapping of the Lips • Nodular elastoidosis—Nodular lesion.
It is a reaction to adverse environmental condi-
tions in which keratin of the vermilion zone loose Lip Ulcers due to Caliber Persistent Artery
its plasticity, so that lip becomes sore, cracked A caliber persistent artery is defined as an artery
and scaly. The affected subjects tend to lick the with a diameter larger than normal near a
lips or to pick at the scales which may make mucosal or external surface. Such artery in the
conditions worse. It is caused by exposure to lip causes chronic ulceration which can be
freezing cold or to hot, dry wind, but acute sun- mistaken for squamous cell carcinoma. The ulcer
burns can cause very similar changes. Manage- is attributed to continual pulsation from the large
ment is by application of petroleum jelly and artery running parallel to the surface.
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 CHAPTER

30 Blood Dyscrasias

Anemia is fall in hemoglobin level below normal Table 30.1: Etiologic classification of anemia
level for given age and sex. It can result from Type Etiology
diverse processes resulting alteration in morpho-
I. Blood Loss
logy of RBCs. These form basis of various classi- A.Acute posthemorrhagic Trauma
fication systems (Table 30.1). anemia
B. Chronic posthe- Lesions of gastrointestinal
morrhagic anemia tract, gynecologic disturbances
Morphologic Classification II. Hemolytic anemia
Thalassemia Hereditary
• Normochromic normocytic anemia Sickle cell anemia Hereditary
– Anemia of acute blood loss Anemia due to enzyme Deficiency of glucose-6-phos-
defieciencies phate dehydrogenase, gluta-
– Anemia associated with leukoplakia thione synthetase, pyruvate
– Aplastic anemia kinase, hexokinase.
• Hypochromic, microcytic anemia Erythroblastosis fetalis Blood incompatibility, immune
mediated
– Iron deficiency anemia Spherocytosis Hereditary, increased
– Thalassemia permeability
• Normochromic, macrocytic anemia III. Impaired Red Cell
Production
– Pernicious anemia Pernicious anemia B12 deficiency secondary to
– Anemia due to folate and B12 deficiency intrinsic factor deficiency.
• Normochromic, microcytic anemia Aplastic anemia Bone marrow suppression
Megaloblastic anemias Deficiency of vit B12, folic acid
– Anemia due to chronic infections. Hereditary elliptocytosis Heredirary
Erythropoietic porphyria Metaboilic defect
Paroxysmal nocturnal Immune mediated
Iron Deficiency Anemia
hemoglobinuria
Iron is essential for synthesis of 'hem' portion of
Causes
hemoglobin. Iron deficiency anemia is caused by
imbalance between iron intake and loss or It is caused by inadequate intake of iron. Malab-
inadequate utilization. sorption of iron due to hypochlorhydria and

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586 Textbook of Oral Pathology

diarrhea. Increased requirement of iron in a gro- aphthous ulceration and candidal lesions can
wing child and in pregnancy. Increased loss of also occur in iron deficiency anemia. Patient may
iron due to injury, recurrent epistaxis and peptic show slow healing after oral surgical procedures.
ulcer. Chronic blood loss such as menstrual and In some cases there may be gingival enlargement
menopausal bleeding parturition. Subtotal or (Fig. 30.1).
complete gastrotomy.
Hematological Findings
Clinical Features
The anemia is microcytic and hypochromic and
It occurs chiefly in women in the 4th and 5th the peripheral smear shows abnormal forms of
decades of life. The patient experiences tiredness, RBCs. There is reduced hemoglobin level, as low
headache, paresthesia and lack of concentration. as 4g/100 ml. There is normal or slightly reduced
Nails become brittle, flattened and often show RBC count. MCV, MCH and MCHC are all redu-
spoon shape (koilonychia). There may be ting- ced.
ling and pins and needle sensation in the extre-
mities. Some patients develop pharyngeal muco- Histopathological Findings
sal thickening and mucosal web formation, There is marked thinning of epithelium, absence
giving rise to dysphagia.
of papillae in the lamina propria and absence of
Gastrointestinal symptoms: Liver and spleen
keratohyaline granules.
may be palpable. There may be gastrointestinal
bleeding and menorrhagia there by setting a Management
vicious circle.
Almost all patients can be treated by oral supple-
Oral Manifestations ments of iron by giving ferrous fumerate or
ferrous sulphate. It is given in dose of 300 mg
In iron deficiency there is pallor of oral mucosa
three to four times a day for a period of 6 months.
and gingiva. The normal pink color is lost due
to lack of oxygenated blood in the capillary bed Plummer Vinson Syndrome
in lamina propria and is associated with lowered
levels of hemoglobin. The generalized atrophy It is also called as Paterson-Brown-Kelly syndrome.
of oral mucosa both in tongue and buccal mucosa It is characterized by dysphagia, iron deficiency
occurs.
There is redness, soreness or burning of ton-
gue. The filiform papillae over the anterior two-
thirds of tongue are the first to undergo atrophy.
In severe cases fungiform papillae are also
affected leaving the tongue completely smooth
and waxy or glistening in appearance.
There is cracking and fissuring at the corner
of mouth. There is softening of epithelium which
leads to linear ulceration of the skin, extending
up to and beyond the mucocutaneous junction.
There may be pain on opening or stretching and Figure 30.1: Gingival enlargement in iron
rarely, bleeding from ulcerated tissues. Recurrent deficiency anemia

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 Blood Dyscrasias 587

anemia, dystrophy of nails (koilonychia) and commonly affected than females. There is usu-
glossitis. ally triad of symptom: generalized weakness,
sore painful tongue and numbness and tingling
Clinical Features of the extremities. Other features are fatiguabi-
It is exclusively found in middle aged women. lity, headache, dizziness, nausea, vomiting, di-
Patient of this syndrome have got characteristic arrhea, with loss of appetite, shortness of breath,
asthenic appearance. It occurs due to the forma- loss of weight, pallor and abdominal pain.
tion of webs in esophagus. Nervous system: Nervous system disorders are also
Vermilion borders of the lip are very thin and present and manifested by sensory disturbances
there is often angular cheilosis. Patients com- including the paresthetic sensation of the
plaint of 'spasm in throat' or food sticking in extremities, weakness, stiffness and difficulty in
throat. There is also complaining of sore mouth walking, general irritability, depression, drow-
and inability to retain dentures. A smooth, red, siness as well as in-coordination or loss of vibra-
occasionally enlarged and often sore tongue with tory sensation. There is also tingling sensation
fissuring is occurs. in the fingers and toes that eventually progress
The width of mouth is narrowed and the oral to numbness.
mucosa is pale and painful. There is also dry Gastrointestinal complaint: Epigastric discomfort,
mouth and spoon shaped nails. There are atro- constipation or diarrhea can be seen in these
phic changes in mucosa of mouth, pharynx, patients.
upper esophagus and vulva.
Oral Manifestations
Histopathological Features
There is glossitis and patient complains of
There is atrophic epithelium and atrophy of painful and burning lingual sensation which
lamina propria and muscles. may be so annoying that the dentist is often
consulted first. The tongue is generally inflamed
Pernicious Anemia often described as 'beefy red' in color, either
entirely or in patches scattered over the dorsum
It is also called as 'primary anemia', 'Addison's
and lateral border of tongue.
anemia' or 'Biermer's anemia'. The term perni-
There is gradual atrophy of the papillae of
cious anemia should be reserved for patients
tongue that eventuates in a smooth and bald
who have B12 deficiency secondary to intrinsic
tongue which is often referred as Hunter's glossi-
factor deficiency.
tis or Moeller's glossitis and is similar to the 'bald
Causes tongue of sandwith' seen in pellagra.
The fiery red appearance of tongue may
It occurs due to atrophy of gastric mucosa undergo remission but recurrent attacks are
resulting in failure to secrete the still unidentified common. Sometimes inflammation and burning
'intrinsic factor'. It is suggested that it is auto- involve the entire oral mucosa. Tongue may
immune disorder, because autoantibodies to show lobulations, which may be secondary to
gastric parietal cells are often found in patients. decrease in saliva production. There is distur-
bance in taste sensation with intolerance to
Clinical Features
dentures and occasional dryness of mouth.
It is rare before the age of 30 years and increase Oral mucosa shows greenish yellow color
in frequency with advancing age. Males are more (frequently observed on the skin) at the junction

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588 Textbook of Oral Pathology

of hard and soft palate, when daylight is used for of gastric hydrochloric acid secretion is a cons-
illumination. tant feature and pH of the gastric content is usu-
ally high. Achlorhydria is associated with atro-
Histopathological Features phy of the gastric mucosa, which commonly
Histologically, oral epithelial cells in pernicious occurs in presence of chronic inflammation. Sch-
anemia reveal enlarged, hyperchromatic nuclei illing test detects the absence of intrinsic factor.
with prominent nucleoli and serrated nuclear
membranes. There is atrophy of epithelium with Management
intra or subepithelial chronic inflammatory cell Most of the patients should be given vitamin B12
infiltration. Cellular atypia can be seen.
parenterally but some of the cases are treated
Laboratory Findings with massive oral dose of vitamin B 12. The
standard dosage is 100 mg IM every 30 days.
The red blood count is decreased often to 3 or
less per cubic millimeter. Many of the cells Thalassemia
exhibits macrocytosis, while some exhibit
poikilocytosis (Fig. 30.2). Decreased WBC count It is also called as 'Cooleys anemia', 'Mediterranean
and mean corpuscular hemoglobin. In advanced anemia' and 'erythroblastic anemia'. Either alpha
cases of anemia, there are polychromatophilic or beta globulin genes may be affected. The resul-
cells, stippled cells, nucleated cells, Howell-Jolly tant red blood cells have reduced hemoglobin
bodies and Cabot rings. Leukocytes are also often are thin and have shortened life span.
reduced in number but are increased in average It is autosomal dominant. It is an inherited
size. impairment of hemoglobin synthesis in which
Bone marrow shows great number of imma- there is partial or complete failure to synthesize
ture red cells or megaloblasts with few normo- a specific type of globin chain.
blasts, indicating maturation arrest at the more
primitive megaloblast state. Achlorhydria or lack Types
• Alpha thalassemia—There is reduction or abse-
nce of ? chain synthesis.
• Beta thalassemia—There is reduction or abse-
nce of beta chains.
• Thalassemia major or homozygous β-thalassemia
— Occurs when the patient is homozygous.
It is also called as Cooley's anemia.
– Hemoglobin H disease—It is very mild form
of the disease in which the patient may
live relatively normal life.
– Hemoglobin Bart disease—In which in-
fants are stillborn or die shortly after birth
• Thalassemia intermedia—It is group of dis-
orders characterized by clinical manifesta-
tions between major and minor.
• Thalassemia minor or thalassemia trait—Occurs
Figure 30.2: Megaloblastic anemia showing macrocytosis when the patient heterozygous.

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 Blood Dyscrasias 589

Alpha Thalassemia because of chronic jaundice. The color is best seen

at the termination of hard palate and in the floor
Alpha chains of hemoglobin are required not only
of mouth.
for HbA but also for HbF, which is the main
Rodent facies—There is marked over develop-
hemoglobin type in fetal life. Therefore, major
ment of maxilla associated with hyperplasia of
type of alpha thalassemia is incompatible with
alveolar process, which results in anterior open
life and results in hydrops fetalis and intrauterine
bite and prominent cheek bone producing
death of fetus. The clinical findings are severe
characteristic 'rodent facies'.
alveolar bone loss, pronounced spacing of maxi-
The maxillary teeth are protrudes with
llary anterior teeth and mongoloid appearance.
spacing between them with a short upper lip due
to a lag between the growth of the maxilla and
Beta Thalassemia
the growth of upper lip. Due to high concentra-
Hemolysis is not primarily due to lack of β-globin tion of iron discoloration of dentin and enamel
chains but it is because of the free alpha chains maybe evident.
which form insoluble aggregates that precipitate Chip munk facies—There is also saddle nose,
within the RBCs and cause damage to the cell prominent malar bone and pneumatization of
membranes. maxillary sinus. As a result of these skeletal chan-
ges, the upper lip is retracted giving the child a
Clinical Features 'chip munk' facies. There is poor healing after
It occurs between the ages of 6 to 24 months and dental treatment.
after the age of 6-8 months, development and
growth of the child is retarded. Survival time is Hematological Findings
short. The patient first presents with pallor of The anemia is hypochromic and microcytic. The
skin, fever, chills, malaise, generalized weakness, peripheral smear shows abnormal RBCs.
prominent cheek bone and mild hepatospleno- Reticulocyte count is increased. Bone marrow
megaly. shows increased erythropoietic activity.
Mongoloid appearance—Bone marrow hyper-
plasia in early life may produce frontal head Diagnosis
bossing and there may be marked over-
Serum bilirubin and fecal and urinary urobilino-
development of malar bone which is associated
gen are elevated because of severe hemolysis. An
with a short nose having a depressed bridge
elevated fetal hemoglobin is present.
giving the appearance of mongoloid. Deposition
of iron in various organs (due to multiple
Histopathological Findings
transfusions) leads to signs and symptoms of
organ failure. Most patients die in childhood due The presence of typical safety pin cells and
to anemia and cardiac failure. nucleated red blood cells in circulation are
characteristic.
Oral Manifestations
Treatment
There is excessive overgrowth of maxilla causing
excessive lacrimation and nasal stuffness. The Blood transfusion, splenectomy, chelating agent,
oral mucosa is pale and has a lemon yellow tint folic acid.

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590 Textbook of Oral Pathology

Sickle Cell Anemia sed susceptibility to infection. Most of persons

It is autosomal dominant. It was first described may expire before the age of 40 years. There is
by Herrick in 1910. This is the most common type also presence of leg ulcer and gall stones. When
of hemoglobinopathy in which there is a substi- associated with folate deficiency, there may be
tution of amino acid glutamine on position growth retardation and also delayed puberty.
6 present in the chain of the HbA, by valine; giving Hyperplasia of marrow in first year of life
rise to an abnormal Hb, i.e. hemoglobin S. In expands the marrow cavity producing bossing
homozygous individuals, whole of HbA is repla- of the skull, prominent malar bones and
ced by HbS and this is known as 'sickle cell protuberant teeth.
disease' and in heterozygous individuals, only Sickle cell crisis — There is a long quit spell of
50% of HbA is replaced by HbS and this is known hemolytic latency occasionally punctuated by
as 'sickle cell trait'. exacerbations called as sickle cell crisis.
It is chronic hemolytic blood disorder charac- Infarction can only occur in bone and spleen
terized by abnormal hemoglobin (deoxygenated but, other tissues may also be involved. In
hemoglobin) which under low oxygen tension, infants, fingers and toes are commonly affected.
results in sickling of cell. Thrombosis of vessels in brain cause severe
When HbS is deoxygenated, it forms struc- neurologic disorders like stroke, convulsion,
tures know as 'tactoids' which distort the RBC coma, downiness as well as speech visual and
membrane and produce characteristic sickle hearing disturbances. Occlusion of smaller
shaped cell which are destroyed by RE cells. vessels results in headache and cranial nerve
Sickle cells increase blood viscosity and tend to neuropathy including palsy and paresthesia.
reduce blood flow leading to thrombosis and
tissue infarction. Oral Manifestations
In addition these, cells are phagocyte in large
The oral mucosa will show pallor and jaundice.
number by mononuclear-phagocyte system
There may be delayed eruption and hypoplasia
which reduce their life span and give rise with
of the dentition, secondary to their general
hemolysis. Patients may develop severe folic acid
development. Patient is more prone to develop
deficiency due to increased erythropesis.
osteomyelitis. This may be due to hypovascula-
Clinical Features rity of the bone marrow secondary to thrombosis.
Patient may present with paresthesia of men-
It is common in females and mostly the clinical
tal nerve which may be secondary to occlusion
symptoms become evident before the age of
involving the nerves and blood supply. Mongo-
30 years. Clinical manifestations begin only after
loid facies with high cheek bones and bimaxillary
several months as fetal Hb protects against
proganthism is present. It is due to marrow
sickling phenomenon. It includes dehydration,
hyperplasia resulting in an increase in hard
chills and infection but some time the attack
palate length and palate alveolar ridge angle.
occurs spontaneously. There is fatigue, weakness
and shortness of breath. Severe abdominal pain,
Diagnosis
muscle and joint pain, at high temperature which
may result in circulatory collapse also occur. It is confirmed by laboratory findings. A smear
There is painless hematuria. of peripheral blood usually shows typical sickle
There is enlargement of heart and murmur shaped red blood cells and hemoglobin level is
is found in most of the patients. There is increa- decreased.

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 Blood Dyscrasias 591

Hematological Findings mouth. Atrophy of filiform and fungiform pap-

The RBC count may reach level of 10 lac cells or illae leads to completely atrophic smooth fiery
less per cu mm with Hb levels from 5 to 12 gm/ red surface of tongue (Hunter's glossitis).
dl. The sickle hemoglobin molecule undergoes Angular cheilitis and dysphagia due to pharyn-
gelation or crystallization, when deoxygenated gitis and esophagitis. Yellowish brown pigmen-
and this typically distorts the erythrocyte, produ- tation of oral mucosa may occur due to increased
cing the sickle or boomerang shape. This sickle circulating bile pigments.
shaped cells then 'logjam' and produce stasis
within the microvasculature. Damage to erythro- Hematological Findings
cyte membrane also occurs in sickle cells and The peripheral smear shows a macrocytic blood
leads to their fragmentation and intravascular picture with many abnormal forms of RBCs.
hemolysis. Reticulocyte count is raised. There is associated leucopenia and sometimes,
thrombocytopenia. Bone marrow shows pre-
Histopathological Features sence of megaloblasts and erythroid hyperplasia.
Management
Management
Prevention of episode by avoiding the precipita-
ting factors is an important aspect of treatment. When hemoglobin level is less than 4g/dl blood,
Patients should avoid becoming chilled, dehy- transfusion should be given. Oral administration
drated or exposed to hypoxia (high attitude). of vitamin B12 should be given. Folic acid supple-
Regular folic acid supplement (5 mg/daily) ments should also be given.
and blood transfusion should be given.
Aplastic Anemia
Megaloblastic Anemia
It is a rare disorder characterized by peripheral
This results due to deficiency of vitamin B12 and blood pancytopenia (anemia, leucopenia and
folate or both, resulting in disordered cell thrombocytopenia) associated with bone ma-
proliferation leading to megaloblastic anemia. rrow suppression. In most cases bone marrow
All megaloblastic anemias have erythroid pre- suppression is not known and hence is known
cursors known as megaloblasts. It is charac- as idiopathic aplastic anemia. Fanconis anemia
terized by increase in mean corpuscular volume. is an inherited anemia that manifests in early
childhood.
Clinical Features
There are symptoms of anemia like weakness, Etiology
loss of appetite and palpitations. Patient may get
Common drugs which can cause aplastic anemia
periodic diarrhea. In severe cases, skin may show
are benzene derivatives, chloramphenicol,
faint lemon yellow tint and spleen may be pal- amidopyrine, organic arsenicals, colloidal silver,
pable. Many cases show paresthesia of finger and
bismuth, mercury, sulfonamides, penicillin and
toes and dementia may also be seen.
anticancer drugs.
Patient with bacterial disease such as tuber-
Oral Manifestations
culosis and viral infections like hepatitis and
There is burning sensation in tongue, hyper- infectious mononucleosis can cause pancyto-
sensitivity, paresthesia and later, dryness of the penia. Long term continuous exposure to small

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592 Textbook of Oral Pathology

amounts of external radiation or to internally Management

deposited radium or thorium has been followed
Stimulation of hemopoiesis by androgens, bone
by the development of aplastic anemia.
marrow transplantation and anti-fibrinolytic
agents.
Clinical Features

It can occur at any age, but is common in young Acute Posthemorrhagic Anemia
adults. Patient may feel weakness after slight The anemia caused by blood loss may occur in
physical exertion and exhibits pallor of skin. variety of conditions causing bleeding. When
Breathlessness, headache, ankle edema are blood loss occurs in large amounts in a short
common clinical features. Numbness and ting- period of time, anemia may develop even though
ling of extremities with edema are often encoun- iron stores remain adequate. It is called as acute
tered. Anemia may be severe enough to cause posthemorrhagic anemia.
anginal pain or congestive cardiac failure. There
may be bleeding from various sites like skin, Clinical Features
nose, vagina and gastrointestinal tract associated The manifestations of hemorrhagic anemia
with fever due to infection. depend on the rate and magnitude of bleeding;
the time elapsed since it took place and the site,
Oral Manifestations whether it is external or internal. When the blood
loss is about 500 to 1000 ml, most of the patients
The mucosa shows pallor. In some cases, spon-
do not present any symptoms, but few may
taneous hemorrhage may occur from the gin-
present with weakness and sweating. With rapid
giva.
loss of 1000 to 1500 ml, a previously healthy
Petechiae, often are present on the soft palate
individual may experience lightheadedness and
and in severe cases, there may sub-mucosal
hypotension.
ecchymosis. Large ragged ulcers covered by gray
When 1500 to 2000 ml of blood is lost, symp-
or black necrotic membrane may be present,
toms like sweating, thirst, shortness of breath,
which are the result of generalized lack of resis-
clouding or loss of consciousness are seen. The
tance to infection and trauma.
pulse becomes rapid and low in volume, skin
becomes cold and clammy and there is a fall in
Hematological Findings blood pressure. When rapid loss of blood exceeds
RBC count is remarkably diminished, as low as 1 2000 to 2500 ml, severe state of shock is reached.
million cells/mm3. WBC count is as low as 2000/
mm3 and platelet count may fall below 20000/ Hematological Features
mm3. The classical finding is that of pancytopenia Plasma volume and red cell mass are reduced in
along with reduction of absolute reticulocyte proportional amount. Anemia is normocytic and
count. Bleeding time is prolonged and clotting normochromic. Erythropoietin secretion is
time is normal. Anemia is normocytic with some stimulated which in turn gives rise to hyper-
degree of macrocytosis. Bone marrow is fatty, plasia of marrow erythroid elements within 3 to
acellular, and few developing cells. 5 days. Increased in reticulocyte number. Neutro-
Histopathological features. Oral ulcer show philic leukocytosis often follows hemorrhage
many microorganism and inflammatory cells. and is maximum after 2-5 hours.

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 Blood Dyscrasias 593

Management mation. The problem is complicated by Rh anti-

Restoration of blood volume. Intravenous gens which are termed as C, D and E. Out of
infusion of saline, dextrin, albumin or plasma. this, D antigen is the strongest and is responsible
Whole blood replacement is the therapy of for the clinical manifestations of erythroblastosis
choice. fetalis.

Chronic Posthemorrhagic Anemia Clinical Features

This develops when there is chronic blood loss Some infants are stillborn. Those that are born
in small amount over prolonged period of time alive have anemia with pallor, jaundice, compen-
and is due to depletion of iron in body. Other satory erythropoiesis (both medullary and extra-
findings are the same as that of iron deficiency medullary) and edema resulting in fetal hydrops.
anemia. Kernicterus — It may manifest itself by apathy
and poor feeding and later by mental retarda-
Erythroblastosis Fetalis
tion, irritability and cranial nerve palsies.
It occurs due to isoimmune antibodies. It is also
called as 'hereditary disease of newborn' (HDN). Oral Manifestations
Congenital hemolytic anemia due to Rh
It may manifest in teeth by the deposition of
incompatibility results from destruction of fetal
blood pigment in enamel and dentin of develop-
blood brought about by a reaction between
ing teeth giving them a green, brown or blue hue.
maternal and fetal blood factors. The Rh factor,
It occurs in those portions of the teeth which are
named after Rhesus monkey, was discovered by
being laid down during the time when icterus
Landsteiner and Wiener in 1940 as a factor in
was at its height.
human RBC which reacts with rabbit antiserum
Enamel hypoplasia is also reported in some
produced by administration of red bloods cells
cases of erythroblastosis fetalis and it usually
from Rhesus monkey.
involves the incisal edges of the anterior teeth and
middle portion of the deciduous cuspid and first
Pathogenesis
molar crown. There is ring like defect called as
It occurs due to inheritance, by the fetus, of a 'Rh hump'.
blood factor from the father that acts as a foreign
antigen to mother. The transplacental transfer Laboratory Findings
of this antigen, actually transplacental leak of red
The red blood count at birth may vary from less
cells, from fetus to mother results in immuni-
the 1,000,000 cells per cubic millimeter to near a
zation of mother and formation of antibodies.
normal level. The icterus index is very high and
When the fetal red cells cross placenta they may
may reach a level of 100 units. The peripheral
stimulate the production of maternal antibodies
smear shows large number of immature RBCs.
against the fetal antigens.
There is also evidence of hemolysis.
Some of these antibodies then cross into fetal
circulation and cause the destruction of fetal red
Pyruvate Kinase Deficiency Anemia
cells. If the father is Rh positive and the mother
is Rh negative, fetus inherits Rh-positive anti- When there is pyruvate kinase deficiency during
gens, which may act as antigen to the mother glycolysis in the RBC, there is discrepancy
and immunize her with resultant antibody for- between the ATP generating capacity of RBC and

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594 Textbook of Oral Pathology

energy needed, leading to loss of pliability which will lead to loss of cell membrane and gradually
result in distorted and rigid cells which are the cell becomes spherical in shape and is
ultimately destroyed by the spleen. destroyed by spleen, giving rise to hemolytic
anemia.
Clinical Features
There is anemia of varying degrees, jaundice and Clinical Features
splenomegaly or pronounced neonatal jaundice Symptoms are usually present in childhood.
is common findings. There is mild to moderate hepatosplenomegaly,
jaundice and anemia. A hemolytic and aplastic
Laboratory Findings crisis may be precipitated by infection or may
Anemia is normocytic and normochromic with occur with any cause. Such patients will have
reticulocytosis. Irregular form of RBCs may be shivering, fever, marked weakness, vomiting,
seen. Serum bilirubin levels are moderately ele- abdominal pain, dyspnoea and palpitation.
vated. Bone marrow hyperplasia also occur.
Hematological Findings
Treatment The peripheral smear shows presence of sphero-
Anemia is best managed by packed cell trans- cytes. Reticulocyte count is increased. Hb ranges
fusion. After the age of 5 to 10 years, splenectomy from 5 to 12 gm/dl.
is the treatment of choice.
Management
Glucose-6-phosphatase Deficiency Anemia Splenectomy is the treatment of choice, but it
should be done after the child is 6 years old. Folic
Glucose-6-phosphatase is required to maintain
acid 5 mg daily.
glutathione in the reduced state which protects
the RBCs from metha-hemoglobin formation.
Hereditary Elliptocytosis
Clinical Features It is a genetically determined abnormality of the
In normal state there is no anemia but acute red cell shape associated with variable degree
hemolysis is characterized by jaundice, pallor of hemolysis.
and dark urine with or with abdominal and back
pain. It remains asymptomatic unless RBCs are Clinical Features
subjected to injury by exposure to certain drugs Most of the patients have no clinical manifesta-
like anti-malarial, vitamin K, aspirin and certain tions but few may show signs of chronic hemo-
infection like Salmonella, E coli, etc. lytic anemia.

Hematological Findings Hematological Findings

Peripheral smear shows abnormal forms of RBC The peripheral smear shows larger number of
and reticulocytosis. Increase in WBC count. elliptocytes. Mild anemia of normocytic, normo-
chromic type.
Hereditary Spherocytosis
Erythropoietic Porphyria
In this disorder, red blood cells are excessively
permeable to sodium ion. The osmostic fragility Is a group of inherited or acquired disorders
of red cells is abnormal in these conditions. It characterized by excessive production, accumu-

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 Blood Dyscrasias 595

lation and excretion of some porphyrins and their There is passage of dark urine usually in morning
precursors or byproducts. It is a metabolic defect sample with subsequent samples of urine being
within the maturing erythrocytes resulting in clear.
excessive production of uroporphyrinogen-I.
Hematological Findings
Clinical Features
There are findings of hemolytic anemia in asso-
It occurs soon after birth. The urine is burgundy ciation with reticulocytosis. There may be leuco-
red in color or turns red on exposure to light. penia and thrombocytopenia.
Photosensitivity of exposed parts of body leads
to formation of blister and scars. There is also Management
hemolytic anemia and splenomegaly. Administration of alkali may control hemolysis
but on cessation of treatments massive hemolysis
Oral Manifestations may occur due to accumulation of sensitive cells
There is deposition of porphyrin in dentin, to a during the period of therapy.
lesser extent in the enamel which imparts red or
brown color to deciduous and permanent teeth Polycythemia Vera
(erythrodontia). The staining by uroporphyrin It is defined as abnormal increases in the number
can be confirmed by ultraviolet light which will of red blood cells in the peripheral blood, usually
produce fluorescence. with an increase hemoglobin level.
Bullous erosions of oral mucosa can be seen. It is also called as 'polycythemia rubra vera',
In advanced cases, pigmented atrophic scars on 'Osler's disease', 'Vaquez's disease' and 'erythremia'.
lip are seen resemble the keloid structures. The There is an uncontrolled proliferation of the
patient is hence, unable to close his mouth. erythroid stem cells leading to excess of erythroid
cell mass in the body (RBCs). There is accom-
Hematological Findings panying increase in the granulocyte and megak-
About 50% normoblasts, reticulocyte and 50% aryocytic elements though to a lesser degree.
RBCs exhibit intense red fluorescence under
fluorescence microscope. Clinical Features
The disease is more common in males and usually
Management occurs in middle age or later. Common symptoms
Splenectomy, in cases of severe cases, hemolysis are lassitude, loss of concentration, headache,
may be carried out. dizziness and blackout, slurring of speech,
pruritis, mental confusion and indigestion.
Paroxysmal Nocturnal Hemoglobinuria Paresthesia is common, usually involving the
cranial nerves. The skin appears flushed or
It is a very rare disease in which the red cells have diffusely reddened. Spleen is palpable in most
acquired susceptibility to antibodies which are
of the patients. Superficial veins are dark enlar-
normally in the blood.
ged and distended. The tip of the finger usually
has a cyanotic appearance. There is marked
Clinical Features
purplish red discoloration especially of the head
The patient has severe to moderate anemia and and neck, feet and hands, which give the patient
mild jaundice. The spleen is usually palpable. an extremely angry appearance. Thrombotic
596 Textbook of Oral Pathology

complications may occur and peptic ulcerations throat, stomatitis and regional lymphadenopathy
are common. as well as headache, arthritis, cutaneous infection
and conjunctivitis. Less frequently patient
Oral Manifestations experience lung, urinary tract infection as well as
rectal and vaginal ulcer. In some patients,
There is purplish red discoloration of the ears,
oral mucosa, gingiva and tongue. The tongue amyloidosis can occur which is due to repeated,
increased antigenic stimulation during
may appear as if it had been painted with crystal
neutropenic episodes. The symptoms are milder
violet. The gingiva is markedly swollen and
bleeds spontaneously, but with no tendency to as compared to agranulocytosis.
ulcerations. Petechiae of the oral mucosa are
Oral Manifestations
common. In such patients, severe hemorrhage
may follow surgical procedures. Severe gingivitis and painful ragged ulcers that
have a core like center are found on the lip,
Hematological Findings tongue, palate, gums and buccal mucosa which
heal after about two weeks with scarring. Isola-
The hemoglobin level is greater than 18 g/dl
ted painful ulcer may occur which correspond
with an associated elevation of WBC and platelet
to the period of neutropenia.
count. The bone marrow is hypercellular with
erythroid hyperplasia, increased number of
Hematological Findings
megakaryocytes and granulocytes.
Normal blood count, over a period of 4-5 days,
WHITE BLOOD CELL DISORDERS begins to show a decline in the neutrophil count
compensated by an increased in monocytes and
Cyclic Neutropenia lymphocytes. At the peak of the disease, the
It is also called as 'periodic neutropenia'. It is a neutrophils may completely disappear for one
rare disorder characterized by periodic or cyclic or two days.
diminution in circulating neutrophils due to
Management
failure of stem cells of bone marrow. It is also
called as periodic neutropenia. One-third cases There is no specific treatment and monitoring
are inherited as autosomal dominant trait and the patient for infection during neutropenic
2/3rd appear spontaneously during the first few period and vigorous early management of infec-
year of life. The patient is healthy between tion. In some cases use of corticosteroids, adreno-
neutropenic periods; but at regular intervals, the corticotropin (ACTH) or testosterone modulates
absolute neutrophils count falls below 500/mm3. sharp reduction in marrow function. Oral hy-
In some patient it comes to zero. giene should be maintained and patient should
be recalled for oral hygiene every 2-3 months.
Clinical Features
The disease is frequently present during infancy Lazy Leukocyte Syndrome
and childhood and both sexes appear to be It is a result of loss of chemotactic function of
equally affected. The frequency of neutropenic the neutrophil. The neutrophils present in the
episodes vary from once in 2 to 4 weeks which blood can not migrate to the site of tissue injury
last for 3 to 5 days with 21 days gap being the although phagocytic and bactericidal activities
commonest. The patients manifest fever, sore are normal.
 Blood Dyscrasias 597

Clinical Features of vitamin B12 and folic acid. Disease causing

It becomes apparent at the age of 1-2 years when sequestration of neutrophils includes systemic
complication occurs due to infections. The most lupus erythematosus and Felty's syndrome. It can
common clinical manifestations are stomatitis, be associated with leukemia, pancytopenia and
otitis media and bronchitis. hypersplenism.

Oral Manifestations Clinical Features

Gingivitis and stomatitis are common oral It can occur at any age but it somewhat more
finding of this disorder. In some cases periodon- common in adults, particularly in woman. It is
tal disease may be present. also common in professional and in hospital as
they have easy access of the offending drugs and
Hematological Findings often use drug sample injudiciously.
The total leukocyte count is slightly low but the The onset may be sudden or gradual. The
absolute neutrophil count is as low as 100 to 200 condition begins with sore throat, high fever and
cells/mm3. The bone marrow contains normal often rigors, which may be followed by prostra-
number of mature neutrophils. tion. The skin appears pale and anemic and in
some cases, jaundiced. There is rapidly advan-
Agranulocytopenia cing necrotic ulceration of throat and mouth with
It is also called as 'granulocytopenia', 'agranulocytic little evidence of pus formation. In most of the
angina'. It is a serious disease characterized by cases patient dies within 3-5 days due to toxemia
marked leucopenia with reduction and absence and septicemia.
of neutrophilic leucocytes.
Oral Manifestations
Types
The most common sites are gingiva and palate,
• Primary agranulocytosis — In this, etiology is tonsil and pharynx. There may be associated
unknown. pain, excessive salivation and spontaneous oral
• Secondary agranulocytosis — In it, cause is hemorrhage. The oral lesions appear as necroti-
recognized. zing ulcerations of oral mucosa, tonsils or/and
• Mild neutropenia — When 1000/mm3 to 2000/ pharynx.
mm3 neutrophils are present. The lesions appear as ragged and necrotic
• Moderate neutropenia — When 500/mm3 to and are covered with a gray black membrane.
1000/mm3 neutrophils are present. The necrotic tissue is often foul smelling. There
• Severe neutropenia — When fewer than 500/ is lack of inflammation at the margin of lesions.
mm3 neutrophils are present. The disease spreads quickly in gingival tissues
• Agranulocytosis — When no neutrophils are causing destruction of supporting structures and
seen in peripheral smear. inevitable loss of deciduous and permanent
teeth.
Causes
Hematological Findings
It is caused by certain drugs like aminophylline,
chlorpromazine and phenylbutazone, benzene, Majority of patients show a leukocyte count
bismuth, chloramphenicol, sulfonamides and use below 2000 cells per cu mm and granulocyte count
of cytotoxic drugs or antimetabolics. Deficiency below 100 cells per cu mm. Hemoglobin and
598 Textbook of Oral Pathology

platelet counts are normal. The histopathological Treatment

features of ulcer shows that reduce or absence of Immediate and proper treatment with antibiotics
neutrophils. It may show bacterial invasion. of the infection as soon as they occur is most
important. Vincristine, prednisolone and
Management
ascorbic acid have been tried as the treatment of
If hemoglobin is less than 10 g/dl, transfusion of this disorder.
red cell concentrate is given. In some cases, white
cell transfusion can be given. Septicemia can be Infectious Mononucleosis
controlled by parenteral antibiotic therapy along It is also called as 'glandular fever'. It is a benign
with corticosteroid therapy. A combination of acute infectious disease caused due to the
carbenicillin, methicillin and gentamicin is Epstein-Barr virus, a herpes virus which infects
commonly used because of broad coverage against the B-lymphocytes. It is commonly seen in
most organisms. adolescents and young adults. The mechanism
of human transmission is not entirely known but
Chédiak-Higashi Syndrome
one important mean is thought to be through
It is a congenital autosomal recessive defect of deep kissing so this condition is also called as
granulocytes and melanocytes. Abnormal 'kissing disease'. EBV is present in oropharyngeal
granules are seen in all blood granulocytes resul- secretions and mixed saliva during active phase.
ting in decreased chemotactic and bactericidal
activity, although phagocytosis remains intact. Clinical Features
It varies from 10 to 40 days and the disease occurs
Clinical Features chiefly in children and young adult in 15-30 age
The characteristic clinical feature of this disease groups. Anterior and posterior cervical nodes are
consists of oculocutaneous albinism, photo- enlarged and only slightly tender on palpation.
phobia, nystagmus and recurrent infections of The patient usually complaint of sore throat
the respiratory tract and sinuses. There may be accompanied by fever usually 101°F to 103°F and
neurological and gastrointestinal disturbances. extreme fatigability. Occasionally, there is a
The disease may be associated with malignant complaint of headache, photophobia, nausea,
lymphoma. vomiting, diarrhea and presence of erythema-
tous macular rash (Morbilliform skin rash).
Oral Manifestations Physical examination reveals enlarged
palatine tonsils with copious amount of cheesy
Ulcerations of the oral mucosa, severe gingivitis yellow exudate filling tonsillar crypt. Enlarge-
and glossitis are the common oral lesions. There ment of the superficial lymph nodes, particularly
may be rarely loss of teeth due to periodontal the posterior cervical and splenomegaly are
disease. common manifestations.

Hematological Findings Oral Manifestations

Hematological studies show presence of giant Tiny petechiae appear on the soft palate, labial
abnormal granules in the granulocytes in the and buccal mucosa during the course of the
peripheral blood as well as in their precursors disease. Acute gingivitis and stomatitis may be
in the bone marrow. present and the lesion normality persisting for
 Blood Dyscrasias 599

3 to 11 days. The organism responsible for gingi- Clinical Features

vitis is fusospirochetes.
Predominantly in females with some cases being
Intraorally, the most prominent sign is
familial. It is usually asymptomatic and free of
enlargement and inflammation of the tonsils
infection which is due to compensatory mono-
along with sore throat and difficulty in swallo-
cytosis, which accounts of normal number of
wing. Quite commonly, the tonsils are covered by
phagocytes at the site of tissue injury. Some pati-
a white or grayish pseudomembrane.
ents may exhibit recurrent bacterial infections
One third of the patients with hemorrhagic
like recurring upper respiratory tract infections,
tendency exhibit oronasopharyngeal bleeding,
otitis media, bronchitis and furunculosis.
including bleeding from gingiva. Transient oral
ulcerations and lymphadenopathy can also Oral Manifestations
occur.
Severe, rapidly advancing periodontal diseases.
Hematological Findings Gingivae appear intensely red with granuloma-
tous margins. Severe gingival recession, advan-
There is an absolute and relative increase in
ced bone loss, mobility, denuded roots and loss
mononuclear cells which exhibit pleomorphism of teeth. The second most common finding is
and an oval and kidney shaped nucleus with a
recurring oral ulcers and in some cases, maxillary
nongranular or foamy cytoplasm. The hemo-
sinusitis can also occur.
globin and platelet counts are normal. An in-
crease in white blood cell count and positive Laboratory Findings
‘Paul-Bunnel test’ are pathognomonic of this
The bone marrow of patients shows a normal
disease.
number of immature cells but decrease in num-
Diagnosis ber of mature neutrophils. This is called as matu-
ration arrest. Absolute neutrophils count may be
It is the most common, serologic diagnostic test below 500/mm3.
for infectious mononucleosis. Leukocyte count
is between 4000 to 15000/dl. Heterophill anti- Management
body test positive, atypical lymphocytes and It is not required if the patient is asymptomatic.
clinical signs and symptoms make a diagnostic
triad. BLEEDING AND COAGULATION DISORDERS
Idiopathic Thrombocytopenic Purpura
Management
It is also called as 'Werlhof's disease', 'purpura
It can be treated by antiviral drugs, corticosteroid, hemorrhagic' and 'primary thrombocytopenic
anesthetics.
purpura'. It is a disease in which there is an abnor-
mal reduction in the number of circulating blood
Chronic Idiopathic Neutropenia
platelets with normal or raised number of mega-
It consists of group of diseases which includes karyocytes in the bone marrow. It is thought to
familial neutropenia, chronic benign neutro- be an autoimmune disorder in which a person
penia, chronic neutropenia and hyperplastic becomes immunized and develops antibodies
neutropenia. Etiology is unknown, but they are against his own platelets. It can be a result of
characterized by a decrease in production of decrease in production of platelets or increased
neutrophils in the bone marrow. destruction of platelets or, both.
600 Textbook of Oral Pathology

Clinical Features

Thrombocytopenic purpura is characterized by

spontaneous appearance of purpuric hemorr-
hagic lesions of skin, which vary in size from
tiny red pin point petechiae to large purplish
ecchymoses and sometimes, even massive
hematoma. The patient also exhibits a bruising
tendency. Epistaxis, hematuria and melena are
common findings. Intracranial hemorrhage is
rare, but can be seen in children and the symp-
toms are headache, dizziness and confusion. Figure 30.3: Idiopathic thrombocytopenic purpura
showing hemorrhage

Oral Manifestations

The first manifestation of the disease can be seen

in oral cavity in the form of excessive bleeding
after tooth extraction. Submucus petechiae and
ecchymosis commonly occur especially at the
junction of the hard and soft palate (Figs 30.3
and 30.4). It appears as numerous tiny, grouped
clusters of reddish spots, only a millimeter or less
in diameter. Petechiae do not blanch on pressure
which is the distinguishing feature between
purpura and telangiectasia. In severe cases, Figure 30.4: Idiopathic thrombocytopenic purpura
showing purpic lesion on the soft palate
extensive spontaneous gingival bleeding may be
seen and this may form foci of secondary Thrombotic Thrombocytopenic Purpura
infection.
It is immunologically mediated. It is characteri-
Hematological Findings zed by occlusion of small arterioles and capilla-
ries of many organs by thrombi formed of fibrin
The platelet count is usually below 60,000 cells and platelets.
per cu mm. The bleeding time is prolonged but,
the clotting time is normal. The bone marrow Clinical Features
reveals megakaryocytic hyperplasia. When
It generally occurs in young adults and more
severe bleeding occurs, there may be associated commonly in females than in males. There is
iron deficiency anemia. thrombocytopenia, hemolytic anemia, fever, tra-
nsitory neurologic dysfunction and renal failure.
Management
Histopathological Features
Corticosteroids, splenectomy, transfusion, local
hemostatic agents should be given to control the There are widespread microthrombi in the
bleeding. arterioles, venules and capillaries in all tissues
 Blood Dyscrasias 601

and organs throughout the body. The intravas- Hematological Findings

cular thrombi are composed of loose aggregates
Prolonged bleeding time, normal platelet count,
or platelet that becomes organized into amor-
failure of aggregation of platelets impaired
phous plugs, which are often replaced by fibrin.
adherence of platelets and depressed levels of
This fibrin deposit more readily seen on PAS stain.
factor VIII are suggestive of this disorder. Low
AHG levels and abnormal platelet retention to
Management
glass beads. The platelet count and prothrombin
Corticosteroids, platelet aggregation inhibitors, consumption are normal. The clotting time usu-
splenectomy and exchange transfusion. ally is normal but capillary fragility is increased.

von Willebrand's Disease Management

It is also called as 'pseudohemophilia', 'vascular Bleeding episode is best controlled by transfu-

hemophilia' and 'vascular purpura'. It a is rare dis- sion of plasma or cryoprecipitation and by local
order which is inherited as an autosomal domi- control of homeostasis.
nant trait in which there is defect in all three
components of the hemostatic mechanism; the Bernard-Soulier Syndrome
capillaries, platelets and coagulation mechanism.
It is transmitted as an autosomal dominant trait
It is most common hereditary clotting disorder.
and is characterized by prolonged bleeding time
In it, there is quantitative and qualitative defi-
and defective aggregation of platelet. The only
ciency in larger molecule portion of factor VIII
treatment is platelet transfusion during acute
molecule. von-Willebrand's factor activity is
bleeding episodes.
responsible for platelet adhesion to subendo-
thelium, regulation of the plasma level of factor
Aldrich Syndrome
VIII coagulant activity and thereby normal
hemostatis. It is also called as 'Wiskott-Aldrich syndrome'.
It is x-linked recessive condition.
Clinical Features

Excessive bleeding either spontaneously or Clinical Features

following even minor trauma, is the chief feature It is characterized by thrombocytopenia, eczema,
of the disease. The most common sites of bleed- increased susceptibility to infection and a
ing are nose, skin and gingiva. Bleeding into the prolonged bleeding time. Patients commonly
gastrointestinal tract, epistaxis and severe manifest boils, otitis media, bloody diarrhea and
menorrhagia are also common. Bleeding tenden- respiratory infection. There is common occu-
cies usually appear early in childhood and rrence of malignant lymphoma, which is an
decrease in middle and old age. important feature of this disease. Bleeding occurs
from nose, skin and gastrointestinal tract.
Oral Manifestations
Oral Manifestations
Gingival bleeding and post-extraction bleeding
are the most common oral manifestations. The Palatal petechiae are frequently present. Spon-
disease may be discovered after dental extraction. taneous bleeding from the gingiva.
602 Textbook of Oral Pathology

Laboratory Findings Causes

Prolonged bleeding time and there is considerable Polycythemia and myeloid leukemia, anemia,
anisocytosis, alternation in the size and shape of tuberculosis and sarcoidosis. Hyperadrenalism,
platelets with most platelets smaller than normal. rheumatoid arthritis and bronchial carcinoma.
There is decreased production and defective
maturation of platelets since normal megakaryo- Clinical Features
cytes may be seen in the marrow.
It is a rare disorder of the elderly associated with
a tendency to bleed and to have thrombic episo-
Familial Thrombasthenia
des. Epistaxis, bleeding into the gastrointestinal
It is also called as 'Glanzmann's disease'. It is tract as well as bleeding into the genitourinary
hereditary, chronic hemorrhagic disease trans- tract and central nervous system is common.
mitted as an autosomal recessive trait. Hemorrhages in skin are also common.

Clinical Features Oral manifestations

There is excessive bleeding, either spontaneous Spontaneous gingival bleeding and excessive
or following minor trauma. Both sexes may be and prolonged bleeding after extraction of tooth
affected and onset of menarche may be a critical is also common.
event. Purpuric hemorrhages of skin are
common as are epistaxis and gastrointestinal Laboratory Findings
bleeding. Hemoarthrosis is also noted in some
cases. The platelet count is increased and there is abnor-
mal aggregation in response to several aggre-
Oral Manifestations gating agents. The clotting time, prothrombin
time are normal but bleeding time is frequently
Spontaneous bleeding from the oral cavity
prolonged.
particularly gingival bleeding.
Management
Laboratory Finding
Certain cytotoxic drugs, heparin, corticosteroid
Bleeding time is prolonged while clot retraction
and aspirin in small dose may help to counter
is impaired. Platelet count is normal as the clott-
act the thrombactive tendency. It responds to
ing time. The aggregation of platelet by epine- radioactive phosphorus.
phrine, ADP and thrombin is defective.
Hemophilia A
Management
It is a hereditary disorder of blood coagulation
No specific treatment but patient in oral surgery characterized by excessive hemorrhage due to a
can be given microfibriallar collagen preparation
prolonged coagulation time. Deficiency of factor
with fibrinolytic inhibitors.
VIII or anti-hemophilic factor is the cause of
hemophilia. It is transmitted as X-linked rece-
Thrombocytosis or Thrombocythemia
ssive character carried on X-chromosome. The
It is characterized by an increase in the number males are clinically affected and the females are
of platelets in the blood in excess of 1000 × 106/ carriers of the trait. Hemophilia A occurs 10 times
dl. It is of two types, i.e. primary and secondary. more commonly than hemophilia B.
 Blood Dyscrasias 603

Clinical Features Christmas Disease or Hemophilia B

Bleeding manifestations usually begin after 6 It occurs due to hereditary deficiency of factor IX
months of age, when the child begins to move or functionally defective factor IX. It is transmitted
about and tends to fall and sustain injuries, i.e.
as X-linked recessive character through
when spontaneous hemorrhage is noted by the
chromosome. It is very rare, compared to hemo-
parents. The most common manifestation is
philia A. Clinical features are same as hemo-
hemorrhage into joints which is spontaneous and
philia. In the laboratory hemophilia A may be
associated with warmth and muscle spasm.
differentiated from hemophilia B by modifica-
Repeated episodes cause damage to the joint
tion of the prothrombin consumption time or the
with wasting of the related muscle, leading to
partial thromboplastin time.
deformity and crippling. Hemorrhage into sub-
cutaneous tissues, internal organs and muscu-
lature also are frequent and potentially disabling Factor V Deficiency or Parahemophilia
complications. Superficial trauma gives rise to It is a rare hemorrhagic disorder, clinically simi-
uncontrolled bleeding. Intracranial bleeding is lar to hemophilia, caused by a deficiency of factor
relatively rare unless associated with trauma. V or proaccelerin.
Oral Manifestations
Clinical Features
Traumatic injury of the oral cavity may lead to
the diagnosis of hemophilia. The anatomic sites It is inherited as an autosomal dominant trait
involved in persistent oral bleeding are the affecting both sexes. Spontaneous epistaxis,
frenum of lip and the tongue. There is prolonged bleeding into the gastrointestinal tract and
bleeding after tooth extraction. Hematoma of the menorrhagia are common. Cutaneous ecchymo-
floor of mouth may occur and blood may spread sis and hamartomas are frequently seen although
via the fascial spaces and produce a hematoma petechiae are rare. Intraocular hemorrhage and
of the larynx, with consequent respiratory hemorrhage into the central nervous system
embarrassment. Physiological processes of tooth have been also reported.
eruption and exfoliation may be associated with
severe and prolonged hemorrhage. Gingival Oral Manifestations
hemorrhage is extremely rare and when it occurs,
it is the result of gingival injury. Spontaneous gingival bleeding occurs in some
cases and prolonged bleeding after extraction of
Hematological Findings tooth is observed.
Clotting time is prolonged, but however the
bleeding time, platelet count and prothrombin Laboratory Findings
time are all normal. The prothrombin consump- Both clotting and prothrombin time are prolon-
tion time and the partial thromboplastin time ged but, bleeding time is normal. The basic defect
may be prolonged in severe cases. is reduction in plasma proaccelerin.
Management
Management
Replacement therapy — Various forms of replace-
ment therapy are available like plasma, cryopre- Transfusion and freshly frozen plasma are given
cipitate and factor VIII concentrates. when there is excessive hemorrhage.
604 Textbook of Oral Pathology

Hereditary Hemorrhagic Telangiectasia giectatic areas are not pulsating. As the affected
individual grows, the bleeding episode tend to
It was first described by Osler in 1901 and it fre-
increase in frequency and intensity. They often
quently bears his name (Osler-Rendu-Weber syn-
drome). It is transmitted as an autosomal domi- give rise to profuse hemorrhage, such as episodes
nant trait and characterized by bleeding from of epistaxis. Severe bleeding may also occur from
mucus membrane and telangiectatic lesions on the gastrointestinal tract.
skin and mucosa.
Oral Manifestations
Clinical Features Oral cavity, lips and tongue are most commonly
They are found most commonly on the skin of the affected. Cherry red, often slightly raised, pin
face, finger, toes and on the oral mucus membrane. point or slightly larger lesions resembling a
The lesion may be present in childhood but more crushed spider is seen at any intraoral site,
often appear in puberty and become progressively especially at the lip. Severe oral hemorrhage may
worse with increasing age. The disease is be experience several times a day for weeks. At
characterized by multiple localized angiomatas times, there is gush of blood when involved areas
or telangiectases on the skin. The lesion bleeds are simply touched with cotton. Hemorrhage can
easily, even after slightest trauma. Bleeding is not be encountered during dental treatment which
caused by clotting factor deficiency but as a result encroach the affected area.
of rupture of the weak capillaries. The typical
lesion is cherry-red to purplish macule or small Management
papule that resembles a ‘crushed spider’. Sclerosing agent such as sodium morrhuate or
The lesion blanches on pressure and regains sodium tetradecyl sulfate injected into the lesion
it's color when the pressure removed. The telan- is useful.
 CHAPTER

31 Bone Pathology

Bone is a dense calcified tissue which is Classification

specifically affected by a variety of disease that 1st
often causes it to react in a dynamic fashion.
These diseases of bone may arise at any age; some Fibro-osseous lesions of medullary bone origin
are congenital and present at birth, while other • Fibrous dysplasia
• Fibro-osteoma
develop in early childhood or in young
• Cherubism
adulthood.
• Juvenile ossifying fibroma
• Giant cell tumor
FIBRO-OSSEOUS LESIONS • Aneurysmal bone cyst
• Jaw lesions in hyperparathyroidism
Normal bone is replaced by benign fibrous tissue • Paget’s disease.
showing varying amounts of mineralization. The 2nd
subject of benign fibro-osseous enlargement of
Fibro-osseous lesions of periodontal origin
the jaws has many facets, the most sinister of
• Periapical cemental dysplasia
which is the possibility that a healthy good
• Florid osseous dysplasia
looking person may take on a monstrous • Cemento-ossifying fibroma
appearance. • Cementifying fibroma
Fibroosseous lesion is not a specific diagnosis • Ossifying fibroma.
but a process. They are a diverse group of
processes that are characterized by replacement Fibrous Dysplasia
of normal bone by fibrous tissue containing a It arises from the bone forming mesenchyme in
newly formed mineralized tissue. Broadly they the spongiosa and develops by proliferation of
can be of the following categories: fibrous tissue. Lichtenstein in 1938 coined the
• Developmental or hamartomatous lesions term ‘fibrous dysplasia’. It is also called as
• Reactive or dysplastic lesions fibrocystic disease, osteitis fibrosa localisata, focal
• Neoplasms. osteitis fibrosa and fibro-osteodystrophy.
606 Textbook of Oral Pathology

Etiology Subclinical Fibrous Dysplasia

There is no general agreement as to the etiology Many a times an unsuspected lesion of fibrous
of lesion. It appears to have no familial, dysplasia comes to light accidentally on routine
hereditary or congenital basis. radiographic examination, without any clinical
evidence of the suspected disease. Such examples
Developmental—Jaffe and Lichtenstein
are termed as sub- clinical fibrous dysplasia.
considered it as a developmental anomaly
caused by aberrant activity in the bone forming Clinical Features
mesenchymal tissue. Most theories favor a
Monostotic fibrous dysplasia: Monostotic fibrous
developmental anomaly because the disease
dysplasia involves only one bone and presents
begins in early life and is active during the
no extra-skeletal effects, other than occasional
growth period.
pigmented skin lesions. Most frequent sites are
Endocrine disturbances—Sternberg and Joseph ribs, femur, maxilla and mandible. Fibrous
considered complex endocrine disturbances with dysplasia discovered in young patients, usually
local tissue susceptibility as the cause. in children younger than 10 years affecting both
the sexes equally.
Classification
Polyostotic fibrous dysplasia (Jaffe’s type):
1st Polyostotic fibrous dysplasia involves multiple
bones, with female to male ratio of 3:1. The skin
• Monostotic fibrous dysplasia—Only one bone
lesions consist of irregularly pigmented, light
is involved.
brown melanotic spots, described as ‘café au lait’
• Polyostotic fibrous dysplasia—More than one
spot. The most common site for these are the
bone is involved.
back, buttocks, thighs, shoulders, chest, neck and
• Jaffe’s type—Fibrous dysplasia involving
face in the mentioned order.
variable number of bone, accompanied by
Recurrent bone pain is the most common
pigmented lesions of the skin or ‘café au lait’
presenting skeletal symptom. Skeletal lesions
spots.
may be unilateral in distribution or may involve
• Albright’s syndrome—A severe form of fibrous
nearly all bones of the body. Spontaneous
dysplasia involving nearly all the bones in
fracture is a common complication. In rare cases,
the body, accompanied by pigmented lesions
continuous and inexorable extension result in
of the skin plus endocrine disturbances of
great deformity and blindness.
various types.
Albright’s Syndrome
2nd
Albright’s syndrome, in addition show,
According to Stewart endocrinal disturbances like precocious puberty,
• Monostotic—Indicates involvement of a single goiter, hyperthyroidism, hyperparathyroidism,
bone. Cushing’s syndrome and acromegaly. Albright’s
• Monomelic—It refers to the involvement of syndrome is exclusively found in females.
one extremity and is rarely found. Vaginal bleeding has been noted. Secondary
• Polyostotic—Many bones involved. sexual characteristics such as pubic and axillary
• Albright’s syndrome hair and development of breasts are evident by
 Bone Pathology 607

the age of 5 years. It may result in crippling often remain impacted and may affect the
deformities or fracture. Precocious puberty is rare eruption of normal teeth.
in boys and is manifested as gynecomastia. Long
bones are frequently affected. Bone and skin Craniofacial Fibrous Dysplasia
lesions found in polyostotic form are unilateral.
If fibrous dysplasia extends to involve the
Skeletal lesions become static with the cessation
maxillary sinus, the zygomatic process, floor of
of growth but proliferation may continue,
orbit and sometimes toward the base of the skull,
particularly in the polyostotic form.
it is known as craniofacial fibrous dysplasia.
Oral Manifestations It results in severe malocclusion and marked
Monostotic: Maxilla is more commonly affected facial deformity. Craniofacial lesions may lead
than mandible, with most changes occurring in to anosmia (loss of sense of smell), deafness and
the posterior region. Most common area involved blindness. There may be proptosis of the affected
is premolar-molar area. There may be unilateral eye.
facial swelling, which is slow growing with intact
overlying mucosa (Fig. 31.1). Swelling is usually Polyostotic (Jaffe’s type)
painless but patients may feel discomfort in some Expansion and deformities of jaws. The eruption
cases and while others complain of frank pain. pattern of teeth is disturbed because of loss of
Enlarging deformities of alveolar process mainly support of the developing teeth. Asymmetry of
buccal and labial cortical plates. In mandible, it facial bones. There is ballooning of jaws, so there
causes protruberant excrescence of the inferior
is gross enlargement and deformity. In some
border of mandible.
cases, intraoral pigmentation can be seen.

Laboratory Examination
Serum calcium and serum phosphorus
concentration, as well as serum alkaline
phosphatase activity usually are within normal
limits. But in rare instances, particularly when
the polyostotic lesions are numerous and active,
the serum alkaline phosphatase levels may be
elevated in 50% of the cases.

Histopathological Features
Figure 31.1: Fibrous dysplasia showing swelling in the palate
Monostotic fibrous dysplasia: The lesion is
The teeth present in the affected area are either essentially a fibrous bone made-up of
malaligned and tipped or displaced. Dental proliferating fibroblast in a compact stroma of
anomalies such as supernumerary teeth have been interlacing collagen fibers. Irregular trabeculae
reported in connection with the monostotic of bone are scattered throughout the lesion, with
fibrous dysplasia. The most commonly affected no definite pattern of arrangement. Some of these
site is maxillary midline and mandibular trabeculae are C-shaped and described as
premolar region. These supernumerary teeth Chinese character shaped (Fig. 31.2).
608 Textbook of Oral Pathology

These trabeculae are usually woven bone, but coarse, woven bone, irregular in shape but
may be lamellar. There is a permanent evenly spaced, showing no relation to functional
maturation arrest in the woven bone stage and pattern (Fig. 31.3). The osteocytes are quite large
it was proposed that lesion demonstrating and collagen fibers of these trabeculae can often
lamellar bone transformation should not be be seen into the fibrous tissue (Fig. 31.4). These
diagnosed as fibrous dysplasia. trabeculae have typically wide osteoid seams.
Osteoclastic activity can may be seen where the
calcification of osteoid extends to the surface of
the trabeculae.

Management
Surgical removal of lesion: Osseous contouring — It
is necessary for correcting the deformity for
esthetics or pre-esthetic purposes.

Figure 31.2: Chinese letter pattern bony trabeculae

seen in fibrous dysplasia

Figure 31.4: Fibrous dysplasia

(Courtesy: Dr Sangamesh Halawar)
Cherubism
It is also known as ‘familial fibrous dysplasia of
the jaws’, ‘disseminated juvenile fibrous
dysplasia’, ‘familial multilocular cystic disease
of the jaws’ and ‘Hereditary fibrous dysplasia of
the jaws’. The clinical entity was first described
by Jones in 1933 who coined the term
‘Cherubism’ reflecting the characteristic chubby
facial appearance of affected children. It is
Figure 31.3: Fibrous dysplasia autosomal dominant inherited fibro-osseous
disease that affects only the jaws causing bony
Polyostotic Fibrous Dysplasia
expansion.
The lesions are composed of fibrillar connective Cherubism is a rare hereditary develop-
tissue within which are numerous trabeculae of mental condition, manifested in childhood by
 Bone Pathology 609

bilateral enlargement of jaws giving a “cherub” Clinical Features

like appearance to the face. “Cherubs” are Early childhood between the ages of 2 to 4 years
referred to the plumped cheek little angels in the males are affected about twice as frequently as
paintings of Renaissance. females. It shows predilection for angle of
mandible bilaterally and occasionally posterior
Classification maxilla (Fig. 31.5).
It depends on the severity and location of the In the rapidly increasing stage, the child
lesion and the extent to which jaws are affected. assumes a chubby, cherubic facial appearance
• Grade I: The fibro-osseous expansion tends to (Fig. 31.6), especially if combined with involve-
be bilateral and symmetrical. It is primarily ment of the orbital floor with upward displace-
in the ramus of the mandible. ment of the globe and exposure of the scleral
rims. The patient may have difficulty in speech,
• Grade II: In more severe cases, the ramus and
deglutition, mastication, respiration and limited
the body of the mandible are involved
jaw movement.
resulting in congenital absence of the third
Bilateral enlargement of mandible in this
and occasionally the second mandibular
condition produces full, round lower face.
molar teeth. In this group, the tuberosity
Bilateral enlargement of maxilla gradually
region of the maxillae are also affected. follows.
• Grade III: In these cases, the lesions affect the
mandible and maxilla entirely and may result Eye raised to heaven: Pulling or stretching of skin
in considerable facial deformities. of the cheek, depresses the lower eyelid, exposing
a thin line of sclera and resulting in the so called
“eyes raised to heaven” look.
Tumor encroachment on the orbital floor
often causes partial obliteration of the palatal
vault with resulting ‘V’ shaped cleft.
Swelling is firm and hard on palpation.
Overlying mucosa is intact and non-painful. The
alveolar process are so wide, so as to occupy
almost the whole of the roof of the mouth; the

Figure 31.5: Cherubism showing swelling intraorally

Etiology
It is caused by anomalous development of dental
structures, Latent hyperparathyroidism,
a hormone dependent benign neoplasm,
trauma, an aberration in ossification, and
disturbance in the development of bone forming
mesenchyme. Figure 31.6: Chubby face appearance seen in cherubism
610 Textbook of Oral Pathology

actual palate being reduced to a narrow fissure

between the two approximating alveolar process.
There may be enlargement of sub-
mandibular lymph nodes. There is rapid increase
in size up to 7-8 years of age, after which the
lesions become static or progress very slowly
until puberty. After puberty the maxillary lesions
tend to regress. The mandibular lesions progress
slowly up to the age of 20 years and then regress;
the facial appearance almost returns to normal
in the 4th and 5th decades of life.
The fibrous replacement of bone displaces the
deciduous dentition. The primary teeth may be
irregularly spaced and some may be absent.
There is premature loss of primary teeth.The
developing permanent teeth are affected, giving
rise to displaced unerupted or absent teeth along Figure 31.7: Cherubism
with malocclusion. (Courtesy: Dr Sangamesh Halawar)

Laboratory Findings Management

Serum calcium, phosphorus and alkaline Surgical procedures should be delayed, as long
phosphatase levels are within normal limits. But as possible, as the cystic lesion defect usually
in active cases, serum alkaline levels may be becomes static and regresses during adulthood.
raised. Occasionally, surgical contouring of the lesions
is necessary to improve esthetics and in case of
Histopathological Features active growth.
Histopathology is characterized by similar
picture as that of central giant cell granuloma. Central Giant Cell Granuloma
Vascular fibrous tissue consisting of giant cells. It is a non-neoplastic bone disease reactive to
The giant cells are small and aggregated focally. some unknown stimulus. It was first described
In some cases, perivascular cuffing by the in the jaws by Waren 1837. It has been called as
eosinophils may be seen. osteoclastoma, myeloid sarcoma, chronic
It is characterized by presence of great hemorrhagic osteomyelitis and giant cell
number of large multinucleated giant cell in a reparative granuloma.
loose delicate fibrillar connective tissue stroma
containing large number of fibroblast and small Clinical Features
blood vessels (Fig. 31.7). Sprinkling inflammatory Lesion of adolescent and young adult with 60%
cells can be found in this condition. Epithelial cases in younger than 20 years and 74% cases in
remnants of developing teeth are sometimes younger than 30 years. Mandible is twice as
scattered throughout the lesion. Perivascular frequently involved than maxilla, with anterior
eosinophilic cuffing of small capillaries in the half showing greatest incidence with fairly high
lesion is sometimes found. percentage crossing the symphysis. The
 Bone Pathology 611

Figure 31.9: Central giant cell granuloma

Figure 31.8: Central giant cell granuloma giant cells

commonest site being the anterior and bicuspid

region in mandible and the canine fossa and
ethmoid region in maxilla.
The earliest sign of the lesion may be
expansion of bone with premature loosening and
shedding of deciduous teeth. There is jaw
swelling associated with facial asymmetry.
Usually painless, but local discomfort may be
noted. Palpation may elicit tenderness. Growth
is slow. Teeth in the area may become mobile
but maintain their vitality, until they are
exfoliated.
Figure 31.10: Giant cell granuloma
Histopathological Features (Figs 31.8 to 31.11)
It is made up of a loose fibrillar connective tissue
stroma with many interspersed proliferating
fibroblast and small capillaries. Multinucleated
giant cell are prominent through the connective
tissue which may vary in size from case to case
and may contain several dozens nuclei.
There are also numerous foci of old
extravasated blood and associated hemosiderin
pigment. The majority of giant cells contain
round nuclei but, some cells may have pyknotic
appearance.

Management
It is treated by enucleation, curettage and partial Figure 31.11: Giant cell granuloma showing few giant
resection cells and bony trabeculae
612 Textbook of Oral Pathology

Paget’s Disease involved jaw. Movement and migration of

It is also called as ‘osteitis deformans’. It was affected teeth, malocclusion and in edentulous
discovered in 1877 by Sir James Paget. There is patient poor fit of denture. Increase in alveolar
abnormal resorption and apposition of bone in width associated with flattening of palate when
one or more bones. The disease is initiated by an maxilla is involved. As the disease progresses
intense wave of osteolytic activity with the mouth may remain open exposing the teeth
resorption of normal bone resulting in an as the lips are too small to cover the enlarged
irregularly shaped resorption cavity followed by jaws.
vigorous osteoblastic activity forming woven Extraction sites heal slowly and incidences
bone after variable period. of osteomyelitis are higher. Extraction may be
further complicated by excessive bleeding from
Clinical Features
highly vascular abnormal bones in the lytic phase
It is seen most frequently in Britain and less of disease.
frequently in North America and Western
Europe. It occurs predominantly in patients over
Laboratory Investigations
40 years of age with a slight predilection for men.
Male to female ratio is 2:1. It is prone to occur in Serum alkaline phosphatase level is increased,
the axial skeleton especially the skull, femur, occasionally, attending the level of 200 or more
sacrum and pelvis. KA units. Serum calcium and serum phosphorus
First complains may be that patient needs to levels are within normal limits. Urinary
buy a hat of larger size because of skull hydroxyproline levels increase from normal
enlargement. Bone pain is a consistent symptom levels of 440 mg/24 hours to 1 gm/24 hours.
and most often directed towards weight bearing
areas. Deafness occurs due to involvement of the Histopathological Features
petrous portion of temporal bone with
compression of cochlear nerve in the foramen. Collagen fibers within the lamellar bone loose
Mental disturbance, dizziness also occur. The their normal orientation and run in many
patients may have ill defined neuralgic pain as a directions. Normal hematopoietic marrow is
result of restriction of foramina and canals, which replaced by cellular large vascular channels
occurs due to pressure created by mass on resulting in marked increase in vascularity.
structures passing through the foramina. A highly fibrous connective tissue replaces
Bowing of legs, curvature of spine and the marrow. Basophilic reversal lines are seen
enlargement of skull occur in some cases. The in the bone. These lines indicate the junction
involved bones are warm to touch because of between the alternating resorptive and formative
increased vascularity and are prone to fracture. phases of bone and result in “jigsaw puzzle” or
Grotesque facial appearance caused by bony “mosaic pattern” of bone (Fig. 31.12).
deformities. Broadening and flattening of the One of the most characteristic features of this
chest and spinal curvature. The patient assumes bone if the formation of ‘mosaic’ bone, a
waddling gait. descriptive term used to indicate the appearance
of bone which has been partially resorbed and
Oral Manifestations
then repaired, leaving deeply staining
Maxilla is involved three times more commonly hematoxyphlic reversal lines. Osteoclasts
than mandible. It is bilaterally symmetrical in the increase in size and number and are
 Bone Pathology 613

Florid Osseous Dysplasia

It is also called as ‘gigantiform cementoma’,
‘chronic sclerosing osteomyelitis’, ‘sclerosing
osteitis’, ‘multiple enostosis’ and ‘sclerotic
cemental masses’. It is considered to be a
widespread form of periapical dysplasia. It is
derived from the cells in or near the periodontal
ligament space.
Robinson defined it as, an abnormal reaction
of bone to irritation or stimulation and the term
florid osseous dysplasia includes chronic diffuse
sclerosing osteomyelitis and sclerotic cemental
Figure 31.12: Paget’s disease showing reversal line
masses. It is inherited as an autosomal dominant
trait.

Clinical Features
Females are exclusively affected. Most common
age is middle age, with a mean age 42 years with
predilection for blacks. The lesion is restricted
to the jaw bone with mandible being most
commonly affected.
There is a painless expansion of the alveolar
process of mandible. Patients may complain of
intermittent poorly localized pain in the affected
bone area, with or without an associated bony
Figure 31.13: Paget’s disease
(Courtesy: Dr Sangamesh Halawar) swelling. Mucosal ulceration with fistulous tract
may be present. Teeth are vital.
morphologically abnormal. Later, in the disease
process osteoblastic activity increases while Histopathological Features
osteoclastic activity decreases and the bone
becomes sclerotic. Inflammatory edema of Presence of homogenous dense calcified masses.
marrow is common and focal collection of Cementum may be seen.
lymphocytes may be observed (Fig. 31.13). The
proliferation of bone and concomitant hyper- Management
cementosis result in obliteration of periodontal If teeth present effective oral hygiene should be
ligament. maintained since with this disease, patients
exhibit poor healing and osteomyelitis may
Management
develop after tooth loss.
Calcitonin, a parathyroid hormone antagonist Ossifying fibroma, cementifying fibroma and
produced by the thyroid gland, suppresses bone cemento-ossifying fibroma:–
resorption and also relieves pain and decrease These are characteristically encapsulated
serum alkaline. neoplasms consisting of highly cellular fibrous
614 Textbook of Oral Pathology

tissue in which bone formation occurs. They may Cementifying fibroma: It may occur at any age, but
show a locally aggressive behavior. There is it is more common in the young and middle aged
remarkable similarity in clinical and radiological adults with a mean age of 35 years. Female are
features of these lesions. There is also considerable affected more commonly than males by ratio of
similarity and even overlap in histological features 2:1. There are mild deformities and displacement
of these lesions. For this reason it is suggested that: of teeth. It is slow growing. Cortical plates of
• They are three separate/distinct benign bone and overlying mucosa or skin are almost
tumors identical in nature, except for the cells invariably intact.
undergoing proliferation.
• The osteoblasts with bone formation in Cemento-ossifying fibroma: Pain and paresthesia
ossifying fibroma, cementoblasts with may result from pressure on adjacent nerves. The
cementum formation in cementifying lesion grows spherical in shape buccally and
fibroma and tumors characterized by both lingually causing bulging of lower border of the
types of cells, but probably the same mandible. Cortical erosion is rare.
progenitor cells, thus giving rise to the well
Histopathology Features
recognized hybrid form of the tumor, i.e. the
cemento-ossifying fibroma. Ossifying fibroma: Large number of fibroblasts,
• It is understood that, these are three sub- with flat elongated nuclei are present within the
classifications of an otherwise identical lesion. network of interlacing collagen fibers.

Clinical Features
Ossifying fibroma: It is a rare neoplasm and occurs at
any age, but usually is found in the young adult with
females more commonly affected than males. It
shows a predilection for premolar-molar area of the
mandible. Mostly, in facial bones in mandible and
in maxilla, it is often located in the canine fossa and
zygomatic arch. In some cases, there may be
involvement of the anterior base of skull and of
temporal bone.
Occasional facial asymmetry is seen in some
of the cases. When in maxilla, symptoms may
include nasal stuffiness and epiphora on the Figure 31.14: Ossifying fibroma showing Chinese
shaped island of bone
affected side. There may be associated
exophthalmos, with visual disturbances, Chinese letter shaped islands of bone or
depending on the extent of compression of its calcification distributed throughout the
orbital content by the tumor. The lesion is slow
connective tissue. As the lesions mature, the
growing and in some cases, there is displacement
islands of ossification increase in number, enlarge
of teeth. Bony cortex and covering mucosa
and ultimately coalesce (Figs 31.14 and 31.15).
remain intact. The lesion may be slow growing
initially, with a rapid increase in size in a Cementifying fibroma: Cementifying fibroma is
relatively short time. If sinus is affected it may composed of cellular fibrous connective tissue
fill the sinus completely and expands the sinus that contains cementum like spherules. It is
wall. composed of many delicate interlacing collagen
 Bone Pathology 615

ultimately becomes a solid block of calcified

cartilage leaving inadequate space for
hemopoiesis. It is characterized by low bone
mass and micro architectural bone fragility. It is
usually rarefaction of bone resulting from
deficiency of bone matrix rather than deficit
mineral.

Types
• Primary (associated with aging).
• Secondary (reduction of histologically normal
bone that has been accelerated by abnormal
Figure 31.15: Ossifying fibroma
(Courtesy: Dr Sangamesh Halawar) or iatrogenic circumstance such as
corticosteroid or heparin therapy or condition
fibers arranged in discrete bundles, interspersed
such as malnutrition and scurvy).
by large number of active, proliferating
fibroblasts or cementoblast. The connective
Mechanism of Bone Loss
tissue characteristically presents many small foci
of basophilic masses of cementum like tissue It is caused by imbalance between bone
(Fig. 31.16). resorption and bone formation with an
These are irregularly round, ovoid or slightly exaggeration of resorption, reduction in bone
elongated, often lobulated. As the lesion formation or combination of both.
matures, the islands of cementum increase in Postmenopausal and senile: It occurs due to overall
number, enlarge and ultimately coalesce. decrease in anabolic hormones (estrogen) in
postmenopausal women. There may be lag in
formation of bone and since bone resorption is
continued, results in osteoporosis. After the age
of 60 years, generalized atrophy of bone occur
(senile osteoporosis). Senile osteoporosis is
caused by decrease in calcium absorption,
vitamin D absorption and metabolism with age,
which in turn decreases the anabolic hormones,
muscular proteins and flow of blood to bone. By
virtue of this condition, bone resorption occurs.
Another cause of bone resorption in adults is
that, in elderly person altered hormonal function
lead to formation of small thrombi which plug
Figure 31.16: Cementifying fibroma
small vessels in bone and cause loss of bone
(Courtesy: Dr Sangamesh Halawar)
vitality and hence resorption.
Osteoporosis Cushing’s syndrome: The symptoms are caused
There is reduction in the inorganic constituent. by an increased output of glucocorticosteroids,
There is abnormal persistence of calcified especially cortisol. The excess cortisol acts to
cartilage. Spongy portion of affected bone produce osteoporosis by two ways:
616 Textbook of Oral Pathology

• It contributes to the degradation of proteins Clinical Features

and severely limits the formation of bone It is either present at birth (congenital) or
matrix by reducing the amount that each developed in early life (infantile). Bones are
osteoblast synthesizes. fragile and susceptible to fracture. These
• It promotes the formation of osteoclasts from fractures typically affect the forearm (colle’s
the osteogenic undifferentiated cells and thus fracture), spine (vertebral fracture) and hip
enhances bone resorption.
(femur fracture).
Drug-induced osteoporosis: Prolonged Congenita form inherited as an autosomal
administration of cortisol and cortisone shows recessive disorder is invariably fatal in early life
Cushing like syndrome and cause osteoporosis due to massive hemorrhage, anemia and rampant
in the same way as that in Cushing syndrome. bone infections occurring due to progressive loss
Contraceptive drug can also cause osteoporosis; of bone marrow and their cellular products.
as after administration of contraceptive drug, Percussion over the affected vertebrae is painful.
there is increase in level of cortisol. Combination Osteoporotic patient may notice gradual loss
of these two compounds leads to production of of height due to shortening of trunk. In advanced
abnormal megakaryocytes, which in turn leads cases, clinical onset is often characterized by
to formation of abnormal sticky platelets. These attack of severe pain which is aggravated by
sticky platelets fuse to form thrombi which movements and occurs after trauma.
occlude small vessels in the tissue due to which Osteomyelitis may occur due to relatively
the bone dies and get resorbed. avascular and there may be bone pain.
Malnutrition state: Sufficient protein must be
Oral Manifestations
absorbed from intestine to supply constant need
for matrix formation. Deficiency causes Marked predilection for development of
osteoporosis and may result from protein poor osteomyelitis. Fracture of jaws during tooth
diet or from GIT disturbances such colitis, regional extraction. Enamel hypoplasia. Microscopic
arteritis. Vitamin C deficiency may weaken dentinal defect. Arrested root development,
sinusoidal vessel walls in medullary bone which retardation of tooth eruption. Delayed eruption
tend to dilate and rupture, resulting in pooling of and early loss of teeth, missing teeth and
blood and hypoxia, which leads to loss of vitality malformed teeth. Teeth are poorly calcified and
and removal of bone by physiologic osteoclast. are prone to caries.
There is interaction between the progesterone
compounds and increased level of cortisol by Laboratory Findings
stress, leading to formation of multiple small Patients manifest anemia due to replacement of
thrombi. The thrombi occlude small vessels of hemolytic marrow by bone. Hepatomegaly may
osseous tissue, which results in death and be present. RBC count below 1,000,000 per cubic
resorption of bone, leading to osteoporosis. meter. Increased serum phosphatase levels.
Thyrotoxic osteoporosis: It is usually seen in
children with hyperthyroidism. Thyroxin Histopathological Features
mediates the action of cortisol on bone and an Endosteal production of bone with an apparent
excess of thyroxin results in a more efficient concomitant lack of physiologic bone resorption.
utilization of steroids. Thus, there is increased Osteoblasts are prominent but osteoclasts are
resorption. seldom found. Trabeculae are in disorder
 Bone Pathology 617

arrangement. Marrow tissue is fibrous. Bone is Oral Manifestations

markedly deficient in collagen matrix.
Mandible is one of the commonest bones affected
Infantile Cortical Hyperostosis in infantile cortical hyperostosis. Patient may
have malocclusion. Enamel hypoplasia may be
It is also called as ‘Caffey’s disease’, ‘Caffey- present. Residual asymmetric deformity of
Silverman syndrome’. It was first described in mandible, usually in the angle and ramus area.
detail in 1945. It is characterized by unusual
cortical thickening of certain bones and it occurs Laboratory Findings
in two forms, i.e. autosomal dominant form and Anemia, leukocytosis, elevated erythrocyte
sporadic form. sedimentation rate, monocytosis and increased
serum alkaline phosphatase levels.
Etiology
It may be an embryonic osteodysgenesis Osteopetrosis
consequent to local defect in blood supply to the It is also called as ‘Marble bone disease’, ‘Albers-
area. Inherited defects of arterioles supplying the Schonberg disease’, ‘osteosclerosis fragilis
affected part results in hypoxia producing focal generalisata’. It is a rare disorder characterized
necrosis of overlying soft tissue and periosteal by an increase in density of bones, which
proliferation. becomes hard and brittle. This is a result of
Allergy as the basis of disease, edema and endosteal bone production with a lack of
inflammation producing periosteal elevation and concomitant bone resorption and remodelling.
subsequent deposition of calcium.
Hereditary being an autosomal dominant Clinical Features
trait.
Almost every one has a varying degree of poor
Clinical Features skeletal development, if the disease appears early
It is equal in males and females, with average in life. Subsequent skeletal deformities occur
onset at 9 weeks of age, with most of the cases later due to bending of the long bones and
arising before 6 months. Bones commonly improper healing after pathological fractures. As
affected are mandible, clavicle, scapula, frontal a result of continuous bone deposition and lack
bone and ulna. There is bilateral involvement in of bone resorption, the foramina of cranial nerves
every case. Of all these bone, clavicle and are constricted, hence there is loss of hearing,
mandible are the most common sites. Infants will disturbed vision, which diminish progressively.
develop fever and become hyper-irritable. Facial nerve palsy is also seen. Due to
Soft tissue swellings have a sudden onset, displacement of hematopoeitic bone marrow
especially in the facial area and early in disease, anemia ensues and the hematopoeitic function
they may be warm and tender. Swelling is devoid is assumed by the liver, spleen and the
of clinical signs of inflammation on the overlying lymphnodes resulting in hyperplasia of
skin. Swelling disappears slowly, but may lymphoid tissues and hepatosplenomegaly.
suddenly recur at the same place or new site may There is also frontal bossing, obliteration of
be involved. Other features are pseudoparalysis, maxillary sinus and possible hydrocephalus and
pleurisy and dysphasia. mental retardation.
618 Textbook of Oral Pathology

Oral Manifestations Types

Osteomyelitis of jaws associated with • Congenital or Vrolik’s type: It is present at birth.
osteopetrosis probably follows the obliteration • Tarda or Lobstein’s type: It is recognized later
and fibrosis of marrow is caused by reduced in life. It is also called as osteopsathyrosis.
osseous circulation. It is most common in
Clinical Features
mandible and occasionally, in maxilla. Paranasal
sinus may become readily obliterated. There may Many infants with this disease are stillborn or
be enamel hypoplasia, defective dentine, die shortly after birth. Extreme fragility and
disturbed tooth development, small pulp and porosity of bones with an attendant proneness
tendency towards caries. of fracture. Fracture heals readily but new bone
is of similar imperfection. It is common for
Laboratory Findings fracture to occur while the infant is walking or
crawling. Hyperplastic callus formation, which
Red blood cell count below 1,000,000 cells per cu
may mimic osteosarcoma, take place.
mm. The serum calcium and phosphate levels
There is occurrence of pale blue sclera
are usually within normal limits. Elevated serum
which is thin; pigmented choroids show
acid phosphate levels have been reported in
through and produces the blue color.
patients with benign dominant osteopetrosis.
There is deafness due to osteosclerosis; laxity
of ligament and peculiar shape of the skull. There
Histopathological Features
is also abnormal electrical reaction of muscle.
It is characterized by the endosteal production Increased tendency for capillary bleeding.
of bone, with an apparent concomitant lack of
physiologic bone resorption. Osteoblasts are Oral Manifestations
prominent, but osteoclasts are seldom found. The
Most of the times, osteogenesis imperfecta is
predominance of bone formation over resorption
associated with dentinogenesis imperfecta.
typically leads to the persistence of cartilage cores
of bony trabeculae, long after their replacement Some times, there is also hypoplasia of teeth.
should occurred in endochondral bones. The Sometime there is class I malocclusion and
trabeculae are disorderly in arrangement and the greater incidence of impacted 1st and 2nd
marrow tissue present is usually fibrous. molars. Deciduous teeth are poorly calcified and
semitranslucent or waxy. Appearance of teeth
Management is faint dirty pink, half normal size, with globular
crowns and relatively short roots in proportion
No effective treatment. Avoid major surgery in to other dimension.
patients with osteopetrosis. Performing dental
extraction has a risk of osteomyelitis and jaw
Clinical Types
fracture.
Neonatal lethal type: It is characterized by multiple
Osteogenesis Imperfecta fractures in infants and the child seldom
survives.
It is also called as ‘brittle bone’, ‘Lobstein disease’.
It is a serious disease of unknown etiology. It Severe nonlethal type: Disease is not evident until
represents a hereditary autosomal dominant late childhood and patient shows fracture of
trait. bone with minimum trauma. Although the
 Bone Pathology 619

fractured bone heals up rapidly, considerable Management

skeletal deformity and dwarfed stature often Breathing support and feeding assistance are
develop. necessary during infancy. Surgical closure and
Moderate and deforming type: It is associated with orthodontic treatment are indicated.
dentinogenesis imperfecta and blue sclera.
Marfan’s Syndrome
Mild and nondeformity type: The patients are
clinically normal, but they have increased It is also called as ‘Marfan-Achard syndrome’,
tendency for bone fracture, due to trauma. ‘arachnodactyly’. It is hereditary disease
transmitted as autosomal dominant trait. It is
Histopathological Features basically a disease of connective tissue related
It exhibits thin cortices, some times being to defective organization of collagen which is
composed of immature spongy bone, while the abnormally soluble.
trabeculae of cancellous bone are delicate and Clinical Features
often show microfractures. Osteoblastic activity
Excessive length of the tubular bones resulting
appears retarded and imperfect and for this
in disproportionately long thin extremities, the
reason thickness of long bones is deficient. There finger and toes are long, thin and tapering so
is failure of fetal collagen to be transformed into that the name ‘spider finger’ has been applied.
mature collagen. Progressive intermolecular The shape of face and skull is characteristically
cross-linkage of adjacent collagen molecule is long and narrow. Hyperextensibility of joint with
only sometimes evident, which a characteristic habitual dislocations, kyphosis or scoliosis and
of normal collagen maturation. flatfoot.
Bilateral ectopia lentis: It caused by weakening or
Pierre-Robin Syndrome
rupture of the suspensory ligaments.
It is also called as ‘Robin anomalad’. It result Cardiovascular complications like aortic
arrested development. aneurysm and aortic regurgitation, valvular
defects and enlargement of the heart are
Clinical Features common.
It consists of cleft palate, micrognathia and
glossoptosis. Arrested development and ensuing Oral Manifestations
hypolasia of mandible ultimately produce the High-arched palatal vault is very prevalent. Bifid
characteristic bird facies. Mandibular hypoplasia uvula, malocclusion and multiple odontogenic
yields a retrognathic appearance. Due to this, cysts of maxilla and mandible. There may be
there is abnormal descent of tongue between the temporomandibular dysarthrosis.
palatal shelves resulting in cleft palate.
There is respiratory difficulty due to failure Down’s Syndrome
of support of tongue musculature because of It is also called as ‘Trisomy 21 syndrome’ and
micrognathia, allowing the tongue to fall down ‘mongolism’. It is associated with subnormal
and backwards, partially obstructing the mentality in which an extremely wide variety of
epiglottis. There may be congenital heart defects, anomalies and functional disorders may occur.
other skeletal abnormalities and ocular lesions. It results from excessive chromosomal material
Mental retardation is also a common finding. involving all or a portion of chromosome 21.
620 Textbook of Oral Pathology

Type usually small and fingers are stubby. The base

of the skull is small and constricted as a result of
• Typical type: Trisomy–21 with 47 chromo-
retarded growth of the cartilaginous portions.
somes (95 percent of cases).
The calvarium is large and bulges frontally and
• Translocation type: 46 chromosomes.
laterally. There is also depression of the bridge
• Chromosomal mosaicism.
of nose.
Lumbar lordosis with prominent buttocks
Clinical Features
and protruding abdomen is often present. Joints
Flat face, large anterior fontanelle, open sutures. exhibit limitation of motion. Arms do not hang
There is also brachycephalic skull shaped with freely at the sides and the elbows can not be
frontal prominence and occipital flattening. They straightened.
are almond shaped with superior-lateral or
Oral Manifestations
Mongolian obliquity. Small slanting eyes with
epicanthal folds. There is also ocular Maxilla is often retruded because of restriction
hypertelorism. There is flattened nasal bridge. of growth at the base of skull which may produce
There is also sexual underdevelopment, cardiac relative mandibular proganthism. Congenital
abnormalities and hypermobility of the joints. missing teeth and disturbances in shape of teeth
may also occur.
Oral Manifestations
Histopathological Features
Macroglossia with protrusion of tongue, fissured Retardation or even aplasia of zone of
tongue or pebbly tongue from enlargement of provisional calcification of endochondral
papilla. There is open mouth, frequent growth. Cartilage columns lack orderly
prognathism and high arched palate. There is arrangement, fail to calcify properly and are not
hypoplasia or aplasia of maxillary sinus. Teeth resorbed and replaced by bone in the usual
are malformed, enamel hypoplasia and fashion. Chondrocyte development is defective;
microdontia. It attributable to small maxillary the orderly longitudinal growth of bone is
arch relative to the mandibular arch. Universal disrupted, resulting in stunting of bones.
severe destructive periodontal disease that do
not appear to be local in origin. Skeletal Fluorosis (fluoride toxicity)

Achondroplasia There are two types of fluoride toxicity:

• Acute
It is also called as ‘chondrodystrophia fetalis’. It is a • Chronic
disturbance of endochondral bone formation, which
results in a characteristic form of dwarfism. It is a Acute Fluoride Toxicity
hereditary condition, which is transmitted as an
Clinical Features
autosomal dominant trait.
There is nausea, vomiting and epigastria distress.
Clinical Features Excess salivation, diarrhea and mucous
Patient is quite short, usually less than 14 meters discharge with headache and sweating. There is
in height and thickened muscular extremities, also barely detectable pulse, hypotension, cardiac
brachycephalic skull and bowed legs. Hands are arrhythmias and disturbance in electrolyte
 Bone Pathology 621

balance. In some cases complication like Massive Osteolysis

respiratory and metabolic acidosis and coma can
It is also called as ‘vanishing bone’, ‘disappearing
arise.
bone’, ‘phantom bone’, ‘progressive osteolysis’ or
Management ‘Gorham syndrome’. It is characterized by
spontaneous, progressive resorption of bone with
All attempts should be to eliminate the toxic dose ultimate total disappearance of bone.
of fluoride from the body.
Clinical Features
Chronic Fluoride Toxicity
It is most common in older children and young
Clinical Features and middle age adults, affecting both sexes
Patient is having pain in the joints of hands, feet equally. The most commonly affected bones are
and spine. There is also stiffness of gait, the clavicle, scapula, humerus, ribs, ilium,
limitation of movement occurs. In most severe ischium and sacrum. The disease may or may
cases, there is almost crippling of movement and not be painful, begins suddenly and advance
extreme pain because the spine and joints
become rigid and virtually, immobilization of the
individual occurs.

Generalized Cortical Hyperostosis

It is also called as ‘Von Buchem disease’ and it
represents an excessive deposition of endosteal
bone throughout the skeleton in a pattern
suggestive of a hereditary condition, with an
autosomal recessive characteristic.

Clinical Features
Facial appearance of this patient may be swollen,
particularly with widening of the angles of
mandible and at the bridge of nose. In some
patients, there is loss of visual activity and loss
of facial sensation; some degree of facial paralysis
and deafness occurs and all this is due to cranial
nerve involvement through closure of foramina.
Intraorally, there is sometimes overgrowth of
alveolar process.

Histopathological Features
Figure 31.17: Vanishing bone disease showing replacement
The bone is normal dense bone, but without of bone by blood vessels (Courtesy: Dr Chandrasekhar
evidence of remodelling. Kadam)
622 Textbook of Oral Pathology

rapidly, until the involved bone is replaced by a Histopathological Features

thin layer of fibrous tissue surrounding a cavity.
There is replacement of bone by connective tissue
containing many thin walled blood vessels or
Oral Manifestations
anastomosing vascular spaces lined by
The patients may present with pain or facial endothelial cells (Fig. 31.17).
asymmetry, or both and there is pathologic
fracture of bone following even minor trauma. Management
There may be complete destruction of mandible; There is no specific treatment although surgical
maxilla is less commonly affected. resection ceases the progress of the disease.
 CHAPTER

32 Skin Disorders

Mucocutaneous lesions are a group of disease Microorganisms like Mycoplasma

and conditions that affect the skin as well as the pneumoniae and herpes simplex virus.
mucous membrane. These lesions affecting the Vaccination, radiation therapy and
oral mucosa may be arising due to infections, occasionally other disease like Crohn’s disease,
allergies, autoimmune diseases. Some of the ulcerative colitis and infectious mononucleosis.
lesions may be hereditary in origin which are
called as genodermatosis. Clinical Features

Erythema Multiforme It is most frequently seen in children and young

adults and is rare after the age of 50. It affects
It is acute inflammatory disease of the skin and males more than females. Most common area,
mucous membrane that causes a variety of skin involved are hands, feet, extensor surfaces of
lesions, hence the term ‘multiforme’. elbow and knees. It has got acute or explosive
onset with generalized symptoms such as fever
Forms
and malaise. It may be asymptomatic and in less
• Self-limiting form. than 24 hours, extensive lesions of oral mucosa
• Severe form may be present as Stevens-Johnson may appear.
syndrome or toxic epidermal necrolysis.
Symptoms: It is characterized by macule or
• Herpes associated erythema multiforme.
papule, 0.5 to 2 cm in diameter, appearing in
Etiology segmental distribution. Typical skin lesions, of
erythema multiforme may be non-specific
Immune-mediated disease that is indicated by
macule, papule and vesicle.
the deposition of immune complexes in the
superficial microvasculature of the skin and Bull’s eye: Target or iris or bull’s eye lesion
mucous membrane or cell mediated immunity. consists of central bulla or pale clearing area,
Drugs like sulfonamides, trimethoprin, surrounded by edema and band of erythema.
nitrofurantoin, phenylbutazone, digitalis, birth This gives concentric ring like appearance.
control pills and penicillin. Morbidity is high due to secondary infection,
624 Textbook of Oral Pathology

fluid and electrolyte imbalance or involvement of Oral Manifestation

lungs liver and kidneys.
Erythema multiforme occurs along with skin
lesions in 45% of the cases. Lip is prominently
Stevens-Johnson Syndrome
involved followed by buccal mucosa, palate,
It is the severe form of erythema multiforme with tongue and face. Oral lesions start as bullae, on
widespread involvement, typically involving an erythematous base and break rapidly into
skin, oral cavity, eyes and genitalia. irregular ulcers. Patient cannot eat or swallow
It commences with abrupt occurrence of and drools blood tinged saliva. The lesions are
fever, malaise, photophobia and eruptions on larger, irregular, and deeper and often bleed very
oral mucosa, genital mucosa and skin. freely. In full blown cases, lips are extensively
Cutaneous lesions: Cutaneous lesions are similar involved and large portions of the oral mucosa
to erythema multiforme and are hemorrhagic, are denuded of epithelium. Sloughing of mucosa
often vesicular or bullous. and diffuse redness with bright red raw surface
is seen. Healing occurs in two weeks.
Eye lesions: It consists of photophobia,
conjunctivitis, and corneal ulcerations. Histopathological Features
Keratoconjunctivitis sicca has also been
The cutaneous or mucosal lesions generally
described and blindness may result, chiefly from
exhibit intracellular edema of the spinous layer
intermittent bacterial infection.
of epithelium and edema of superficial
Genital lesions: Genital lesions are reported to connective tissue, which may produce
consist of non-specific urethritis, balanitis and subepidermal vesicle (Figs 32.1 and 32.2).
vaginal ulcers.
Complications: Other reported complications are
related to respiratory tract involvement such as
tracheo-bronchial ulcerations and pneumonia.
Oral mucous membrane lesions in Stevens-
Johnson syndrome may be extremely painful
and so, mastication becomes impossible.
Mucosal vesicles or bullae occur, rupture and
leave a surface covered with a thick, white or
yellow exudate. Erosions of the pharynx are also
common. Lips may exhibit ulcerations with
bloody crusting and are often painful.

Toxic Epidermal Necrolysis

It is also called as ‘Lyell’s disease’. It occurs
secondary to drug reaction and results in Figure 32.1: Erythema multiforme showing vesicle
formation
sloughing of skin and mucosa in large sheets. It
is managed in burn centers where necrotic skin There is zone of liquefaction degeneration in
is removed under general anesthesia and healing the upper layer of the epithelium with
takes place under sheets of porcine xenografts. intraepithelial vesicle formation and thinning,
 Skin Disorders 625

with frequent absence of the basement membrane. Types

Dilatation of the superficial capillaries and
• Pemphigus vulgaris
lymphatics in the upper most layer of the
• Pemphigus vegetans
connective tissue is prominent and mixed
• Pemphigus foliaceous
inflammatory infiltrate with numerous
• Pemphigus erythematosus
eosinophils also seen.
• Paraneoplastic pemphigus—It is autoimmune
disease which is associated with malignancy
like lymphoma, leukemia, etc.

Pemphigus Vulgaris
Mechanism
Binding of IgG antibody to pemphigus antigen
leads to epithelial cell separation by triggering
complement activity or plasminogen plasmin
system. Separation of cell takes place in lower
layer of stratum spinosum.

Clinical Features
It is seen in 5th to 6th decades of life and male to
female ratio is 1:1, with whites more commonly
Figure 32.2: Erythema multiforme showing vesicle affected.
formation
Symptoms—Thin walled bullae or vesicles
Vesicle formation is seen as intraepithelial or varying in diameter from few mm to several
the subepithelial region. Necrotic eosinophilic centimeters arise on normal skin or mucosa.
keratinocytes are seen in the blister area.
Signs— These lesions contain a thin, watery fluid
Inflammatory cells are in abundance in the vesicle
shortly after the development, but this may soon
region.
become purulent or sanguineous. They rapidly
Management break and continue to extend peripherally,
eventually leaving large areas of denuded skin.
Mild cases: Soft liquid diet and topical anaesthetic
mouth wash. Nikolsky’s sign—Characteristic sign of the disease
is that pressure to an apparently normal area will
Moderate to severe erythema multiforme: 30 mg/day
result in formation of new lesion. This
prednisolone or methyl prednisolone for several
phenomenon is called as Nikolsky’s sign. It
days and tapered after the symptoms subside.
results from upper layer of skin pulling away
Prophylactic Acyclovir to prevent HSV related
from the basal layer. It is caused by perivascular
erythema multiforme.
edema which disrupts the dermal-epidermal
Pemphigus junctions.
It is autoimmune disease involving the skin and Asboe-Hansen sign—After giving application of
mucosa and characterized by intraepithelial pressure to an intact bulla, the bulla will enlarge
vesicles or bullae formation. by extension to an apparently normal surfaces.
626 Textbook of Oral Pathology

The course of pemphigus vulgaris is a Clinical Features

variable one; the disease terminating in death or
It occurs in 1 to 9% of the cases. The flaccid bullae
recovery within a few days or weeks or it may
become eroded and forms ‘vegetations’ on some
get prolonged over a period of months or even
of the erosions. These fungioid masses become
years.
covered by purulent exudate and exhibit
Oral Manifestations inflamed borders, frequently occur first on nose
and in the mouth or axilla. The disease usually
In 90%, of the cases oral lesions develop and in terminates in pemphigus vulgaris.
60% cases they occur first. Initial lesion most
frequently occurs on buccal mucosa because the Oral Manifestations
epithelium demonstrates less intercellular
substance and fewer intercellular junctions Gingival lesions may be lace like ulcers with
making the area more susceptible to purulent surface on red base or have granular
acantholysis. Palate and gingiva are other or cobblestone appearance.
common sites of involvement.
Pemphigus Foliaceous
Symptoms and signs—Oral lesions begin as classic
It is a relatively mild form of pemphigus, which is
bullae on noninflamed base with formation of
shallow ulcers as bullae break rapidly. Thin layer most common in older adults. It is manifested by
characteristic early bullous lesions which rapidly
of epithelium peels away in an irregular pattern
rupture and dry to leave masses of flakes on scales
leaving denuded base.
suggestive of an exfoliative dermatitis or eczema.
Nikolsky’s phenomenon—The oral lesions may
Brazilian pemphigus—It is a mild endemic form of
exhibit Nikolsky’s phenomenon and may be
pemphigus foliaceous found in tropical regions,
denuded by peripheral enlargement of the
lesion. particularly in Brazil. It is seen in children and
frequently in family groups.
Lesions bleed easily and are tender on
palpation. The pain may be so severe that the
patient is unable to eat. The lesion may have Pemphigus Erythematosus
ragged borders and be covered with white or It is also called as Senear-Usher syndrome. It is
blood tinged exudate. Edges of the lesion may form of disease which is characterized by the
extend peripherally. Diffuse erythematous occurrence of bullae and vesicles concomitant
involvement of gingiva. with the appearance of crusted patches
resembling seborrheic dermatitis or even lupus
Pemphigus Vegetans
erythematosus. Most cases ultimately terminate
Types in pemphigus vulgaris or foliaceous. The skin
manifestations in any form of pemphigus may
• Neuman type—It is more common and early
be accompanied by fever and malaise.
lesions are similar to those seen in Pemphigus
Vulgaris.
Histopathological Features Bond
• Hallopeau type—In hallopeau type, pustules,
not bullae, are the initial lesions which are Basic defect is intraepithelial and is demonstrated
followed by verrucous hyperkeratotic as acantholysis (loss of cell to cell attachment) in
vegetations. stratum spinosum. The cellular outlines are
 Skin Disorders 627

Figure 32.3: Pemphigus vulgaris showing acantholysis

(Courtesy: Dr Sangamesh Halawar)
Figure 32.4: Pemphigus vulgaris showing
round, rather than polyhedral; intercellular suprabasilar vesicle formation
bridges are lost (spongiosis) and the nuclei are Indirect immunofluorescent antibody test—
large and hyperchromatic (Fig. 32.3). Antibodies against intercellular substance can be
Characteristic ‘suprabasilar split’ intra- seen in the serum of patient. The titers of antibody
epithelial separation, which occurs just above the are directly related to the level of the clinical
basal layer of the epithelium, is seen (Fig. 32.4). disease.
Row of tombstones—Sometimes, the entire Direct test—Antibody will bind the
suprabasal or superficial layers are striped off immunoglobulin deposit in the intercellular
leaving only the basal layers which is called as substance and show positive fluorescence under
the row of tombstones. fluorescence microscope.
Acantholysis—The spinous cell layer lose their cell
contacts and the loose cells assume a rounded
shape, forming characteristic cells—“the Tzanck
cells.”
The diagnostic test of the cytological study
of the contents of the vesicle or the lesion shows
these cells are termed as “Tzanck test”.
Tzanck smear—It is done to demonstrate Tzanck
cells which often are found lying freely within
the vesicular space (Fig. 32.5).
The underlying connective tissue shows mild
to moderate chronic inflammatory infiltrate.
The underlying connective tissue is densely
filled with chronic inflammatory cells, which may
also enter the vesicular fluid. As the vesicle and
bulla rupture, the ulcerative lesion becomes
infiltrated with polymorphonuclear leukocytes and Figure 32.5: Tzanck smear showing presence of
the surface may show suppuration. Tzanck cell
628 Textbook of Oral Pathology

Management (Fig. 32.6). The vesicle contain fibrinous exudate

It can be managed by corticosteroids, immuno- admixed with occasional inflammatory cells.
suppressive drugs, Plasmapheresis and admin-
istration of 8- methoxypsoralen, followed by ex-
posure of peripheral blood to ultraviolet radia-
tion.

Bullous Pemphigoid
It is also called as ‘parapemphigus’, or ‘aging
pemphigus’. In this, the initial defect is
Figure 32.6: Bullous pemphigoid showing separation of
subepithelial in the lamina lucida region of the the epithelium at the basement membrane zone
basement membrane. It is associated with anti-
basement membrane antibodies which are These cells are usually eosinophils. In severe
detected in the basement membrane. inflammation, the basal keratocytes of mesen-
chymal demonstrates spongiosis. It is called as
Clinical Features eosinophilic spongiosis.
It occurs chiefly in adults over the age of 60. It is It also shows stratified squamous epithelium,
self-limiting and rarely lasts over 5 years. Bullae thin epithelium and subepithelial bulla
do not extend peripherally and remain localized; formation is the characteristic feature of bullous
heal spontaneously. pemphigoid. The vesicle shows moderate
Skin lesions begin as generalized nonspecific numbers of the acute inflammatory cells as
rash, commonly on the limbs, which appear as eosinophils and chronic inflammatory cell
blisters on inflamed skin; itching precedes. It infiltrate within it.
may persist for several weeks to several months
Laboratory Test
before ultimate appearance of vesiculobullous
lesions. These vesicle and bullae are relatively Indirect immunofluorescence antibody test—Lesions
thick walled and may remain intact for some will demonstrate circulating IgG antibodies
days. Rupture does not occur although, it leave against basement membrane antigen.
raw eroded areas which heal rapidly. Direct immunofluorescence testing and compliment
Oral Manifestations fixation test—Positive specimen will demonstrate
IgG and complement in the basement membrane
Oral lesions are smaller, form more slowly and zone.
are less painful. Gingival lesions consist of gen-
eralized edema, inflammation, desquamation Management
and localized areas of discrete vesicle formation.
Systemic steroids in lower doses, for shorter
Vesicles and ultimately erosion may develop not
period combined with immunosuppressive
only on the gingival tissue but any other area
drugs and dapsone.
such as the buccal mucosa, palate, floor of the
mouth and tongue.
Benign Mucous Membrane Pemphigoid
Histopathological Features It is also called as ‘Cicatricial Pemphigoid’. It is
The vesicle and bullae are subepidermal a disease of unknown etiology but probably is
and non-specific. Epithelium appears normal autoimmune in nature.
 Skin Disorders 629

Clinical Features cells and eosinophils. Lesion which are chronic

in nature shows granulation tissue and fibrosis.
It occurs more commonly in patients over 50 years
of age and female to male ratio is 2:1. Typically,
Laboratory Findings
the vesiculobullous lesions occur on the oral
mucous membrane, conjunctiva and skin. The Direct immuno fluorescent study will show
other mucous membranes involved are those of positive fluorescence for immunoglobulin and
nose, larynx, pharynx, esophagus, vulva, vagina complement in basement membrane zone, i.e.
and penis. in intercellular substance of prickle cell layer of
Adhesions may develop between bulbar and epithelium.
palpebral conjunctivae resulting in obliteration Low titers of serum antibody is seen.
of the palpebral fissure with opacity of the Immunofluorescence studies revealed the
cornea, frequently leading to blindness. presence of tissue bound basement membrane
Involvement of esophagus and trachea may zone antibodies as well as circulating anti-
cause strictures leading to difficulty in basement membrane zone antibodies in the
swallowing or breathing. It may lead to scarring serum of some patients.
of affected area.
Management
Oral Manifestations Topical and intralesional steroids, dapsone therapy
It occurs on gingiva, buccal mucosa and palate. and immunosuppressive therapy are given.
The mouth may be the only site involved. The
mucosal lesions are also vesiculobullous in Familial Benign Chronic Pemphigus
nature, but appear to be relatively thick walled It is also called as Hailey-Hailey disease. It is
and for this reason, may persist for 24 to 48 hours uncommon disease transmitted by irregular
before rupturing and desquamation. After dominant gene.
rupture of vesicle, surface epithelium is lost
leaving raw red bleeding surface. Clinical Features
Gingiva is edematous and bright red,
It is usually manifested during adolescent or young
involvement is patchy and diffuse. There may
be formation of ulcer, which surrounded by zone life with no predilection for sex. The most common
sites are flexure surfaces of the axillae and groin,
of erythema. There may be erosion on cheek and
the neck and the genital area. Heat and sweating
vesicles on palate and narrower peripheral
extensions. The oral lesions rarely produce scars. amplify the outbreak of the lesion while
spontaneous remission may occur in cold weather.
Histopathological Features Signs and symptoms—The lesions develop as
small groups of vesicles appearing on normal or
The vesicle and bullae are subepidermal rather
erythematous skin, which soon rupture to leave
suprabasilar and there is no evidence of
eroded, crusted areas.
acantholysis. The basement membrane structure
appears to detach with the epithelium from the Nikolsky’s sign—They enlarge peripherally but
underlying connective tissue. There is non- heal in center with Nikolsky’s sign positive.
specific chronic inflammatory infiltrate of Tender and enlarged regional lymph nodes may
connective tissue chiefly lymphocytes, plasma also be present.
630 Textbook of Oral Pathology

Oral Manifestations are occasionally affected. When the blister heals

within 2 to 10 days, there is no resultant scarring
Oral lesions are similar to those occurring on the
or permanent pigmentation. The disease appears
skin and it develops as crops of vesicle, which
to improve at puberty and prognosis is good for
rapidly ruptures leaving raw eroded areas.
normal life span. The localized form is limited to
Histopathological Features hands and feet only and tends to exacerbate in
hot weather.
It is similar to pemphigus vulgaris but
acantholysis is more extensive. Characteristic Epidermolysis bullosa dystrophic dominant: The
feature of this disease is occasional intercellular onset is at infancy and it may delay until puberty.
bridges persist so that adjacent epithelial cells The blister commonly develops on the ankles,
still adhere to each other. This gives classic knees, elbows, feet and head. Healing results in
description of the ‘dilapidated brick wall’ effect. scarring which is sometimes keloid in type. Hair
may be sparse, while nails are usually dystrophic
Epidermolysis Bullosa or absent with milia present.
Palmar-plantar keratoderma with hyper-
It is a dermatological disorder in which bullae or
hidrosis also may occur with ichtyosis and
vesicles occur on skin or mucous membrane
sometimes hypertrichosis.
surface spontaneously, shortly after minor
trauma. Epidermolysis bullosa dystrophic recessive: It has
onset at birth or very shortly thereafter. It is
Classification characterized by formation of bullae
• Epidermolysis bullosa simplex spontaneously or at sites of trauma, friction or
• Generalized form pressure. The typical sites of involvement are the
• Localized form (Weber-Cockayne syndrome, feet, buttock, scapula, elbows, finger and occiput.
recurrent bullous eruption of hands and feet) Nikolsky’s sign is positive in this type of
• Epidermolysis bullosa dystrophic, dominant. epidermolysis bullosa. The bullae contain a clear,
• Epidermolysis bullosa dystrophic, recessive. bacteriologically sterile or sometime blood tinged
• Junctional epidermolysis bullosa fluid. When these bullae rupture or are peeled off
(Epidermolysis bullosa latalis, junctional under trauma or pressure, they leave raw, painful
bullous epidermatosis, Herlitz’s disease). surface. The bullae heal by scar and milia
• Epidermolysis bullosa acquista (acquired)— formation which may result in afunctional club-
It is similar to dystrophic form of the disease like fists. The hair may be sparse, while the nails
but usually with an adult onset. are usually dystrophic.
Junctional Epidermolysis Bullosa: It is extremely
Clinical Features severe form of the dystrophic recessive form, which
Epidermolysis bullosa simplex: It is inherited as is incompatible with prolonged survival. It has
autosomal dominant trait and manifests at birth onset at birth, absence of scarring, milia,
or shortly thereafter. It is characterized by pigmentation and death within three months of
formation of bullae or vesicle on the hands and age. The bullae are similar to recessive form but
feet at site of friction or trauma. The knees, they develop simultaneously and sheets of skin
elbows and trunk are rarely involved and nails may actually be shed.
 Skin Disorders 631

Oral Manifestations Epidermolysis bullosa dystrophic dominant: The

bullae in this form develop as a result of
Epidermolysis bullosa simplex: Bullae of the oral
separation through the very thin, irregular PAS
cavity are reported in occasional cases of
positive basement membrane which becomes
generalized epidermolysis bullosa, but teeth are
divided. The basal layer appears normal,
not affected.
although flattened on the roof of the blister and
Epidermolysis bullosa dystrophic dominant: Bullae the underlying connective tissue shows absence
can occur in this type. Sometimes, oral milia can of elastic and oxytalan fibers.
be seen but teeth are unaffected.
Epidermolysis bullosa dystrophic recessive: The
Epidermolysis bullosa dystrophic recessive: They separation and bulla formation occur
may be preceded by the appearance of white spots immediately beneath the poorly defined PAS
or patches on the oral mucous membrane or by positive basement membrane which remains
development of localized areas of inflammation. attached to the roof of the blister. Fragment of
These bullae are painful especially when they basement membrane may adhere to the dermis
rupture or when the epithelium desquamates. but basal layer of cells is normal. The pre-elastic
Scar formation results in obliteration of sulci and and oxytalan fibers in the connective tissue are
restriction of the tongue movement. Hoarseness increase in number.
and dysphagia may occur as a result of bullae of
Junctional Epidermolysis Bullosa: It is similar and
larynx and pharynx.
identical to those occurring in the dystrophic
Esophageal involvement produces serious
recessive disease.
strictures. Dental defects like rudimentary teeth,
congenitally absent teeth, hypoplastic teeth and
crowns denuded of enamel may be seen. Diagnosis

Junctional Epidermolysis Bullosa: Oral bullae are The diagnosis should be simple when the family
frequently very extensive and because of their history, reaction to trauma and the lesions
extreme fragility produce serious feeding appearing most profusely on the extremities are
problems. Severe disturbance in enamel and taken into consideration.
dentin formation of deciduous teeth also occur.
Management
Histopathological Features
Large blisters should be pricked and the blister
Epidermolysis bullosa simplex: Vesicle and bullae fluid released. Dressing, to minimize reaction
are developed as a result of destruction of basal may be helpful. Super infections should be
and suprabasal cells so that some nuclei may treated with appropriate local or systemic
persist on the floor of the blister. The individual antibiotics.
cells become edematous and show dissolution
of organelles and tonofibrils with displacement
Dermatitis Herpetiformis
of the nucleus to the upper end of cells. In
localized form, bullae are intraepidermal and It is also called as ‘Duhring-Brocq disease’. It is a
suprabasal in location. Electron microscopy shows rare, benign, chronic, recurrent dermatologic
perinuclear edema and subnuclear cytolysis. disease of unknown etiology.
632 Textbook of Oral Pathology

Clinical Manifestations Pityriasis Rosea

It occurs between 20 to 55 years of age, with males It is an acute skin eruption of unknown etiology.
affected at least twice as frequently as females.
These occur most frequently on buttocks, Clinical Features
extremities as well as on the face, scalp and It is more common in spring and autumn and it
sometimes, the oral cavity. involves young adults chiefly, with no sex
predilection.
Symptoms—the first manifestation of the disease
is usually pruritis and severe burning followed Prodormal features—The generalized outbreak is
by the development of erythematous papules, frequently preceded by the appearance of a
vesicles, bullae or pustules. The patient usually ‘primary lesion’ or ‘herald spot’ seven to ten days
shows increased severity in summer months. previously. It is characterized by the appearance
of superficial, light red macules or papules,
Oral Manifestations generalized over most of the skin surface.
Vesicles and bullae rupture rapidly to leave areas Signs and symptoms—The spot is brighter red and
of superficial ulceration at any intraoral site is larger (3 to 4 cm in diameter) than the multiple
the characteristic finding. eruptions which follow its appearance. The
individual exanthematous lesion is commonly
Histopathological Features ovoid, with long axis parallel to the natural lines
The lesion begins with accumulation of of cleavage of skin and are covered by a thin
neutrophils and eosinophils in the dermal; papillae silvery scales. The lesion often manifests mild
producing a microabscess. The connective tissue aching headache and low grade fever and
becomes necrotic and the overlying epithelium cervical lymphadenopathy.
separates, usually forming a subepithelial vesicle Oral Manifestations
with destruction of the basement membrane.
There is also perivascular infiltrate of The oral lesion occurs either concomitantly with,
lymphocytes. There is deposition of IgA along or subsequent to the skin manifestations. It can
the basement membrane. Electron microscopic occur on buccal mucosa although tongue and
study shows blister formation below the lamina palatal lesions have been reported. Oral lesion
lucida. appears as erythematous macule, with or without
central area of grayish desquamation. The lesion
Laboratory Findings may be single or multiple, irregular in shape,
occasionally showing raised borders and may
Direct immunofluorescence staining is positive vary in size from few mm to 1-2 cm in diameter.
at the epidermal-dermal junction. Patient may
develop eosinophilia and sensitivity to halogens Histopathological Features
(chlorine, bromine, iodine and fluorine), both by
It consists of slight acanthosis and focal parakeratosis
patch test and after ingestion. with microvesiculation or simply sprinkling of
leukocytes within the epithelium. There is also
Management
edema, hyperemia and perivascular infiltration of
Use dapsone. lymphocytes, plasma cells and histiocytes.
 Skin Disorders 633

Management the connective tissue with hyperpigmentation. In

It requires no treatment since the disease is self- some cases acanthosis of epithelium is noted.
limiting and generally undergoes rapid
spontaneous regression. Management
No treatment is necessary for skin disease. Dental
Incontinentia Pigmenti
defects can be corrected with the assistance of
It is also called as ‘Bloch-Sulzberger syndrome’. It orthodontics and prosthetic dentistry.
is transmitted as sex-linked dominant trait.
Acanthosis Nigricans
Clinical Features
Types
It appears shortly after birth and exclusively seen
in females. • Benign—It may be present at birth or occur
later in childhood; appears to be genetic in
Symptoms—It is characterized by the appearance
origin, inherited as a dominant characteristic.
of erythematous and vesiculobullous lesions on
• Malignant—It is associated with internal
the trunk and extremities which frequently malignancy like adenocarcinoma of stomach
disappear and reappear. Then, they are
and occurs in older age group.
gradually replaced by white keratotic, lichenoid,
• Pseudoacanthosis nigricans—It is most com-
papillary or verrucous lesions, which then persist mon and is associated with endocrinopathy.
for some months. Some of the lesions in infants
are brownish gray macules in a streaked, patchy Clinical Features
distribution over the trunk and extremities,
occurring subsequent to the verrucous keratotic The most common areas involved are axilla,
lesions. palms and soles and face and neck.
There is heavy melanin pigmentation of Sign and symptoms—Skin lesions are symmetric
epithelium, dropping down into cluster of with mild hyperpigmentation and mild papillary
chromatophores in the upper dermis hypertrophy of only small patchy areas. In some
(incontinence), which gives the disease its name. cases, it is heavily pigmented, aggressively
Other defects can be seen like cataract, optic verrucous lesion involving much of the skin. The
atrophy, strabismus, retrolental fibroplasia, verrucous lesions are often pigmented and
central nervous system involvement and lesions generalized pruritis is also a common finding.
of skeletal system.
Oral Manifestations
Oral Manifestations
The tongue and lips are most commonly
Both the deciduous and permanent dentitions may involved, followed by buccal mucosa. There is
be affected. There is delayed tooth eruption, peg hypertrophy of the filiform papillae producing a
and cone shaped crowns, congenitally missing shaggy, papillomatous surface on the dorsal tongue.
teeth, malformed teeth and additional cusps. The lips may be enlarged and covered by
papillomatous growths, particularly at the angle
Histopathological Features
of mouth. The buccal mucosa may show a velvety
It shows cleft in the epithelium which consists white appearance with occasional papillary
of eosinophils. There are also macrophages in lesions.
634 Textbook of Oral Pathology

Histopathological Features blood vessels, resulting in excessive bruising as

well as defective healing of skin wounds.
There is marked acanthosis, coupled with peculiar
Rupture of large arteries as well as of intestine
parakeratosis. Upward finger like projection of
often occurs, producing a life-threatening
dermal papillae occur. There is also thinning of
situation.
adjacent epidermis and presence of pseudo horn
cyst (Fig. 32.7). Rubber man—In some patients, skin extensibility
Keratinized stratified squamous epithelium is pronounced, the patient is known as ‘rubber
shows papillary projections. Hyperkeratosis is a man’.
prominent feature. Skin lesions have more Hypertelorism, a wide nasal bridge, epicanthic
deposition of melanin and less acanthosis. Oral folds, protruding ears and frontal bossing are often
lesions show more spinous cell acanthosis and present. Freely movable subcutaneous nodules are
less melanin deposition. frequently found, which represent fibrosed lobules
of fat. The scarring of skin following wound healing
in these patients is unusual, as the scars tend to
spread rather than contract in time.

Oral Manifestations
Oral mucosa is of normal color but is excessively
fragile and bruises easily. The gingival tissue
appears fragile and bleeds after tooth brushing.
Hypermobility of temporomandibular joint
resulting in repeated dislocations of the jaw have
been reported. There may be lack of normal
scalloping of the dentinoenamel junction,
formation of irregular dentin and increased
tendency to form pulp stones with hypoplastic
changes in enamel.

Figure 32.7: Acanthosis nigricans showing papillary Laboratory Findings

projection, parakeratosis and hyperpigmentation. (Courtesy:
Dr Sangamesh Halawar) Clotting time is normal, but capillary fragility
test is usually positive.
Management
Management
There is no treatment for this disease.
There is no specific treatment for this disease,
Ehlers-Danlos Syndrome but surgical procedures should be carried out
carefully as healing problems can exist.
It is also called as ‘Cutis Hyperelastica’ and it is a
group of hereditary disorders of connective tissue. Psoriasis
It is common a dermatological disease
Clinical Features characterized by white, scaly papules and plaque
There is hyperelasticity of skin, hyper- on an erythematous base that preferentially
extensibility of the joints and fragility of skin and affects the extremities and scalp.
 Skin Disorders 635

Etiology and Precipitating Factors

It has been suggested a possible inheritance
pattern, possibly transmitted as simple dominant
trait. β-hemolytic streptococcal infection often
precedes psoriasis. Metabolic disturbances and
endocrine dysfunction can also precede psoriasis.
Antimalarials, β-blocker and lithium may
worsen psoriasis and the rash may ‘rebound’
after stopping systemic corticosteroids or potent
local corticosteroid. Anxiety precipitates some
exacerbation. Mental anxiety and stress can
increase severity of the disease. In areas of skin
damage such as scratches or surgical wound.

Clinical Features
It commonly affects adults and arises in 2nd and
3rd decades of life. It is commonly occur on
extremities, and scalp. It is usually chronic with
acute generalized exacerbations. It is more severe
in winter and less severe in the summer as a
result of increase exposure to ultraviolet light.
Symptoms—It is characterized by occurrence of
Figure 32.8: Patient is having dusky appearance on leg in
small sharply defined, dry papules each covered patient of psoriasis
by delicate silvery scale which appear as
resembling a thin layer of mica (Fig. 32.8). • Erythrodermic psoriasis—The skin becomes
universally red and scaly or more rarely just
Auspitz’s sign—If the deep scale is removed one
red with very little scale present.
or more bleeding points are seen.
• Pustular psoriasis—It is a severe form of
Papules are enlarged at periphery and may
form large plaques which are roughly psoriasis with eruption of minute pustule
symmetrical. After removal of scale the surface with shedding of nails is common.
of skin is red and dusky in appearance.
Oral Manifestations
Clinical Types
Oral lesions are reported on lip, buccal mucosa,
• Stable plaque psoriasis—It is most common palate, gingiva and floor of mouth. They appear
type. The lesions are red with dry, silvery- as plaques, silvery, scaly lesions with an
white scaling, which may be obvious only erythematous base. Sometime, they are multiple
after scraping the surface. papular eruptions which may be ulcerated or as
• Guttate psoriasis—It is seen in children small, papillary elevated lesions with scaly
adolescents and may follow streptococcal surface.
sore throat. Individual lesions are droplet- Usually, four types of oral manifestations
shaped, small and scaly. occur in patient with psoriasis:
636 Textbook of Oral Pathology

• The first type consists of minute well defined

gray to yellowish white lesion that is round
or oval.
• The second type is characteristic by white,
elevated, lacy, circinate lesion on the oral
mucosa including the tongue. These
eruptions are parallel to eruption skin.
• The third type consists of a fiery red erythema
of the oral mucosa, including the tongue is
seen in acute form.
• The fourth type of oral lesion described in
psoriasis is a geographic tongue that occurs
most frequently among the patients with
psoriasis. Figure 32.9: Psoriasis showing test tube
shaped rete pegs
Histopathological Features
Intraepithelial microabscess formation (Munro’s
abscess) occurs. It is characterized by uniform
parakeratosis, absence of stratum granulosum
and elongation and clubbing of rete pegs. This
appearance of rete pegs is termed as test tube
appearance (Figs 32.9 and 32.10).
The epithelium over the connective tissue is
thin and it is from this point; bleeding occurs
when the scales are removed. Tortuous, dilated
capillaries extending high in the papillae are
prominent.
Figure 32.10: Psoriasis showing elongation of retepegs,
Management hyperkeratosis and inflammation of the papillary connective
tissue (Courtesy: Dr Sangamesh Halawar)
Topical agents
• Emollients—It has modest effect in terms of • Corticosteroids—Use of potent topical
reducing scale. corticosteroid on the face or hair margins
• Dithranol—It is gold standard therapy with should be under close and expert medical
dithranol. It normalized differentiation and supervision.
inhibits proliferation when applied to
Ultraviolet light—It is mainstay of management
psoriatic plaques.
of patient with moderate to severe psoriasis. It
• Tar—Crude tar is used which is pro-
is used 3-7 times in a week.
inflammatory and action same as dithranol.
• Calcipotriol—It is vitamin D agonist and it PUVA therapy—Psoralens are natural
reduces the thickness of plaque. photosensitizes found in number of plants. It
• Retinoid—It diminishes the induration, includes clearance to greater degree than any
scaling and redness of plaque. other therapy.
 Skin Disorders 637

Systemic treatment—Three main systemic agents reminiscent of white sponge nevus. A nonspecific
are i.e. methotrexate, oral retinoid and cyclos- thickening of the parakeratin and spinous cell
porin. layer is seen.

Pachyonychia Congenita Management

It is also called as ‘Jadassohn-Lewandowsky There is no treatment for this disease.
syndrome’. It is extremely uncommon disease,
inherited as an autosomal dominant Porokeratosis
characteristic with incomplete penetrance. It is also called as ‘Mibelli’s disease’ and it is
autosomal dominant. It is characterized by faulty
Clinical Feature keratinization of the skin followed by atrophy.
It usually occurs shortly after birth with no sex
predilection. It consists of dystrophic changes in Clinical Feature
the fingernails and toenails, hyperkeratotic cal- It often begins in early childhood but progression
luses of the palms and soles, follicular keratosis of disease is extremely slow. It appears to occur
about the knees and elbows, hyperhydrosis or in males with greater frequency than in females.
excessive sweating of the hands and feet. It occurs most commonly in extremities
There is marked thickening, increasing particularly in hands and feet, as well as shoulder,
toward the free border with nail bed becoming face and neck and the genitalia.
filled with yellowish keratotic debris, often Symptoms—It consists initially of crateriform
causing the nail to project upward at the free keratotic papules which gradually enlarged to
edge. It is associated sparse hair and corneal form elevated plaques. In some cases, there is ring
dyskeratosis producing corneal opacities have like keratotic lesion of the skin with atrophic
been reported. center.
Bullae formation occurs on the feet, and
secondary infection of these may lead to Signs—It is ranging in size from a few millimeters
crippling deformity. Thickening of the laryngeal to several centimeters. The plaques are
commissures, tympanic membrane and nasal surrounded by a distinct raised border of
mucosa and mental retardation are also reported. epidermal proliferation. The nails commonly
become thickened and ridged. The central
Oral Manifestation portion of the lesions ultimately becomes
atrophic, leaving permanent scarring.
The most common site of occurrence are buccal
mucosa, tongue and lips. They consist of focal or Oral Manifestation
generalized white, opaque thickening of the mu-
It is most commonly seen on upper lip and palate.
cosa. There is frequent oral aphthous ulceration
There is numerous small slightly opalescent ring
is seen. In some cases, inflammation of angle of and serpenginous and hyperemic border studded
mouth is seen. Natal teeth are also present. over the palate.

Histopathological Feature Histopathological Features

The mucous membrane exhibits intracellular The elevated horny margin of the lesion exhibits
edema or vacuolization of the spinous cell hyperkeratosis and acanthosis with a deep groove
638 Textbook of Oral Pathology

filled with parakeratin and a characteristic Histopathological Features

absence of the usual underlying granular layer. It The characteristic finding in skin lesion is
constitutes the ‘coronoid lamella’ which is
hyperkeratosis, papillomatosis, acanthosis and
characteristic of the lesion.
peculiar benign dyskeratosis. Dyskeratosis is
The connective tissue beneath the coronoid
characterized by typical cells called corps, ronds
lamella may exhibit lymphocytes infiltrate. The
and grains (cell within cells).
central portion of the lesion shows epithelial
The corps ronds are larger than normal
atrophy and occasionally dyskeratosis.
squamous cells and have a round, homogenous,
basophilic nucleus with a dark eosinophilic
Management
cytoplasm and distinct cell membrane.
There is no treatment for the disease except for Grains are small, elongated parakeratotic
removal of individual lesions. cells situated in the keratin layer. Acantholytic
cells are commonly found floating in the lacunae
Keratosis Follicularis produced by this intraepithelial separation.
It is called as ‘Darier’s disease’, ‘Darier-White
Management
disease’. It is autosomal dominant trait.
Administration of large dose of retinoid is given.
Clinical Feature
Warty Dyskeratoma
It is usually manifested during childhood or It is also called as ‘isolated Darier’s disease’ which
adolescence and has equal sex distribution. They bears histologic similarity to keratosis follicularis
are generally distributed above the forehead, but it present as single isolated focus.
scalp, neck, and over the shoulders.
Clinical Feature
Sign and symptoms—The cutaneous lesion appears
small, firm papules. They are red when they first It usually occurs in older age group with male
appear but characteristically become grayish predominance. The skin lesion occurs on face,
brown or even purple. It can ulcerate and crust scalp, neck and upper chest. They appear as
over. In the skin fold, the lesion tend to coalesce elevated nodules, umbilicated, with raised
and produce verrucous or vegetating macerated, borders and varying in color from yellow or
foul smelling masses. Characteristic nail changes brown to gray or black. They appear as
are consisting of splintering, fissuring, invariably single lesion varying in size from 1 to
longitudinal streaking and sublingual keratosis. 10 mm in diameter. Purulent drainage as well
as bleeding occurs in some cases.
Oral Manifestation
Oral Manifestation
Keratotic papule occur on oral mucosa
It is very rare and it is if present found most
particularly on hard and soft palate, gingiva,
commonly on the alveolar ridge and palate. It
tongue have whitish appearance. They are
appears as small whitish area of the mucosa with
multiple whitish papules which feel rough upon
a central depression.
palpation. In some cases, it has been described
as rough, pebbly areas with verrucous white plaque Focal acantholytic dyskeratosis—It is variant of
or as having cobblestone appearance. Papule warty dyskeratoma in which two or three
become confluent as disorder progress. discrete lesions arising adjacent to one another.
 Skin Disorders 639

Histopathological Features Pseudoxanthoma Elasticum

The microscopic finding of skin and mucosal It is autosomal recessive and the basic defect
lesion are identical except for the absence of a involves the structure of elastin, making it
pilosebaceous structure in the oral lesion. The susceptible of calcification. It is rare hereditary
intraoral lesion exhibits a central orthokeratin or connective tissue disorder, characterized by
parakeratin core beneath which the epithelium generalized degeneration of the elastic fibers
shows a suprabasilar separation resulting in a with a broad phenotypic expression. The clinical
cleft-like space containing acantholytic and picture consists mainly of cutaneous, ocular, and
benign dyskeratotic cells. vascular manifestations.
The connective tissue papillae are covered
usually by a single layer of basal cells while the Clinical Features
underlying connective tissue shows a non- Although widely variable, the age of onset
specific chronic inflammatory infiltrate. Spinous averages 13 years with no predilection for sex.
layer is thickened and contains individually Raised yellowish papules develop on areas of
keratinized cells. thickened, coarsely grained skin especially
Management around the mouth, neck, axilla, elbows.
Brownish gray streaks of the optic fundus
It should be treated by surgical excision. (angiod streaks), recurrent gastrointestinal
Hereditary Mucoepithelial Dysplasia hemorrhage, weak pulse, and failing vision is
common occurrence.
It is described by Witkop and associates in 1978. The typical cutaneous lesions are small
It is autosomal dominant. yellowish papules or larger coalescent plaques
with an appearance similar to plucked chicken
Clinical Features
skin. More severely affected skin results in
It affects all orofacial mucosa and is characterized hanging, redundant folds.
by cataracts, follicular keratosis of the skin, non-
scarring alopecia. Repeated episodes of Oral Manifestations
pneumonia, spontaneous pneumothorax. Mucous membranes, mainly of the inner aspect
of the lower lip are affected.
Oral Manifestation
Oral lesion is fiery red, flat or micropapillary Hound dog appearance—Skin around mouth
frequently involving gingiva and hard palate. becomes redundant, producing a ‘hound dog’
Red enlarged and fissured tongue is common appearance. Lower lip exhibit yellowish
occurrences. intramucosal nodule.

Histopathological Feature Histopathological Features

The oral lesions show a lack of cornified and Intramucosal nodules show large number of
keratinized cells, thinning of the epithelium and thickened and twisted elastic and collagen fibers.
dyskeratosis. Skin histopathology involves swollen, irregularly
clumped and multiply fragmented elastic fibers in
Management the middle and deep reticular dermis, with
No specific treatment for the disease. secondary calcium deposition.
640 Textbook of Oral Pathology

Management PAS positive and show equivocal reaction for

amyloid.
At present, no specific treatment exists. The
knowledge, however, of the potential
Management
complications may lead physicians to take some
necessary precautions. No known treatment for the disease.
Gingivectomy is recommended when diffuse
Hyalinosis Cutis et Mucosae Oris hyperplastic appearing gingival infiltration is
present.
It is also called as ‘lipoid proteinosis’ or ‘Urbach-
Wiethe syndrome’. It is autosomal recessive trait,
White Sponge Nevus
characterized by subdermal and submucosal
infiltration of a hyaline glycoprotein material. It is oral genodermatose, which was described
by Cannon in 1935, hence it is also known as
Clinical Features ‘Cannon’s disease’. It is also called as ‘white
folded gingivostomatitis’, ‘congenital
It is mostly seen in young adults with no sex
leukokeratosis’, ‘pachyderma oralis’ or ‘oral
predilection. It is common in mucosal tissue,
skin, vessel walls, larynx, and brain. It is also seen epithelial nevi. It is autosomal dominant with
irregular penetrance. It appears to represent a
in face, eyelids and neck.
defect in epithelial maturation involving
There may be calcification of dorsum sellae.
Cutaneous lesions commences as vesicles, upon tonofilament formation with impaired normal
desquamation of the superficial strata of cells.
healing acneform scars develop.

Oral Manifestation Clinical Features

Most commonly affected area is lower lip. It can It has no definite sex predilection with children
be seen on tongue, buccal mucosa. Affected most commonly affected and may present at
tissue becomes infiltrated with yellowish white birth and may become intense at puberty. The
elevated pea sized plaque which gradually most common sites are cheek, palate, gingiva,
increased in size in puberty. floor of mouth, portion of tongue. It may be
Radiating tissue may appear at angle of widespread and may involve entire mucosa. It
mouth. Tongue becomes firm and large and can also occur on the mucous membranes of the
bound to floor of mouth. The dorsum of tongue nose, esophagus, genitalia, and rectum.
may lose its papilla and ulcer may develop.
Signs and symptoms—Mucosa appears thickened
Diffuse hyperplastic appearing gingival
and folded or corrugated with soft or spongy
infiltration is present. texture and a peculiar white opalescent line. It
There is restriction of oral function such as
has got sodden, furrowed or wrinkled
saliva flow, tooth eruption, and swallowing.
appearance. The lesion varies in extent from a
There is hypodontia of maxillary lateral incisors small patch to involvement of a large area of
and premolars.
mucosa. Friction may strip superficial keratotic
area leaving zone of normal looking epithelium
Histopathological Features
or raw area. Ragged white area may be present
It shows diffuse hyalinization with prominent which can be removed by gentle rubbing without
hyaline perivascular cuffing. These deposits are bleeding.
 Skin Disorders 641

Histopathological Features
Epithelium thickened showing hyperkeratosis,
acanthosis with basal layer being intact. In some
cases, there is extensive keratosis showing
‘basket-weave’ appearance.

Figure 32.12: White sponge nevus showing marked

increase in the spinous cell layer and vacuolated cells

Figure 32.11: White sponge nevus showing acanthosis

with clear cytoplasm

The cells of the entire spinous layer exhibit

intracellular edema and show pyknotic nuclei.
There is also oraganophilic perinuclear
cytoplasmic condensation (Fig. 32.11).
In addition, parakeratin plugs running deep
into the spinous layer are typically found. The
submucosa may show a mild inflammatory cell
infiltration (Figs 32.12 and 32.13). Figure 32.13: White sponge nevus showing
The characteristic feature of white sponge inflammatory cell infiltration
nevus is the “perinuclear eosinophilic conden-
sation” which is more appreciable in exfoliative sponge nevus in its hereditary pattern but its
cytology. This is proved ultra- structurally to be clinical and microscopic features are different. It
tangled masses of keratin tonofilament. is inherited as autosomal dominant trait and there
is defect in keratinization characterized by
Management cytoplasmic accumulation of tonofilament with
There is no specific treatment for this disease but loss of cellular interdigitation and desmosomes.
prognosis is very good. Palliative procedure to
relieve burning and tenderness. Clinical Features
It is most commonly seen in children with no sex
Hereditary Benign Intraepithelial Dyskeratosis predilection. It is commonly seen in eyes.
It is also called as ‘Witkop-Von Sallman Superficial gelatinous looking plaques occur on
syndrome’. It is superficially similar to the white a hyperemic bulbar conjunctiva.
642 Textbook of Oral Pathology

Eye is characterized by superficial, foamy, mucosa. Lesion frequently involves the corners
gelatinous white plaque overlying the cornea, of the mouth and appears as soft plaques with
sometimes producing temporary blindness. Eye pinpoint elevation when the mucosa is
lesion shows seasonal variation tending to stretched.
appear or increase in severity in the springs and
disappear. There may be photophobia and Histopathological Feature
blindness cause by involvement of cornea by
Buccal mucosa exhibits thickening of the
plaque formation and scarring.
epithelium with pronounced hydropic
Oral Manifestation degeneration. Numerous round; waxy-appearing
eosinophilic cells resembling minute epithelial
They are most commonly seen in buccal mucosa, pearls are present. There is peculiar intraepithelial
floor of mouth, ventral and lateral surfaces of dyskeratosis in addition to acanthosis and
the tongue, the gingiva, and palate. vacuolization.
It appears generally as white, spongy,
macerated lesions of the buccal mucosa with or
Management
without folds.
These lesions vary from delicate, opalescent No treatment is necessary but its potential for
white membranous areas to a rough, shaggy blindness, genetic counseling may be in order.
 CHAPTER
Nutrition and
33 Oral Cavity

Man is a complex biologic unit in a complex and parasitic infection. Hyperthyroidism and
environment. Duncan defined ‘metabolisms as hypermetabolic state which interfere with
sum total of tissue activity as considered in terms utilization.
of physicochemical changes associated with and
regulated by availability, utilization and disposal Clinical Features
of protein, fat, carbohydrate, vitamins, minerals, It occurs between the ages of 1 and 3 years in
water and influence which the endocrine exerts children.
on these processes. Alternation from this normal Symptoms—There is loss of weight and
metabolic process constitutes the disturbances of subcutaneous fats, wasting of muscles,
metabolism. pigmented changes in skin with hair loss,
hypotension, weakness, anemia and edema. In
Protein Deficiency severe cases, there is edema, episodes of
It is also called as ‘marasmus’ or ‘kwashiorkor diarrhea, skin pigmentation, liver enlargement
disease’. Marasmus is an overall deficit of food and poor resistance to infection.
intake, notably energy while the kwashiorkor is Kwashiorkor is distinguished from marasmus
associated with primary dietary protein by the presence of edema and the relatively less
deficiency. Protein is the most important group severe degree of body wasting.
of food stuff. In addition to contributing to cells
and intercellular material, proteins also help in Oral Manifestations
the formation of hormones, enzymes, plasma Bright reddening of tongue with loss of papillae
protein antibodies and numerous physiologically occurs. In kwashiorkor patient may have edema
important substances. of tongue and may develop scalloping around
the lateral margins due to indentation of the
Etiology teeth. Papillary atrophy may be present and
It is prolonged febrile illness, massive burns and dorsum of the tongue assumes an erythematous
large chronic ulcer. Stress, starvation and and smooth appearance.
persistent vomiting and diarrhea. Chronic There is bilateral angular cheilosis, fissuring of
infections like urinary tract infection, tuberculosis lip. Loss of circumoral pigmentation also occurs.
644 Textbook of Oral Pathology

Mouth becomes dry. However, there is reduced • Amyloidosis associated with multiple
caries activity due to lack of substrate carbo- myeloma.
hydrate. Epithelium easily becomes detached • Secondary amyloidosis—Associated with
from underlying tissue, leaving raw bleeding variety of chronic inflammatory disease.
surface. • Localized amyloidosis—It is characterized by
Decreased overall growth of jaws, delayed small localized deposits of amyloid in the
eruption, retarded growth of incisors and molars. skin, bladder and respiratory tract.
The gingiva and periodontal ligament membrane • Familial amyloidosis—It is rare condition such
exhibit varying degrees of degeneration. as familial Mediterranean form or familial
amyloidosis with polyneuropathy.
Deciduous teeth of children may exhibit linear
• Hormone related amyloid—It is associated with
hypoplasia of the teeth.
tumors of endocrine cells which secretes
peptide hormones.
Laboratory Findings
It may cause mild anemia which is normocytic Etiology
and normochromic. The reticulocyte count is It is caused by collagen diseases like rheumatoid
normal and the bone marrow tends to become arthritis, chronic infections like tuberculosis,
hypocellular. osteomyelitis, regional enteritis and ulcerative
colitis and malignant diseases like multiple
Amyloidosis myeloma, Hodgkin’s lymphoma and renal cell
It is also called as ‘amyloid disease’. It is deposition carcinoma.
of amyloid in the tissue.
Clinical Features
Forms of Amyloid It commonly affected organs are kidneys, heart,
gastrointestinal tract, liver, respiratory tract, skin,
• Type A (secondary) amyloid is a fibrillar eyes, adrenals, nerves and spleen. There may be
protein of unknown origin that is seen in primary localized collection of amyloid.
prolonged inflammatory disease, genetic
Symptoms—The general symptoms are fatigue,
disease and syndromes such as familial
weakness, ankle edema, dyspnea, paresthesia,
Mediterranean fever.
orthostatic hypotension and weight loss.
• Type B (primary) amyloid is thought to be
Purpuric spots caused by hemorrhage resulting
immunologic in origin because of sequence form amyloid deposits in the blood vessels.
homology with the NH 2 terminal end of
immunoglobulin light chain. It is commonly Sign—Superficial waxy lesion occurs on the
seen in patient with multiple myeloma and eyelids, nasolabial folds, neck, axilla or perineum
macroglobulinemia. and they may bleed on pressure. Congestive
cardiac failure is a common problem due to
• Type C—It includes amyloid of aging,
amyloid deposits on myocardium. There is
localized nonspecific amyloid and pheochro-
hepatomegaly, malabsorption or colitis may
mocytomas.
develop.
Types Oral Manifestations
• Primary amyloidosis—There is no evidence of Fibrous glycoproteins are deposited in sub-
preceding or existing disease. mucosa as well as in deeper muscular layer of
 Nutrition and Oral Cavity 645

tongue. Skin lesion may diffusely involve the face Classification

or may present as small elevated yellow nodules.
• Erythropoietic porphyria—There is excessive
Symptoms—There are difficulties in chewing, abnormal porphyrins formation in
swallowing or talking. The speech difficulties is developing erythrocytes. It is localized to
due paresis of the vocal cord resulting from bone marrow.
deposit of amyloid in the upper third of larynx. • Uroporphyria
Signs—Amyloid deposition of tongue will result • Protoporphyria
in macroglossia of tongue. It is seen in both primary • Hepatic Porphyria—In it liver is the site of
and secondary form. Tongue is enlarged and excessive porphyrin formation.
studded with small garnet colored enlargements • Acute intermittent porphyria
along with nodes of cheeks and lips. Mobility of • Porphyria variegate
the tongue is decreased. Yellowish nodules are • Porphyria cutaneous tarda
present along the lateral border of the tongue • Hereditary coproporphyria.
and impression from the teeth is also visible. The
Clinical Features
gingiva may be infiltrated and may be bluish,
spongy and hypertrophied. Xerostomia may It is transmitted as nonsex-linked recessive
result from salivary gland involvement. character and both sexes are equally affected.

Histopathological Features Symptoms—The first sign is excretion of red urine

containing uroporphyrin which may be noted
There is extracellular deposition in the at birth or apparent at first two year after birth.
submucosal connective tissue of amorphous Excessive deposition of the excess porphyrin in
eosinophilic material in the gingiva. This material the skin lead to photosensitivity. It is absent in
present is arranged in perivascular orientation or the neonatal period but may become apparent
diffusely. Congo red stain should be used for during first few years after it becomes exposed
staining. In this staining amyloid occurs red. to sunlight.
Diagnosis Signs—Vesicular and bullous eruption appears
Scintiscanning with Tc99m to localized soft on face, back and hand, i.e. exposed parts. Vesicle
contains a serous fluid which exhibits red
tissue deposits. Congo red is used to diagnose
fluorescence. Ruptured vesicle heals slowly and
amyloid which shows birefringence and
leaves depressed pigmented scars. There is often
dichroism.
coexisting anemia. In this disease there is
Management abdominal crisis and psychological or metal
symptoms. There is demyelination of nerve
You can treat amyloidosis by alkylating agents fibers.
like melphalan. Combination therapy using
melphalan, prednisone and fluoxymesterone Oral Manifestations
reported significant improvement. Renal
The porphyrin has an affinity for calcium
transplantation may be indicated.
phosphate and due to this, deposition of
porphyrin occur in dentine. Deciduous and
Porphyria
permanent teeth show red or brownish
It is a group of disorders in which there is discoloration which under ultraviolet light
defective metabolism of pyrrole compound. exhibits red fluorescence due to incorporation
646 Textbook of Oral Pathology

of porphyrins during development. Bullous, • Gaucher’s disease

erosive lesions of oral mucosa may be present. • Niemann-Pick disease
There is also atrophic cheilitis, advanced • Tay-Sachs disease.
periodontal diseases.
Hand-Schüller-Christian disease
DISTURBANCES IN LIPID METABOLISM It is also called as ‘multifocal eosinophilic
granuloma’ ‘chronic disseminated histiocytosis X’ or
Lipids are a heterogeneous group of organic ‘xanthomatosis. It is characterized by widespread
compounds which are relatively insoluble in skeletal and extraskeletal lesions and chronic
water but, soluble in solvent such as ether, clinical course. It is result of error in the
chloroform and benzene. metabolism of cholesterol and its esters.
I. Classification
Clinical Features
• Simple lipids—They are esters of fatty acids
It is more common in boys than girls 2:1. It occurs
with various alcohols, i.e. natural fats and
in early life usually before age of five.
waxes.
• Compound lipids—They are esters of fatty
Classic Triad
acids containing groups other than and in
addition to an alcohol and fatty acids. • Single or multiple areas of punched out bone
• Derived lipids—They are derivatives obtained destruction in skull.
by hydrolysis of the simple and compound • Unilateral or bilateral exophthalmos.
lipids, which still posses the general • Diabetes insipidus.
characteristics of lipids. Involvement of facial bone which is
• Miscellaneous—It includes carotenoids, commonly associated with soft tissue swelling
vitamins E and K. and tenderness causing facial asymmetry. Otitis
media and skin may some time exhibit papular
II. Classification or nodular lesion. Course is chronic with
• Histiocytosis X—It is also called as nonlipid numerous remissions and exacerbation.
reticuloendothelioses, idiopathic histiocytosis
and Langerhans cell disease. It is a inflam- Oral Manifestations
matory reticuloendothelioma condition with
Sore mouth with or without ulcerative lesion,
evidence suggesting that it may be reaction
halitosis, gingivitis and suppuration is present.
to some type of infection. There is
An unpleasant taste, loose and sore teeth with
pathological accumulation of histiocytes and
precocious exfoliation and failure of healing of
eosinophilic leukocytes. It is of 3 types.
tooth socket following extraction. Loss of
• Hand-Schüller-Christian disease.
supporting alveolar bone mimics advanced
• Eosinophilic granuloma of bone (chronic
periodontal disease.
localized Histiocytosis X)
• Letterer Siwe disease (acute disseminated
Histopathological Features
histiocytosis X)
• Lipid reticuloendothelioses—It is disturbance in Proliferative histiocytic phase—Accumulation of
sphingomyelin and glucosyl ceramide collection of leukocytes (eosinophilic) scattered
metabolism. throughout the sheets of histiocytes.
 Nutrition and Oral Cavity 647

Vascular granulomatous phase—Persistence of Signs—Lesion is destructive and well

histiocytes and eosinophils sometimes with demarcated, roughly round or oval in shape. The
aggregation of lipid laden macrophages. area destroyed is replaced by soft tissue. Tissue
of early lesion is soft and brown and since there
Diffuse xanthomatous phase—Abundance of foam
is no necrosis, it is not friable. Later it becomes
cells.
fibrous and grayish.
Fibrous or Healing Phase—Langerhans cells
contain rod shaped cytoplasmic structure called Oral Manifestations
as Birbeck granules. It may occur in jaws and overlying soft tissues.
There is loss of superficial alveolar bone often
Laboratory Findings mimicking juvenile periodontitis. Gingivitis and
bleeding gingiva, pain or ulceration is present.
Anemia and less frequently leucopenia and Loosening and sloughing of teeth often occurs
thrombocytopenia occur. Serum cholesterol level after destruction of alveolar bone. Sockets fail to
is normal but tissue cholesterol level is raised. heal normally.

Management Histopathological Features

Prognosis of this disease is good and half of the Primary cell is histiocyte which grows in sheets
patients undergo spontaneous remission over a and sheet like structures. It may coalesce to form
period of a year. It is usually treated by curet- multinucleated giant cells. When fibrosis occurs
tage or excision of lesion. The lesions which are eosinophils may disappear.
inaccessible are treated by irradiation. Some
Management
patients may be given drug treatment like pred-
nisolone, vinblastine and cyclophosphamide. It is usually treated with curettage and X-ray
therapy. Symptoms usually subside within 2
Eosinophilic Granuloma weeks after treatment.

This term was introduced by Lichenstein and Letterer Siwe Disease

Jaffe in 1940. It is also called as ‘unifocal It is an acute fulminating disease, which
eosinophilic granuloma’. It is the lesion of bone invariably occurs in infants usually before the
which is primarily histiocytes proliferation with age of 3 years.
an abundance of eosinophilic leukocytes by no
intra or extracellular lipid accumulation. Clinical Features
It is fulminating condition that most often occurs
Clinical Features
in infants younger than 1 year of age.
It occurs primarily in older children and young Symptoms—Initial manifestation is skin rash
adults and proportion of male to female is 2:1. involving trunk, scalp and extremities. Rash may
Skull and mandible are common site but femur, be erythematous, purpuric and ecchymotic,
humerus, ribs may be affected. some time with ulceration. Patient may have
Symptom—There may be local pain which may persistent low grade spiking fever with malaise
be dull and steady, swelling and tenderness. and irritability.
General malaise and fever may accompany the Signs—Splenomegaly, hepatomegaly and
eosinophilic granuloma of bone. lymphadenopathy is common. Nodular and
648 Textbook of Oral Pathology

diffuse involvement of visceral organs Clinical Features

particularly in lung and GI tract. Soft tissue and It is mostly seen in adults and progress is slow.
bony granulomatous reaction, hemorrhage,
anemia, failure to thrive. Signs—There is enlargement of the lymph nodes,
spleen and to a lesser extent the liver. There is
hepatosplenomegaly, CNS involvement.
Oral Manifestations
Symptoms—There may be bone pain due to
Enlargement of the gingival tissue occurs. There
changes in bone marrow. There is often bleeding
may be presence of ulcer on the oral mucosa.
from the nose or from the gums. Teeth extraction
Diffuse destruction of bone of maxilla and mandible from the affected area may result in bleeding
which may result in loosening and premature complication.
loss of teeth.
Pingueculae—The skin is sometimes pigmented
Histopathological Features and the conjunctival fibrous tissue may be
thickened and of brownish discoloration, a
Same as others but without foam cells and no condition known as ‘pingueculae’.
fibrosis occurs. Erlenmeyer flask deformities are seen in long
bone which is characteristic features of disease.
Laboratory Findings
Progressive anemia is often present as well as Histopathological Features
leukopenia or thrombocytopenia. Examination of biopsy of spleen and liver show
typical Gaucher cells which is round pale cell,
Management
containing a small eccentric nucleus and wrinkled
It has got very poor prognosis and it usually or crumpled silk cytoplasm. When the bone is
terminates fatally in short period of time. involved the bone marrow shows numerous
large foamy, slightly granular cells with small
Gaucher’s Disease round pyknotic nuclei, which are Gaucher cells.
It is most common of lipid reticuloendotheliosis.
It has got familial tendency and is thought to be Management
due to faulty metabolism of the lipoid, kerasin. The prognosis of malignant infantile form is very
The reticular cells and histiocytes are increased poor; the disease results in death usually within
in number and many of them become infiltrated the first year. Administration of purified gluco-
with kerasin. It is autosomal recessive. cerebrosidase.

Types Niemann-Pick Disease

• Type I (chronic nonneuropathic)—There is no It is also called as ‘sphingomyelin lipidosis’. It is
cerebral involvement. characterized by an abnormal storage of
• Type II (acute neuropathic)—It is characterized phospholipids due to lack of sphingomyelinase.
by hepatosplenomegaly and central nervous It is present in four forms; i.e. type A, B, C and
system disorders. D. Type A and B caused by deficiency of acid
• Type III (subacute neuropathic)—It resembles sphingomyelinase and type C and D occur due
type II but later onset and more protracted to mutation of NPC-1 gene involved with
clinical course. cholesterol processing.
 Nutrition and Oral Cavity 649

Clinical Features found. It is inherited as an autosomal recessive

It involves lungs, liver and the nervous system. trait.
There is hepatosplenomegaly, retarded physical
and mental growth and severe neurological Clinical Features
disturbance. It usually becomes apparent within first two
years of life, progresses during early childhood
Histopathological Features
and adolescence and terminates in death before
There is presence of foam cells, usually an puberty. Head is large, prominent forehead,
enlarged reticuloendothelial cell whose broad saddle nose and wide nostrils,
cytoplasm contains numerous droplets like hypertelorism and puffy eyelids with coarse
inclusion of lipid. Characteristic ‘sea blue’ histiocytes bushy eyebrows, thick lip, large tongue, open
in bone marrow aspiration may be seen. mouth and nasal congestion and noisy breathing.
Management Progressive corneal clouding, hepatosple-
nomegaly resulting in protuberance of abdomen.
It is only symptomatic to control infection. Death A short neck and spinal abnormalities are
usually occurs within 3 years of age. Enzyme typical, while flexion contractures result in the
replacement therapy and organ transplantation is ‘claw hand’.
also carried out.

Tay-Sachs Disease Oral Manifestations

It is caused by lack of hexosaminidase A. it Shortening and broadening of mandible with

results in accumulation of ganglioside within prominent gonions and wide intergonial
lysosomes of neurons. distance. There is typical spacing of teeth. Teeth
are small and mis shapen. There may be gingival
Clinical Features hyperplasia.
It may be mild or severe in nature. In severe form
neuronal degeneration will occur. There is also Histopathological Features
blindness, intractable seizures. There is excessive accumulation of intracellular
mucopolysaccharides in many tissues and organs
Histopathological Features
throughout the body including liver, spleen,
They are same as seen in other lipid reticuloend- reticuloendothelial system, nervous system,
othelioses. cartilage, bone and heart. Abnormal deposits are
found in many sites with involved fibroblasts
DISTURBANCES IN CARBOHYDRATE assuming the appearance of clear (gargoyle) cells.
METABOLISM
Laboratory Finding
Hurler’s Syndrome
It is disturbance of mucopolysaccharide There is elevated level of mucopolysaccharides
metabolism, which is characterized by elevated in the urine.
mucopolysaccharide excretion ratio and an
Management
excessive intracellular accumulation of
chondroitin sulphate B and heparin sulphate in Death usually occurs before the age of ten due
those tissue and organ where they are normally to pneumonia and cardiac failure.
650 Textbook of Oral Pathology

Lipoid Proteinosis Management

It occurs due to disturbance of mucopoly- Symptomatic treatment should be given.
saccharide metabolism or alteration in formation
Disturbance in carbohydrate mechanism
of lipoprotein. It is transmitted as autosomal
dominant trait.
Hereditary Fructose Intolerance

Clinical Features It is transmitted as autosomal recessive trait. It

results from a deficiency in fructose 1-phosphate
It is present since birth. It occurs on lip, oral
aldolase.
mucosa, face, neck, hands, axillae, scrotum,
perineal area, intergluteal cleft, eyelids, knees Clinical Features
and elbows.
It is manifested by hypoglycemia and vomiting
Symptoms—Inability of infant to cry at birth and
after ingestion of fructose containing foods.
hoarseness of voice due to laryngeal involvement Affected individuals rapidly acquire an intense
and dyspnea may be severe.
aversion to all sweets and fruits.
Signs—Patient develops solitary or clustered
yellowish white waxy nodules. It is solid in Oral Manifestations
consistency and fixed to underlying tissue. It There are fewer incidences of caries in these
varies in size from 1 mm to 0.5 cm in diameter. It individuals.
has non ulcerated surface.
Hypoglycemia
Oral Manifestations
It is a subnormal blood glucose level.
Symptoms—Recurrent painful parotitis with
stenosis of parotid duct opening occurs. Causes
Congenital absence of teeth and severe enamel
hypoplasia may occur. Delayed meal, inadequate total caloric intake and
Yellowish white papular plaque on the oral unusual physical exertion. Insulin measurement
mucosa is present. Lips become thickened and error, insulin overdose, the “brittle” diabetic, oral
nodular white while tongue becomes thickened, hypoglycemic agents.
enlarged, very firm to palpation and sometimes
Clinical Features
bound to floor of mouth.
Symptoms—Sudden onset of hunger, sweating,
Histopathological Features shakiness, tremor, anxiety, restlessness,
faintness, weakness, nausea, palpitations.
Hyalinosis of connective tissue occurs which
Progression to mental confusion, bizarre
begin around the sub-epithelial capillaries.
Hyaline material stains intensely with periodic behavior, personality change, reduced level of
consciousness, loss of consciousness, seizures.
acid Schiff stain. There is deposition of lamellar
material around the blood vessels, nerves, hair Signs—Pulse rapid, blood pressure may be
follicle. In the advance stage, there is deposition elevated and moderate hypothermia may be
of amorphous substance within the dermal present due to sweating, hyperventilation and
connective tissue. vasodilatation. Bizarre or aggressive behavior
 Nutrition and Oral Cavity 651

may be present, staggering gait, may appear the tongue. The oral changes sometime described
drunk, cold, clammy skin. as ‘stomatitis’, ‘glossitis’ and ‘stomatitis producing
thrush like picture’. Buccal mucosa is present with
Laboratory Findings red and white spots, erosions, ulcers and
desquamation. Lesion on the tongue is sometime
Blood glucose reduced.
papillated and halitosis is often severe.
Management
Management
Glucagons should be given consult physician and
Two hundred twenty mg of zinc sulfate tds daily
ensure that the airway is patent and protected
produces remarkable improvement.
and that ventilation is adequate in the patient
with a reduced level of consciousness. Hypophosphatasia

DISTURBANCES IN MINERAL METABOLISM It is a hereditary disease transmitted as a

recessive autosomal characteristic. There is
Acrodermatitis Enteropathica deficiency of serum alkaline phosphate. It
resembles rickets. There is low level of serum
It is also called as ‘zinc deficiency’. Rare disease of
alkaline phosphatase activity and elevated
infancy and childhood, transmitted as autosomal
urinary excretion of phosphorylethanolamine
recessive character. Since supplements appear to
which result in formation of defective bone
be curative even pathogenesis of the character- matrix.
istic lesions involves a number of etiologic fac-
tors. Types

Clinical Manifestations • Infantile type

• Juvenile type after six month age
It usually occurs in childhood after weaning. The • Adult type which is mild.
primary signs of the disorder are skin lesion, hair
loss, nail changes and diarrhea. Clinical Features
Erythematous, pustular, moist erosions of the
The onset of the disease is within the first 6
orofacial areas occur as an early manifestation.
months of life. there is anorexia, irritability,
In fully developed conditions the buttocks,
persistent vomiting and mild pyrexia.
elbows, fingers and toes are affected by
vesiculobullous rash similar to that affecting the Infantile form—Severe hypocalcemia, bone
orofacial region. Retarded body growth and abnormalities and failure to thrive manifest the
mental changes also occur with some frequency. infantile form. Most of the cases are lethal.
Juvenile form—Hypophosphatasia of childhood
Oral Manifestations is characterized by increased infection, growth
The buccal mucosa, palate, gingiva and tonsils. retardation and rochite like deformities
Large number of children suffers from including deformed extremities, costochondral
candidosis. The perioral area usually being junction enlargement (rochite rosary) and
affected by weeping erosions, angular fissuring pulmonary gastrointestinal and renal disorders.
and spreading dermatitis. Adults form—The adult form includes fracture
There may be numerous small whitish with a prior history of rickets and osseous
papillomas on the buccal mucosa and borders of radiolucency.
652 Textbook of Oral Pathology

Homozygous involvement—Disease with homo- hydrates, vitamins, minerals and water. The
zygous involvement begins in utero and patient defective absorption may be due to defective
die within 1st year. Bowed limb bone and digestive or defective intestinal absorption.
marked deficiency of skull ossification.
Clinical Manifestations
Heterozygous involvement—In heterozygous,
Excessive amounts of fat are passed in stools,
there is milder effect with poor growth and
inducing a concomitant excessive loss of calcium
fracture and deformities.
which in turn causes calcium deficiency with
Skull—Skull suture close early resulting in ensuing low blood calcium level and occasional
bulging suture and grayal marking on internal tetany. This disturbance in calcium metabolism
surface of skull and brachycephalic shape. may result in osteoporosis and other skeletal
anomalies.
Oral Manifestations
Symptom—It usually begins with intestinal
There is almost total lack of cementum and disturbances including diarrhea, constipation and
normally attached periodontal fiber leading to flatulence. Nervous irritability, numbness and
poor support and premature loss of teeth in tingling of the extremities occur; malaise and
deciduous teeth. There is also delayed eruption generalized weakness are also common.
of permanent teeth. Teeth may be hypoplastic. The
Sign—The skin changes include irregular
pulp chamber and root canal are sometimes
brownish pigmentation particularly on the face,
larger than normal. The roots either fail to
neck, arms and legs and drying of skin with scaly
develop fully or undergo early resorption of the
eruptions.
apices.
The alveolar bone which support the teeth Oral Manifestations
fail to develop normally which result in
There may be severe glossitis with atrophy of
premature loss of primary teeth. There is
filliform papilla, although the fungiform papillae
inflammation of the gingiva.
persist for sometime on the atrophic surface.
Histopathological Features Painful burning sensation of the tongue and oral
mucosa are common. There are small projections
There is abundant production of poorly
which are pink or red in color and the
mineralized osteoid. There may be increased
erythematous swelling and palatal lesions
amount of mineral bone, which is of less mature
appear as multiple aphthous ulcers.
in nature.
Exfoliated teeth show absence of cementum Management
on the root.
Administration of vitamin B12 and folic acid is
done. Diet must be carefully supervised and
MISCELLANEOUS DISORDERS
supplemented with vitamins and minerals.
Malabsorption Syndrome
DISORDERS OF VITAMINS
It is also called as ‘sprue’, ‘idiopathic steatorrhea’,
‘celiac disease’. It includes conditions causing poor Vitamins are organic substances in food which
digestion or absorption to a variable degree of a are required in small amounts but which can not
number of nutrients, fats, proteins, carbo- be synthesized in adequate quantities in the body
 Nutrition and Oral Cavity 653

and which are soluble in either fat or water. tissues. The digestion for the liberation of
Vitamins are needed in small quantities to act as vitamins and its absorption is a result of
a cofactor in a variety of metabolic reactions. Your breakdown of cellular structures in the gut.
body needs only small amounts of vitamins. But Vitamin B-complex is not stored in appreciable
because what the body manufacture is often not amounts in the body tissues except vitamin B12.
enough, these must be obtained from your diet Excretion of vitamins occurs in the kidney.
and from supplements. The oral signs of deficiency of vitamin-B
Vitamins occur in a natural and in a occur in the oral tissues like tongue, mucous
physiologically inactive form and are called as membrane and gingiva. It may result in
provitamins. They become activated only after dermatitis, stomatitis and gastritis and blood and
conversion within the animals. For example, bone marrow disorders. Degenerative changes
vitamin A exists in plants in the form carotene, of brain and nerves are also a characteristic
which is activated in the liver. feature of deficiency since nerve tissue depends
Small amounts of vitamins can be on glucose. In hemopoiesis, vitamin B12 and
synthesized endogenously. For example, vitamin folate are essential for maturation of red cell
D is synthesized from a precursor steroid, niacin precursors.
from tryptophan which is an essential amino
acid, vitamin K and biotin by intestinal Thiamine (vitamin B1)
microflora.
Deficiency of vitamins may be primarily due It is a vitamin for calm nerves. It is also known
to vitamin deficient diet or secondarily because as ‘aneurin’. It was discovered by Eijkman in 1897.
of disturbances in intestinal absorption, transport It is a colorless basic organic compound
in blood, tissue storage or metabolic conversion. composed of a sulfated pyrimidine ring.

Causes of Vitamin Deficiency Absorption and Excretion

• Decreased amount of intake of essential It is readily absorbed from both small and large
nutrients. intestine. The capacity of the human intestine to
• Impaired absorption from the alimentary absorb this vitamin is limited to about 5 mg per
tract. day. It is phosphorylated by the liver and
• Increased metabolism due to rapid growth. kidneys. In tissues, it is found as thiamin
• Inadequate storage, fever and pregnancy. pyrophosphate. Thiamin pyrophosphate is a
coenzyme for decarboxylation of pyruvate to
Water-Soluble Vitamins acetyl coenzyme A. Any excess supply of
Water-soluble vitamins are found in yeast, grain, thiamine is excreted in the urine.
rice, vegetables, fish and meat. They are essential
co-enzymes required in energy releasing Functions in the Body
mechanisms and in hemopoiesis. They also act • Growth—It promotes growth, protects heart
as co-enzymes for metabolism of proteins, muscle and stimulates brain action.
carbohydrates and fats.
• Nervous system—It plays an important role in
the normal functioning of the entire nervous
B-complex Vitamins
system.
Most of B-complex occurs in nature in the bound • Digestion—It aids in digestion especially that
form within the cells of vegetables or animal of carbohydrates.
654 Textbook of Oral Pathology

• Diuretic—It is a mild diuretic and it increases – Peripheral neuritis

urine formation. – Korsakoff’s psychosis.
• GIT—It improves peristalsis and helps
prevent constipation. Wet Beriberi
• Blood cells—It maintain the normal blood It is marked by cardiac dilation with four
count and improves circulation. chamber enlargement, pallor and flabbiness of
• Others—It also reduces fatigue, increases myocardium.
stamina, prevent premature ageing and
Etiology
senility by increasing mental alertness and
promotes a healthy skin. • Diet—It is caused due to eating diets in which
calories are derived from polished rice.
Deficiency Symptoms • Alcoholics—It is commonly seen in chronic
alcoholics due to their poor nutrition in
Nervous disorders—When cells can not metabolize general and also because alcohol interferes
glucose aerobically, it affects the nervous system with intestinal absorption of thiamine.
first since it depends entirely on glucose for its • Others—It is often precipitated by infection,
energy requirements. There is mental pregnancy and lactation.
depression, nervous exhaustion and insomnia.
Pathogenesis
Digestive symptoms—It occurs due to defective
Deficiency of thiamine → incomplete
hydrochloric acid production in the stomach. metabolism of glucose → accumulation of
Patient complains of loss of appetite, poor pyruvic acid and lactic acid in tissue and body
digestion, chronic constipation and loss of fluid → dilation of peripheral blood vessels →
weight. fluid may leak out through capillaries, producing
edema → high cardiac output, heart dilation.
Heart—There is accumulation of pyruvic acid
and lactic acid derived from it, which produces Clinical Features
vasodilatation and increases cardiac output. The
Symptoms—Pain in legs after walking due to
heart muscle becomes lazy and fatigued and the
accumulation of lactic acid.
auricles or the upper chambers of the heart lose
their strength and it gradually enlarges. It may Cardiac signs—There is tachycardia and increased
lead to a condition known as ‘hypertrophy of the blood pressure, cardiomegaly, increased JVP and
heart’. palpitations. There is also presence of sinus
tachycardia and inverted T waves.
BERIBERI Skin is warm due to vasodilation. Edema may
develop rapidly to involve leg, face and trunk.
Prolonged gross deficiency can cause beriberi.
There are three types of beriberi: Histopathological Features
• Wet beriberi There is interstitial myocardial edema, swelling
• Dry beriberi of the myofibrils.
• Infantile beriberi
• Others—Other diseases, which can be Management
associated with it are: Thiamine 50 mg IM for 3 days then 10 mg 3 times
– Wernicke’s encephalopathy daily by oral route.
 Nutrition and Oral Cavity 655

Dry Beriberi reactions. It is stable to boiling in an acidic

solution. It is decomposed by heat. It is also
Clinical Features
destroyed by ultraviolet light.
It is a peripheral neuropathy. In long standing
cases, there is degeneration and demyelination Absorption and Excretion
of both sensory and motor nerve fibers resulting It is readily absorbed from the intestinal tract and
in severe wasting of muscles. Blood pyruvate is phosphorylated in the wall of the intestine. It
levels are normal. is carried to the tissue of the body ad
incorporated into the cells enzymes. It is stored
Oral Manifestations
in liver, kidneys and heart. Riboflavin is excreted
There is hypersensitivity of oral mucosa. Pain in primarily in the urine and bile Sweat are other
the tongue, teeth, jaws and face. minor routes of excretion.

Wernicke’s Encephalopathy Function in the Body

It is commonly seen in alcoholics with persistent • It is essential for growth and general health.
vomiting. There is a classical triad of ocular • Metabolism—It is involved in the metabolism
abnormalities, ataxia and confusion. There are of carbohydrates, fats and proteins. It is
facial symmetrical areas of grayish discoloration. essential for normal tissue maintenance.
There is also bilateral symmetrical ophthal- • Nervous system—It helps in functioning of the
moplegia and ataxia. nervous system.
Histologically, there is hypertrophy and • Digestion—It helps in digestion and prevents
hyperplasia of small blood vessels. constipation.
Injection of thiamine should be given. 50 mg • Eyes—It alleviates eye strain and it is helpful
by slow intravenous injection followed by 50 mg in counteracting the tendency toward
daily by oral route for a week. glaucoma.
• Others—It promotes a healthy skin, nails and
Korsakoff’s Psychosis hair and strengthens the mucous lining of the
mouth, lips and tongue.
In it, there is a predominant abnormality in
mental function which is memory defect. There
is profound impairment of memory recall and Causes of Deficiency
new learning ability. It occurs due to inadequate diet and also
inadequacy of other essential nutrients including
Riboflavin (Vitamin B2) vitamins and proteins.
It is also called as the ‘beauty vitamin’. It is a Secondary deficiency—It may occur due to diseases
yellowish green fluorescent compound soluble of the intestinal tract. Prolong use of psycho-
in water. The word riboflavin is derived from logical drug that interfere with production of
two sources ribose, referring to ribose sugar flavin monophosphate. Chronic alcoholism,
found in several vitamins and enzymes and burns and trauma.
flavin meaning yellow. It is an essential
component of coenzyme flavin mononucleotide Deficiency Symptoms
and flavin adenine dinucleotide, involved mainly It affects the nasolabial fold and ala of the nose
in a wide variety of oxidation-reduction which exhibits a scaly gray dermatitis and
656 Textbook of Oral Pathology

consists of enlarged follicles around the side of and NADP, which are important pyridine
the nose which is plugged with dry sebaceous nucleotides which play an important role in
material. redox reactions involving carbohydrate, protein
and lipid metabolism. Deficiency of niacin leads
Ocular changes—It consist of corneal
to a disease called as ‘pellagra’ which means
vasodilatation, photophobia and superficial and
rough skin.
interstitial keratitis. There may be itching and
burning of the eyes. Function in the Body
Skin and nails—It may also result in dull or oily • Nervous system—It is important for proper
hair, an oily skin, premature wrinkles on the face blood circulation and healthy functioning of
and arms and split nails. the nervous system.
There is also malfunctioning of adrenal • Gastrointestinal tract—It is essential for the
glands, anemia, vaginal itching and cataract. proper metabolism of proteins and
carbohydrates.
Oral Manifestations • Blood vessels—It dilates the blood vessels and
Tongue—Glossitis which begins with soreness of increases the flow of blood to the peripheral
lip and lateral margins of the tongue. Filiform capillary system.
• Hormone—It is essential for the synthesis of
papillae become atrophic while fungiform
sex hormone, estrogen, progesterone and
papillae remain normal or become engorged and
testosterone as well as cortisone, thyroxin and
mushroom shaped giving the tongue a reddened
insulin.
coarsely granular appearance. In severe cases,
• Others—It helps to maintain a normal healthy
the tongue becomes glazed and smooth due to
skin.
complete atrophy of the papillae and exhibits a
magenta color. Pellagra
Lip—Lips become red and shiny because of Causes of Deficiency
desquamation of epithelium. Paleness of lips and
cheilitis which is seen as maceration and • Tryptophan deficiency—If insufficient,
fissuring at the angle of the mouth. There is tryptophan is available for synthesis of niacin.
maceration at angle of mouth with pain on the • Diet—Dietary deficiency of niacin. High
opening mouth, it again results in fissuring and dietary levels of amino acid lucine antagonise
the synthesis of NAD and NADP.
cracking with ulceration. As the disease
• Miscellaneous—Chronic alcoholism, diarrhea
progresses, angular cheilitis spread to the cheek,
and carcinoid syndrome.
the tissues bleed easily and are painful if
secondarily infected. Clinical Features

Management It can be developed in 3 weeks with prodromal

symptoms of loss of appetite, vague
Riboflavin 25,000 to 50,000 mcg is given daily in gastrointestinal disturbances and numbness or
divided doses. burning in various locations.
It is called as disease of 3-Ds
Niacin (vitamin B3)
• Dermatitis
It is also known as ‘nicotinic acid’. Niacin is • Diarrhea
required for the formation of coenzyme NAD • Dementia.
 Nutrition and Oral Cavity 657

Skin—There is an erythema resembling severe rapidly. Superimposed ANUG or Vincent’s

sunburns appears symmetrically overall parts of infection involving the gingiva, tongue and
the body exposed to sunlight and especially on mucosa is common.
the neck. Affected area is well demarcated from
the normal. In acute cases, skin lesions may Management
produce vesiculation, cracking, exudation, Niacin 10 mg or 10,000 mcg per day. Vitamin B
crusting with ulceration and secondary infection.
complex. Alcohol should be stopped.
In chronic cases, dermatitis occurs as roughening
and thickening of skin with brown pigmentation. Pantothenic Acid (vitamin B5)
Alimentary tract—Anorexia, nausea, dysphagia. It is water soluble vitamin of the B complex
Glossitis precedes the skin lesions. Non infective group. It was discovered by Roger Williams in
inflammation may extend through the 1933. Tissue extracts from a variety of biological
gastrointestinal tract. Diarrhea is caused by materials provided a growth factor for yeast. This
atrophy of gastric epithelium followed by growth factor is identified as pantothenic acid,
submucosal inflammation which is then derived from Greek word ‘pantos’ meaning
followed by ulceration. everywhere. It is a pale yellow oily liquid which
is not crystallized, but its calcium crystallizes
Nervous system—Delirium is the most common
readily and this is the form in which it is
mental disturbance in the acute form and
generally available.
dementia in chronic cases. There is also loss of
appetite, irritability and burning sensation in Function in the Body
different areas of the body. • It is a part of enzyme system which play a
vital role in the metabolism of carbohydrates,
Oral Manifestations
fats and protein and in the synthesis of amino
Entire oral mucosa becomes fiery red and painful acids and fatty acids.
and salivation is profuse. • It is also essential for the formation of
Tongue—The epithelium of the entire tongue is porphyrins, the pigment portion of the
desquamated. The filiform papillae are most hemoglobin molecule.
sensitive and disappear first; the fungiform • It stimulates the adrenal glands and increases
papillae may become enlarged. The tongue production of cortisone and other adrenal
becomes red swollen and beefy and in animals, hormones.
the deficiency leads to black tongue. In early • It is primarily used as an anti-stress factor and
stages, only the tip and margins of the tongue protects against most physical and mental
are swollen and red. In advanced cases, the stress.
tongue losses all the papillae and the reddening • It increases vitality, wards off infections and
becomes intense. In this stage, the tongue speeds recovery from ill health.
becomes so swollen that indentation from the • It helps in maintaining the normal growth
teeth are found along the borders of the tongue. and development of the central nervous
The mouth is sore and shows angular stomatitis, system.
cheilitis. • It prevents premature ageing and provides
Symptoms—Tenderness, pain and ulceration protection against any damage caused by
begin at the interdental papillae and spreads excessive radiation.
658 Textbook of Oral Pathology

Deficiency Symptoms Biotin (vitamin B8)

Muscle tissue—Chronic fatigue, muscle cramps, It functions as a coenzyme for four carbohydrates
painful and burning feet and muscular weakness. involved in fatty acid and amino acid meta-
Nervous system—Mental depression, irritability, bolism. Previously was known as ‘vitamin H’.
dizziness and insomnia.
Function in the Body
Gastrointestinal—It may lead to gastric distress
and constipation. • Metabolism—It is involved in the metabolism
of carbohydrates, proteins and fats.
Others—Increases tendency toward infection,
graying and loss of hair, skin disorders, low blood • Hair—It is essential for the growth and health
sugar, low blood pressure and duodenal ulcer. of the hair. It prevents premature graying of
the hair as well as hair loss.
Management • Others—It helps to maintain the skin and
It is given in the dose of 1000 mg daily for 6 nervous system in a sound condition. It
weeks. controls proper distribution of color
pigments.
Pyridoxine (vitamin B6)
It is an important coenzyme in the Deficiency Symptoms
intermedullary metabolism of amino acids and
• Skin—Scaly dermatitis, eczema, seborrhea
complex glycolipids. It is a white crystalline
and prickling of the skin.
substance soluble in water and alcohol.
• Hair—It can cause alopecia and dandruff.
Deficiency Symptoms • Nervous—There is confusion, mental
Nervous—Peripheral neuropathy, mental depression and drowsiness.
retardation, irritability, mental confusion and • Muscle—There is muscular weakness,
nervousness. extreme fatigue and lassitude.
• Others—Anemia, lack of appetite, hearing
Blood—Anemia, albuminuria and leukopenia.
abnormalities and lung infections.
Skin—Dermatitis and eczema. • Oral—The fleshy part of the tongue may
Others—Kidney stones, inflammation of the waste away.
colon, damage to the pancreas, loss of muscular
control, migraine headache and premature Management
senility. • 20 mcg of biotin taken daily for 10 days IM
Oral Manifestation can heal skin lesions.
• Oral biotin to be taken in amount of 400 mcg
Cheilosis—Cracking at the corner of the lip.
daily for 8-12 weeks.
Glossitis—Inflammation of the tongue. • Shampoo coating 1% biotin can be useful in
Others—Angular stomatitis, Tooth decay and controlling excessive hair loss.
halitosis.
Folic Acid (vitamin B9)
Management
It is also known as ‘folacin’ or ‘folate’. It is a water-
10-50 mg daily in divided doses. soluble vitamin. It is a yellow crystalline
 Nutrition and Oral Cavity 659

substance sparingly soluble in water and soluble • Reproductive disorders—Spontaneous

in acid solution. It undergoes fairly rapid abortions, difficulty during labor and high
destruction when heated in neutral or alkaline infant death can also occur. Loss of libido
substances. occurs in males.
• Nervous—Dementia, mental depression and
Functions in the Body fatigue.
• RBC—Folic acid in combination with vitamin
Oral Manifestations
B12 is essential for the formation, maturation
and multiplication of red blood cells. Filiform papillae disappear first and fungiform
• Nerve—It is necessary for the growth and papillae remain prominent. In severe cases,
division of all body cells, including nerve cells fungiform papillae are lost and tongue becomes
and for manufacturing a number of nerve thick, smooth and fiery red. Severe ulcerative
transmitters. stomatitis may be seen. Swelling and redness of
• Hair and skin—It is essential for the health of lips and lateral margin of the tongue.
skin and hair and helps to prevent premature
graying of hair. Hematological Findings
• Pregnancy—It is an important nutrient for the
The blood and bone marrow in megaloblastic
pregnant women and her developing fetus.
anemia due to folate deficiency are similar to
Folic acid also improves lactation.
those in vitamin B12 deficiency except that the
• Others—It helps in building of antibodies
serum and red cell folate levels are low.
which prevent and heal infection. It also
produces nucleic acids, RNA and DNA.
Management
Deficiency Causes A daily dose of 5,000 mcg to 10,000 mcg of folic
acid is sufficient and a maintenance dose of 5000
• Decreased intake—Inadequate diet, impaired
mcg once in week is given in cases of
absorption, malabsorption states and intrinsic
megaloblastic anemia.
intestinal diseases.
• Increased loss—Hemodialysis. Cyanocobalamin (vitamin B12)
• Increased requirement—The body demands Vitamin B 12 is a complex organomatrix
exceed the intake like in pregnancy, infancy, compound called as cobalamine which is cobalt
leukemia, hemolytic anemia. containing porphyrins. It is freely soluble in
• Others—Impaired utilization, diseases of the water. It is resistant to boiling in neutral solution,
upper small bowel where folate is mainly but it is liable to destruction in the presence of
absorbed and idiopathic. alkalis and acids.

Clinical Features Functions in the Body

• Anemia—Deficiency of folic acid cause • Red blood cells—Like vitamin B6, it is essential
anemia which often occurs in pregnant in the production and regeneration of red
women and also children. blood cells.
• Skin—Loss of hair, grayish brown skin • Nervous—It improves concentration,
pigmentation can also occurs. memory, balance and relieves irritability.
660 Textbook of Oral Pathology

• Metabolism—It is necessary for proper sensation occur. Small shallow ulcers resembling
utilization of fats, carbohydrates and proteins aphthous ulcers on the tongue with atrophy of
for body building. It is also used in papillae with a loss of normal muscle tone is
metabolism of folic acid. called as ‘hunter’s glossitis. Fiery red appearance
• Others—It promotes growth and increases of tongue due to inflammation and burning
appetite in children. sensation.
Discomfort in wearing dentures is due to
Causes of Deficiency weakened muscular tone.

• Congenital—Congenital deficiency without

Histopathological Features
gastric atrophy.
There is epithelial atrophy, enlarged basal cell
• Systemic diseases—Diseases of terminal ileum,
nuclei and increased mitosis in basal epithelium.
i.e. Crohn’s disease.
Epithelial dysplasia and nonspecific infiltration
• Defective absorption—There is defective of lymphocytes, plasma cells or polymor-
absorption of vitamin B12. There is chronic phonuclear cells in the lamina propria.
atrophic gastritis with failure of production
of intrinsic factor. Laboratory Findings
• Smoking—Studies show that smokers have
lower levels of vitamin B12 and folic acid than There is decrease in the count of red blood cells.
Cells show macrocytosis and poikilocytosis.
non-smoker.
There is increase in hemoglobin content which
• Others—Inadequate diet and intrinsic factor is proportional to their increased size. MCHC is
deficiency. normal.
In advanced cases, the red blood cell abnor-
Deficiency Symptoms
malities are detected like polychromatophilic
Deficiency of vitamin B12 leads to megaloblastic cells, stippled cells, nucleated cells, Howell-Jolly
anemia or pernicious anemia. However, bodies and Cabot’s ring. Achlorhydria due to
pernicious anemia is a result of deficiency of absence of gastric hydrochloride secretion and
intrinsic factor, which is essential for absorption pH of gastric content is usually high.
of vitamin B12 and hence a deficiency of vitamin
B12. Bone Marrow Findings
It occurs in 5th to 8th decades of life. It is more
common in men than in women. There is Increased number of immature red cells or mega-
generalized weakness, numbness and tingling of loblasts with a few normoblast. Polymacrocytes
the extremities. Fatigue, headache, dizziness, or large PMN’s with large poly labeled nuclei
nausea, vomiting, diarrhea, loss of appetite, are found. Megakaryocytes appear normal.
pallor and abdominal pain.
Management
Oral Manifestations
Oral—In a dose from 6 to 150 mcg. Taken in these
Tongue—There is sore painful tongue, glossitis doses, it helps in the treatment of lack of
and glossodynia. Tongue is inflamed and is beefy concentration, fatigue depression, insomnia,
red in color. Painful and burning lingual anorexia, poor memory and loss of weight.
 Nutrition and Oral Cavity 661

Parenteral—1000 mcg of vitamin given twice • Weakened blood vessels particularly

weekly in cases of anemia. microvessels having least muscular supports.
• Defective synthesis of osteoid which is
Vitamin C derivative of collagen.
It is also called as ‘ascorbic acid’ and ‘antibiotic • Impaired wound healing.
vitamin’. It is a modified simple sugar. It is the
most active reducing agent. Its highest Pathogenesis
concentration is in the pituitary, adenoid, eye
There is defective formation of collagen in
and WBCs. Stress and corticotrophin leads to loss
of ascorbic acid from the adrenal cortex. It is a connective tissues because of failure of
powerful antioxidant. It is necessary for normal hydroxylation of proline to hydroxyproline
maintenance of intercellular substances of which is a characteristic amino acid of collagen.
connective tissue in bone and other tissue of There is also increase permeability of capillary
mesenchymal origin. (hemorrhage), anemia due to erythropoiesis and
defective collagen formation.
Functions of Vitamin C
Clinical Features
• Synthesis—It is important in the formation of
collagen, chondroitin sulfate and Infantile scurvy—Lassitude, anorexia, painful
neurotransmitter. limbs and enlargement of costochondral
junction.
• Maintenance—It is useful for maintenance of
folate pool and mobility and phagocytic Folliculosis—Hair follicle rises above skin and
activity of neutrophils. It is also necessary for there are perifollicular hemorrhages, i.e. tiny
maintenance of bones and proper points of bleeding occurring around the orifice
functioning of the adrenal and thyroid of hair follicles with heaping of keratin like
glands. material.
Hemorrhage may occur in the joint, into
• Absorption—It enhance the absorption of iron
nerve sheath under the nails or conjunctiva.
in the body.
Petechial hemorrhage occurs in buttocks,
• Metabolism—Tryptophan, norepinephrine abdomen, legs, arms, ankle and nail beds.
and tyrosine metabolism require vitamin C. Scorbutic child usually assumes a ‘frog like’
• Others—It promotes healing and protects position and this may reflect as subperiosteal
against all forms of stress. hemorrhage. Epistaxis, anemia and delayed wound
healing. Edema of the limbs and face is a frequent
Deficiency Symptoms finding in severe ascorbic acid deficiency. It may
Mild deficiency may appear in the form of lead to premature aging, thyroid insufficiency
lassitude fatigue, anorexia, muscular pain and and lower resistance to all infections.
greater susceptibility to infection. A prolonged
deficiency may cause scurvy. Oral Manifestations
It occurs chiefly in gingival and periodontal
Scurvy region. Interdental and marginal gingiva is
Prolonged deficiency of vitamin C may result in bright red, swollen, smooth, shiny surface
scurvy. It is characterized by producing an appearance known as ‘scurvy bud’.
662 Textbook of Oral Pathology

In fully developed scurvy, the gingiva becomes Functions—It helps in the transportation of fats
boggy, ulcerated and bleeds easily. Color in the body and prevents accumulation of fat in
changes to violaceous red. the liver. In combination with fatty acid and
Typical fetid breath of the patient with phosphorus, it stimulates the formation of
fusospirochetal stomatitis. In severe cases, lecithin, an important constituent of nerve cells
hemorrhage and swelling of periodontal in the body. It goes directly into the brain cells
ligament membrane occurs followed by loss of to produce a chemical that aids memory.
bone and loosening of teeth which are exfoliated. Deficiency symptoms—It may cause cirrhosis and
fatty degeneration of the liver, high blood
Histopathological Features pressure and atherosclerosis.
Osteoblasts fail to form osteoid. Cartilage cells Doses—It can be given in doses of 1000-2000 mg
of epiphyseal plate continue to proliferate in daily in divided doses.
normal fashion and salts are deposited in the
matrix between the columns of cartilage cells. Inositol
The calcified matrix material is not destroyed so
It is a crystalline compound which has sweet
that wide zone of calcified but non-ossified
taste. It is highly soluble in water and is not
matrix, called the ‘scorbutic lattice’ develops in
destroyed by heat in neutral, acid and alkaline
the metaphysis. As the lattice increases in width media.
a more and more fragile zone develops, so that
eventually complete fracture of the spicule Functions in the Body
occurs with separation and deformity of the
cartilage shaft joint. • It is essential for transportation of fat in the
body.
Laboratory Features • It is important in providing nourishment to
the brain cells.
Anemia in scurvy is mild to moderate but may • It helps to lower cholesterol levels.
be severe. It is usually normocytic and • It also promotes the growth of healthy hair
normochromic and associated with leucopenia and helps to prevent its falling.
and thrombocytopenia, reticulocytosis and • It helps in preventing eczema.
normoblastic hyperplasia of the bone marrow
Deficiency symptoms—It can cause alopecia or
are other changes.
patchy baldness, gastritis hypertension, fatty
infiltration in the liver, hardening of the liver and
Management
eczema.
Vitamin C 250 mg 3 times daily can be given.
Doses—It is given in the dose of 2 gm a day for 6-
10 weeks.
Choline
It is a colorless crystalline compound which Fat Soluble Vitamins
absorbs water quickly. It is highly soluble in
Common Properties
water and alcohol. It is member of vitamin B
group. It is present in foods as well as in the body • They are soluble in fat.
in relatively large amount. The body can make • Bile salts are essential for their absorption.
choline from methionine, an amino acid, with • They are generally stored in the liver.
the aid of vitamin B12 and folic acid. • They are not excreted in urine.
 Nutrition and Oral Cavity 663

Vitamin A (Retinol) corneal ulceration or keratomalacia can

occur. Xerophthalmia due to decrease in
Carotene is a yellow pigment found in vegetable
lacrimal secretion.
foods. It is converted into vitamin A in the body.
• Keratinizing metaplasia—The epithelial cells
Vitamin A or retinol is found in foods of animal
fail to differentiate. This means that the cells
origin, while carotene is provided by foods of
in the basal layer lose their specificity and
both plant and animal origin. Vitamin A is stored
tend to form a stratified squamous
in liver. As a concentrated solution retinol is light
epithelium with keratin production.
yellow in color. It solidifies when cooled and has
Keratinizing metaplasia of epithelial cells is
a mild pleasant odor.
usually evident in several organs such as
bladder, vagina and skin and predisposes
Functions in the Body
them to infection. Drying of skin and atrophy
• Epithelial tissue—Vitamin A helps in of sebaceous glands.
maintaining the integrity of epithelial tissue • Effect on reproductive organs—Degeneration of
such as epithelial layer of skin, respiratory germinal epithelium thus affecting
mucosa and esophagus and gastrourinary reproduction causes sterility in males and
tract. Due to this, it builds up resistance to cornification of vaginal epithelium in
respiratory infection. females.
• Structural integrity—Its function in the • Effects on bone—Imbalance between osteo-
preservation of the structural integrity and blasts (bone forming cells) and osteoclasts
normal permeability of the cell membrane as (bone resorbing cells) causing aberrations in
well as that of membrane of intracellular the shape of bone.
particles such as lysosomes and
mitochondria. Oral Manifestations
• Bone and teeth—It accelerates the normal
Teeth—Defective formation of enamel in teeth.
formation of bones and teeth.
Odontogenic epithelium fails to undergo normal
• Vision—Vitamin A also has a specific role on
histodifferentiation and morphodifferentiation,
the physiological mechanism of vision.
which results in increased rate of cell
• Oxygenation—It also increases permeability of
proliferation. Therefore epithelial invasion of
blood capillaries thereby contributing to
pulpal tissue is characteristic of vitamin A
better tissue oxygenation.
deficiency. There is also distortion of shapes of
• Aging and senility—It also prevents premature
the incisors and the molars.
ageing and senility.
• Synthesis—It is required for synthesis of Hypoplasia of teeth—Since the enamel forming
glucocorticoids and cholesterol. cells are disturbed, enamel matrix is poorly
• Somatic growth—Vitamin A is required for defined so that calcification is disturbed and
somatic growth. enamel hypoplasia results.
Dentin—Dentin too is atypical in structure,
Deficiency Symptoms lacking normal tubular arrangement and
• Effect on growth—Failure of growth in young containing vascular and cellular inclusions.
and collagenous tissue is affected. Caries—There is increased caries susceptibility.
• Effect on eyes—Night blindness, dry Eruption is delayed. In prolonged deficiency,
conjunctiva, Bitot’s spot, corneal xerosis, eruption ceases completely. Alveolar bone is
664 Textbook of Oral Pathology

retarded in its rate of formation. Gingival Forms

epithelium becomes hyperplastic; in prolonged
• D3—It is present in fish liver oils and animal
deficiency it shows keratinization. Tissue is easily
fats. It is called as cholecalciferol.
invaded by bacteria that may cause periodontal
• D2—It is obtained artificially by irradiation
disease and micro abscess formation. Major and
of ergosterol and called as ergocalciferol.
minor salivary glands undergo typical
keratinising metaplasia.
Function in the Body
Management of Deficiency Symptoms The major function of vitamin D is the
Depending upon deficiency symptoms it is given maintenance of normal plasma levels of calcium
in the dose of 7500 – 15,000 mcg per day for one and phosphorous.
month. It is very important for the proper formation
of teeth and bones. It plays an important role in
Hypervitaminosis A prevention of dental caries. It is necessary for the
healthy functioning of parathyroid gland, which
If more than 30,000 mcg of vitamin A is taken regulates the calcium levels in the blood.
daily, it can produce toxic effect in adults if
continued for many months. In infants, toxic
Pathogenesis
effect can be produced by the intake of more than
5,550 mcg per day. • Overgrowth—There is overgrowth of
Toxicity symptoms—Painful joints, thickening of epiphyseal cartilage due to inadequate
long bones, anorexia, low grade fever, loss of provisional calcification and failure of
hair, hepatomegaly, blurred vision, rashes, cartilage cells to form a matrix and
irregular menstruation, fatigue and headache. disintegrates.
• Formation of irregular masses—There is
Acute toxicity—It results from a single massive
persistence of distorted, irregular masses of
dose and it manifests as abdominal pain, nausea,
cartilage many of which projects into the
vomiting, headache, dizziness and sluggishness.
marrow cavity.
Chronic toxicity—It may occur following
• Deposition—Deposition of osteoid matrix on
ingestion of 12,000 mcg or more daily for
inadequately mineralized cartilaginous
prolonged periods. It is characterized by joints
remnants.
pain, hair loss, and dryness and fissuring of lips,
loss of appetite, low grade fever and weight loss. • Disruption—Disruption of the orderly
replacement of cartilage by osteoid matrix
Vitamin D with enlargement and lateral expansion of
osteochondral junctions.
It is also called as ‘sunshine vitamin’. Vitamin D
(1,25-dihydroxycholecalciferol) is one of the • Microfracture—There is abnormal growth of
compound that are grouped together as the capillaries and fibroblasts in the disorganized
hydroxylated cholecalciferol. If vitamin D zone because of microfracture and stresses
deficiency occurs in children and infants it is on inadequately mineralized, weak, poorly
formed bone.
called as ‘rickets’ and if it occur in adults it is
called as ‘osteomalacia’. Deficiency of vitamin D • Deformity—Deforming of skeleton due to loss
tends to cause hypocalcemia. of structural rigidity of the developing bone.
 Nutrition and Oral Cavity 665

Vitamin D Deficient Rickets girdling the thoracic cavity at the lower margin
of the rib cage.
The word ‘Rickets’ refers to any disorder in
vitamin D calcium phosphorous axis which Lumbar lordosis—the pelvis may be deformed.
results in hypomineralized bone matrix that is When an ambulatory child develops rickets,
failure of endochondral calcification. It develops deformities are likely to affect the spine, pelvis
in an area where sunlight is deficient. It results and long bones causing ‘lumbar lordosis’.
from inadequate extracellular level of calcium
and inorganic phosphate, mineral necessary for Oral Manifestations
new bone to calcify. Osteoid builds in excessive Developmental abnormalities of dentin and
amounts because it fails to mineralize properly. enamel, delayed eruption and malalignment of
Rickets occur in infants and children and teeth. There is higher caries index in rickets as
osteomalacia common in adults. compared to normal. There may be hypoplasia
of enamel; enamel may be mottled, yellow gray
Clinical Features in color. There are large pulp chamber, high pulp
It occurs in infants and children. In the first 6 horns and delayed closure of root apices. The osteoid
month of life, tetany, convulsions are common is so soft that teeth are displaced leading to
manifestations due to hypocalcemia. The wrist malocclusion of the teeth.
and ankles are typically swollen. The changes
in bone are found in the epiphyseal plates, Osteomalacia
metaphysis and the shaft. It is also known as ‘adult rickets’ and only flat
Craniotabes—Localized area of thinning are bones and diaphyses of long bones are affected.
sometimes present in the skull, so that a finger It is most commonly seen in post menopause
can produce indentation. This condition is called females with a history of low dietary calcium
as ‘craniotabes’. There is softening of posterior intake and little exposure to UV light.
part of the parietal bone, which may be first sign
of the disease. Patients have a short stature and Clinical Features
deformed extremities. Children with rickets
It is seen in adults and pelvic deformities are
show bowing of legs. Excess of osteoid produces
commonly seen in females. Remodeling of bone
frontal bossing and squared appearance to the
occur in the absence of adequate calcium
head.
resulting in softening and distortion of the
Rickety rosary—Deformation of chest results from skeleton.
over growth cartilage or osteoid tissue at the
costochondral junction producing ‘rickety Symptoms—The majority of patients has bone
rosary’. pain and muscle weakness of varying severity.

Pigeon breast—The weakened metaphyseal areas Signs—There is increased tendency towards

of the ribs are subject to pull of the respiratory fracture, peculiar waddling or penguin gait,
muscles and thus bend inwards creating anterior tetany and green stick bone fractures.
protrusion of the sternum resulting in a ‘pigeon
breast deformity’. Oral Manifestations
Harrison grooves—The inward pull at the margins There is incidence of severe periodontitis in some
of diaphragm creates Harrison’s grooves, cases of osteomalacia.
666 Textbook of Oral Pathology

Biochemical Changes occurrence being inherited as X-linked dominant

trait. Rickets and osteomalacia which does not
Elevation of serum alkaline phosphatase to three
or more times its normal levels. Serum respond to usual doses of vitamin D. Diminished
phosphorus is low due to increased phosphorus intestinal calcium and phosphate absorption.
excretion in response to reduction of serum Normal vitamin D metabolism and absence of
calcium. Serum calcium levels are usually on the other related abnormalities.
lower side.
Clinical Features
Management of Rickets and Osteomalacia It is first recognized in children when they are
Dietary enrichment of vitamin D in the form of about to walk.
milk. If tetany is present, give calcium gluconate Sign—Slight decrease in height of the patient and
IV. Daily dose between 1000-2000 IU of vitamin reduced growth and rickets like bone changes.
D combined with 500-1000 mg of calcium.
Hormonal therapy like flucytosine. Symptoms—There is bowing of legs, enlarged
Curative treatment includes 2000 to 4000 IU epiphysis, bone pain, muscle weakness and
of calcium daily for 6 to 12 weeks followed by a vertebral fracture. Bony outgrowth at the site of
daily maintenance dose of 2000 to 4000 IU for a muscular attachment and around joints may
prolonged period. Patients with osteomalacia due limit the movement.
to intestinal malabsorption require a larger dose
of vitamin D and calcium, i.e. 40,000 to 1, 00,000 Oral Manifestations
IU of vitamin D and 15 to 20 gm of calcium lactate
Widespread formation of globular hypocalcified
per day.
dentin with clefts and tubular defects occurring
Vitamin D resistant rickets (familial in the region of pulp horns. Gross reduction in
hypophosphatemia, refractory rickets) amount and quantity of dentin which results in
It is X-linked trait with some defect in abnormal wide root canal and large pulp
reabsorption of metabolism. chambers with faulty calcification and marked
interglobular space in dentin. Pulp horns are
Pathogenesis elongated and extend high often reaching the DE
Vitamin D promotes the absorption of calcium junction.
and phosphorus from the intestinal tract, Because of this defect, there is commonly
therefore diet deficient in vitamin D produces a invasion of the pulp by microorganisms without
negative calcium balance that in turn leads to demonstrable destruction of the tubular matrix.
under mineralization of the skeletal system. Thus, there is often periapical involvement of a
Thus, rickets is caused by a lack of calcification grossly normal appearing deciduous or
of cartilage and osteoid tissue. permanent tooth followed by the development
of multiple gingival fistulae. Tract is frequently
Causes present to dentinoenamel junction or even outer
The disease is now recognized as a specific enamel surface. This tract remains patent and
disorder characterized by hypophosphatemia may result in early pulpal infection developing
associated with decreased renal tubular in abscess or carious lesion. There is formation
reabsorption of inorganic phosphates. Familial of abnormal cementum.
 Nutrition and Oral Cavity 667

Histopathological Features supplying them. It also dissolves the blood clot

Failure of bone salts to be deposited in the and also prevents their formation.
cartilage matrix between the rows of Electron transport system—It functions as a
hypertrophic cells so that these cells are not cofactor in the electron transport system.
invaded and destroyed by capillaries. The
histological feature is characterized by a broad Healing—It prevents the formation of excessive
zone between the multiplying cartilage cells and scar tissue and in some instance even melts away
the shaft the so called ‘rachitic metaphysis’ which unwanted scar tissue.
is composed of tones of cartilage which extend Prevention—It is required for prevention and
down toward the shaft and are separated form storage of creatinine in muscles. It has ability to
one another by collection of capillaries. This zone prevent hepatic necrosis in animals. It prevents
contains trabeculae made of uncalcified cartilage vitamin A from destruction and helps in its
matrix upon which osteoid is deposited on storage in tissue.
preexisting bony trabeculae.
Deficiency Symptoms
Management
• Reproductive—Abortion of fetus in females
25-hydroxy cholecalciferol in lower doses is
useful than conventional vitamin D. and atrophy of spermatogenic structures in
males leading to permanent sterility.
Vitamin E (Tocopherol) • Muscles—It causes degenerative changes in
It is also called as ‘anti-aging’ factor. The word muscles. There is muscle fiber atrophy which
‘Tocopherol’ is derived from the word tocos is replaced by connective tissue.
meaning child birth and pheros meaning to bear. • Heart—There is necrosis and fibrosis of heart
It is yellow, oily liquid freely soluble in fat muscles.
solvents. • Blood capillaries—Deficiency may lead to
Tocopherol alpha, beta, gamma, lambda have degenerative changes in the blood capillaries
been obtained from natural sources and their which in turn lead to heart and lung diseases,
relationship with fertility and prevention of pulmonary embolism and brain stroke.
muscular dystrophy have been found. They are
not destroyed by heat even at room temperature Oral Manifestations
or above 100°C. They are destroyed by UV light.
Loss of pigmentation, atrophic degenerative
Functions in the Body changes in enamel of vitamin E deficient rates.

Reproductive function—Protective effect of Management

vitamin E on reproduction and prevention of
sterility. All the three layers of embryo ectoderm, Vitamin E is given in the doses of 100-400 mg
mesoderm and endoderm are preserved by daily.
vitamin E.
Vitamin K (Phyloquinone)
Blood flow and clotting mechanism—Vitamin E
dilates the capillary and enables the blood to flow It is essential for the production of a type of
freely into the blood deficient muscle tissue thus protein called prothrombin and other factors
strengthening both the tissue and the nerves involve in the blood clotting mechanism. Hence
668 Textbook of Oral Pathology

it is known as ‘anti-hemorrhagic vitamin’. It is not cases when there is defective production of

easily destroyed by light, heat or exposure to air. prothrombin.
It is destroyed by strong, acids, alkalis and • Oxidative phosphorylation—It acts as a cofactor
oxidizing agents. in oxidative phosphorylation associated with
lipid.
Forms
Effects of Deficiency
• K1—It is the form which occurs in plants.
• K2—It is produced by most bacteria present Prolongation of clotting time and a tendency to
in human intestine if not supplied in the bleed profusely. There may be nasal bleeding.
diet.
Oral Manifestations
Functions in the Body Gingival bleeding can also occur in cases of
• Synthesis—It is essential for the hepatic vitamin K deficiency.
synthesis of coagulation factors II, V, VII, IX
Management
and X.
• Clotting—It prevents hemorrhage only in It is given in dose of 10-20 mg daily.
 CHAPTER
Endocrine
34 Disorders

Hormones vary tremendously in chemical originating in higher centers in the brain and
composition and in biologic activity. Various reaching them by way of the nerve fibers that
disorders of components of the endocrine system link them to that system.
have generalized adverse effects on skeletal
The pituitary gland—It lies within the sella tursica
system due to altered metabolism.
at the base of brain and it is divided into three
distinct lobes. The anterior lobe also called as
ANATOMY AND PHYSIOLOGY
adenohypophysis originates from epithelium of
Rathke’s pouch, the intermediated lobe from
The endocrine system is specifically designed to
dorsal portion of Rathke’s pouch and posterior
integrate and control the human body’s
lobe or neurohypophysis develops from base of
innumerable metabolic activities. Its functioning
third ventricles. Hormone secreted by anterior lobe
components are the endocrine glands. These
are growth hormone (GH), adrenocorticotropic
units can function individually, in series, or in
hormone (ACTH), thyroid stimulating hormone
parallel, their activities being integrated closely.
(TSH), follicle stimulating hormone (FSH),
Communication with each other and with the
luteinizing hormone (LH) and prolactin.
tissues under their control is established by
Hormone secreted by intermediated lobe is
means of hormones, which they produce, store,
melanocytes stimulating hormone and by
and release as required and which are distributed
posterior lobe vasopressin and oxytocin. The
throughout the body by means of the circulating
secretory activities of the pituitary gland are
blood. In most instances, the agent stimulating
modulated by hypothalamus through a series of
or inhibiting their activity is the hormone
complex feedback interaction.
produced by the corresponding target gland. The
exceptions are the adrenal medulla, the posterior The thyroid gland—It is situated in midline of body
pituitary gland, and to a lesser extent, the in the neck, at the level of cricoids cartilage having
pancreas. These glands are connected with the two lateral lobes which are joined by isthmus.
autonomic nervous system, secreting their Third pyramidal lobe also extends from the
hormones in response to electrical stimuli isthmus. Embryologically, the thyroid gland
670 Textbook of Oral Pathology

develops as a downgrowth from the portion of in response to a fall in serum ionized calcium
four pharyngeal pouches. It regulates the basal concentration. PTH directly promotes
metabolic rate, stimulates somatic and psychic reabsorption of calcium from renal tubules and
growth and plays an important role in calcium bones. PTH also has indirect effect, mediated by
metabolism. Follicular cells lining the follicles of increasing conversion of 1, 25 hydorxy
the gland secrete tri-idothyronin and tetra- cholecalciferol, which results in increase calcium
idothyronin (thyroxin) which stimulates basal absorption from the food and enhanced
metabolic rate and somatic and psychic growth mobilization of calcium from bone. The initial
of the individuals. Parafollicular cells lie in effect of PTH on bone is to stimulate osteolysis,
between the follicles and they secrete returning from bone to extracellular fluid.
thyrocalcitonin which promotes deposition of
Adrenal gland—The adrenals are triangular-
calcium salts in skeletal and other tissue and
shaped structure that sits on the superior poles
tends to produce hypocalcemia. 83% of T3 is
of the kidneys. It produces and secretes a number
produced by monodeiodination of T4 in others
of compounds that are essential for maintenance
tissue such as liver, muscle and kidney. T4 is
of life adaptation to stress. Each gland is divided
probably not metabolically active until converted
into adrenal medulla and a cortex.
to T3 and may be regarded as prohormone. T3
and T4 circulate in plasma protein almost entirely Adrenal medulla—It arises form ectodermal
bound to transport plasma proteins mainly tissues and function as a part of the sympathetic
thyroxine binding globulin. It is a minute fraction nervous system. It manufactures and secretes
of unbound or free hormone, which diffuse into two catecholamine, i.e. epinephrine and
tissues and exerts its metabolic action. norepinephrine. Epinephrine supports blood
Production of T 3 and T 4 in the thyroid is pressure by increasing the heart rate.
stimulated by thyrotrophin (thyroid stimulating Epinephrine also increases oxygen consumption
hormone – TSH), a glycoprotein released from by the tissue and glucose release by the liver.
thyrotrophic cells of the anterior pituitary in Norepinephrine increases peripheral resistance
response to the hyperthalamic tripeptide, by its vasoconstrictor effect. Epinephrine and
thyrotrophic releasing hormone. There is norepinephrine also exert important metabolic
negative feedback of thyroid hormones on the effects by promoting lipolysis; increase blood
thyrotrophs such that in hyperthyroidism, when sugar levels by stimulating glycogeneolysis,
plasma concentration of T3 and T4 are raised, elevating body temperature and increases basal
TSH secretion is suppressed and in metabolic rate. These compounds aid the body
hypothyroidism, due to disease of the thyroid in adapting to stress which is important in the
gland; low T3 and T4 are associated with high dental sitting because release of endogenous
TSH level. epinephrine during stressful dental procedure
can produce significant changes in blood
Parathyroid glands—The four parathyroid glands
pressure and pulse rate.
lie behind the lobes of the thyroid. They are not
regulated by pituitary gland, but respond The adrenal cortex—It secretes three major classes
directly to changes in serum ionized calcium of hormone: glucocorticoids or cortisols, which
concentration. Parathyroid hormone (PTH) is a affects the inflammatory process and
single chain polypeptide of 84 amino acid which carbohydrates and protein metabolism, the
are synthesized by the chief cells and released mineralocorticoid aldosterone, which affect water
 Endocrine Disorders 671

and electrolyte balance and sex hormone Signs—Pituitary tumors may also induce
testosterone, estrogen and progesterone. deficiency of other pituitary hormones causing
signs of hypogonadism including decreased
DISEASE OF PITUITARY GLAND libido and menstrual problems in women.

Hyperpituitarism Acromegaly
It results from hyperfunction of anterior lobe of It is more common in males and occurs most
pituitary gland, most significantly with increased frequently in third decade. Bone overgrowth and
production of growth hormone. The usual cause thickening of the soft tissue causes a characteristic
of this condition is a benign, functioning tumor coarsening of facial features termed acromegaly.
of the eosinophilic cells in the anterior lobe of Symptoms—Hand and feet become large, with
the pituitary gland. GH acts directly on some
clubbing of the toes and fingers due to
tissue but most its biological effects are accounted
enlargement of the tufts of the terminal
by stimulation of secretion of insulin like growth phalanges. There is temporal headache,
factor I (IGF-I) and its binding proteins from the
photophobia and reduction in vision. The
lower lobe.
terminal phalanges of the hands and feets
become large and the ribs also increase in size.
Types
• Gigantism—If the increase in the production Oral Manifestations
of GH occurs before the epiphysis of the long
Teeth in gigantism are proportional to the size
bone are closed.
of jaw and the rest of the body and root may be
• Acromegaly—If the increase in the production
longer than normal. The teeth become spaced,
of GH occurs later in life after epiphysis
partly because of enlargement of the tongue and
closure.
party because upper teeth are situated on the
inner aspect of the lower dental arch, due to
Clinical Features
disproportionate enlargement of the two jaws.
Gigantism: Generalized overgrowth of most Mandibular condylar growth is very
tissue in childhood also occurs. Most of the soft prominent. Overgrowth of mandible leading to
tissue and bones respond to the excess hormone prognathism. Mandible may be of extraordinary
by enlarging. Excessive generalized skeletal proportions creating a major discrepancy
growth. between the upper and lower jaws and a
Symptoms—Patient may often have height of 7 to pronounced class III malocclusion. In some cases
8 feet. Patients achieve monstrous size because the growth at the condyle exceeds that of the
of tumors of the pituitary gland. Later in life, it alveolar processes, so that increased in vertical
may show genital underdevelopment and depth of the ramus is greater than that of the
excessive perspiration and they have complained body of the jaw, consequently the upper and
of headache, lassitude, fatigue, muscle and joints lower teeth fail to come into proper occlusion.
pain and hot flashes. There is increase in size of The palatal vault is usually flattened and the
calvarium which may lead to change in the hat tongue increase in size and may cause crenation
size. on its lateral border. The lips become thick and
672 Textbook of Oral Pathology

Negroid. In edentulous patients,’ enlargement of Clinical Features

the alveolus may prevent the comfortable fit of
The underdevelopment is symmetrical,
complete dentures. individual is very small and in some cases, there
may be a disproportional shortening of the long
Diagnosis
bones. The hallmark of this condition is that the
It can be made from the characteristic clinical and growth is retarded to a greater degree that is bone
radiographic findings. Growth hormone and dental development. It may occur because of
concentration can be measured by radioimmuno- growth hormone and cortisol deficiency. Lack of
assay technique. gonadotrophin delays the onset of puberty. The
presence of diabetes insipidus associated with
Management deficient secretion of vasopressin is suggestive of
pituitary dysfunction.
Trans-sphenoidal surgery may result in cure of
Growth hormone secretion is lost resulting in
GH excess especially in patients with lethargy, muscle weakness and increase fat mass
macroadenoma. Octreotide, a long acting in adults. After luteinizing hormone (LH)
analogue of somatostatin, lowers GH. It is secretion becomes impaired, in the male loss of
administered as subcutaneous insulin 2 to 3 libido and impotence and in female
times/day. Dopamine antagonists are also used. oligomenorrhea or amenorrhea. The male
produces gynecomastia and skin becomes fair and
Hypopituitarism wrinkled. The skull and facial bone are small and
Pituitary is the master gland of the body. It results there is delay in maturation of the skeleton and
due to reduced secretion of pituitary hormone epiphysis may remain ununited throughout the
which may occur due to pituitary adenoma that life.
compresses the pituitary gland. It is often the
consequence of growth of pituitary gland or Oral Manifestations
interference with stalk function. It results in Marked failure of development of maxilla and
pituitary dwarfism. Pathologic changes can mandible with lack of condylar growth with short
result from a variety of pituitary gland ramus and this can lead to severe malocclusion
malfunction. Total absence of all pituitary and crowding of the teeth.
secretions is known as panhypopituitarism. The two important hormones excreted by this
Hypopituitarism which commences after gland— the somatotrophic and the thyrotrophic
puberty is called as ‘Simmond’s disease’. are responsible for the normal eruption of teeth
and the alveolar growth. Thus, in case of hypo-
Causes
function of this gland the tooth eruption is
It is congenital or due to destructive disease of hampered. The dental arch is smaller than normal
pituitary gland such as infarct occurring before and thus, cannot accommodate all the teeth
puberty. Space occupying lesions involving the resulting in crowding and subsequent
sella turcica like craniopharyngioma, adenomas malocclusion. The clinical crown appears smaller
and sarcoidosis. Sheehan’s syndrome is a form of than normal because even through eruption
hypopituitarism caused by infarction of the occurs, it is not complete. Eruption is delayed and
pituitary associated with postpartum hemorrhage. so the shedding of the deciduous teeth.
 Endocrine Disorders 673

Management nodular goiter or toxic adenoma. Excessive

thyroxin causes generalized increase in metabolic
It is usually directed towards removal of the cause
rate of all body tissues. In patient with
or replacement of the pituitary hormone or those
thyrotoxicosis, dental treatment can precipitate
of its target glands.
an acute emergency like ‘thyroid crisis’ or
Progeria ‘thyroid storm’.

It is transmitted as autosomal dominant trait. This Definition

condition is rare and underlying cause is entirely
Hyperthyroidism is excessive functional activity
dependent on pituitary dysfunction. It is regarded
of the thyroid gland that can be caused by
as premature senility in an individual of infantile
Graves’ disease, excessive replacement of or
proportions.
overdose of thyroid hormone.
Clinical Features
Causes
Affected infants appear normal at birth, but the
It is caused by exophthalmic goiter, toxic
typical clinical features become manifested
within the first few years. Patient exhibits adenoma, ectopic thyroid tissue, Grave’s disease,
multi-nodular goiter, thyroid adenoma, a
alopecia, pigmented areas of the trunk, atrophic
pituitary disease involving anterior portion of the
skin, prominent veins and loss of subcutaneous
fat. The individual have high pitched squeaky gland, choriocarcinoma, excess pituitary TSH,
autonomous struma ovarii, polyostotic fibrous
voice beak-like nose and hypoplastic mandible.
dysplasia.
The face is pointed, with the nose resembling the
beak of a bird. The head is large, while mandible
Clinical Features
is small. Exophthalamus may be present and joint
deformities. The lips are thin. The intelligence of It has predilection for females between 20 to 40
this patient is either normal or above normal and years of age.
even at early age patient behave like old person. Sign—Thyroid is diffusely enlarged, smooth,
possible asymmetrical and nodular, a thrill may
Oral Manifestations
be present, may be tender. Abdomen, liver and
Accelerated formation of irregular dentin. spleen may be enlarged.
Delayed eruption of teeth. Symptoms—It includes nervousness, fine tremors,
and muscle weakness, mood swings from
Management
depression to extreme euphoria, emotional
No treatment and patient usually die before the liability, hyper-reflaxia, ill- sustained clonus,
age of 27 years. proximal myopathy, bulbar myopathy and
periodic paralysis. There is weight loss despite
Hyperthyroidism normal or increases appetite, diarrhea, bowel
It is also called as ‘thyrotoxicosis’ and it is a alterations, anorexia, vomiting and hyper-
defaecation.
syndrome in which there is excessive production
of thyroxin in thyroid gland. It is associated with Cardiac features—there is also palpitation,
diffuse toxic goiter and less frequently, with toxic excessive perspiration, irregular heart beat.
674 Textbook of Oral Pathology

Increased metabolic activity leads to increased Management

circulatory demands, tachycardia and increased
Antithyroid drugs—It would be appropriated to
pulse pressure and sometimes, congestive cardiac
give anti-thyroid drugs for 12 to 18 months to
failure. Exertional dyspnea and ankle edema,
those in whom a single episode was anticipated.
blood pressure normal, systolic hypertension
Carbimazole – for 0-3 weeks — 40-60 mg daily
may be present. Angina and cardiomyopathy
in divided doses, for 4-8 weeks — 20-40 mg daily
and exacerbation of asthma.
In thyrotoxicosis, patient may have bulging in divided doses and for maintenance phase —
eye and partial paralysis of the ocular muscles, 5-20 mg daily.
retraction and jerky movement, corneal ulcer- Other treatment modalities include subtotal
ation, optic neuritis, ocular muscle weakness, thyroidectomy, radioactive iodine, and β-
papilloedema, loss of visual activity, exophthal- adrenoreceptor antagonist.
mos. There is amenorrhea, oligomenorrhea, in-
fertility, spontaneous abortion and loss of libido, Hypothyroidism
impotence. It is caused by insufficient secretion of thyroxin
Others symptoms—There is increased sweating, by the thyroid gland. As a failure of thyrotrophic
pruritis, oncholysis, pigmentation, Vitiligo, function on the part of the pituitary gland or an
digital clubbing and pretibial myxoedema atrophy or destruction of the thyroid gland leads
(bilateral nonpitting edema). Heat intolerance, to an inability of the thyroid to produce sufficient
sweaty and warm extremities, thin shiny skin, hormone to meet the requirement of the body.
pretibial myxoedema, increased pulse rate and
early fatigue, lymphadenopathy, thirst and Definition
osteoporosis occurs.
Decreased or deficient secretion of thyroid
Oral Manifestations hormones is caused by thyroiditis, insufficient
thyroid replacement, post thyroidectomy, post
There is advanced rate of dental development radioactive iodine therapy.
and early eruption with premature loss of
primary teeth. Generalized decrease in bone
Types
density or loss of some areas of edentulous
alveolar bone. Early jaw development and • Cretinism—If failure of hormone occurs in
alveolar bone atrophy. infancy.
• Juvenile myxoedema—If it occurs in childhood.
Laboratory Investigation • Myxoedema—If it occurs after the puberty. In
it, there is subcutaneous deposition of
Plasma levels of T3 and T4 are increased; free
hydrophilic mucopolysaccharides.
thyroxin index is raised in this disorder. Thyroid
stimulating hormone (TSH) decreased. Anemia
Clinical Features
may be moderate to severe degree and is seen in
patient with prolonged duration of the disease. Cretinism and juvenile myxoedema—It may be
The anemia is hypochromic and abnormal forms present at birth or become evidence within the
of RBC may be seen. first few months after birth.
 Endocrine Disorders 675

Symptoms—Hoarse cry, constipation, feeding Maxilla is overdeveloped and mandible is

problems in neonates, retarded mental and underdeveloped. Retarded condylar growth
physical growth. leads to characteristic micrognathia and open bite
relationship.
Signs—There is delayed fusion of all body
Tongue is enlarged by edema fluid and due
epiphysis and delayed ossification of paranasal
to it tongue may protruded continuously and
sinus, partially pneumatization. There is
such protrusion may lead to malocclusion of
protruberant abdomen with umbilical hernia.
teeth. The base of skull is shortened leading to a
The hairs are sparse and brittle, the finger nails
retraction of the bridge of the nose with flaring.
are brittle and the sweat glands are atrophic.
Face is wide and fails to develop in longitudinal
Myxoedema: Symptoms—It may include weakness, direction. Lips are puffy, thickened and
fatigue, cold intolerance, and lethargy, dryness of protruding.
skin, headache, menorrhagia, and anorexia. In late Myxoedema—Macroglossia and enlarged lip as a
stages there is slowing of intellectual and motor result of the deposition of water and protein. Facial
activity, absence of sweating, modest weight gain, swelling of non-pitting type and mandible is
constipation, peripheral edema, pallor, underdeveloped. There is greater tendency to
hoarseness, decreased sense of taste and smell, periodontal disease, with alveolar destruction and
muscle cramps, aches and pains, dyspnea, loosening of the teeth.
anginal pain and deafness.
Signs—Dull expressionless face, peri-orbital Laboratory Findings
edema, sparse hair and skin that feels droughty Thyroid stimulating hormone (TSH) increased
to touch. Temperature normal and the patient and T3 and T4 decreased. In ECG, a classical sinus
may be disorientated which may indicate bradycardia with low voltage complexes and ST/
impending myxoedematous coma, pulse T wave abnormalities. There is raised cholesterol
decreased, blood pressure normal, diastolic level and triglycerides level and low serum
hypertension may be present. Facial pallor, sodium.
puffiness of face and eyelids (myxoedema),
occasional purpura, thickened nose and lips in Complications
more advanced cases, note scars in neck from
It is caused by coronary artery disease, congestive
thyroidectomy. Thyroid gland may be enlarged,
heart failure, increased susceptibility to infection,
thin brittle nails, coarse thin hair, dry rough skin;
mental disturbances including depression.
displaced apical beat may be present. Delayed
return of deep tendon reflexes, pleural effusion
Diagnosis
may be present. There are also watery eyes, brittle
hair and patchy alopecia. Clinical signs and symptoms and radiographic
features along with laboratory test revealing
Oral Manifestations reduction in serum T 3 and T 4 levels are
confirmatory.
Cretinism and juvenile myxoedema: Dental
development delayed and primary teeth slow to
Management
exfoliate. Enamel hypoplasia can also be seen.
Abnormalities of dentin formation lead to enlarge Thyroid preparation—patients are managed by
pulp chamber. thyroid preparation. Mainly use is levothyroxin,
676 Textbook of Oral Pathology

which is available as 25, 50 and 100 mg tablets. It is of vitamin D or from deficient metabolism of
customary to start slowly and a dose of 50 mg/day vitamin D in the liver or kidney. It effects to
should be given for 3 weeks and finally to 150 mg/ restore serum calcium level at the expense of
day. In the elderly and in patient with ischemic heart the lots of calcium in bone.
disease, the initial dose should be 25 μg/day. • Tertiary—Occasionally, parathyroid tumor
after long standing secondary hyperpara-
Hyperparathyroidism thyroidism develops this condition known as
It is an endocrine disorder in which there is an tertiary hyperparathyroidism. The increased
excess of circulating parathyroid hormone. parathyroid level produces increased bone
Excess PTH stimulates osteoclast to mobilize resorption and a resultant hypercalcemia.
calcium from skeleton leading to hypercalcemia • Ectopic—Due to excessive parathyroid
in addition to PTH, increased renal tubular re- hormone synthesized in patient with
absorption of calcium. malignant disease.
Following is the sequence of event which
gives an idea of the reaction promoted by this Clinical Features
hormone. Female to male ratio is 3:1. Mainly in 30 to 60
The bone and the kidney are the target organs years of age.
of parathyroid hormone which mediates the
osteoclast to resorb bone actively. When the bone Symptoms—Renal calculi are common and
is resorbed, calcium is released in the hematuria, back pain, urinary tract infection and
extracellular fluid and the serum calcium level hypertension are common. Peptic ulcer,
is elevated. The parathyroid hormone acts on the psychiatric effect like emotional instability, bone
epithelium of kidney tubules causing diuresis of and joint pain, and sometime pathologic fractures
phosphorus resulting in decrease in serum occurs. Gastrointestinal difficulties such as
phosphorus level. At the same time, it induces an anorexia, nausea, vomiting and crampy pain may
increase in calcium re-absorption from glomerular be present.
filtrate. Parathyroid hormone may also increase Signs—Bone deformities occur such as bending
the absorption of calcium from the intestine but of long bone occasional fracture and collapse of
this is not definitely established. Hence in a vertebrae and formation of pigeon chest. It is
healthy person injection of parathyroid hormone associated with muscle weakness, fatigue, weight
produces an elevated plasma calcium level, a loss, insomnia, headache, polydypsia and
decreased plasma phosphorus level and an polyuria.
increased alkaline phosphatase level.
Oral Manifestations
Types
There is gradual loosening drifting and loss of
• Primary—There is autonomous secretion of
parathyroid hormone (PTH) by hyperplasia, teeth, malocclusion. There is pathological fracture
of bone. Cystic lesion involving jaws are seen over
benign and malignant tumor of one or more
10% of cases.
of the four parathyroid glands.
• Secondary—Compensatory increase in output
Histopathological Features
of PTH in response to hypocalcemia. The
underlying hypocalcemia may result from an There is osteoclastic resorption of the trabeculae
inadequate dietary intake or poor absorption of the spongiosa and along the blood vessels in
 Endocrine Disorders 677

the haversian system of the cortex. Fibrosis Etiology

especially in the marrow spaces is marked. Surgical damage to parathyroid gland and their
Fibroblasts replace resorbed trabeculae and the vascular supply during thyroid gland procedure.
fibrotic islands. There is recent and old Parathyroid damage from radioactive iodine 131.
hemorrhage with much hemosiderin in evidence. Autoimmune destruction of parathyroid gland
Large tortuous blood filled sinusoidal channels and may be Idiopathic.
are lined by a flat endothelial layer. The
surrounding tissue is fibroblastic and hypercellular. Clinical Features
Multinucleated giant cells lie adjacent to the Symptoms—It can lead to tetany in the form of
sinusoids and osteoid trabeculae tend to orient carpopedal spasm of the wrist and ankle joint.
themselves in close proximity to the vascular Stiffness in hands, feet and lips. There is also
spaces. paresthesia of hand feet and around the mouth.
Tingling in the circumoral area, fingers and toes.
Laboratory Findings
Patients may complain of anxiety, depression,
The serum calcium level is raised and serum epilepsy and chorea. Reduction in intellectual
phosphorus level is decreased and serum alkaline capacity due to calcification within the brain.
phosphatase level is elevated in primary Signs—Patient with pseudohypo-parathyroidism
hyperparathyroidism and in secondary manifest short stature due to early closure of
hyperparathyroidism, the serum calcium level is certain bony epiphysis. The face is round and the
decreased whereas the serum phosphorus and hand shows shortening of the metacarpal bones,
alkaline phosphatase level are elevated. so that fingers are short.
Trousseau’s sign—It is elicited by occluding blood
Diagnosis
flow to the forearm for 3 minutes with
Serum alkaline phosphatase and serum calcium sphygmomanometer cuff applied to the arm and
level is increased. Decreased blood phosphorous raising the pressure above systolic level. This will
level. Increase in circulating hormone induce carpopedal spasm.
demonstrated by radioisotope studies.
Oral Manifestations
Management Hypoplasia of enamel, delayed eruption, external
It often regresses without surgery and the root resorption and root dilacerations. There is
rarefaction disappears. Surgical excision of also blunting of molar roots.
adenoma. The oral administration of vitamin-D
Chvostek sign—A sharp tap over the facial nerve
in secondary type can prevent skeletal
in front of ear causes muscle twitching of facial
demineralization in most of the cases. muscle around the mouth which is called as
Chvostek sign. Chronic candidiasis is also some
Hypoparathyroidism and
time present.
Pseudohypoparathyroidism
It is an uncommon condition in which there is Diagnosis
insufficient secretion of parathyroid hormone. The serum calcium level is decreased usually
Pseudo-hypoparathyroidism is a condition in below 7mg/dl. Serum phosphate level
which there defect is in the response of tissue target correspondingly elevated. Urinary calcium is low
cell to normal level of parathyroid hormone. or absent.
678 Textbook of Oral Pathology

Management • Endocrine disease — Excess endogenous

production of hormonal antagonists to insulin,
Supplemental calcium and vitamin D depending
e.g. growth hormone in acromegaly.
on severity of the hypocalcemia and the nature
of the associated signs and symptoms. In severe • Drug induced like corticosteroid, thiazide
cases, intravenous administration of calcium diuretics and Phenytoin.
gluconate is the treatment of choice. • Genetic syndrome like Down syndrome,
Klinefelter’s syndrome and Turner’s syn-
Diabetes Mellitus drome.
• Impaired glucose tolerance
It may cause by autoimmune response. Principal • Gestational diabetes
laboratory sign are hypercalcemia. It is common
endocrine disorders characterized by chronic
Etiology
hyperglycemia and abnormalities in
carbohydrate and lipid mechanism. Type I diabetes mellitus
• Viruses—Several viruses have been implicated
Definition including infection with mumps coxsackie B4,
Inappropriate hyperglycemia due to tissue retrovirus, rubella and cytomegalovirus and
resistance to insulin action, reduced insulin Epstein Bar virus. Virus particle known to
secretion or both. cause cytopathic or autoimmune damage to
beta cells have been isolated from the
Etiology pancrease.
• Diet—Bovine serum albumin (BSA) a major
It is caused by disorders of carbohydrate
constituent of cow’s milk has been implicated
mechanism resulting from insulin deficiency or
in triggering type I diabetes. It has been shown
ineffectiveness, producing hyperglycemia and
that a child who has taken cow’s milk early in
glycosuria. Genetic, autoimmune and pancreatic
infancy has been more prone to develop type I
dysfunction.
diabetes mellitus as compared to other who
Types has taken breast milk.
• Stress—It may precipitate the development of
Primary type I diabetes by stimulating the secretion of
• Type I or Insulin dependent (IDDM) — It counter regulatory hormones and possibly by
occurs due to deficiency. modulating immune activity.
• Type II or Non-insulin dependent (NIDDM) • Immunological factors—There is evidence that
— It occurs due to insulin resistance. type I diabetes is a T cell mediated autoimmune
• Non-obese disease. There is also HLA linked genetic
• Obese predisposition. Monocular cell infiltration of
• Maturity onset diabetes of the young (MODY). pancreatic islets restoration in selective
Secondary destruction of insulin secreting cells and
• Pancreatic disease (pancreatitis, hemochro- induction of remission by immuno-
matosis, neoplastic disease, pancreatectomy, suppressive drugs such as cyclosporine
cystic fibrosis). suggest it immunological etiology.
 Endocrine Disorders 679

Type II diabetes mellitus recurrent urinary tract infections, recurrent skin

• Genetic—The majority of causes for type II infections (especially of feet) and reversible
diabetes are multifactorial. Various types are paresthesia of fingers or toe.
associated with it like hepatocyte nuclear Nocturia, weight loss, fatigue, obesity usually
factor, glucokinase, and mitochondrial DNA present in older age group, nausea, vomiting.
and insulin receptors. Temperature, blood pressure may be elevated and
• Environmental factors peripheral pulses may be reduced.
– Lifestyle—Over-eating, especially when
combined with obesity and under activity Oral Manifestations
is associated with developmental of type
It will influence the onset and course of
II diabetes.
periodontal disease. Patient with diabetes are
– Malnutrition—It is proposed that
more prone to develop periodontal disease than
malnutrition in utero and the infancy may
are those with normal glucose metabolism. There
damage beta cell development at a critical
is greater tendency for bleeding on probing. The
period predisposing to type II diabetes
patient may exhibit a fulminating periodontitis
later in life.
with periodontal abscess formation and inflamed
– Age—Age is an important risk factors for
painful abscess and even hemorrhagic gingival
type II diabetes as it is principally disease
papillae, this factor culminated and gave rise to
of middle aged and elderly affecting 10%
tooth mobility, i.e. loose teeth.
of the population over the age of 65.
It will show more severe and rapid alveolar
– Pregnancy—During normal pregnancy,
bone resorption and are more prone to develop
insulin sensitivity is reduced through the
action of placental hormone and this affect periodontal abscess. Insulin dependent diabetic
glucose tolerance. The term gestational children tend to have more destruction around
diabetes refers to hyperglycemia occuring the first molars and incisors than elsewhere. As
for the first time during pregnancy. such diabetes mellitus does not cause periodontal
• Insulin resistance occurs in type II diabetes is disease directly, but it alters the response of the
due to an abnormal insulin molecule, an periodontal lesion to local irritants, hastening
excessive amount of circulating antagonists bone loss and retarding postsurgical healing of
and target tissue defect. the periodontal lesions. Gingival fluid in the
diabetes has more glucose level which favors the
growth of microflora.
Clinical Features
Diabetes is considered to be factor for median
• Polydypsia—There is excessive intake of fluid. rhomboidal glossitis as frequency of abnormal
• Polyuria—There is excessive urine passage. blood sugar level in diabetes and predisposition
• Polyphagia—There is excessive hunger. of these subjects to candidiasis. There is also
There is presence of acetone breath. Visual impairment of blood supply to dorsum of tongue
difficulty ranging from progressive color due to arteriosclerosis changes in the blood vessels
blindness to total blindness that have disease supplying the area. Impairment of local immune
more than 20 years. Coronary artery disease and mechanism which decrease the concentration of
stroke are frequent complication. Langerhans of cells in the lesion.
Diabetic neuropathy can cause marked It is infection with Candida albicans which
irritability. Recurrent vaginal (yeast) infections, occurs due to encouragement of local
680 Textbook of Oral Pathology

multiplication of Candida albicans due to impaired Clinical Features

glucose level and immune mechanism. Dry socket It is more common in males and, while found in
develops in diabetes hence they show delayed all age groups, it is most frequently seen in the 3rd
healing and impaired immunological balance. It and 4th decade.
is often associated with variety of otherwise
Symptoms—Feeble heart action, general debility,
unexplained oral symptoms such as burning
vomiting, and diarrhea and severe anemia. Patient
sensation, atypical paresis, dysetheria and
complained of postural hypotension.
dysgeusia.
Diabetes neuropathy is recognized as Sign—The disease is characterized by bronzing
polymorphic condition as when manifested as of skin, a pigmentation of the mucous membrane.
polyneuropathy. On the assumption the Decrease cortisol level interferes with the
trigeminal nerve might be involved. manufacture of carbohydrates from protein,
Increased caries activity occurs due to causing hypoglycemia and diminished glycogen
excessive fluid loss patient complained of storage in the liver. Neuromuscular function is
xerostomia. There is also atrophy of lingual inhibited, producing muscle weakness. There is
papillae with fissuring and dry tongue. There is also reduced resistance to infection, trauma, and
delay in healing of oral wound due to decreased stress.
polymorphonuclear chemotaxis. There is also Oral Manifestation
angular cheilosis, altered taste sensation, oral
lichen planus, and diffuse enlargement of parotid The pale brown or deep chocolate pigmentation
of the oral mucosa, spreading over the buccal
gland.
mucosa form the angle of the mouth and/or
developing on the gingiva, tongue, lips may be
Management
first evidence of disease.
Treatment modalities include, diet control, oral
hypoglycemic drugs like sulfonylurea, Histopathological Features
biguanides, Alfaglucosidase inhibitors, Insulin Biopsy of oral lesion shows acanthosis with silver
therapy. positive granules in the cells of the stratum
germinativum.
Addison’s Disease
Laboratory Investigation
It is also called as chronic adrenal insufficiency
of the adrenal cortex. It was first described by The associated anemia is normocytic and
Addison in 1855. normochromic associated with reticulocytosis.
There is reduction in the red cell mass. There is
Causes high blood levels potassium and low
concentration of sodium and chloride. Elevated
It is caused by tuberculosis, metastatic carcinoma,
blood urea nitrogen.
intradermal hemorrhage, amyloidosis,
hemochromatosis, adrenal infarction and
Management
congenital adrenal hypoplasia. Drugs which can
cause Addison’s disease are aminoglutethimide, Glucocorticoids replacement—Cortisol is the drug
ketoconazole and etomidate. of choice. In patients who are not critically ill,
 Endocrine Disorders 681

Hydrocortisone 15 mg on waking and 5 mg at 6 PM is truncal obesity with prominent supra-clavicular

in evening. Supplement mineralocorticoid – can and dorsal cervical fat pads giving rise to the
be given. ‘buffalo hump’ appearance at the base of neck.
The distal extremities are usually thin. Weakness,
Adrenogenital Syndrome hypertension, or concurrent diabetes is usually
present.
It refers to any situation in which there is
overproduction of androgens. It results when Symptoms—There is alternation in hair
hyperplasia or tumors of the adrenal cortex occur. distribution. Dusky plethoric appearances with
It may appear at 3 different times of life, i.e. at formation of purple striae appear on abdomen.
birth, in childhood and in adult. Clinical features There is also weight loss, menstrual irregularity,
vary according to appearance of lesion. In female hirsutism, backache, obesity, hypertension can
child, it produces pseudohermaphroditism, while also occur.
in male child, it produces macrogenitosomia praecox.
Oral Manifestations
In the females, it produces masculinization and
in males it produce sexual precocity. In females it In children, growth and development including
produces virilism and in males it produces skeletal and dental age may be retarded. In some
feminization. If the disease begins early, instances, there may be osteoporosis of the jaws.
premature eruption of the teeth may occur.
(Administration of corticosteroid or estrogen). Management
If the lesion in the pituitary gland is the cause,
Cushing’s Syndrome therapy usually consists of combination of
Cushing’s syndrome arises from excess secretion surgery and radiotherapy.
of glucocorticoids by the adrenal glands. It is Drugs used—Metyrapone in dose of 2-6 g per day
described by Harvey Cushing in 1932. in divided doses by mouth. Other drug which is
given is aminoglutethimide which is anticonvul-
Etiology sant, and it acts by blocking steroid synthesis.
Adrenal adenoma, adrenal carcinoma, adrenal
Adrenal Insufficiency
hyperplasia and basophilic adenoma of the
anterior lobe of pituitary gland. Exogenous Causes
corticosteroid. ACTH secreting tumor of the It is relatively rare and usually occurs in
anterior pituitary associated with adrenal cortical connection with an acute septicemia and is called
hyperplasia. Ectopically located adrenal like as ‘Waterhouse Friderichsen syndrome’. This
tumor like in ovary. Alcohol excess, major occurs in patient who are on long-term steroid for
depressive illness and primary obesity. the treatment of variety of systemic condition
developed adrenal insufficiency.
Clinical Features
Female to male ratio is 3:5, seen in 3rd and 4th Types
decades. • Primary—It occurs due to disorders of pituitary
Signs—Rapidly acquired obesity about upper or adrenal glands (primary adrenal
portion of the body and rounded moon face. There insufficiency is called as Addison’s diseases).
682 Textbook of Oral Pathology

• Secondary—It occurs due to chronic Symptoms—There is anxiety, fatigue and

administration of corticosteroid resulting in hypotension and inability to tolerate stress.
the suppression of endogenous steroid. Abdominal pain, nausea, vomiting, and cold
clammy skin, lethargy, partial or complete loss of
Predisposing Factors consciousness is also present. There is also mental
• Following sudden withdrawal of steroid confusion occur in acute adrenal insufficiency and
hormones in a patient who has primary susceptibility to infection and hypertension.
adrenal insufficiency. Signs—Oral, conjunctival, and vaginal mucosa
• Following sudden withdrawal in a patient often show patches of pigmentation. In some
with normal adrenal cortices but with a patient, particularly in the dark-skinned races,
temporary insufficiency resulting from cortical patchy area of de-pigmentation surrounded by
suppression by exogenous corticosteroid hyperpigmentation may be seen. Hyperpigmen-
administration. tation of buccal or labial mucosa is seen in many
• Following stress such as physiologic or
patients. These patches may be brown, gray or
psychological stress.
blue in color.
• Following bilateral adrenalectomy or
removing of a functioning adrenal tumor that Laboratory Investigation
had been suppressing the other adrenal gland.
• Following sudden destruction of the pituitary Plasma cortisol levels are low and fail to raise
gland. after administration of ACTH. Plasma ACTH
• Following injury to both adrenal gland by levels are elevated and serum sodium and
trauma, hemorrhage, infection, thrombosis or chloride and urinary 17-ketosteroird and 17-
tumor. hydroxy-corticosteroids are low.

Clinical Features Management

It occurs primarily in children but can occur in Replacement therapy with a combination of
adults. It is characterized by rapidly fulminating glucocorticoids (cortisol), mineralocorticoids
septic course, a pronounced purpura and death (fludrocortisone), and anabolic steroids. In adrenal
within 48 to 72 hours. In this, patient is not able to crisis, the patient must be treated vigorously. In
tolerate the stress. mild case, hydrocortisone alone might be sufficient.
 CHAPTER
Diseases Affecting
35 Nerves and Muscles

NEUROMUSCULAR DISORDERS Wolf thought that ischemia at various

portions of the trigeminal pathway might be
Trigeminal Neuralgia responsible for the paroxysms of pain.
It is also called as ‘Tic Douloureux’, trifacial Jannetta and others have shown subtle
neuralgia or Fothergill’s disease. Tic douloureux’ changes of a compression-distortion pheno-
menon which is usually caused by arterial loops
is only used when the patient suffers from
of atherosclerotic vessels. Vessels become
spasmodic contractions of the facial muscles.
elongated with advancing age and with
Trigeminal neuralgia is an extremely painful
atherosclerotic involvement gain abnormal
condition as it is unique to humans. It is a
positions- by wedging into the space between
syndrome in which symptoms are sufficiently
the pons and trigeminal nerve. It is postulated
distinctive to permit a reliable diagnosis solely
that with progressive material elongation,
on the basis of history. fascicles of adjacent nerves later suffer myelin
injury and pain results.
Etiology
Secondary Trigeminal Neuralgia
It can be idiopathic or secondary.
Conditions such as carcinoma of the maxillary
Idiopathic Trigeminal Neuralgia antrum, nasopharyngeal carcinoma and tumors
of peripheral nerve root can cause secondary
Dental pathosis is believed by some investigators trigeminal neuralgia. Benign tumors, as well as
to be involved with the onset of trigeminal intracranial vascular anomalies may present
neuralgia. It may occur secondary to excessive trigeminal pain. Multiple sclerosis is usually
traction on the various divisions of the fifth nerve, associated with trigeminal neuralgia.
being influenced by maxillo-mandibular
relationship, allergic and hypersensitivity Clinical Features
reaction causing edema of the trigeminal nerve It usually occurs in middle and old age, the
root. disease seldom occurs before 35 years of age.
684 Textbook of Oral Pathology

Incidence increases with age due to degenerative Histopathological Features

changes of the nerve fibers. It most frequently Trigger point may show fibrosis and infiltration
occurs in women. It is more common on the right by chronic inflammatory cells. In some cases,
side and the lower portion of the face is more there is focal area of myelin degeneration are
frequently affected. seen along the course of cranial nerves.
Nature of pain — The pain is paroxysmal, lasting Glossopharyngeal Neuralgia
only a few seconds to a few minutes and is
Clinical Features
usually of extreme intensity. It may be described
by the patient as resembling ‘knife like stabs’ It is a variant of tic douloureux that can mimic
‘lightening’, ‘electric shock’, ‘stabbing’ or oral pathologic condition in which pain is
‘lancinating’ type of pain. During the intervals confined to the distribution of the nineth cranial
between these violent experiences, there is nerve. This neuralgia occurs without any sex
usually no pain or a mild or dull ache. predilection in the middle aged or older persons.
The pain is generally unilateral.
Aggravating factors — The pain is provoked by
Nature of pain — It manifests as sharp
obvious stimuli to the face. A touch, a draft of
excruciating, electric like, lancinating paroxysms
air, any movement of the face as in talking,
of pain in the ear, pharynx, nasopharynx, tonsils
chewing, yawning or swallowing may evoke a
or the posterior portion of the tongue.
lancinating attack. Later the pain may be so
Glossopharyngeal neuralgia has a tendency
severe that the patient lives in constant fear of towards remissions and exacerbations. Pain free
an attack. Often there is a transitory refractory intervals of seconds, minutes, hours, days, and
period after the attack. As the attack occurs, the years are common. The patient usually has a
patient may clutch his face as if in terror of the trigger zone in the posterior oro-pharynx or
dreaded pain. tonsillar fossa. An important and frequent trigger
Triggers zones — ‘Trigger zones’ which is the initiation of the act of swallowing.
precipitate an attack when touched, are common
Geniculate Neuralgia
on the vermilion border of the lips, the ala of the
nose, the cheeks, and around the eyes. The It involves the intermediate nerve of Wrisberg,
patient learns to avoid touching the skin over an important component of the facial (VII) nerve.
the trigger zones which frequently makes him It is caused pathological involvement of the
go unwashed or unshaven for days. sensory intermediate nerve root of the VIIth
The pain is confined to the trigeminal zone, cranial nerve due to neuroma; vascular
nearly always unilateral and, if bilateral, is malformations, etc are the cause. Females are
successive rather than concomitant. The affected more commonly than males. It occurs
mandibular and maxillary divisions are more more commonly in old aged persons.
commonly involved than the ophthalmic; In Nature of pain — The pain may be felt in front
some instances, these two divisions may be and deeply in the ear with occasional pain in the
simultaneously affected. The pain never crosses palate and the tongue and deeply in the facial
the midline. The neurological examination musculature. When the triggering is caused by
findings are normal with no objective sensory touching the ear, topical anesthesia of the
loss along the trigeminal nerve. external auditory canal may arrest it.
 Diseases Affecting Nerves and Muscles 685

Management Etiology
Medical treatment It usually occurs after exposure to cold. But many
• Trichloroethylene inhalation, anti-cholinergic workers believe that it is a chance finding. It may
drugs, Dilantin, be a causative factor as Bell’s palsy occurs after
• Carbamazepine (tegretol) has a special effect extraction of teeth and after injection of local
on the paroxysmal pain. The use of this drug anesthetic. Extraction and injection may cause
causes paroxysms to become separated by damage to the nerve and subsequent paralysis.
intervals of freedom for weeks, months or Surgical procedures, such as removal of parotid
even years. This is considered to be the best gland tumor in which the facial nerve is
conservative treatment for trigeminal sectioned can also cause facial paralysis. It may
neuralgia. As an initial dose, 100 mg is given cause by ischemia of the nerve near the
twice daily until relief is established. At no
stylomastoid foramen, resulting in edema of the
time the daily dose should exceed 1200 mg.
nerve by its compression in the bony canal and
Side effects include dizziness, unsteady gait,
finally, paralysis.
gastrointestinal distress, skin rashes and
Familial and hereditary occurrence is also
aplastic anemia. Recently, baclofen an
reported in cases of Bell’s palsy. Tumors of
antispastic drug is also being used.
• Anti-inflammatory agents like indomethacin cranial base, parapharyngeal space and
and short courses of steroids have been found infratemporal fossa often cause 7th nerve palsy.
to be useful. Others causes of Bell’s palsy are Melkersson -
Rosenthal syndrome, acute otitis media and
Surgical treatment: Injection of the nerve with atmospheric pressure changes.
anesthetic solution, injection of the nerve with
alcohol, nerve sectioning and nerve avulsion Clinical Features
(peripheral neurectomy), electrocoagulation of
gasserian ganglion, percutaneous radio- Women are more commonly affected than men
frequency trigeminal neurolysis, decompression and usually, it occurs in the middle age group.
and compression and rhizotomy, bulbar It arises more frequently in spring and fall, than
trigeminal tractotomy. at any other time of the year. It begins abruptly
as paralysis of the facial musculature, usually
Bell’s Palsy unilaterally.
It is also called as ‘7th nerve paralysis’ or ‘facial Symptoms — In some cases, it is preceded by pain
paralyses. on the side of the face which is ultimately
involved, particularly within the ear, temple,
Pathogenesis mastoid area or at the angle of the jaw. On the
The cortical tract communicating with the motor affected side, eye can not be closed and wrinkles
nucleus ambiguous of facial nerve crosses over are absent on that side. There is watering of eye,
to get innervated into the lower face which leads to infection.
musculature. Upper face fibers are ipsilateral It is associated with Melkersson-Rosenthal
proximal to the nucleus. A cortical lesion will syndrome. When the patient smiles, the paralysis
cause contralateral lower face palsy; lesions of becomes obvious since the corner of the mouth
brainstem, main trunk or peripheral fibers will does not rise nor does the skin of the forehead
result in total hemifacial paralysis. wrinkles or the eyebrows raise.
686 Textbook of Oral Pathology

Oral Manifestations Symptoms — The patient exhibits preauricular

The muscular paralysis manifests itself by flushing and sweating of the involved side of
dropping of the corner of mouth, from which face, following ingestion of food or visual
saliva may dribble. The patient has a typical stimulation by foods. Patient may sometimes feel
masklike or expressionless appearance. Speech and pain while eating. The severity of sweating is
eating is difficult and occasionally, taste sensation increased by tart food. Profuse sweating may be
on the anterior portion of tongue is lost or altered. evoked by parenteral administration of
Food is retained in the upper and lower buccal pilocarpine or eliminated by the administration
and labial mucosal folds due to weakness of of atropine.
buccinator.
Sign — Local skin temperature is raised without
Management sweating. Temperature may rise to 100º F. Pres-
ence of cutaneous hyperesthesia in front and
The use of vasodilator drug like histamine has above the ear, area supplied by the auriculotem-
been proved beneficial in some cases. poral nerve.

Auriculotemporal Syndrome Crocodile tears — In it, patient exhibits profuse

lacrimation when food is eaten particularly hot
It is also called as ‘Frey’s syndrome’ or ‘gustatory
sweating’. and spicy food.

Minor Starch-Iodine test — 1% iodine solution is

Pathogenesis painted on affected area. After the solution is
It is an unusual phenomenon which arises as a dried, area is coated with layer of starch. After
result of damage to the auriculotemporal nerve. patient having taken food, moisture of sweat will
Auriculotemporal nerve, in addition to sensory mixed with iodine on the skin. It will results in
function, also supplies parasympathetic fibers to blue color.
parotid gland. The fibers regenerate, become
misdirected and follow the course of sympathetic Management
fibers to the skin and sweat gland. Intracranial division of auriculotemporal nerve
Parasympathetic fibers would therefore has been reported to be successful. To control
induce salivation; inadvertently stimulate the gustatory sweating, may be maintained for up
preauricular-dermal sweat gland and arterioles, to 3 days, by topical application to the affected
causing hydrosis and vasodilation.
skin by 1% glycolpyrrolate lotion or cream.

Etiology Myositis Ossificans

It follows surgical operations such as removal It is a condition in which fibrous tissue and
of a parotid tumor or ramus of mandible. It may heterotopic bone form within the interstitial
follow superficial parotidectomy. It may occur tissue or muscle, as well as in associated tendons
due to birth trauma. and ligaments. Secondary destruction and
atrophy of the muscle occurs, as this fibrous
Clinical Features
tissue and bone interdigitate and separate the
There is no age and sex predilection. muscle fibers.
 Diseases Affecting Nerves and Muscles 687

Types Signs — The overlying skin may be red and

• Localized myositis ossificans or traumatic inflamed. Intramuscular mass is palpated at 2 to
myositis ossificans 3 weeks. The lesion may appear fixed or it may
• Progressive myositis ossificans or generalized be freely movable on palpation.
myositis ossificans.
Oral Manifestations
Localized Myositis Ossificans
It involves the muscles of face particularly,
It is also called as ‘post-traumatic myositis masseter and temporal following single
ossificans’ or ‘solitary myositis’. traumatic injury. Some difficulty in opening of the
mouth occurs.
Etiology
It is caused by acute or chronic trauma or heavy Histopathological Features
muscular strains caused by certain occupation
It exhibits varying stages from hemorrhage,
or sports. Traumatization of the periosteum of
degeneration of muscle and connective tissue
an adjacent bone with the displacement of
hyperplasia to chondrification and ossification. The
osteoblasts into the muscle and subsequent
osteoid and bone trabeculae formed often
formation of bone occurs. Activation of periosteal
trapped viable muscle fibers but these may
implants already present in muscle by trauma
ultimately disappear. The trabecular pattern is
or hemorrhage. Metaplasia of the pluripotential
often extremely bizarre with the cartilage and
intermuscular connective tissue into the bone
myxomatous tissue present which may resemble
and metaplasia of fibrocartilage can also be
callus formation. The more mature tissue is
causative factors.
usually found on the periphery of the lesion.
Pathogenesis
Management
Injury → hemorrhage into the muscle or
associated tendon or fascia → the hemorrhage Sufficient rest should be given with limitation
organized and undergoes scarring → during of use. Excision after process becomes stationary.
healing process cartilage is formed →
calcification of cartilage → ossification of Progressive Myositis Ossificans
cartilage. It is characterized by formation of bone in
tendons and fascia with subsequent replacement
Clinical Features of adjacent muscle by expanded bony mass. In
It can occur at any age, sex and more often in some cases, there is history of hereditary and
young persons. The most commonly involved familial pattern.
muscles are the masseter and sternocle-
idomastoid. But in some cases, lateral pterygoid Clinical Features
muscle can be involved.
It usually affects children before 6 years of age.
Symptoms — Site of trauma remains swollen, It is seen more in males as compared to females.
tender and painful much longer than expected. It may advance rapidly or there may be long
In some cases, there is a mild discomfort period of relative inactivity with intermittent
associated with a progressive limitation of bursts of activity. It starts in muscles of neck and
motion. upper back and moves to extremities. Soft tissue
688 Textbook of Oral Pathology

swelling that is tender and painful and may show The initial symptoms usually consist of difficulty
redness and heat. in walking with leg pain and paresthesia.
Gradual increase in stiffness and limitation
Signs — Atrophy of foot, leg and hand muscles
of motion of neck, chest and back and extremities
ultimately occurs with the appearance of a
occurs. Ultimately entire groups of muscles
typical foot-drop, steppage gait and stork-legs.
become transformed into bone resulting in
limitation of movements. It is associated with Amyotrophic Lateral Sclerosis
congenital shortness of first metatarsal and It generally occurs between the ages of 40 and
metacarpal bones, shortness of little bone. 50 years and affects males more frequently. It is
Interphalangeal joint may be fused. The masseter also called as Lou Gehrig disease (he is famous
muscle is frequently involved so that fixation of baseball player who died of these disease).
jaw occurs. Precipitating factors include fatigue, alcohol
The patient becomes transformed into a rigid intoxication and trauma. Infections like syphilis,
organism called as ‘petrified man’. Patient dies influenza, typhus and epidemic encephalitis can
during 3rd or 4th decades. Premature death is also lead to amyotrophic lateral sclerosis.
usually results from respiratory embarrassment.
Symptoms — The initial symptoms consist of
weakness and spasticity of limbs, difficulty in
Histopathological Features
swallowing and talking with indistinct speech
The muscle in this disease is gradually replaced and hoarseness. Atrophy, flaccidity, symmetric
by connective tissue which undergoes osteoid weakness, slowness of movements and
formation and subsequently ossification. In some impairment or loss of palatal movements may
cases cartilage formation may become evident. also occur.
Signs — In earlier stages, the tongue is slightly
Motor System Disease
weakened, leaving articulation relatively
The motor system disease results from unaffected. In the middle stages, a gradual and
degeneration of the corticospinal and anterior generalized weakening of tongue occurs,
horn cells. accompanied by spasticity which results in
reduced rate, range and force of articulatory
Types tongue movements. In the later stages, there is
• Progressive muscular atrophy virtually unintelligible articulation.
• Amyotrophic lateral sclerosis Progressive Bulbar Palsy
• Progressive bulbar palsy It generally occurs in the 5th and 6th decades of
life with familial pattern in some instances.
Clinical Features
Symptoms — It is characterized by difficulty in
Progressive muscular atrophy swallowing and phonation, hoarseness, facial
It usually occurs in childhood. It shows a weakness and weakness of mastication. Chewing
strong hereditary pattern, affects males more is difficult as facial muscles become weakened.
frequently than females.
Signs — Atrophy of facial, masseter, temporal
Symptoms — It is characterized by progressive muscles and tongue, with fasciculation of the
weakness of limbs with associated muscular face and tongue. There is also impairment of
atrophy, reflex loss and sensory disturbances. palate and vocal cords.
 Diseases Affecting Nerves and Muscles 689

Management Management
Course of this disease is fatal. Temporary Patient should be referred to neurologist for
remission can occur. further management.

Multiple Sclerosis
MUSCLE DISORDERS
It is also called as ‘disseminated sclerosis’.
Muscular Dystrophy
Etiology
There are mainly two types of muscular
The lesions are allergic hypersensitivity dystrophy:
manifestations of the nervous tissue due to • Severe generalized familial muscular
antigen-antibody reactions. The lesions are due dystrophy
to scattered venous thrombosis in the nervous • Mild restricted muscular dystrophy
system associated with altered coagulation of
blood. The lesions are due to repeated, transitory Severe Generalized Muscular Dystrophy
localized vasoconstriction in various portions of
It is described as a rapidly progressive muscle
the nervous system, precipitated by emotional
disturbances or fatigue. disease, usually beginning in early childhood
and presenting a strong familial transmission.
Clinical Features
Clinical Features
It occurs chiefly in younger age group with an
onset of symptoms between the ages of 20 and It is the most common form of muscular
40 years. There is slight female predilection with dystrophy and predominately affects males. It
familial occurrences. begins in childhood, usually before the age of 6
years and rarely after 15 years.
Symptoms — There is fatigability, weakness and
stiffness of the extremities with ataxia or gait Symptoms — The earliest symptom is inability
difficulty, involving one or both legs. Other to walk or run change to which, the children fall
symptoms includes are area of superficial or readily and associate with muscular enlargement
deep paresthesia, personality and mood and weakness.
deviation towards friendliness and cheerfulness, Sign — The muscular enlargement ultimately
variety of ocular disturbances including visual proceeds to atrophy and the limbs appear flaccid.
impairment as a manifestation of retrobulbar It is the atrophy which is responsible for the
neuritis, nystagmus and diplopia. postural and ambulatory defects, such as
Charcot’s triad — It consist of intentional tremors, waddling gait.
nystagmus, dysarthria and scanning speech.
Oral Manifestations
Oral Manifestations
The muscles of mastication, facial ocular,
Facial and jaw weakness occur in some patients. laryngeal and the pharyngeal muscles are
Staccato (a series of short, detached sound or usually involved, only late in the course of
words) type of speech is interesting feature of disease. Due to lack of muscle tension, teeth can
this disease. In some cases, both trigeminal not be kept properly aligned in the arch. Locking
neuralgia and Bell’s palsy have been reported. and clicking of the jaw occur.
690 Textbook of Oral Pathology

Histopathological Features ‘tapir-lips’. The patient is unable to whistle or

smile. There may be severe open bite and
There is gradual disappearance of muscle fibers, as
development of diastema.
the disease progresses until ultimately no fibers
may be recognized. Persistent fibers may show
variation in size in the earlier stages of disease, Histopathological Features
some being hypertrophic but others atrophic. There is some variation in size of muscle fibers
and moderate infiltration of fiber bundles by
Laboratory Findings
connective tissue. Individual fibers ultimately
Serum creatinine phosphokinase levels are become atrophic.
elevated in all males.
Management
Management
Some patients undergo temporary periods of
There is no treatment for this disease. Physical remission or even complete arrest.
therapy may help prolong the use of specific
muscle group.
Myotonias
Mild Restricted Muscular Dystrophy Types
It is a slowly progressive proximal myopathy • Dystrophic
which primarily involves the muscles of • Congenital
shoulder and face and has a weak familial • Acquired.
incidence. It is transmitted as an autosomal
dominant trait. Dystrophic Myotonia

Clinical Features It is also called as ‘myotonic dystrophy’ or

‘dystrophic myotonica’. It is inherited as an
The disease begins at any age from 2 years to 60
autosomal dominant trait.
years. Although onset is in the first two decades
of life.
Clinical Features
Symptoms — The earliest symptoms are inability It dose not appear until the 3rd decade of life,
to raise the arms above the head and inability to
but may be seen earlier, i.e. even in childhood.
close the eyes, even during sleep as a result of
Atrophy of the muscles is seen usually in the
weakness of the facial muscles. hands and forearms. It can be seen in muscles of
Signs — Scapular muscles become atrophic and face, jaws, neck and levators of eyelids. There is
weak with subsequent alteration in the posture. associated weakness of the muscles. There is
Cardiac abnormalities including cardiomegaly alteration in the facial muscles, which consist of
and tachycardia are often present and many ptosis of the eyelids and atrophy of the masseter
patients die due to sudden cardiac failure. and sternocleidomastoid muscles.
Myopathic facies and swan neck — The masseteric
Oral Manifestations
atrophy produces a narrowing of the lower half
The lips develop a characteristic looseness and of the face which, with ptosis and generalized
protrusion, which have been described as weakness of the facial musculature gives the
 Diseases Affecting Nerves and Muscles 691

patient a characteristic ‘myopathic facies’ and Management

‘swan neck’.
There is no specific treatment for this disease.
Pharyngeal and laryngeal myotonia also
exhibit weakness manifested by a weak,
Acquired Myotonia
monotonous nasal type of voice and subsequent
dysphagia. Recurrent dislocations of the jaw are also It refers to spasm of muscles which are more
reported in this disease. Other features are intense than typical myotonia.
testicular atrophy, cataract, hypothyroidism with
Clonus — If these spasms are intermittent, it is
cold extremities, slow pulse, loss of hair and
called as ‘clonus’ (myoclonic contraction).
functional cardiac changes.
Trismus — If spasms are constant, it is called as
Histopathological Features ‘trismus’ (myotonic contractions).

There is enlargement of scattered muscle fibers and

Myasthenia Gravis
the presence of centrally placed muscle nuclei in
long rows. True hypertrophy in some fibers, is It is a chronic disease characterized by
found, as well as in isolated fibers which show progressive weakness of the skeletal muscles,
extreme degenerative changes including nuclear particularly those innervated by the cranial
proliferation, intense basophilic cytoplasmic nerves. It is characterized by easy fatigability of
staining and phagocytosis. the striated muscles secondary to disorders at
the neuromuscular junction.
Congenital Myotonia
Etiology
It is transmitted as an autosomal dominant trait
with incomplete penetrance in some families. It There is a defect in the neuromuscular
is also called as Thomson’s disease. transmission, which bears amazing resemblance
to those abnormalities which occur after
Clinical Features administration of ‘curare’. There appears to be a
fault in the acetylcholine mechanism, with the
It commences early in the childhood and may
be first noticed because of difficulties in learning motor end organs being normal. It may occur
to stand and walk. The muscles of the thighs, in the endocrine system due to thymus
forearm and shoulders are especially affected as hyperplasia or tumors of the thymus. It may be
well as are the muscles of the neck, masseter and related to pregnancy, menstruation and
facial muscles. hyperthyroidism.
Muscular contraction induces a severe,
painless muscular spasm, and an actually delay Clinical Features
in relaxation. Electrical and physical stimulation
It occurs in adults in the middle age group with
of a muscle produces the characteristic
a predilection for women.
prolonged contraction or ‘percussion contraction’.
Blinking with strong closure of the eyes will Symptoms — It is characterized by a rapidly
sometimes produce a prolonged contraction of developing weakness in voluntary muscles,
the lids. following even minute activities. Diplopia and
692 Textbook of Oral Pathology

ptosis along with dropping of the face, lends a dermatitis, myositis and sometimes neuritis and
sorrowful appearance to the patient. The neck mucositis.
muscles may be so weak that the head can not
be held up without support. Clinical Features
It may occur in patients of any age ranging from
Signs — Patients become exhausted, looses
very young children to elderly, but majority
weight becomes further weakened and may
occur in the 5th decade of life. There is no sex
eventually become bedfast. Death frequently
predilection.
occurs from respiratory failure.
Symptoms — It begins with erythematous skin
Oral Manifestations eruptions, edema, tenderness, swelling and
weakness of the proximal muscles of limbs. Fever
The patient’s chief complains may be difficulty may be associated with it.
in mastication, deglutition and dropping of the jaw.
Speech is often slow and slurred and disturbance Signs — The weakness of muscle is progressive
in taste sensation occurs. Dysphagia and and characteristically spread to the face, neck,
regurgitation of food are common. larynx, pharynx and heart. The skin becomes the
There may be weakness of the tongue and palatal seat of violaceous erythema and edema with a
muscles. Protrusive movements of the tongue predilection for the eyelids, malar area and dorsa
may become weak leading, at times to posterior of hands. The typical skin lesions include
collapse of the organ with airway obstruction. ‘heliotrope’ (lilac-colored) changes around the face
and fingers.
The edema which gives the skin a puffy
Histopathological Features
consistency including the face, leaves a reticulated
Focal collection of small lymphocytes or telangiectatic erythema when it subsides.
lymphorrhages is found surrounding small blood Calcinosis cutis — The skin lesions frequently
vessels in the interstitial tissue of the affected
calcify and form calcium carbonate nodules with
muscles. In few cases, foci of atrophy or necrosis
a foreign body reaction which is known as
of muscle fibers have been described. calcinosis cutis.

Management Calcinosis universalis — The term calcinosis

universalis is applied when these calcified
Anticholinesterases — Pyridostigmine and masses are found generalized throughout the
neostigmine, administered intramuscularly soft tissues.
improves the strength of the affected muscles
few minutes. Plasmapheresis has been temporary Oral Manifestations
value in patient with severe exacerbation.
The oral lesions consist of diffuse stomatitis and
pharyngitis are extremely common. Involvement
Dermatomyositis
of the muscles of jaw, tongue and pharynx may
It is also called as ‘polymyositis’. It is an acute or a cause problems in eating and phonation. The oral
chronic disease of unknown etiology and is mucosa may show dark red or bluish erythema.
characterized by a gradual onset with vague and In the early stages, tongue is swollen and later
indefinite prodromata, followed by edema, becomes harder and gradually it becomes
 Diseases Affecting Nerves and Muscles 693

atrophic. The tongue may become rigid owing to Laboratory Findings

severe calcinosis. Telangiectatic lesions of
It manifests as a mild anemia or leukocytosis. In
vermilion border of lips and cheeks may also occur.
addition, creatinuria is a constant finding as well
There is purplish black intrinsic staining of teeth.
as elevated levels of serum transaminase and
Histopathological Features aldolase.

The muscle fibers in dermatomyositis exhibit

Management
widespread degeneration and hyalinization. In
advanced cases, the muscle fibers disappear Prognosis—Muscle involvement may become
leaving only the fibrous stroma. Many fibers severe enough to confine the patient to bed or
show vacuolization, granulation and fragmentation cause death owing to failure of respiratory
with phagocytosis of disintegrating fibers. muscle.
 CHAPTER
Syndromes of the
36 Orofacial Region

INTRODUCTION • Cause—For example, Crush syndrome

• Pathogenesis—For example, Dumping syn-
The heritage of the term syndrome is ancient. It
drome
was used by Hippocrates to denote a group of
• Anatomic location—For example, oculo-dento-
regularly concurrent symptoms which resulted
digital syndrome.
from several causes. The word syndrome has its
• Main symptoms—For example, progressive
origin from the Greek literature which means,
hemifacial atrophy
“running together” (syn—together and dro-
mos— running). The word syndrome has been Eponyms: Eponyms are used to describe syn-
used in English language since 1541. dromes and diseases since the middle of the
At this time there is neither complete agree- nineteenth century when they were employed
ment concerning what constitutes a syndrome by neurologists to separate groups of similar
nor there is a uniform method of nomenclature. clinical entities – One of the most obvious merits
Syndrome is defined as “A group of signs and is the honor bestowed upon several keen obser-
symptoms or a set of symptoms usually three or vers who have dotted the medical and dental
more, which occur together characteristic of a history.
morbid condition”. Or “It is the aggregate of The etiological factors for the occurrence of
signs and symptoms associated with any morbid the syndromes are not definite. Many times it
process and constituting together the picture of occurs due to genetic influence or genetic dispo-
the disease.” sition. Racial predominance is also seen as a cau-
Syndromes being a multisystem disease are sative factor. Offsprings of an affected individual
difficult to classify in a specific pattern. It is a usually have chance of acquiring the disorder if
grouped together symptom complex which is of inherited. The syndrome sometimes becomes
varied etiology and clinical presentation. progressively more severe in succeeding genera-
tions.
Classification of Syndromes Syndromes may also occur due to immuno-
Many syndromes are classified according to: dysfunction or an abnormal immune response
• Eponym—For example, Morquio-Brailsford to microbial antigen – Dysfunction of certain
syndrome glands and vascular anomalies can also result in
698 Textbook of Oral Pathology

form of syndromes. Diagnosis of a syndrome is SYNDROMES ASSOCIATED WITH

problematic because of the extreme variability CRANIOFACIAL ANOMALIES
of its clinical expression. OF GENETIC ORIGIN
Although the prognosis in some of the condi-
Genetic Factors
tions is extremely poor, mortality is drastically
reduced with early diagnosis and recent advan- Genetic information in humans is carried by 23
ced treatment modalities available. Treatment pairs (46) of chromosomes, known as autosomes
can be directed at correction or reconstruction which carry information regarding the structure
of the existing deformities. During infancy and and functions of the body one pair (2) is known
childhood the patient should be examined as the sex chromosome, determines the sex of
frequently to rule out suspicious conditions. the individual (XY in male and XX in female).
Genetically derived-disorders can be
Growth and development should be observed
considered under three major groups:
closely for evidence of mental impairment or
• Chromosomal disorders
abnormalities of sexual development. This will
• Single gene (monogenic) disorders
facilitate early identification of potentially dan-
• Multifactorial disorders.
gerous syndromes.
Syndromes as already stated are difficult to Chromosomal Disorders
classify in a specific pattern as it is a grouped
The disorder in this group is due to errors in
together multisystem complex of signs and sym- number and/or structure of the chromosomes.
ptoms. For sake of convenience, orofacial syn- For example, Down syndrome (Trisomy 21)
dromes are compiled together under the follo- results from an additional autosomal
wing headings: chromosome, while Klinefelter syndrome is
• Syndromes associated with craniofacial ano- associated with an additional X chromosome in
malies of genetic origin male, i.e. XXY. Deletion of part of chromosome
• Syndromes associated with skin and pigmen- 5 results in the “Cri – Du – Chat’syndrome.
tation
Single Gene Disorders
• Syndromes associated with salivary and
lacrimal glands The disorders in this group are due to defects in
• Syndromes associated with teeth, a single gene which refers to the primary error
• Syndromes associated with oral mucous in the DNA code. These disorders are inherited
membrane, lips and cheek, gingiva, tongue, following Mendelian laws and are subdivided
according to the chromosome on which the
• Syndromes associated with nerves,
abnormal (mutant) gene is situated and by the
• Syndromes associated with blood and vas-
nature of the trait.
cular malformations The inheritance is known as autosomal when
• Syndromes associated with immunodefi- the abnormal gene is situated on an autosome
ciency, and the trait is said to be X-linked when such a
• Syndromes associated with hormonal distur- gene is situated on the X chromosome.
bances, These traits may either be “dominant” or
• Syndromes with benign oral neoplastic or “recessive” (autosomal dominant or recessive,
hamartomatous components. and X-linked dominant or recessive).
 Syndromes of the Orofacial Region 699

Autosomal dominant inheritance has the Multifactorial Inheritance

following characteristics:
This involves interaction between both
• Every affected person has at least one affected
environmental and genetic factors. The
parent.
environmental factors include: 1) trauma 2)
• Both males and females are likely to be affected
neoplastic disease 3) toxic drugs 4) metabolic
and are capable of transmitting the disease
factors and 5) infections.
• Transmission is generally seen (father to son
or daughter and mother to son/daughter) Genetics of Craniofacial Malformation
with no skipping of generation
• Affected persons typically transmit the trait Causes of congenital malformation
to half their offspring • Genetic Factors
These general rules however are modified by – Chromosomal disorders
• Penetrance—Carrier of abnormal genes may – Single gene disorders
be so mildly affected to be entirely indistin- – Multifactorial disorders (polygenic and
guishable from normal persons. A gene is environmental) at birth and disorders of
said to be “penetrant” if it is expressed in a later life
given carrier • Nongenetic factors
• Expressivity—A mildly affected parent may – Maternal infections
– Maternal metabolic derangements and
have severely affected child or vice versa.
genetic diseases. (Diabetes, hyper-tension,
There is variation in the expression
thyroid disorders)
• New mutation—An individual who does not
– Maternal exposure to radiation
carry a gene for a particular inherited
– Disturbances of embryonic differentiation
condition may rarely have an affected infant.
and fetal growth.
Autosomal Recessive Inheritance Some Craniofacial malformation syndromes
The characteristic are: with autosomal dominant inheritance
• The trait usually appears only in sibs and not • Blepharophimosis syndrome
in their parents • Waardenberg’s syndrome
• On average one in four of the sibs are affected • Treacher-Collin syndrome
• Consanguity is generally seen among the • Van der – Woudes syndrome
parents of the affected individual • Poplitial pterygium syndrome
• Males and females tend to be equally affected • Oculo-dento-digital syndrome
• Affected individuals will generally have • EEC (Ectrodactyly, ectodermal dysplasia
normal children but all of the children will clefting)
be carrier of the condition. • Craniostenosis syndromes:
– Crouzon syndrome
Sex-linked Inheritance – Saethre – chotgen syndrome
When the X chromosome carries the abnormal – Pfeiffer’s syndrome
gene, the disorder is said to be X-linked and – Apert syndrome.
when the Y chromosome carries it, the condition Some craniofacial syndromes with recessive
is said to be X-linked dominant disorders, e.g. inheritance
(the hypoplastic form of amelogenesis, e.g. • Cryptothalmes syndrome
hypohidrotic ectodermal dysplasia) • Orofacial digital syndrome
700 Textbook of Oral Pathology

• Roberts syndrome Essential oral features include high arched

• Carpenters syndrome. vault of the palate with or without clefts, delayed
eruption of teeth, malformed roots, super-
X-linked Inheritance Syndromes numerary teeth and frequent impactions.
• Albinism—Deafness syndrome A skull X-ray reveals the features together
• Amelogenesis imperfecta (two types) with the obtuse mandibular angle which is
• Coffin—Lowry syndrome increasingly reported in these patients.
• Aarskog syndrome Impacted teeth in affected individuals are
• Focal dermal hypoplasia reported to be lacking cellular cementum.
• X—linked hydrocephaly
• Lowe syndrome Apert’s Syndrome (Acrocephalysyndactyly)
• Mucopolysaccharide II (Hunter)
Acrocephalysyndactyly is believed to be
• Orofacial-digital syndrome.
transmitted by an autosomal dominant gene
Multifactorial (MFR) occurring sporadically in about 1 – 2000 000 of
the general population.
• Sturge-Weber syndrome
Clinical features include ovoid skull,
• Mobius syndrome
brachycephalic and often presents a horizontal
• Frontonasal dysplasia syndrome
supraorbital groove. Premature closure of the
• Goldenhar syndrome
sutures occurs and often the anterior fontanelle
• Aglossia – adactylia syndrome
is open due to late closure.
• Cleft lip – cleft palate malformations
Features of hand and feet include synda-
• Craniosynostosis.
ctylism. Osseous or cutaneous fusion of fingers
Cleidocranial Dysplasia is usually present.
Oral features include a high palatal vault and
Cleidocranial Dysplasia is an autosomal
the presence of posterior palatal and uvular
dominant condition although in less than half
the cases it occurs spontaneously. clefts. Dental malocclusion is consistent.
The condition is characterized by the defects Other oral features include mandibular
in the clavicles, cranial vault and occasionally prognathism, malocclusion and retarded
with other selected and dental findings. eruption.
As the name suggest, the frequent changes Ellis-van Creveld Syndrome
seen in the syndrome are in the clavicles and the
cranium. The clavicles are unilaterally or Synonym: Chondroectodermal dysplasia
bilaterally hypoplastic or aplastic. When Ellis-van Creveld syndrome consists of
hypoplastic the deficiency is seen at the acromial anomalies derived from the ectodermal
end allowing the individual to approximate their mesodermal germ layers. The disorder is
shoulders in front of the chest. X-ray of the chest believed to be inherited as an autosomal
reveal the exact defect. recessive trait. The disorder is congenital. Defects
The skull is large with frontal, parietal and are seen in the extremities, teeth, nails and hair.
occipital bossing. Delayed mineralization of the Rarely cardiac anomalies also have been noted.
sutures is a constant finding of the syndrome. Person is of short stature from birth.
There is usually a midfacial hypoplasia present Polydactyly with or without syndactyly is
resulting in relative prognathism of the present. Nails are dystrophic and hairs are
mandible. sparse.
 Syndromes of the Orofacial Region 701

Oral Manifestations—Deciduous teeth show The affected individual is mentally retarded,

hypoplasia occasionally and partial anodontia short stature and exhibits brachycephaly with a
of permanent dentition is seen. Prognathism of relatively flat occiput. Fontanelles close later and
lower jaw is present. There is obliteration of labial frontal sinuses are hypoplastic.
suture due to gingival frenal attachments. The ears are small and the iris shows
speckling (Brushfiel’s spots). Disorders involving
Crouzon Syndrome the pelvis, skin hair and genital occur with
Synonym: Craniofacial dysostosis variable frequency. Facial features include a
Craniofacial dysostosis is an autosomal small head and an open mouth.
dominant condition, characterized by cranial Oral manifestation—Short mouth, large tongue
synostosis, ocular proptosis and hypoplastic and tongue thrust beyond the lips. Maxillary
maxilla. lateral incisors show abnormality. They are
The appearance is quite characteristic either conical or missing developmentally.
because of exophthalmos, hypertelorism and the Microdontia is often present. Delayed eruption
under development of the maxilla. Exophth- and malocclusion are other findings. The palate
almos is due to shallow orbits. is high arched and narrow, the usual may be
Maxillary hypoplasia is responsible for the bifid. Necrotizing ulcerative gingivitis is
relative mandibular prognathism which is a reported to be common in these individuals.
consistent feature of this syndrome. The upper In about 95% of the affected individual’s
lip is usually short and the nose is parrot like. condition arises as the result of the failure of
The cranium is brachycephalic. paired chromosomes to disjoin and enter
Oral features include, malocclusion, cleft lip separate daughter cells during meiosis.
and palate with V shaped palate. Some of the Both chromosomes (number 21) from parental
occasional findings include peg shaped teeth and cell enter the gamete destined for fertilization and
partial anodontia. at fertilization a third chromosome (number 2) is
added from the normal gamete of the other
Trisomy Syndromes
parent. The result is a zygote with three
Trisomy syndromes represent autosomal chromosomes (number 21) which is known as
chromosome disorders. Several disorders in this trisomy 21.
group have been discovered. Only those with
craniofacial and associated dental anomalies will Trisomy 18 Syndrome
be dealt with in the following discussion. They
Synonym: Edward syndrome
are:
• Trisomy 21 syndrome (Down’s syndrome) Children with trisomy 18 are quite striking at
• Trisomy 18 syndrome (Edward syndrome) birth with intrauterine retardation of growth and
• Trisomy 13 syndrome (Patau syndrome) characteristic facial appearance. Trisomy 21 and
trisomy 13, non-dysfunction is the cause of this
Trisomy 21 syndrome. Increased maternal age is an
important factor in its causation.
Synonym: Down’s syndrome The affected individuals are mentally
Trisomy 21 occurs in one in about 650 births. This retarded and show hypertonicity. The index
syndrome is characterized by several features finger overlapping the third finger and the fifth
involving almost all organs of the body. over fourth are the striking features. Oral
702 Textbook of Oral Pathology

features – small mandible high arched palate and Oral features include micrognathia, palate
occasional cleft palate. defects and absence of hard and often the soft
palate.
Trisomy 13 Syndrome
Hajadu-Cheney syndrome
Synonym: Patau syndrome
Synonym: Acro-osteolysis
In the trisomy 13 syndromes, affected
individuals show 46 normal chromosomes but Acro-osteolysis is a rare autosomal disorder
a portion of chromosome 13 is attached to characterized by short stature, disintegration of
another chromosome or is present as a fragment. the terminal phalanges of fingers and toes,
The majority of cases are due to nondisjunction. premature loss of teeth and abnormal shape of
The physical features are characteristic. They skull.
include microcephaly, microphthalmia, ocular The affected individual is generally short. A
hypertelorism, microphthalmia, ocular hyper- long nose, low frontal hairline and flared ears
telorism, malformed ear, deafness, polydactyly are the characteristic facial features. The
and heart anomalies. phalanges of fingers and toes exhibit acro-
Oral features include a small mandible, high osteoses and clubbing of nails present.
arched palate and occasional cleft palate and The skull is dolicocephalic. Sutures are
bifid uvula. As with all trisomy syndromes. usually open. Oral features include premature
Chromosomal counts confirm the diagnosis. loss of teeth.

Cerebrohepatorenal Syndrome Anderson Syndrome

Synonym: Familial osteodysplasia
Synonym: Bowen syndrome
The recently reported syndrome of familial
The cerebrohepatorenal syndrome is believed to
osteodysplasia is an autosomal recessive
be having an autosomal recessive inheritance.
disorder, characterized by craniofacial and
The condition is characterized by craniofacial
anomalies, renal cortical cysts, hypoprothrom- skeletal anomalies, presence of hyperuricemia
binaemia and several other occasional findings and diastolic hypertension.
involving genitals, spleen and pancreas. Oral features include mandibular
Oral features include micrognathia, prognathism, hypoplastic maxilla and resultant
protruding tongue and high arched palate. malocclusion. The mandible without wide angle,
increased body length and reduced ramus and
Cerebrocostomandibular Syndrome body height is an essential features of the
syndrome.
The cerebrocostomandibular syndrome is an
autosomal recessive condition. This disorder is Caffey-Silverman syndrome
characterized by mental retardation, thoracic
Synonym—Infantile cortical hyperostosis
deformities and mandibular and facial
anomalies. Infantile cortical hyperostosis is characterized by
Clinical features include microcephaly, long the bilateral mandibular swellings, hyper-
trunk, rib anomalies producing gaps and irritability and fever occurring in infants.
tracheal cartilage malformation producing a The bilaterally symmetrical swellings which
barking cough. occur over the ramus, angle and the body of the
 Syndromes of the Orofacial Region 703

mandible are very striking. The soft tissue is Facies are quite striking in that two unrelated
tender and edematous, often undergoing patients look like siblings. X-rays and blood
remission and exacerbations. Similar bony and chemistry help in the diagnosis as serum alkaline
soft tissues changes may be seen in ribs, ulna, phosphatase is elevated.
femur, clavicle, tibia and irritability are seen as
preceding symptoms in about 70% of the affected Mohr Syndrome (OFD II)
infants.
Synonym: OFD Orofacial digital syndrome II
Hematological and biochemical findings
include raised erythrocyte sedimentation rate, The Mohr syndrome is an autosomal recessive
leukocytosis anemia and increased alkaline disorder characterized by oral, facial and digital
phosphatase levels. defects. The affected individual is moderately
This disorder is characteristically seen in the short, exhibiting digital deformities such as
infants, the onset being usually between the brachydactyly, syndactyly or polydactyly. Facial
second to sixth months after birth, although a features include the midline cleft lip and the
few cases have been reported in utero. Disorders broad and bifid tip of the nose.
to be considered in differential diagnosis include Oral features include the lobate tongue, high
osteomyelitis and parotid gland inflammation arched palate, hypoplastic body of mandible,
or tumors. and the absence of mandibular incisors.
The syndrome resembles the orofacial digital
Median Cleft Face Syndrome I syndrome, which is X-linked and occurs only
Synonym: Frontonasal dysplasia in females.
Frontonasal dysplasia is a disorder of unknown
Treacher Collins Syndrome
genetic causes (possibly multifactorial)
characterized by facial disorders such as nasal Synonym: Mandibulofacial Dysostosis
clefts and notches, preauricular tags and ocular Treacher Collins syndrome primarily affects
hypertelorism. Extracranial anomalies are structures developing from the first branchial
usually not found in this syndrome. arch but it also involves the second branchial
Oral features may include cleft lip and arch, to a minor degree.
malocclusion. The persons affected have a convex facial
profile with a prominent nose and retrusive chin.
Melnick Needles Syndrome It is generally a bilateral anomaly with a
Synonym: Osteodysplasty characteristic faces including downward sloping
Melnick Needles syndrome is an autosomal of the palpebral fissures, coloboma of the lower
dominant condition characterized by the eyelid, mandibular and midface hypoplasia and
generalized bone dysplasia and abnormal faces. deformed pinnas.
The facial appearance is quite typical with
marked exopthalmos, full cheeks, large ears, Etiology and Pathogenesis
micrognathia and a transversely long mouth. It is transmitted by an autosomal dominant
Radiographically the skull exhibits delayed mode of inheritance although about half the
closure of the basic of the skull and mastoid cases are due to spontaneous mutation. Affected
process. There are also skeletal deformities siblings are remarkably similar and the
involving clavicle, vertebrae, radius, tibia, fibula syndrome becomes progressively more severe in
and humerus, micrognathia and malocclusion. succeeding generations.
704 Textbook of Oral Pathology

This disorder is relatively rare with an Treatment and Prognosis

incidence between 0.5 and 10.6 cases per 10,000 It is directed at correction or reconstruction of
births. Stapedial artery dysfunction results in the the existing deformities. Neutralization of
defects of the stapes and incus and first arch conductive hearing loss through surgery and
vessels supplying the maxilla. hearing aids is helpful. Ophthalmic surgery is
The expression begins as early as the sixth to done to correct eye defects.
seventh embryonic week. Mandibular
abnormalities such as improper orientation and Pierre-Robin Syndrome
hypoplasia of the mandibular elevater muscles The Pierre-Robin syndrome manifest clinically
result due to failure of developments of ancillary as microganthia, glossoptosis and high arched
vascular supply by inferior alveolar artery. or cleft palate in the neonate. It can occur as an
isolated finding or as a component of various
Clinical Findings syndrome or development anomalies.
Treacher Collins syndrome is a combined Primary findings are retrognathia and
development anomaly of first and second hypoplasia of mandible. Respiratory and feeding
branchial arch. Varying degrees of hypoplasia problems are prevalent and may result in
of the mandible, maxilla, and zygoma is seen. episodic airway obstruction, infant hypoxia,
Abnormalities of medial pterygoid plates and malnutrition and failure to thrive.
hypoplasia of lateral pterygoid plates are
Etiology and Pathogenesis
common. Notched or linear colobomas of the
outer third of the lower eyelid are found in 75% Incidence is 5.3 to 22.7% per 100,000 births with
of patients. The facial appearance is characteristic 39% of the infants exhibiting no additional
and described as bird like or as fish like. abnormalities 25 % have known syndromes and
Microdontia or underdeveloped ear and a 36% have one or more anomalies that are not a
narrow extension of the hair over the part of a known syndrome.
preauricular region known as “hair like” are Fetal malposition and interposition of the
common in this syndrome. tongue between the palatal shelves have long
Oral findings include cleft palate in about been considered the etiologic catalysts for palatal
30% of the patients and macrostomia in 15% of deformity and micrognathia. Recent evidence
the patients. Cleft palate and malocclusion is suggest that the primary defect may be due to
present. The underdeveloped zygomatico- genetically influenced metabolic growth
maxillary complex leads to a clinically severe disturbances of the maxilla and mandible rather
mid face deficiency. than to mechanical obstruction by the tongue
Radiographic findings include downward during embryogenesis.
sloping floors of the orbits, peaked long nasal
contor, aplastic or hypoplastic zygomatic process Clinical Features
of the temporal bone and obtuse mandibular Infants present without severe micrognathia and
angle. Lateral cephalograms demonstrate mandibular hypoplasia. A 'U' shaped high
antegonial notching and broad concave nature arched palate is usually present. Glossoptosis is
of the inferior border of the mandible helps to the result of the retro positional attachment of
distinguish the condition from other syndromes genioglossus muscle because of the retrognathic
involving the mandible. mandible. The geniohyoid muscle is fore
 Syndromes of the Orofacial Region 705

shortened so that support to the hyoid bone and Oral findings include high arched palate with
strap muscles of the larynx is also compromised. dental crowding. The face appears long and
narrow.
Treatment and Prognosis The cardiovascular system shows mitral
valve prolapsed in 75 to 85% of the cases. Aortic
Treatment is supportive to overcome feeding
dilatation is there of the ascending aorta leading
problems and the patient should be monitored
to aortic regurgitation.
for airway obstruction.
Ocular findings include dislocation of the
Marfan's Syndrome lens (ectopic lentis). Retinal detachment is
infrequent but myopia (short sightedness) is a
It is a heritable disorder of connective tissue common finding.
characterized by abnormalities of the skeletal,
cardiovascular and ocular systems. Diagnosis is Treatment and Prognosis
problematic because of the extreme variability
of clinical expression. The syndrome is notable Subluxation of the lens of the eye, chest cavity
for a number of cases of sudden catastrophic deformities potential for pneumothorax are
death occurred in affected athletes (undia- serious prognostic indicators. Treatment consists
gnosed). of annual medical examination with a
cardiovascular, emphasis frequent ophthal-
Etiology and Pathogenesis mologic examination, scoliosis screening and
echocardiography. Mortality has been drastically
It is an autosomal dominant inherited in 1 in reduced with the use of composite grafts to
100,000 individuals. There are no ethnic, racial replace the aortic value and region containing
or gender predictions. The Marfan's gene is the aortic aneurysm.
believed to produce a change in one of the
proteins that provide strength to a complete
Ehlers-Danlos Syndrome
connective tissues probably collagen. The gene
has been located on chromosomes 15 and will The Ehlers-Danlos syndrome is an uncommon
provide for diagnostic testing in pairs at risk. inherited disorder of connective tissue, clinically
Diagnosis is based on characteristic characterized by joint hypermobility and skull
abnormalities of the musculoskeletal ocular and hyperextensibility. There are inherited defect in
cardiovascular systems and a positive family collagen metabolism. In addition to the skin and
history. joint anomalies, severe gastrointestinal and
cardiovascular complications may occur and
Clinical Features coexist.
The condition has been classified into eight
Patients characteristically present with a tall
variants. The periodontal form (EDS type VIII)
slender stature with relatively long legs and
is characteristic by rapidly progressing
arms, large hands with long fingers and loose
periodontal disease resulting in complete tooth
joints. The arms, legs and digits are dispro-
loss in second or third decade of life.
portionately long compared to the patient trunk.
Chest deformities present with a protrusion or
Etiology and Pathogenesis
indentation of the breast bone. Even the normal
thoracic kyphosis is absent and the back is Various subtypes of Ehlers-Danlos syndrome are
straight. inherited as autosomal dominant, autosomal
706 Textbook of Oral Pathology

recessive and X- linked traits. There is defect in patients should be evaluated and monitored.
the synthesis and structure of type III collagen. Sudden death can occur. Surgical intervention
A deficiency of the enzyme lysyl hydroxylase must be tempered in light of connective tissue
resulting in decreased amounts of collagen fragility unsuccessful owing to suture failure.
hydroxylsine has also been reported. The Wound healing is delayed and prolong bleeding
conversion of precollagen to collagen is may occur following injury.
prevented.

Clinical Feature SYNDROMES ASSOCIATED WITH SKIN

AND PIGMENTATION
There is marked hyper elasticity of skin and
extreme laxity of the joints. The skin can be Hereditary Ectodermal Dysplasia
stretched for several centimeters and when
released it resumes its contor. Skin has velvety In order to be considered an ectodermal
appearance with high degree of fragility and dysplasia, the disorder should meet the
bruisability. Minor trauma may produce following criteria suggested by Solomon etal
ecchymosis, bleeding and large gaping wounds • The disease must be congenital
with poor healing tendencies and "cigarette • The disease must be diffuse ( not localized)
paper" scar formation especially on the forehead
and must involve the epidermis as well as at
and lower legs and over pressure points.
least one of its appendages (hair, sebaceous
Articular hypermobility is variable. Extreme
glands, nails, mucosa, mucosal glands and
joint laxity leads to spontaneous dislocation of
joints (back knee) flat feet, etc. teeth)
Cardiovascular anomalies include mitral • The epidermal component may involve
valve prolapsed and rupture of major blood keratinocytes, melanocytes or Langerhans
vessels. Pulmonary problems include cells or any combination there of
spontaneous pneumothorax and respiratory • The disease is not progressive, infact patients
impairment secondary to chest wall deformities. often improve somewhat over a period of
Orofacial features include a narrow maxilla years
flattened midface and wide nasal bridge. • Increased delineation of syndromes with
Fragility of gingival and mucosal tissues may be ectodermal defects has led to considerable
problematic. Marked extensibility of the tongue
difficulty in developing a coherent
enabling contact with the tip of the nose has been
classification of this disorder
described.
Dental findings include deep anatomic • The ectodermal dysplasia are classified by
grooves and excessive cuspal height of the Friere-Maia and Pinhero for the purpose of
molars and premolars. Maceration of roots and delimiting the field as conditions with at least
pulpal calcified structures have been noted. one of the following four features :
– Trichodysplasia
Treatment and Prognosis – Dental defects
Prognosis depends on severity of the systemic – Onychoysplasia
manifestation. The cardiovascular status of all – Dyshidrosis.
 Syndromes of the Orofacial Region 707

Classification of the Ectodermal Dysplasis Contd...

Disorder Inheritance Congenital ED of the face Heterogenous ?

Subgroup 2-3
Subgroup 1-2-3-4
Nail dystrophy-deafness syndrome Autosomal dominant
Anhidrotic X-linked ED X-linked semidominant
Triphalangeal thumbs-
Hypohidrotic ED Autosomal recessive
hypoplastic distal
(autosomal recessive)
Phalanges-onychodystrophy Autosomal recessive
Xeroderma, talipes, and Autosomal dominant
Subgroup 2-4
enamel defect
Marahall's ED with ocular and Autosomal dominant
Rosselli-Gulienetti syndrome Autosomal recessive
hearing defects
Rapp-Hodgkin hypohidrotic ED Autosomal dominant
Ectrodactyly-ED-clefting syndrome Autosomal dominant
Subgroup 1-2-3
Other Ectodermal Dysplasias
Hidrotic ED(Clouston's syndrome) Autosomal dominant
Trichodento-osseous syndrome Autosomal dominant A large number of further ectodermal dysplasias
Trichorhinophalangeal syndrome Heterogenous recessive are associated with alterations of the oral mucosa
Ellis-van Creveld syndrome Autosomal recessive or of the teeth. They are listed in the following
Schof-Schulz Passarge Syndrome Autosomal recessive table.
Dentao-oculocutaneous syndrome Autosomal dominant ? Syndrome Oral manifestations Other features

Odontotrichomelic dysplasia Autosomal recessive ?

Cranioectodermal Small decidous teeth; Hypoplastic
Tooth and nail syndrome Autosomal recessive enamel;
Subgroup 1-3-4 dysplaisia hypodontia; shortened arms,
Freire-Maia syndrome ? Dolichocephaly; taurodonitism Finger toes; hair
anomalies
Subgroup 2-3-4
Trichodento osseus Amelogenesis Curly hair,
Hypoplastic enamel - onycholysis Autosomal dominant
imperfecta sclerotic bone
hypohidrosis syndrome enamel hypoplasia,
Tooth and nail syndrome Autosomal recessive unerupted teeth;
Gorlin's syndrome Autosomal recessive ? taurodontism

Oculodentadigital syndrome ? Trichoonychodental Hypodontia: Fine short hair;

syndorome conical teeth Thining of the
Monilethrix and anodentia Autosomal dominant
Lateral ends of
Oral - facial digital syndrome X- linked dominant ? the eyebrows
(type I)
Trichoonychodental Taurodontism, enamel Fine curly hair;
Subgroup 1-3 syndrome defects Thin dysplastic
Curly hair-ankyloble Autosomal dominant nails
pharon-nail Dysplasia Curry-Hall Small and conical Short Limbs;
Palanoplantar hyperkeratosis Heterogenous syndrome deciduous teeth;
Retained incisors; Polydactyly, nail
and alopecia
small dysplasia
Onychotrichodysplasia Autosomal recessive permanent teeth
with neutropenia Otodental Globodontia; Sensorineural
dysplaisia taurodontism; hearing loss
Subgroup 1-4
microdontia; hypodontia

Contd... Contd...
708 Textbook of Oral Pathology

Contd... Clinical Features

GAPO: Failure of both Frontal bossing The patients exhibit a soft, smooth, thin, dry skin
(growth retardation dentitions midface
with partial or complete absence of sweat glands.
alopecia to erupt hypoplasia
Pseudoanodontia Such persons cannot perspire and they
and Optic atrophy) consequently suffer from hyper pyrexia and an
Johanson-Blizzard Hypodontia in both Hearing loss inability to endure warm temperatures. The hair
syndrome dentitions. Roots of panceratic
of the scalp, a eyebrow are fine scanty and blond.
deciduous teeth are dysfunction
short and deformed, mental
crowns are conical Retardation
Oral Manifestation
microcephaly; They manifest anodontia or oligodontia.
hypoplastic
Complete or partial absence of teeth with
alanasi
Waardenburg's Cleft lip/palate Deafness; hair frequent malformation of any teeth present.
syndrome depigmentation. Where some teeth are present, they are
LEOPARD No mucosal lentigines Triangular face commonly truncated or cone shaped when
syndrome with complete anodontia exists, the growth of the jaw
(lentigines, but there may be hypertelorism is not impaired.
and ptosis
electrocardiographic grandular cell
anomalies, ocular myoblastomas Ellis–van Creveld Syndrome
hypertelorism, Synonym: Chondroectodermal dysplasia
pulmonary stenosis,
abnormal genitalia,
(described under syndromes of craniofacial
retarded growth, malformations).
deafness) Chondroectodermal dysplasia is characterized
Congenital Hyperkeratosis Deafness by a number of ectodermal disturbances
erythrokeratoderma Occasional carcinoma erythroker- including involvement of the nails and teeth as
atoderma
With sensorineural
well as chondrodysplasia polydactyly and
hearing loss congenital heart disease. Hypoplastic nails with
koilonychias is present.
The most constant finding is a fusion of the
Hereditary Hypohydrotic (Anhydrotic) middle portion of the upper lip to the maxillary
Ectodermal Dysplasia gingival margin, eliminating the normal
mucolateral sulcus. Natal teeth or absence of
Hereditary hypohidrosis (anhidrotic) ectodermal teeth in the anterior segment frequently is a
dysplasia is a specific syndrome characterized feature. Delayed tooth eruption is always there.
by a congenital dysplasia of one or more Teeth are cone shaped irregularly spaced and
ectodermal structures and their accessory demonstrate enamel hypoplasia.
appendages, manifested primarily by
• Hypohidrosis Stevens-Johnson Syndrome
• Hypotrichosis At one time considered to be a separate disease ,
• Hypodontia. Stevens- Johnson syndrome is now recognized
It affects skin, hair, nails, eyes, teeth, facies, as simply a severe bullous form of erythema
sensorineural apparatus and adnexal glandular multiforme with widespread involvement
structures in various combinations and of typically including the skin, oral cavity, eyes and
varying severity. genitalia.
 Syndromes of the Orofacial Region 709

It commences with the abrupt occurrence of Incontinentia Pigmenti

fever, malaise, photophobia and eruption of the Incontinentia pigmenti, also known as Bloch-
oral mucosa, genitalia and the skin. The Sulzberger syndrome, is an uncommon genetic
cutaneous lesions in this mucocutaneous-ocular disease with an X- linked dominant mode of
disease are similar to those of erythema inheritance. This distinctive multisystem
multiforme, although they are commonly disorder is characterized by abnormalities of skin
hemorrhagic and are often vesicular or bullous. pigmentation.
Linear rows of blisters on the extremities are
Mucocutaneous Lymph Node Syndrome seen at birth. Occasionally the trunk is also
Synonym: Kawasaki disease involved. Later on the blisters are replaced by
warty lesions that may persist until first year of
This syndrome was first described by Kawasaki
age. In the final stage, there is hyper-
in 1967 in the Japanese children. The etiology of
pigmentation, caused by melanin deposition in
this disease is still unknown but is suggested to the upper dermis. Mental retardation, seizures,
be of viral disease or a "collagen - vascular" microcephaly, and other CNS disorders and
disease. ocular and skeletal anomalies occur in about 30
% of the patients. In 90 % of patients major dental
Clinical Features
anomalies such as partial or complete anodontia,
The diseases have been reported to occur in peg-shaped or conical deformities of the teeth,
children of age 3 to 12 years. The most frequent enamel disorders, and delayed eruption are
symptoms as determined by the epidemiological found (Bjellerup. Carrney; Milam et al; Vogte and
study group of the Japanese MCLS research Matheson). The oral mucosa is not involved.
committee are:
BROAD GROUPS OF PIGMENTARY
• Fever for 5 or more days with no response to
DISORDERS
antibiotics
• Bilateral congestion of ocular conjunction A multitude of factors, genetic and /or acquired
• Changes in the extremities peripherally related to melanin and /or some other histologic
including indurative edema, erythematous substrates needs to be considered in properly
palms and soles and membranous understanding the etiopatho-genesis in a
desquamation of fingers and toes particular case and its management. Disease of
• Lips and mouth show dryness, cracking and hypo- and hyperpigmentation can for all
swelling of tongue papilla practical purposes be grouped into two broad
divisions:
• Polymorphous exanthema of torso without
a. Cause showing cutaneous pigmentary alter-
vesicles or crust
ation as the most significant and predominant
• Acute purulent swelling of cervical lymph
sign if not the only exclusive clinical feature,
nodes. representing in most instances a relative lack
Other findings include diarrhea, proteinuria of excess of melanin pigmentation.
leukocytosis and increased sedimentation rate. b. Those cases showing these disorders as one
Cardiac abnormality may occur .While the vast of the clinical features in associations with
majority of the cases are self limiting and non- other cutaneous and/or extracutaneous
fatal, occasional deaths may occur as a result of manifestation which often appear more
cardiac complications. significant.
710 Textbook of Oral Pathology

That is the pigmentary disorders may be Bony lesions usually first present during the
either first decade with pain, pathological fractures,
• Depigmentary and hypopigmentary or swellings and deformities. In females precocious
• Hyperpigmentary of various shades. puberty at the age of 5 yr may occur in the form
of pubic hairs, vaginal bleeding and breast
Peutz-Jeghers Syndrome enlargement. Biochemical raised serum alkaline
Synonym: Periorificial Lentiginosis phosphatase level may be observed in the
This is an autosomal dominant disorder showing presence of widespread bone involvement.
pigmented macules on the oral mucosa and skin Histopathologically hyperpigmented
associated with gastrointestinal polyposis. macules and normal skin show a normal number
There is no sex or racial preponderance. A of melanocytes with absent or occasional
family history is found in 60% of cases. macromelanosomes in the melanocytes and
Pigmented macules show an increased amount keratinocytes. The disease is not usually lethal.
of melanin in the basal layer of the epidermis Pathological fractures normally unite easily.
without any associated increase in the number
of melanocytes. Macules are present at birth, SYNDROMES ASSOCIATED WITH
infancy or early childhood. The oral mucosa is SALIVARY AND LACRIMAL GLANDS
always involved with affection of buccal mucosa,
gums hard palate and lower lips. • Sjögren’s syndrome
Lesions are irregularly distributed round, • Heerford’s syndrome
oval or irregularly patch brown or black in color • Riley - Day syndrome
and 1 to 5 mm in diameters. Also present on the • Felty's syndrome.
face, hands, feet and nails. Gastrointestinal
polyps are benign hamartoma with relatively Sjögren's syndrome
less potential for malignant transformation. They
Synonyms: Sicca syndrome, Gougerat - Sjögren’s
may occur throughout the gastrointestinal tract
syndrome, rheumatoid sialadenitis.
but are commonly observed in the small
intestine. The symptoms of GI polyps are Sjögren’s has described this syndrome in 1933.
repeated abdominal colic, rectal bleeding, Sjögren’s syndrome is a condition originally
hematoma, anemia usually start between 10 and described as a triad consisting of kerato-
30 years of age. Apart from an increased risk of conjunctivitis sicca, xerostomia and rheumatoid
malignancy life expectancy is normal. arthritis. Primary Sjögren’s syndrome is so called
when patients present only with dry eyes and
Albrights Syndrome dry mouth (Sicca complex). The secondary
Albrights syndrome consists of fibrous dysplasia, Sjögren’s syndrome involves or develops
large pigmented cutaneous macules endocrine systemic lupus erythematosus, polyarteris
dysfunction with precocious puberty and nodosa , polymyositis or scleroderma as well as
somatic over growth. Cutaneous pigmented rheumatoid arthritis.
macules are usually present or appear soon after
Etiology
birth and are usually light brown in color. They
have irregular serrated margins and are Various causes of this disease have been
asymmetrically distributed, (with in the suggested, genetic, hormonal, infections and
midline). immunologic among others. It is generally
 Syndromes of the Orofacial Region 711

assumed that the antigen-antibody reaction is • In one case there may be intense lymphocytic
not the sole cause of this disease. As majority of infiltration of the gland replacing all the
those affected are women, hormonal dere- acinar structures although the lobular
gulation plays a role. architecture is preserved, there may be
Laboratory findings support the autoimmune proliferation of the ductal epithelium and
etiologic role. Bertram has reported that 75% of myoepitheliun to form "epimyoepthelial
a series of 35 patients with Sjögren’s syndrome islands". Both of these histologic changes are
had in their sera antisalivary duct antibody. In identical with those occurring in the benign
addition the sicca complex and Sjogren’s lymphoepithelial lesion (Mikulicz's disease).
syndrome have been found to be associated with • The third alteration may be simply an
the HLA system, specifically HLA - DR3 and
atrophy of the glands sequential to the
DR4.
lymphocytic infiltration.
Clinical Features
Laboratory Findings
The disease occurs predominantly in women
over 40 years of age, although children or young Primary Sjögren’s syndrome have shown a
adults may be affected. The female: male ratio is polyclonal hyperglobulinemia and may develop
10:1. The typical features are dryness of mouth cryoglobulins. Multiple organ or tissue specific
and eyes as a result of hypofunction of the antibodies are found, including anti-salivary
salivary and lacrimal glands. This often results duct antibodies, rheumatoid factor and
in painful, burning sensations of the oral mucosa. antinuclear antibodies.
In addition various secretory glands of the nose, An increased sedimentation rate is present
larynx, pharynx and tracheabronchial tree in 80% of these cases. The anti-salivary duct
(buccopharyngolargyngitis sicca) as well as of antibody present is three times more common
the vagina are involved with this dryness. in secondary Sjogren’s syndrome than the sicca
Sialochemistry studies have demonstrated complex.
significantly elevated levels of IgA, potassium
and sodium in the patients of sicca complex. Treatment and Prognosis: There is no satisfactory
Rheumatoid arthritis is an integral part of the treatment for Sjögren’s syndrome. Patients are
secondary Sjögren’s syndrome. It has been treated symptomatically. Keratoconjunctivitis is
shown that patients with rheumatoid arthritis treated with lubrication with artificial tears
have certain different clinical manifestations containing methyl cellulose. Xerostomia is
than patients with sicca complex, despite similar treated with the saliva substitutes.
histologic findings and some laboratory findings.
In this regard patients without rheumatoid Heerfordt's Syndrome
arthritis that is sicca complex more frequently
Synonym: Uveoparotid fever. Uveoparotitis.
manifest parotid gland enlargements, lympha-
denopathy, purpura, Raynaud’s phenomenon, Uveoparotitis is considered to be a form of
kidney involvement and myositis. sarcoidosis in which characteristically there is
firm, painless usually bilaterall enlargement of
Histologic Features parotid glands, accompanied by the inflam-
Three types of histologic alterations have been mation of the uveal tracts of the eye and cranial
described: nerve involvement.
712 Textbook of Oral Pathology

The submaxillary and sublingual glands may SYNDROMES AFFECTING TEETH

be similarly involved and even the lacrimal
glands maybe swollen, all features suggestive of • Gardner's syndrome
Mikulicz disease or Sjögren’s syndrome. A • Trichodento-osseous syndrome
chronic low grade fever is often present and the • Naegali-franceschettis Jadassohn syndrome
patient may complain of lassitude, malaise and • Johansson - Blizzard syndrome
vague gastrointestinal disturbances or even • Occulodentodigital dysplasia
nausea and vomiting. Xerostomia is common. • Fanconi's syndrome
The most common eye lesion in uveoparotitis • Lacrimal - auriculodentodigital syndrome
and often the earliest symptoms is uveitis, but • Cleidocranial dysplasia
conjunctivitis, keratitis and corneal herpes • Hereditary ectodermal dysplasia
among others also have been reported. • Nevoid basal cell carcinoma syndrome.
Although the uveitis may begin unilaterally,
it eventually becomes bilateral and in most cases Gardner's Syndrome
result in some permanent visual impairment.
Gardner's syndrome is an autosomal dominantly
The most common nerve paralysis is of seventh
nerve which may be unilateral or bilateral, which inherited disorder with variable penetrance,
is said to occur in 1/3rd to 1/2 of the cases. The characterized by multiple supernumerary teeth,
signs and symptoms of this syndrome multiple osteomas of bony skeleton, intestinal
dissappear within time, although some swellings polyposis. And various skin and soft tissue
of the parotid glands and visual disturbance may tumors. Multiple sebaceous cyst are also
persists. associated.

Etiology
Riley-Day Syndrome
Manifestation of Gardner’s syndrome is thought
Riley-Day syndrome is characterized by
to be due to abnormal growth of all three
sialorrhea, excessive perspiration, defective
primordial germ layers.
lacrimation and erythematous blotching of the
skin.
Clinical Features
Other Features Multiple polyps of the colon appear in the second
decade of life. Although asymptomatic,
Wide fluctuance in blood pressure, emotional
malignant changes or risk of colonic cancer is
instability cold hands and feet and hyporeflexcia.
almost 100% by the age of fifty. Skeletal
It is manifested first in infancy by impaired
abnormalities are localized cortical skeletal
swallowing and sucking and by absence of tears,
growth is retarded and the ability to walk and thickening of long tubular bones and osteomas
sit and speech is delayed. There is marked of the mandibular rami are characteristic of this
absence of fungi form papillae on the tongue. syndrome.
Occurs commonly or almost inclusively in Jews Dental anomalies noted are supernumerary
and is inherited as an autosomal recessive trait. teeth, odontomas, multiple unerupted teeth and
Sailorrhea is especially marked during multiple caries. Sebaceous or epidermoid cyst
excitement. The syndrome is thought to result appear later in life over the face, scalp and
from an inborn error in catecholamine meta- extremities. Lipoma are frequently noted in the
bolism. subcutaneous tissue. Fibromas are also reported.
 Syndromes of the Orofacial Region 713

Diagnosis Features
Family history of colonic malignancy. Osteomas A characteristic face in which the nose is thin
of the mandible and cutaneous lesions are and also hypoplastic microphthalmia or
indicators of the possible symptomatic colonic microcornia. Enamel hypoplasia and syndactyly
polyps. and camptodactyly of the fourth and fifth fingers.

Trichodento-osseous Syndrome
Fanconi's Syndrome
This autosomal dominantly inherited familial
Synonym: Familial pancytopenia. Familial pan-
syndrome affects the hair, teeth and bones.
myelophthisis.
Approximately 50% of the affected patients
complain of brittle nails that peel frequently. It is inherited disorder of childhood charac-
Dental manifestations are enamel terized by diffuse cutaneous pigmentation,
hypoplasia, impacted teeth, taurodont teeth and skeletal and dental changes, hematological
multiple dento alveolar abscesses, a thickened anomalies, mental retardation and hypo-
cortical bone enamel is thin with normal dentin. gonadism. There is enamel hypoplasia reported
with this syndrome. Cutaneous pigmentation is
Naegali-Franceschetti-Jadassohn Syndrome present with 85% of the cases characterized by
This is a rare autosomal dominant inherited generalized dusky or olive brown pigmentation
syndrome characterized by the development of on lower trunk and neck.
reticulate brown or slate - gray pigmentation, Hematological abnormalities include
yellowish discoloration of teeth palmoplantar progressive hypoplastic anemia, neutropenia
hyperkeratosis and hypohidrosis with heat and thrombocytopenia. Skeletal anomalies are
intolerance. short and broad hands with tapering fingers,
absence of thumbs, aplasia of the radius and
Johansson-Blizard Syndrome microcephaly.
This syndrome is characterized by hypoplasia
of the nasal alae: Lacrimoauriculodentodigital Syndrome
• Oligodontia Lacrimoauriculodentodigital syndrome is an
• Scalp and hair defects autosomal dominant malformation complex that
• Sensory neural defects affects craniofacial structures including teeth and
• Short stature salivary glands. The first case of lacrimo-
• Psychomotor retardation and malabsorption. auriculodentodigital syndrome was first
reported by Levey in 1967.
The nose is beak like and the teeth are peg shaped
and hair is sparse. Height, weight and head The features are:
circumference are all subnormal and osseous Lacrimal—Absence of the lacrimal puncta
development is delayed. nasolacrimal ducts and lacrimal sacs.

Oculodentodigital Dysplasia Dental—Dental dysplasia hypodontia,

hypoplasia, amelogenesis imperfecta, high
Oculodentodigital dysplasia is an autosomal
narrow palate.
dominantly inherited syndrome that affects the
eyes, teeth, digits, skeleton and the nervous Digital—Arachnodactyly, radioulnar synostosis,
system. hypoplasia of the first digit.
714 Textbook of Oral Pathology

Ears—Protuberant ears that are cup shaped. Melkersson-Rosenthal Syndrome

Sensory hearing loss.
Synonym: Mieschers's disease
Nevoid Basal Cell Carcinoma Syndrome (Bifid This condition is characterized by recurring
Rib Syndrome Or Gorlin-Goltz Syndrome) spontaneous swelling of one or both lips (which
may become permanent) has no cause. The
The dental/oral manifestation manifestation of this syndrome is facial swelling,
The developing teeth may get displaced facial neural paresis and plicated tongue. The M-
because of the odontogenic keratocysts R Syndrome generally appears in adults. Many
associated with this syndrome. There is cases reveal possible additional involvement of
deformity of teeth and may not develop till areas away from the face such as hands and
middle age of life. chest. Additional symptoms include secretary
disturbances of the salivary and lacrimal glands.
SYNDROMES ASSOCIATED WITH LIPS AND
CHEEK
van der Woude's Syndrome
• Ascher's syndrome The van der Woude’s syndrome was first
• Melkersson-Rosenthal syndrome described by Demarquay Rischet. It is also called
• van der Woudes syndrome as Demarquay - Rischet syndrome. Its
• Hereditary hemorrhagic telangiectasia manifestations are:
• Cowden syndrome • Congenital lip fistulas, especially of the lower
• Zinsser - Engman - Cole syndrome. lip
• Cleft lip and cleft palate
Ascher Syndrome • Hypodontia
Ascher syndrome, first described in 1920, is the • Flattened nose and
combination of a progressive enlargement of the • Skeletal and cardiac abnormalities.
upper lip (double lip) lid laxity (blepharo-
chalasis) and goiter. Hereditary Hemorrhagic Telangiectasia
The lip abnormality may be identical to the
Synonym: Osier's disease, Osier - Weber- Rendu
acquired double lip or it may be the result of
disease.
chronic recurrent then persistent lip swelling.
This is a relatively common autosomal dominant
Thus, Ascher syndrome does have some parallels
disorder. It was first described by Bird, Garland
with Melkersson - Rosenthal syndrome, but only
and Aiming. HHT is characterized by
the upper lip is involved and the horizontal ford
widespread vascular involvement with recurrent
is almost always seen. The lips feel soft and
episode of spontaneous bleeding with multiple
lobulated.
telangiectasia of the skin and mucous
Excessive salivation may be present - It is
membranes.
considered a localized disorder of elastic tissue
because both the eyelids and upper lip seem to be
Clinical Features
deficient in this structural component. There may
also be endocrine problems including thryoid The first and the most common clinical sign is
goiter acromegaly and menstrual irregularities. persistent nose bleeds which begins in
 Syndromes of the Orofacial Region 715

childhood. Tiny red macules and papules are Zinsser-Engman-Cole Syndrome

seen around the mouth. The lesions blanch
Synonym: Dyskeratosis Congenita
readily on diascopy. These are telangiectasia
throughout the gastrointestinal system and This extremely rare syndrome is characterized
lungs. CNS manifestation is migraines and by keratinization of the oral mucosa, reticular
epilepsy. Recurrent GIT bleeding may occur. cutaneous hyperpigmentation, nail dystrophy
There may be associated liver spleen vascular and pancytopenia. There is sex linked recessive
anomalies. inheritance pattern. The initial lesions may be
noted as early as fourth or fifth year as a
reticulated hyperpigmentation on the face. neck
Oral Manifestations
and trunk. Purpuric lesions can be seen as the
The oral mucosa has telangiectasia on the hematology abnormalities develop. The most
vermilion border of the lip, the lip mucosa, commonly observed involvement of the oral
tongue a buccal, mucosa and palate and less mucous membrane (sites being tongue and
oftenly gingiva. Oral bleeding in observed as a buccal mucosa) is characterized by extensive
frequent complaint after tooth brushing. keratinization, primary lesion that consists of a
Multiple telangiectasias have been reported in bulla that eventually ruptures apparently leads
the CREST syndrome. to eventual keratinization. The likelihood of
C — Calcinosis cutis squamous cell carcinoma developing is well
R — Raynauds phenomenon documented and all cases should be followed
E — Esophageal dysfunction very carefully for such occurrence.
S — Sclerodactyly
T — Telangiectasia. SYNDROMES ASSOCIATED WITH TONGUE

• Orofacial digital syndrome

Cowden Syndrome • Meckels syndrome
Synonym: Multiple Hamartoma syndrome. • Mobius syndrome
• Hurler syndrome
Cowden syndrome is a rare inherited geno-
• Maroteux. Lamy syndrome
dermatose.
• Beckwith hypoglycemic syndrome
The major features include:
• Aglossia - adactylia syndrome
• Multiple hamartomatous papules and
• Melkerson - Rosenthal syndrome
nodules of the skin and oral mucosa
• Burning mouth syndrome
• Together with thyroid and breast anomalies
Many syndromes associated with tongue are
and
• Polyposis of the gastrointestinal tract. characterized by macroglossia. They include the
This syndrome was named after the first following:
patient reported. Almost all patients have
The Winchester Syndrome
mucocutaneous abnormalities, typically facial
papules or nodules (usually hair follicle The Winchester syndrome clinically resembles
hamartoma with histologic features of a storage disorder but lacks the metabolic defects.
trichilemmomas). Or nodules on neck, hands Patients have joint deformities, corneal opacities,
and forearms and of the palms and soles. a coarse facies, thickened skin and macroglossia.
716 Textbook of Oral Pathology

Anhydrotic Ectodermal Dysplasia/the Christ Orofacial Digital Syndrome

- Siemes - Fouraine Syndrome
OFD type 1
It is characterized by skin anomalies, alopecia, Orofacial digital syndrome has been subdivided
dental abnormalities and in some cases by an into several disorders but the classic or type 1
enlarged tongue. OFD is of primary interest. It is inherited as an
X-linked dominant triat so that only females are
Zellweger Syndrome affected because the gene is lethal in males. The
first case was described by Papillon - Leage and
Its features are dwarfism, cardiac defects, facial
Psaume in 1954.
anomalies, cutis laxa and macroglossia.
Features of OFD type 1 are
Hajadu-Cheney Syndrome Clefts of the jaw and tongue in canine region.
Others features are syndacyly, brachydactyly
It is one form of congenital aeroosteolysis and
and polydactyly), small nostril, a lobulated
has microglossia as a feature.
tongue with hamartoma. Aberrant hyperplastic
oral frenula which appears to lead to the clefting
Beckwith-Wiedemann Syndrome
of jaws, tongue and upper lip.
Patients have an omphalocele, microglossia and
hypertrophy of various organs or even Hurler Syndrome
gigantism. Often during pregnancy hydrominon Synonym: Mucopolysaccharidosis
develops because of difficulty in swallowing Hurler syndrome is a disturbance of muco-
caused by enlarged fetal tongue. polysaccharide metabolism exhibiting a variety
Many of the metabolic storage or lysosomes of classical clinical features. It is characterized
disorders are characterized by macroglossia; the by an elevated mucopolysaccharide excretion
patients usually have so many other problems level in the urine.
that the macroglossia almost never is the
presenting complaint. Clinical Features
Included in this group are:
The disease usually becomes apparent at the age
• The mucopolysaccharidosis
of two, progresses during early childhood and
• Hurler syndrome adolescence and terminates in death usually
• Hunter syndrome before puberty.
• Sanfillippo's syndrome
• Sulfatidoses Facies—Large head with prominent forehead,
• Austin syndrome broad saddle nose and wide nostrils, thick lips
• Gangliosidoses and large tongue, open mouth and nasal
congestion. Progressive corneal clouding is
• GMI or landing syndrome
classic feature with hepatosplenomegaly. Flexion
• Cornelia de lange's syndrome II with muscle
contraction results in "claw hand". The
and tongue hyperplasia, abnormal ears and
individuals are dwarf and mentally retarded.
CNS abnormalities
• Leroy syndrome
Oral Manifestation
• Mannosidoses and
• Glycogen storage diseases mainly type II or Short and broad mandible with prominent
Pompe's disease. gonions. Delayed eruption of teeth, localized
 Syndromes of the Orofacial Region 717

areas of bone destruction in the jaws may be Treatment and Prognosis

found which appear to be representing There is no treatment for the disease but the
hyperplastic dental follicles with large pools of prognosis appears to be good, barring compli-
metachromatic material, probably mucopoly- cations.
saccharide. Gingival hyperplasia is almost
always present. Enlarged tongue is present. Aglossia Adactylia Syndrome
This syndrome in which absence of the tongue
Histopathology is associated with failure of digits to develop is
Excessive accumulation of intracellular extremely rare. It was first described by De
mucopolysaccharide in many tissues and organs Jussier.
and throughout the body. There is elevated level
Systemic Manifestations
of mucopolysaccharide in the urine.
Extremities involvement may be severe, from
Mobius Syndrome complete peromelia to absence of single digit.

Synonym: Congenital facial diplegia Oral Manifestation

Congenital facial diplegia is a nonfamilial The tongue is absent in majority of the cases. The
deficient development of cranial muscles sublingual muscular ridge may be enlarged. The
consisting of facial diplegia with bilateral mandible is usually small and poorly developed.
paralysis of the ocular muscles, particularly the
abducens. The cause of the disease is now Burning Mouth Syndrome
recognized as degeneration of the sixth and Clinical Features
seventh cranial nerve muscles although it was
Burning mouth syndrome [burning and pain of
originally thought to be a primary nuclear
the mouth or of the tongue, painful tongue.
hypoplasia with secondary muscle atrophy.
Glossodynia] without any visible changes is
common with many causes.
Clinical Features The patient complains of intense and
It is manifested in the first few days of life by unbearable pain that interferes with eating,
failure to close the eyes during sleep. Because of sleeping and virtually every other body function.
The tongue may appear as red or atrophic or the
the partial or complete facial paralysis, the infant
mucosa may appear entirely normal. The
exhibits no change in the facial expression even
complaints are usually limited to tongue but may
when crying or laughing. There is difficulty in
involve the entire mucosa.
mastication, saliva frequently drools from the Burning mouth syndrome is most common
corners of the mouth and speech is severely in middle aged women especially around the
impaired. time of menopause.
The majority of patients have other associated Neurologic disorders may also present with
congenital defects, including external mouth or tongue symptoms, most often
ophthalmoplegia, deafness, paresis of the unilateral. If the pain seems localized to one side
tongue, soft palate or jaw muscles, clubfoot, of the tongue, a microbiologic evaluation may
mental defects and epilepsy. be indicated.
718 Textbook of Oral Pathology

SYNDROMES ASSOCIATED WITH GINGIVA Ramon Syndrome

The characteristic features of this syndrome are:
• Tuberous sclerosis
- Gingival fibromatosis. Hypertrichosis,
• Zimmerman - Laband syndrome
cherubism, mental retardation and epilepsy.
• Rutherford syndrome
There is characteristic perivascular fibrosis in
• Ramon syndrome
gingival biopsies.
• Cowden syndrome.
Other associated features include juvenile
• Papillon-Lefevre syndrome
rheumatoid arthritis; the gingival fibromatosis
• Klippel Weber syndrome
usually always precedes cherubism.
• Melkersson-Rosenthal syndrome
Papillon - Lefevre Syndrome
Tuberous Sclerosis
This is an inherited disorder transmitted as an
(Described under syndromes with benign oral autosomal recessive trait. The disease usually
neoplastic and hamartomatous components). manifests during early childhood as circum-
There is gingival fibromatosis or there may be scribed erythematous hyperkeratotic plaques on
single or multiple fibromas of the gingiva, oral the palms and soles. Hyperkeratosis may extend
mucosa and skin. to the sides of hands and feet, knees and elbows.
Transverse grooves of nails occur.
Dental changes are:
Zimmerman Laband Syndrome • Gingivitis
Synonym: - Laband syndrome • Periodontitis
• Premature loss of deciduous teeth by the age
Laband syndrome (LS) is a rare disorder of 4 or 5 years.
characterized by gingival fibromatosis, • Deep periodontal abscess may be present.
abnormalities of the nose and /or ears and • Permanent teeth are also lost in the same
absence and /or hypoplasia of the nails or manner.
terminal phalanges of the hands and feet. Other
more variable features include hyper Klippel - Trenaunay - Weber Syndrome (KTW)
extensibility of joints, hepatosplenomegaly, mild KTW syndrome was reported by Klippel and
hirsuitism and mental retardation. Trenaunay in 1900. It consists of a classical triad
The first case was described by Zimmennan of clinical features and angioma formation. The
in 1928; Lanband et al described the first familial predominant features of the KTW syndrome are
occurrences in 1964. asymmetric limb hypertrophy. Cutaneous
hemangiomas and pigmenta.
Varicose veins located on the legs, abdomen
Rutherford Syndrome
and trunk. Other abnormalities include
This syndrome has autosomal dominant macrodactyly hyperpigmented nevi on skin,
inheritance -The manifestations are telangiectasia, macrocephaly and enlarged
• Congenially enlarged gingiva genitalia.
• Delayed tooth eruption
• "Curtain like" corneal opacities Oral Manifestation
Associated features are: Mental retardation Angiomatous involvement of lips, buccal
and dentigerous cysts. mucosa, tongue, palate, gingiva and oropharynx.
 Syndromes of the Orofacial Region 719

In addition, enlarged maxilla, premature The auriculotemporal syndrome is not a

eruption of teeth and marked displacement of common condition. Nevertheless the possibility
teeth on the affected side producing dental of its occurrence must always be considered.
malocclusion have also been reported.
Treatment
SYNDROMES ASSOCIATED WITH NERVES
Treatment of the auriculotemporal syndrome by
• Auriculotemporal syndrome (Frey's the intracranial division of the auriculotemporal
syndrome) nerve has been reported to be successful.
• Reader’s syndrome (Paratrigeminal
syndrome) Paratrigeminal Syndrome (Reader’s
• Horton's syndrome (cluster headache) syndrome)
• Migranous syndrome (migraine)
• Horner's syndrome The paratrigeminal syndrome is a disease
• Jaw Winking Syndrome (Marcus - Gunn characterized by severe headache or pain in the
Phenomenon). area of trigeminal distribution with signs of
ocular sympathetic paralysis. The sympathetic
Auriculotemporal Syndrome symptoms and homolateral pain in the head or
Syndrome: Frey's syndrome, Gustatory sweating eye occur without vasomotor or trophic
disturbances. These signs and symptoms usually
The auriculotemporal syndrome is an unusual
appear suddenly. The disease appears to be the
phenomenon which arises as a result of damage
most common in males, chiefly those of middle
to the auriculotemporal nerve and subsequent
age.
reinnervation of the sweat glands by
Paratrigeminal syndrome presents some of
parasympathetic salivary fibers.
the signs of the homer's syndrome but can be
Etiology differentiated from it by the presence of pain and
little or no change in sweating activity on the
The syndrome follows some surgical operation
affected side of face. The cause of this disease is
such as removal of parotid tumor or the ramus
unknown but dramatic improvement has been
of mandible or parotids of some type that has
found after elimination of dental infection.
damaged the auriculotemporal nerve. After a
considerable amount of time following surgery
during which the damaged nerve develops, Horton’s Syndrome
innervating sweat glands which then function Synonym: Sphenopalatine neuralgia, cluster
after salivary, gustatory or psychic stimulation.
headache, periodic migranous neuralgia.
Clinical Features Sphenopalatine neuralgia is a pain syndrome
The patient typically exhibits flushing and originally described by Sluder as a symptom
sweating of the involved side of the face, chiefly complex referable to the nasal ganglion. Vial
in the temporal area during eating. The profuse described a similar syndrome but believed it
sweating may be evoked by the parenteral involved the vidian nerve and concluded that
administration of pilocarpine or eliminated by the condition reported by sluder should be
the administration of atropine or a procaine block termed "vidian neuralgia". It is considered to be
of the auriculotemporal nerve. a variant of migraine.
720 Textbook of Oral Pathology

Etiology susceptible patients it may become manifest in

The suggested etiology is that it is caused by the preheadache phenomenon. Then, as part of
vasodilatation involving the internal maxillary an attempt to maintain cranial homeostasis there
artery a branch supplying the sphenopalatine is decrease in constrictor in certain other cranial
region. The theory of deviation of nasal septum arteries, particularly branches of the external
being one of the causes is also questioned. carotid. These secondary effects, possibly
harmonal as well as neurogenic in origin are the
Clinical Features source of the headache.
This syndrome is characterized by unilateral
Clinical Features
paroxysm of intense pain in the region of the
eyes, maxilla, ear and the mastoid, base of the Usually seen in second decade of life. The
nose and beneath the zygoma. frequency of attacks is extremely variable as they
Sometimes the pain extends into the occipital may occur at frequent intervals over period of
area as well. The paroxysm of pain has a rapid year or on only a few occasions during the
onset, persists for about 15 minutes to several lifetime of patient. A prodromal stage
hours and then disappears as rapidly as they (preheadache phenomena) is noted by some
began. There is no "trigger zone". The pain occurs patients consisting of lethargy and dejection
atleast once in a day regularly. This disappears several hours before the headache. Visual
after some weeks or months and the period of phenomena such as scintillation hallucinations
freedom may be for months or years. or scotomas are often described. The headache
In addition to pain sensation, other pain consists of severe pain in the temporal,
complaints may be sneezing, swelling of the frontal and retro-orbital areas although other
nasal mucosa, severe nasal discharge may be sites such as parietal, post auricular, occipital or
there after painful attacks. Also accompanied are sub occipital are also occasionally involved. The
epiphora or watering of the eyes, bloodshot eyes pain is usually unilateral but may become
and paresthesia. Men are more commonly bilateral and generalized. The pain is described
affected than women. as a deep aching, throbbing type.
Treatment Treatment and Prognosis
Cocainization of the sphenopalatine ganglion or The treatment of migraine includes a wide
alcohol injection of this structure is one of the variety of drugs ranging from acetylsalicylic acid
widely used therapies. Surgical resection of the and codeine to ergotamine, methyl salicylic and
ganglion is done in some cases. nor epinephrine.
Migraine Syndrome (Migraine) The prognosis of the disease is good, since
the condition is not dangerous, and may undergo
Migraine is a syndrome presenting manifes- complete and permanent remission.
tations of diffuse disturbances in body functions
occurring during or after stress, characterized by Horner's Syndrome
severe periodic headache, irritability and nausea.
The cause of the disease is unknown but has Synonym: Sympathetic ophthalmoplegia
been postulated to be a discharge of autonomic Horner’s syndrome is a condition characterized
centers in the forebrain leading to constriction by: miosis or contraction of the pupil of the eye
in portions of the cerebral arterial tree. In due to paresis of the dilator of the pupil. Ptosis:
 Syndromes of the Orofacial Region 721

drooping of the eyelid due to paresis of the smooth condition "Marian Amat Syndrome "or "inverted
muscle elevator of the upper eyelid. Anhidrosis Marcus-Gunn phenomenon" is usually seen after
and vasodilatation over the face due to the peripheral facial paralysis. In this condition,
interruption of psudomotor and vasomotor the eye closes automatically when the patient
control. opens his mouth forcefully and fully as in
Its chief significance lies in the fact that it chewing and tears may flow.
indicates the presence of a primary disease. The
lesions in the brain stem, chiefly tumors or SYNDROMES ASSOCIATED WITH BLOOD
infections in the cervical or high thoracic
• Plummer - Vinson syndrome
vertebrae will occasionally produce this
• Chediak - Higashi syndrome
syndrome. Preganglionc fibers in the anterior
• Sweets syndrome (Acute febrile neutrophilic
spinal Roots to the sympathetic chain in the low
Dermatosis)
cervical and high thoracic area are rather
• Lazy Leukocyte syndrome.
commonly involved by infection, trauma or
pressure as by aneurysm or tumor to produce Plummer-Vinson Syndrome
Horner’s syndrome.
Synonym: Iron deficiency anemia.
Jaw-Winking Syndrome (Marcus-Gunn The Plummer - Vinson syndrome is one
phenomenon; Pterygoid. Levators manifestation of iron- deficiency anemia and was
synkinesis) first described by Plummer in 1914 and by
Vinson in 1922 under the term "hysterical
This interesting condition consists of congenital
dysphagia" Not until 1936; however was the full
unilateral ptosis with rapid elevation of ptotic
clinical significance of this condition was
eyelid occurring on movement of the mandible
recognized. Ahlbom then defined it as a
to the contralateral side. It is commonly
predisposition for the development of carcinoma
recognized in the infant by the mother, when
in the upper alimentary tract. It is in fact one of
breast feeding her baby she notices one of its
the few known predisposing factors in oral cancer.
eyelids shoot up. As in the case reported by Smith
and Gans.
Clinical Features
The syndrome may be later in life but also is
seen to be as hereditary in some cases. Males are While an iron deficiency anemia can occur at any
more commonly affected than females. The left age Plummer - Vinson syndrome occurs chiefly
upper eyelid is more frequently involved than in women and in the fourth-fifth decade of life.
the right. It is also thought that about 2% of all Presenting symptoms of the anemia and the
cases of congenital ptosis are due to this syndrome are cracks or fissures at the corners of
condition. There are numerous theories the mouth; a lemon tinted pallor of the skin, a
concerning the etiology of this disease and these smooth red, painful tongue with the atrophy of
have been reviewed by Simpson. The most filiform and later fungiform papillae and
widely accepted is that the levators palpebral resulting from esophageal stricture of the web.
muscle is connected not only with the third Koilonychias or spoon shaped finger nails which
nucleus, but also with the external pterygoid are brittle and break easily have been reported
portion of the fifth nucleus. in many patients. Splenomegaly has also been
However there is some evidence of reported in 20 to 30 percent of the cases. The
supranuclear involvement. An interesting depletion of iron stores in the body, manifested
722 Textbook of Oral Pathology

as iron deficiency anemia may be the direct cause phobia, nystagmus and recurrent infections. The
of the mucous membrane atrophy, since the degree of albinism and the structures involved
integrity of epithelium is dependant upon are quite variable as is pigmentary dilutions of
adequate serum iron levels. The atrophy of the structures.
epithelium predisposes to the development of Recurrent infections usually involve the
carcinoma in these tissues. This relationship was respiratory tract and skin. Occasional other
first noted by Albom in the patients suffering findings include neurologic problems, a variety
from carcinoma of pharynx and upper part of of gastrointestinal disturbances, generalized
esophagus. lymphadenopathy and hepatosplenomegaly.
The disease has been sometimes associated with
Laboratory Finding the malignant lymphomas.

Blood examination reveals a hypochromic Oral Manifestations

microcytic anemia of varying degree while
Ulcerations of the oral mucosa, severe gingivitis
megaloblasts typical of pernicious anemia. The
and glossitis are the commonly described oral
red blood cell count is generally between 3, 000,
lesion.
000 and 4, 000,000 cells per cubic mm; and the
hemoglobin is invariably low. The anemia of the Laboratory Findings
iron deficiency type can be confirmed by lack of
Hematological studies show that the patients
a reticulocyte response following administration
classically exhibit giant abnormal granules in the
of vitamin B12. Serum iron is low and there is
peripheral circulating leukocytes in their marrow
an absence of free hydrochloric acid in the
precursors and in many other cells of the body
stomach. The achlorhydria is generally the cause as well. These granules are the hall mark of the
of the faulty absorption of iron since the absence syndrome and are invariably present. They are
of hydrochloric acid prevents the conversion of thought to represent abnormal lysosomes and
unabsorbable dietary ferric iron to the absorbable bear resemblance to toxic granulations and
ferrous state. The exfoliated squamous epithelial Dohle bodies. Pancytopenia is sometimes
cells also showed changes such as deficiency of presents
keratinized cells, a reduced cytoplasmic
diameter of cells with a paradoxical enlargement Treatment and Prognosis
of the nucleus, an increase in the nucleoli,
There is no specific treatment for this disease and
presence of double nucleus and karyohexis.
death usually occurs within first few years of life
as a result of secondary infection or hemorrhage.
Chediak-Higashi Syndrome
Chediak-Higashi syndrome is an uncommon Sweet's Syndrome
genetic disease which is often fatal in early life
as result of a lymphoma like terminal phase, Synonym: Acute febrile neutrophilic dermatosis
hemorrhage or infection. It is transmitted as an Sweet's syndrome was first described by Sweet
autosomal recessive trait. in 1964. He identified young women who had
sudden development of erythematous nodules
Clinical Features associated with fever and malaise. Typically the
The characteristic clinical features of this diease skin lesions consists of coleasing, plague forming
consist of Oculocutaneous albinism, photo- papules which at first sight give the illusion of
 Syndromes of the Orofacial Region 723

vesiculation but are solid on palpation. • Klippel - Trenaunay syndrome

Histopathologically the lesion show papillary • Von Hippel-Lindau syndrome
dermal edema and an intense neutrophilic • Parkes- Weber syndrome
infiltrate, but do not represent an infection or • Beckwith-Wiedemann syndrome.
leukemic infiltrate.
In majority of the cases a febrile infection of Maffucci’s Syndrome
the upper respiratory tract, tonsillitis or influenza Cutaneous hemangiomas appearing in infancy
like disease precedes the clinical symptoms by 1 are associated with hard nodules of the bones,
to 3 weeks. Twenty percent of the acute especially distal, which are enchondroma on
myelogenous leukemia patients have histology.
documented this manifestation as an early sign. Abnormal bone growth, interference with
epiphyseal cartilage and pathology fractures can
Lazy Leukocyte Syndrome
cause gross deformity which is added to by
First described by Miller, Oski and Harris in enlargement of the vascular swellings into large
1971. Lazy leukocyte syndrome is caused by loss masses.
of the chemotactic functions of the neutrophils. Maffuccis syndrome is associated with an
The bone marrow contains normal numbers of increased risk of malignancies like
mature neutrophils, but the patients have severe chondrosarcoma, fibrosarcoma, angiosarcoma,
neutropenia because the cells are unable to lymphangiosarcoma, osteosarcoma and ovarian
migrate from the marrow to the peripheral tumors.
blood.
Blue Rubber Bleb Naevus Syndrome
Clinical Manifestations
Synonym: Blans syndrome
The infections complications are apparent at age
1 to 2 years. The most common infections noted Blue-Rubber bleb nevus syndrome is seen as an
are gingivitis, stomatitis, otitis media and association of multiple cutaneous and
bronchitis. The total WBC count is in the low gastrointestinal tract hemangiomas, the skin
normal range. But the absolute neutrophil count lesions are typically bluish, rubbery, nipple like
is as low as 100/mm3 to 200 mm3. Erythrocytes compressible angioma, along with smaller blue
and platelets are normal. The diagnosis is based marks and larger cavernous hemangiomas.
on neutrophil mobilization tests showing lack Gastrointestinal bleeding, hematuria, meno-
of normal response. rrhagia and epistaxis may be the presenting
features if other visceral angioma are present.
SYNDROMES ASSOCIATED WITH Cases may be sporadic or familial with
VASCULAR MALFORMATIONS autosomal dominant inheritance

Syndromes associated with Hemangiomas Diffuse Neonatal Hemangiomatoses

• Maffucci's syndrome
This term encompasses a benign form in which
• Blue-Rubber bleb nevus syndrome
the child may have hundreds of small cutaneous
• Diffuse Neonatal Hemangiomatoses
hemangiomas without visceral involvement and
Syndromes associated with Port wine stain a disseminated form in which organ
• Sturge-Weber syndrome involvement is present. The latter group is prone
724 Textbook of Oral Pathology

to complications like hemorrhage, disseminated SYNDROMES ASSOCIATED WITH

intravascular coagulation. Cardiac failure and IMMUNODEFICIENCY
compression of vital structures. Systemic
corticosteroids are usually indicated. • Acquired immunodeficiency syndrome
• Reiter’s syndrome
Sturge-Weber Syndrome • Behcet’s syndrome.
Synonym: Encephalofacial angiomatoses Reiter’s Syndrome
A facial port-wine stain in the distribution of the The underlying cause of this disease syndrome
first branch of the trigeminal nerve is associated is unknown although there appears some genetic
with homolateral leptomeningeal angiomatosis, influence in some cases. An abnormal immune
usually over the posterior parietal and occipital response to microbial antigens is now considered
lobes of the cerebral cortex. It is postulated to be to be a likely mechanism for the multiple
a developmental defect of certain ectodermal manifestations of this disease. (Syndrome)
and mesodermal elements closely approximated The clinical features include:
in the brain and meninges at 4 to 8 weeks of Arthritis, non - gonococcal urethritis, and
gestational age. conjunctivitis or uveitis. The urethritis and
The port wine stain is present at birth and is conjunctivitis or uveitis. The urethritis generally
unilateral, but may cross the midline and also precedes the appearance of other lesions.
involve mucosae. Patients with additional Mucocutaneous lesions may be seen in up to half
involvement of in the distribution of the second of the patients.
branch of the trigeminal nerve have a high risk Orally the lesions have been described as
of ocular complications, Some patients also have relatively painless aphthous type lesions (Ulcers)
bilateral facial lesions or involvement of the occurring almost anywhere in the mouth.
trunk or extremities. Tongue lesions are like that of the geographic
Convulsions of the grand mal type generally tongue. The duration of the disease varies from
appear during the first year of life and behavioral weeks to months and recurrences are not
problems, subnormal intelligence, contralateral common. Non steroidal anti-inflammatory drugs
hemiparesis and EEG abnormalities (usually are used for the treatment of this disease.
unilateral or focal) may be seen. Ocular
involvement (40%) is in the form of ipsilateral Behcet’s Syndrome
glaucoma, congestion strabismus and loss of Exact etiology of this syndrome is unknown
vision. Characteristic S-shaped intracranial although the underlying disease mechanism
calcifications are found in the leptomeninges may be very similar to the one associated the
within the first few months of life, on CT scans aphthous ulcers. Genetic predisposition and viral
and on X-rays after the age of 2 years. They etiology has also been suggested.
increase in density until the end of second decade
when 50 to 60 % of patients show this on Clinical Features
radiography. They are thought to be due to The lesions of this syndrome typically affect the
anoxic injury from vascular stasis and increased oral cavity, the eye, and the genitalia. Recurrent
vascular permeability in the areas of the arthritis of the wrists, ankles and knees may be
angioma. seen. Cardiovascular manifestations are usually
 Syndromes of the Orofacial Region 725

of thrombotic type and neurologic manifestations is characterized by a rapidly acquired adiposity

are in the form of headaches. Oral manifestations about the upper portion of the body, moon like
of this syndrome appear identical to the ulcers of appearance of the face. A tendency to become
the aphthous stomatitis. The ulcers are usually of round shouldered and develop a "buffalo-hump"
the minor aphthous type and are found in the at the base of the neck. Other features include
typical aphthous distribution. Ocular changes are muscle weakness, vascular hypertension,
found in most of Bechetix patients. glycosuria not controlled by insulin and
Uveitis, conjunctivitis and retinitis are more albuminuria.
common inflammatory processes. Genital The oral pathologist’s primary concern with
lesions are ulcerative in nature and may be the this peculiar disease state lies in the bone changes.
cause of significant pain and discomfort. In children there may be osteoporosis and
Inflammatory bowel disease has also been premature cessation of the epiphyseal growth,
reported. while in adults there is a severe osteoporosis.
Histopathology—T lymphocytes are predomi- The pathogenesis suggested is based on the
nant. Neutrophilic infiltrates in which the cells SFN (sugar-fat-Nitrogen) hormone group of
appear within the vessel walls have also been steroids. The 'N' hormone stimulates
described. osteogenesis and closing of the epiphysis and 'S'
hormone leads to a retardation of osteoblastic
Treatment—Consist of systemic steroid therapy.
activity and reduction in matrix formation.

SYNDROMES ASSOCIATED WITH Adrenogenital Syndrome

HORMONAL DISTURBANCES
This condition results when there is hyperplasia
• Cushing syndrome of the adrenal cortex. Depending on the age at
• Adrenogenital syndrome onset and the sex of the person affected the
• General adaptation syndrome clinical signs occur. These manifestations are
• Hutchison - Gilford syndrome pseudohermaphroditism, sexual precocity and
• Waterhouse - Frederichsen syndrome. virilism in women or feminization in men. If the
disease begins early, premature eruption of the
Cushing Syndrome teeth may occur.
Cushing syndrome is a result of hormonal excess
General Adaptation Syndrome (GAS)
resulting from any of the following: Hyperplastic
adrenal cortices without any other clinically General adaptation syndrome represents the
evident endocrine lesion. signs and symptoms occurring due to prolonged
• Adrenal cortical adenoma or carcinoma "stress" as a part of individual adaptation
• Ectopically located adrenal like tumor for, e.g. mechanism. The adrenal gland and stress has a
of an ovary. close relationship. This theory was proposed by
• ACTH secreting tumor of the anterior Hans Selye.
pituitary Adrenal changes result due to prolonged
• Non-pituitary carcinoma for, e.g. of a lung stress leading to mobilization of lipids and
or pancreas. ultimate exhaustion atrophy of the cortical cells.
When this syndrome is associated with The hormones of the adrenal cortex are necessary
spontaneous bilateral adrenal hyperplasia, it is for cellular enzymes to catalyze the energy
referred to as Cushing’s disease. This syndrome producing processes of cells. All 'stresses' agents
726 Textbook of Oral Pathology

stimulate adrenal function through stimulation ageing process. Delayed eruption is also reported.
of pituitary to secrete ACTH. If excessive
amounts of hormones are produced, eventually Water House - Frederichsen Syndrome
pathologic changes occur in those tissues which Acute adrenal cortical insufficiency is relatively
respond to the stimulation and the diseases of rare and it occurs in connection with Water
adaptation, (hypertension, periarteritis nodosa House-Frederickson syndrome. This disease
and others) result. primarily occurs in children but also occurs in
The stages are: adults. It is characterized by a rapidly
• Ist "Alarm reaction" which consists of a shock fulminating septic course, a pronounced purpose
phase and then a counter shock phase and death within 48 to 72 hours - Meningococi,
• IInd "Adaptation stage" in which a person’s streptococci and pneumococci are the organisms
resistance to original stressor is greater but most responsible for the disease. At autopsy, the
his resistance to other stressor agents is conspicuous change is bilateral adrenal
lowered hemorrhage.
• IIIrd - If the stressor is continued. He The use of antibiotics and cortisone has
eventually enters a stage of exhaustion and changed the course of the disease from its usual
dies. fatal termination to recovery in some cases.
If the stressor is removed, he enters a stage
of convalescence and recovers. SYNDROMES WITH BENIGN ORAL
NEOPLASTIC OR HAMARTOMATOUS
Progeria (Hutchinson - Gilford syndrome) COMPONENTS
Progeria is a very rare disease originally • Von Recklinghausen’s neurofibromatosis
described by Hutchison in 1886. It is of unknown • Nevoid basal cell carcinoma syndrome.
etiology and is characterized by dwarfism and • Multiple mucosal neuroma syndrome
premature senility. It is thought to be transmitted (multiple endocrine neoplasia type III)
as an autosomal recessive trait. • Tuberous sclerosis
• Acanthosis nigricans
Clinical Features • Albrights syndrome.
Affected infants appear normal at birth, but the
typical clinical features become manifest within Von Recklinghausen’s Neurofibromatosis
the first few years. The patients all have amazing Two distinct varieties of this classic syndrome
resemblance to each other, exhibiting alopecia, are now recognized:
pigmented areas of the trunk, atrophic veins and Neurofibromatosis 1: which is often
loss of subcutaneous fat. The individuals have a associated with oral lesions.
squeaky voice, a beak nose and a hypoplastic Neurofibromatosis 2: (bilateral acoustic
mandible. The intelligence these individuals is neurofibromatosis) which is caused by a gene
either normal or above normal. Even at an early on a different chromosome is much less
age person resembles a wizened little old person. common, and while often accompanied by other
central nervous system tumors is less frequently
Oral Manifestations associated with obvious peripheral neuro-
As described by Gardner, there is accelerated fibromatosis or oral lesions.
formation of irregular secondary dentin, Neurofibromatosis 1 is inherited as an
apparently a manifestation of the premature autosomal dominant condition. But only half the
 Syndromes of the Orofacial Region 727

cases exhibit a family history. The syndrome is Carcinoma Syndrome

characterized by the simultaneous occurrence Synonym: Gorlin - Goltz syndrome, Jaw-cyst-Bifid
usually on the trunk, axilla and pelvic area of rib syndrome.
light brown pigmentation (cafeaulait spot, light This syndrome was first described by Binkely
brown macules with a smooth outline "like the and Johansson in 1951. This has been thoroughly
coast of California"). The finding of six or more reviewed by Gorlin and his co-workers.
macules with a diameter of 1.5 cm or greater is
diagnostic of neurofibromatosis. Clinical Features
Cafeaulait spots are also found in 10 % of the This syndrome has the following variable
normal population, especially in fair skinned manifestations:
persons. Similar skin lesions with the same name • Cutaneous anomalies: Basal cell carcinoma,
occur in Albright’s syndrome. other benign cyst and tumors, palmar pitting,
Other findings are axillary freckling (Crowe's palmar and plantar keratosis and dermal
sign) and a wide variety of nerve and nerve calcinosis
sheath tumors in both central and peripheral • Dental Osseous anomalies: Multiple
nervous systems. odontogenic keratocyst, jaw prognathism, rib
The peripheral lesions are often anomalies (often bifid) and vertebral
indistinguishable from those called as anomalies.
neurilemmoma (tumors of the nerve sheath: • Ophthalmologic abnormalities: Hypertelorism
schwannoma). Both neurofibroma and with wide nasal bridges, congenital blindness
neurilemmoma are encapsulated S-100 positive and strabismus
tumors that show patterns of whorled connective • Neurologic anomalies: Mental retardation,
tissue elements histologically with readily dural calcification, agenesis of corpus
recognizable axons with or without myelin callosum.
sheath. • Sexual abnormalities: Hypogonadism in males
The central lesions because of their location and ovarian tumors.
within a bony cavity often in association with • Oral manifestations: The keratocyst may
various nerve roots lead to neurologic symptoms, displace the developing teeth and result in
mental retardation and vertebral anomalies large their deformity.
infiltrating lesions that occur both peripherally
and centrally and lead to severe deformity and Multiple Endocrine Neoplasia Syndromes
are referred to as 'plexiform neuroma'. (MEN Syndromes)
Pheochromo cystomas (tumors of the adrenal This is a group of syndromes characterized by
medulla and paraganglia). tumors of various endocrine organs occurring
Approximately 5% of the patients with neu- in association with a variety of other pathologic
rofibromatosis have well developed oral lesions features. Steiner and his associates have
and macroglossia. The tongue being the most classified these syndromes into:
common oral location for neurofibroma both in MEN I - TYPE I and
this syndrome and in solitary oral neurofi- MEN II - TYPE II
broma. Khairi and associates have decribed type III
(MEN III) other workers have subdivided type II
Basal Cell Nevus or Nevoid Basal Cell into II a (synonymous with original type II) and II
728 Textbook of Oral Pathology

b (synonymous with type III). Red brown macules and papules are seen about
the mouth in the nasolabial folds. CNS findings
Clinical Features include epilepsy mental retardation.
MEN I : Consists of tumors of hyperplasia’s of the
pituitary, parathyroid, adrenal cortex and the Oral Features
pancreatic islets occurring in association with The oral cavity frequently shows distinctive
peptic ulcers and gastric hypersecretion.
changes. Angiofibroma, fibromas or papillomas
MEN II: (Sipples syndrome, II a) is characterized are found on the gingiva, hard and soft palate,
by parathyroid hyperplasia or adenoma but no buccal mucosa and tongue. They may be white
tumors of pancreas. However in addition these or yellow although they lack any distinctive tint.
patients have pheochromocytomas of the Both dentitions have tiny pits arising from
adrenal medulla and medullary carcinoma of the enamel defects. The pits are often an early
thyroid gland. There is no peptic ulcer. diagnostic clue. A high palate, macroglossia, cleft
MEN III: (II B) it is characterized by lip and palate and hemangiomas have been
mucocutaneous neuroma, pheochromocytomas described.
of the adrenal medulla, medullary carcinoma of
the thyroid. Other abnormalities include Acanthosis Nigricans
hypertrophied corneal nerves, other skeletal
defects and gastrointestinal difficulties, oral Acanthosis nigricans (AN) is a misnomer.
manifestations; Patients develop velvety dark patches
particularly in flexural areas, but also in the
MEN III: Is particularly having the constant mouth. There is papillomatosis and
component of multiple oral neuroma. The
hyperkeratosis but no acanthosis and no increase
neuroma are most common on the lips tongue
in pigment, it appears that the deranged
and buccal mucosa. They produce "Bumpy Lips"
epithelial growth is directed through epidermal
since the neuroma present at birth or may
growth factor receptors. Several type of AN can
develop later appear as small elevated sessile
be identified.
nodules on the vermilion producing puffy lips.
• Tumor-related: In many cases, when tumor is
On the tongue they are commonly present on
removed AN disappears.
the anterior third.
• Endocrine: In a variety of endocrine
syndromes AN is a frequent finding
Tuberous Sclerosis • Juvenile or familial AN: Reported in some
Synonyms: Pringle-Bourneville syndrome families
adenoma sebaceum. • Drug induced: A variety of drug, primarily
Tuberous sclerosis (TS) is an autosomal nicotinic acid can produce presumably by
dominant disorder with marked variability of acting on growth factor receptors.
expression in a given family.
Oral Features
Clinical Features The perioral lesions may be velvety brown
The key skin findings are hypopigmented patches particularly at the corners of the mouth.
macules (ash-leaf macules) connective tissue Multiple papillomatous lesions can be found on
nevi, (shagreen patch) and multiple angiofibroma. the lips, especially at the commissures. The
 Syndromes of the Orofacial Region 729

tongue shows elongated filiform papillae, deeper Von Hippel-Lindau Syndrome

folds and may appear enlarged. The buccal
An autosomal dominant condition is classically
mucosa may be also thickened and show
present as bilateral retinal angiomatosis leading
increased folds. There may also be gingival
to visceral impairment and blindness and
hyperplasia. In contrast to skin lesions, the oral
cerebellar or medullary hemangioblastoma.
lesions are usually not pigmented. The
Renal angioma and/or renal cell carcinoma,
papillomatous lip changes resembling angular
hepatic angioma and phaeochromocytoma may
cheilitis are limited almost exclusively to tumor,
occur. Port wine stains and café au lait spots are
- related AN. The course of the disease is chronic
seen in 5 percent of patients.
and there is no satisfactory treatment for this
disease.
Parkes -Weber Syndrome
Klippel-Trenaunay Syndrome Arteriovenous fistulas are seen with features of
the klippel-Trenaunay syndrome, viz. Port wine
Classically, a port wine stain usually of the lower stain, venous varicosities and limb hypertrophy.
limb is associated with limb hypertrophy, They may be suspected by a thrill or continuous
lymphoedema, venous varicosities and bruit at the involved site. Their diagnosis should
obstructed, absent or deeper vessels. be confirmed by arteriography for large fistulae
The cutaneous lesions be angioma or or by diagnostic methods using isotopes.
angiokeratomas. The bone and soft tissue
hypertrophy may be both linear (almost 100% Beckwith -Wiedemann Syndrome
of patients) and circumferential (75%) and Synonym: Exophthalmos - macroglossia -
develops gradually. Varicose veins are present gigantism syndrome (EMG syndrome).
which may be obscured clinically by edema. A facial portwine stain, macroglossia, visceral
hyperplasia and hemihypertrophy are
components of this syndrome.
730 Textbook of Oral Pathology

CHAPTER
Forensic
37 Dentistry

INTRODUCTION presentation of dental evidence in both civil

and criminal legal procedures.
Forensic odontology is the subject concerned with • Identification—Personal identification, either
the application of medical and paramedical individually or in context of mass disasters.
science knowledge to certain branches of law, • Age assessment—To calculate the age of
both civil and criminal. The medico-legal patient.
information obtained from the examination of • Bite mark investigation—Investigation of
teeth and jaws falls in the preview of forensic
criminal cases where bite marks are involved
odontology. Upon exposure to physical injury
and the interpretation of bite marks.
and putrefaction, the human dentition, the
• Human abuse—Recognition of domestic, and
enamel of which is the hardest substance in the
child abuse.
body outlasts all other tissues. The material used
in restoration is also extremely resistant to • Lip print—Comparison and identification of
destruction by chemical and physical elements. lip print.
The fundamental principles of dental • Legal aspect—Legal aspect of dental
identification are those of comparison (when traumatology.
ante-mortem records of the proposed deceased
are available) and exclusion (when ante-mortem Record Management
records of other persons are available). In the
The dental record is a legal document of dentist
absence of the ante-mortem records attempt to
which contains information about subjective and
elicit dental information is made by interrogating
objective finding of the patient. It also includes
the relatives and friends of the deceased, which
may frequently prove unreliable. pathological report, radiographs, and clinical
Forensic odontology may be defined as the photographs of the patient.
application of dental science to the Treatment plan which is given to the patient
administration of the law and the furtherance of should be updated in the record. All the letter of
justice. It involves:- reference, letter of consent, insurance and
• Record preparation—The correct handling and financial statement should be stored in the record
examination and the proper preparation and of the patient.
 Forensic Dentistry 731

The progress note of the patient should contain • The availability of documentation of the ante-
information about the restorative and therapeutic mortem status of the dentition in the form of
procedure which are carried out on to the patient. dental treatment records and diagnostic
Summaries of the telephonic conversation radiographs.
with the patient, consultant, insurance company Each individual has 32 teeth with 5 surfaces
representative and legal authorities should be each with their own character of size, shape,
maintained in the record. position and spacing with the result that no 2
Record should be signed by the personnel. sets of teeth are alike. Teeth extracted after death
Any change made in the record should not be leave a completely different socket from those
erased but a line should be crossed on it, so that removed during life. When the tooth is removed
it is readable. This will help to remove any fraud or dental work of any sort is carried out the teeth
intention to alter record. pattern is changed and its record may exist with
It is common nowadays that dental record the dentist. In natural decomposition teeth are
to be maintained electronically. Nowaday some practically indestructible. They are not easily
software programs are developed to maintain destroyed by fire. Being sheltered in the oral
patient dental information. This is advantageous cavity they are generally not damaged. Teeth as
as it can be easily networked and transferred. well as dentures made of acrylic resin are
Record should be kept minimum of 7 to 10 generally resistant to the action of corrosive
years. In the case pediatric patient record should acids. The identification from data of
be maintained until the patient reaches the age authenticated teeth depends entirely upon the
to maturity. accuracy and completeness of authenticated
records made during life. The record should
include.
IDENTIFICATION
• The number and situation of teeth present
Forensic odontology is concerned with the and number and situation of teeth lost.
identification of both living and deceased person. • Arrangement, irregularities, erosion, caries,
fillings, bridge crown work and dentures.
Role of Teeth in Establishing the Identity • Exact shape of edentulous arch.
• Some of the common identifying features of
Dental Comparison teeth pertain to faulty development, faulty
alignment, presence of stains, localized wear
Dental comparison affords a potentially
on certain teeth and missing teeth.
straightforward and simple means of
establishing identity. The method of dental Faulty development—Teeth may be undersized,
identification depends upon:- oversized, notched or present some other
• The relative resistance of the mineralized irregularity as a result of faulty development and
dental tissues and dental restoration to malformation. Hutchinson’s teeth constitute a
changes resulting from decomposition or classical example of malformation of the incisor
harsh environment extremes such as in congenital syphilis. These changes are most
conditions of temperature and violent conspicuous in central incisors which are usually
physical forces. small, widely spaced, notched and less broad at
• The unique individual characteristic of the the cutting edge than at the gum margin giving
dentition and dental restoration. them the appearance of tip of screw driver.
732 Textbook of Oral Pathology

Faulty alignment—The defect in the alignment may socket leaving a slight depression and the socket
be in the space between teeth, e.g. widely spaced outline is not visible on X-ray examination. In
teeth or overriding teeth. Between the teeth of the recently recovered remains postmortem tooth loss
upper and lower jaw when there is protrusion of discloses a clean socket devoid of blood clot. In
upper incisors resulting in overlap of lateral skeleton in which postmortem loss of teeth is
incisors the bite pattern is known as overbite and common, the bony rim of alveolus is sharp and
the reverse pattern is known as cross-bite. feathered.

Stains—Pan (betel leaf, tobacco) chewing habit

Procedures in Dental Identification
stains the teeth with dark brown or black deposits.
Yellowish or dark brown stains on the back of It should include not only the oral cavity of the
incisor teeth is common in cigarette smokers. victim but when applicable, the surrounding
Chalky white or yellowish brown areas of scène as well, especially in case of conflagration
discoloration are found in fluorosis. Metal or injuries when only the remnants of the dental
poisoning may cause pigmentation of gums and arches may remain scattered and rubble and
thereby suggest a cause of death. Copper causes debris. Dental examination in mass disaster
green and mercury and lead a blue black line on victims often by necessity must be accomplished
the gums. Gum hyperplasia induced by under accident site and/or temporary mortuary.
phenytoin may aid in identification and suggest
Recovery of dental structure—The task of dental
epileptic seizure as a cause of death.
identification begins at the site of discovery of the
Localized wear on certain teeth—A pipe smoker may body. When the gross postmortem changes
have localized wear of teeth either on incisors or affecting the teeth have occurred, such as charring,
at angle of mouth due to position of pipe. Notched disintegration and fragmentation in fires and high
incisors from holding thread, pins, nail, between impact accident, meticulous care in their recovery
teeth on day to day basis may suggest the and conveyance to the mortuary are of most
occupation of tailor or hairdresser or cobbler. important.
Missing teeth—The missing tooth may have been Displaced teeth in decomposed bodies or
lost ante- mortem or postmortem. Antemortem loss skeletal remains should be saved, labeled and
of teeth due to trauma at or near the time of death later secured to the intraoral position using
if frequently associated with fracture of thin bony adhesive cement. Most open alveoli are the result
plate surrounding the alveolus. In loose tooth of postmortem tooth loss. In recently recovered
which has fallen out it is not so. Extraction or remains postmortem tooth loss disclose a clean
tooth loss in living person is followed by bleeding socket devoid of blood clots.
from its socket which stops in about 24 hrs or Instrument—Instruments used for dental
sometimes 2 to 3 days, when the clot forms in the examination includes explorer, mirror, tissue
raw socket. By about 14 days, the clot is forceps, heavy duty autopsy scalpel, handle and
obliterated by fibrous tissue and the alveolar rim blades, tissue clamps, irrigating syringe, rubber
is smoothened by resorption of bone. By about 5 autopsy gloves, polythene specimen bags, gauze
to 6 months, gradual new bone formation fills the sponges (for tooth cleansing) and a source of
socket but its outline is still visible on X-ray illumination (flash light or battery operated head
examination. By about 6 months to 1 year lamps); and dental examination form should be
remodeling of new bone completely obliterates the used.
 Forensic Dentistry 733

Reconstruction and examination—Examination • In high impact accidents such as aircraft and

should be performed by two persons thoroughly high speed road crashes much mechanical
familiar with dental terminology, one actually damage can occur and teeth and jaws may
performing the examination and one recording fracture and disintegrate.
the data. The recorder should view the actual • Failure to recover significant material may
teeth in order to record the basic morphological result in failure to identify a body.
pattern of the restoration or cavities. In some cases,
Errors in examination—Errors can easily be made
it is necessary to remove the jaw from the body for
in the examination and recording of the
more detailed examination and future reference.
postmortem dental material.
Transcription of dental records—The point to be
Inadequate ante-mortem data—Errors in charting
considered while charting are missing teeth,
teeth treated and insufficient descriptive details
unerupted teeth, supernumerary teeth,
about the treatment provided are common. Other
restoration, prosthesis, dentures, decayed, broken
difficulty arises when a dentist has retired and
teeth, malposition, overlapping, crowding and
destroyed his records.
spacing, peculiar shape of teeth.
Technique for Identification in Mass Disaster
Identification of Edentulous Bodies
These include X-ray, UV light, postmortem
Frequently dentures are present in the mouth of
serology and DNA profiling.
unknown bodies or may be found elsewhere. If
the denture can be identified and it can be shown
X-ray
to fit the mouth of the deceased, a reliable
identification can be made. The most reliable All bodies which are found under suspicious
means of identification of denture is for them to circumstances and which are rendered
be permanently marked with the name of the unrecognizable due to prolonged immersion in
patient or some code during manufacture. water, burning by fire and acid or by any other
destructive means such as explosion should be
Problems in Identification routinely radiographed. A dental radiograph
when available constitutes one of the most
Condition of material recovered
valuable pieces of evidence for identification
• It depends upon the circumstances
purpose. Panoramic X-ray technique provides
surrounding the death and the care exists in
excellent pictorial dental record.
its collection and transport.
The CAPMI (computer assisted postmortem
• Incineration produces damage to teeth ranging
identification) system compares dental record of
from mild scorching of the surface to severe
victims of mass disaster and enables rapid
charring of the enamel and dentin with
identification of air crash, flood and explosion
crumbling of the crown
victims. Radiography can provide information
• Sustained very high temperature will result
in relation to age, sex, race, and occupation,
in calcinations of the teeth with considerable
diagnosis of certain conditions and identification
overall shrinkage.
and cause of death.
• Burnt teeth are usually very fragile and suffer
separation of the enamel and often gross Age—Age can be established by radiography of
disintegration of the crowns. bones and teeth (for root calcification).
734 Textbook of Oral Pathology

Calcification of costal cartilage and osteo-arthritic Even in putrefied bodies, blood group antigen may
changes in large joints and the spine also help. be detectable for serological studies. The bone
marrow in skeletal remains may still retain
Sex and race—May be deduced by radiography in serologically detectable antigens.
some cases.
Occupation—This can sometimes be deduced from DNA Profiling
X-ray. The whole range of pulmonary This is useful if suitable tissue (blood, semen
occupational diseases such as silicosis, asbestosis stored in bank) is available. If such tissue is
may show specific radiographic findings. The available a DNA profiling or autopsy derived
radial artery in laborer’s using pneumatic drill tissue should be compared by single probe
may show calcification; coal carriers and analysis with that of parent, children, sibling and
professional wrestlers are liable to calcified if be necessary other relatives. This is now used
lesions of the ligamentum nuchae. Football worldwide in aircraft and other major accidents.
players may show calcified hematoma of the thigh The techniques which are used for DNA
profiling are Restriction fragment length
muscle.
polymorphism (RFLP) and polymerase chain
Identification—It is possible by comparison of reaction (PCR). RFLP results in splitting source
postmortem and ante-mortem X-ray. DNA into thousand of fragment. PCR can do
evaluation of denture DNA or minute quantity
Cause of death—Fracture of bones seen on X-ray
of DNA.
may indicate their ante-mortem origin and these
include depressed fracture of skull, fracture of AGE ASSESSMENT
hyoid, fracture dislocation of cervical vertebrae,
severe injury to bones by cutting instrument or Chronological age assessment may be an
fracture of several ribs which are incompatible important factor in establishing the identity of
with life. Foreign bodies in the upper respiratory the living or deceased person. It is also important
tract provide valuable clue. Evidence of poisoning in legal proceedings when specific charge for
by heavy metals and signs of diseases such as particular offence may depend on whether the
alleged offender is a juvenile.
malignant growth may be apparent.
Visual observation—Stage of eruption of the teeth
UV Rays and evidence of changes due to function such as
attrition can give an approximate estimate of age.
An ultraviolet lamp can be used to locate and
define tattoo marks and scars on burned and Radiography—It can provide a great detail, the
decomposed remains, and to segregate bones in gross stage of dental development of the dentition.
cases of mix-up. When examined by UV light Histological—It requires preparation of the tissue
washed blood stains are readily seen and seminal for detailed microscopic examination, which can
stains give a bluish white fluorescence. determine more accurately the stage of
development of the dentition.
Postmortem Serology
Physical and chemical analysis—It is done to
A known postmortem grouping of an individual determine alterations in ion levels with age have
serves to narrow the range of possible identities. been proposed.
 Forensic Dentistry 735

BITE MARKS

One of the two major interests of the forensic

odontologist is one that has direct relevance to
the pathologist, in that it concerns the
interpretation of trauma to the body surface.
Dental evidence is used to identify the
perpetrators of a crime who happened to have
left their teeth marks in some substances left at
the scene. Generally, it is easier to exclude a
certain person than to identify one conclusively
from the bite mark.

Definition Figure 37.2: Bite marks on inmate subject

A bite mark is a patterned injury produced by teeth

Causes
on animate (Fig. 37.1) or inanimate (Fig. 37.2)
objects, is caused by small enamel defects on the • Child abuse—It can be found anywhere on the
incisal edge of incisor teeth creating individual body, favorite sites being the arms, hands,
characteristics during biting procedures. It can be: shoulders, cheeks, buttocks and trunk. Most
• Tooth mark—Mark left by a tooth (human or of the time, it is inflicted by the mother.
non-human). • Sexual assaults—It usually occurs in cases of
• Arch mark—Mark produced by four or five rape. Most common site of it is breast and
adjacent teeth in the same arch. nipples but neck, shoulders, thighs, abdomen,
pubis and even vulva may be attacked.
• It may also be inflicted on police officers when
attempting to arrest resisting offenders.
• Sports—Bites can occur in sporting events
especially football and some forms of
wrestling. In this, it can occur anywhere but
hands, fingers, nose, forearms, ears and even
lips may be target.
• Self-inflicted—Falls onto the face or a fit may
cause the tongue and lips to be badly bitten.
Some persons deliberately bite themselves,
sometime to fabricate injuries for a variety of
motives ranging from gain to psychiatric
disorders.

Importance of Bite Marks

It can permit precise identification because the
alignment of teeth is peculiar to each individual
(Figs 37.3 and 37.4). The mark may be on the article
Figure 37.1: Bite marks seen on animate subjects of food found at the scene of crime or on human
736 Textbook of Oral Pathology

Method of Preservation of Bite Marks

The doctor should be asked how the bite mark
may be best preserved. When the substance is
plastic such as butter, cheese, lard, or chocolate,
it should be stored in a refrigerator to prevent
melting or gradual flowing. It should not be deep
frozen as this may cause brittleness and cracking.
Fruits should be preserved in Campden solution,
a metabisulphite fluid used for fruit bottling. If it
is not there then 5% acetic acid in 40% aqueous
formaldehyde solution can be used.
Figure 37.3: Human dentition consists of 32 teeth with
5 surface Whatever preservation is recommended, the
object should be adequately photographed with
film plane at right angles to the bite and a scale
placed in the focal plane. Death freezes a bite mark
but a subdermal hemorrhage in living person
disappears within 20 minutes hence it is important
to take an immediate photograph of the bite mark.

Classification of Bite Marks

• Nonhuman (animals)
• Human (Fig. 37.1)
• In foodstuffs (in a part eaten foodstuff
abandoned by offenders at the scene of crime)
(Fig. 37.2)
• On nonbiological object (on pencils, pipe
stems and detonators)
• In human skin
Noncriminal (erotic bite).
Figure 37.4: Bite mark is represented distorted
image of human dentition Criminal—It can be offensive (upon victim by
being to realize that a bite on human flesh can assailant) and defensive (upon assailant by victim).
have marks not necessarily due to break in the
Bite Marks on Foodstuff
continuity of the skin but due to a small subdermal
or thin deep hemorrhage. Foodstuff bite marks can be classified into
Bite marks are always contaminated by saliva 3 types:–
and therefore contain amylase, ptyalin and blood • Type I—Bites that are found in material such
group which can be determined by the cast of as chocolate, which fracture readily with
suspect of mouth is made and transparency of limited depth of tooth penetration.
his bite compared to that of the unknown bite. It • Type II—Bites formed in foods where teeth
should be remembered that bite marks on skin obtain a good grip and then bitten piece is
are modified by its elasticity when the teeth are removed by fracturing it from main material,
withdrawn. e.g. apples.
 Forensic Dentistry 737

• Type III—Bites in which teeth bite right

through the bitten material, e.g. cheese. These
bite exhibits extensive scrape marks and may
give an indication of the relative position of
upper and lower incisor teeth in centric
occlusion.
Depth of penetration of the teeth—Where there is
only minimum penetration of teeth into the food,
the record is of biting edges and where there is
greater penetration the record is of the labial
aspect of the teeth.
Time and temperature—Bite mark in foodstuff may
produce the exact mesial-distal dimension of the
teeth provided the record is taken immediately
after the bite was made. Dimensional and color
changes are expected to occur in the foodstuffs
due to effect of temperature.
Figure 37.5: Assault bite mark
Bite Marks in Human Skin
Human tissue has been described as one of the
least dependable substances for the registration
of bite marks as the bite marks in the tissue is
affected by several variables.

Status of the Tissue

Site—Type of tissue/condition of skin. For
example, loose skin or excessive subcutaneous
fat commonly demonstrate easy and extensive
brushing leading to a poor bite mark definition.
Areas of fibrous tissue or high muscle content
tend to bruise less easily and thus are more likely
to demonstrate a bite mark. Figure 37.6: Sexual bite mark
Age—Infants and elderly individual tend to
bruise more easily than other age groups marking The Influencing Factor
the detection of bite marks more difficult.
Bite mark can be influenced by time elapsed
Sex—Females tend to bruise easily than males. between the actual biting and when the
Once produced a bite mark will be evident for a impression is made. Depression of the skin as
longer period of time on females as compared to occurs in most bite marks will recover within 10-
males. 20 minutes after the bite, although discoloration
In human, bite mark can be sexual or assault and swelling may be present 24-72 hours on a
type (Figs. 37.5 and 37.6). living object.
738 Textbook of Oral Pathology

The force exerted—The skin appearance of bite • If the contour of the part bitten is irregular or
mark may vary from bruising, abrasion, and markedly curved, then only part of dental arch
indentation to actual lacerations. is in contact with the tissues.
• If the bite is forcible, then extensive
The number of teeth—In general, the more teeth
subcutaneous bruising may spread and blur
marks present in the bite marks, the better is the
the outline.
likehood of identification utilizing that bite mark.
• If the bite is inflicted many days before, then
The type of teeth—In many cases, the bite mark is healing of abrasion and lacerations and
seen to be comprised of the upper anterior teeth. absorption of bruising will leave progressively
The investigator should take note of the width of less detail.
teeth. • When the teeth are forcibly applied the typical
appearance is of two ‘bows’ with their
The reaction of the surrounding tissue – Bite marks
concavities facing each other and a gap at
made hours or days before the event will show
each end.
inflammatory changes and signs of healing
• Love bites—they are caused by firm application
microscopically.
of the lips, which form an airtight seal against
the skin, and then sucking action reduces the
Types of Bite Mark
air pressure over the center. This causes
• Definite bite mark shower petechial hemorrhage to appear from
• Amorous bite mark rupture of small venules in the superficial
• Moderately aggressive bite mark layer of the subcutaneous tissue. If forcible the
• Aggressive bite mark petechiae are confluent and a frank bruise or
• Very aggressive bite mark. even hematoma develops.

The Nature of Bite Marks Investigations of a Bite Mark (Figs. 37.7 to 37.13)

Though called bite marks, but some times it may Firstly, the bite mark should be carefully and fully
not be from actual teeth. The lips can transiently photographed. The photograph should be taken
mark the skin, if forcibly nipped, especially on from different angles, but especially from directly
children. Suction can produce a crop of punctate perpendicular viewpoint, with the plane of film
hemorrhages, either small petechiae or larger at right angle to that of the lesions. An accurate
ecchymoses merging into a confluent central scale should always be held near the lesion, as
bruise. It is caused by front teeth from canine to close as possible. The lesion should be almost fill
canine with a gap at either side representing the the camera frame in some shots to capture as much
separation of upper and lower jaw. detail as possible. When photography is
A human bite is near circular or a shallow completed, swabs of the bite should be taken to
oval. A deep parabolic arch or ‘U-shaped’ can try to recover saliva. Plane cotton-wool swab are
only be animal in origin. The teeth may cause clear, gently rubbed onto the bite. They should be then
separate marks or they may run into each other to deep frozen unless send straight to the serology
form a continuous or intermittently broken line. laboratory.
As the time progresses, original teeth marks After this, impression of the bite can be taken.
spread out and blur. Teeth marks may be abrasion, It is done by pouring a plastic substance over the
bruises or laceration or a combination of any two bite mark, which then hardens, so as to produce
of three. The clarity of bite marks depends on a negative cast of the lesion. It is usually made with
number of factors: a rubber-or silicone based medium containing
 Forensic Dentistry 739

Figure 37.7: Sample bite taken on apple Figure 37.10: Bite mark of suspect is taken

Figure 37.8: Impression is taken of the bite and cast is Figure 37.11: Positive replica of bite mark is made
made

Figure 37.9: Study case is made Figure 37.12: Cast of suspect is made
740 Textbook of Oral Pathology

abnormalities of the teeth. Photographs of

dentition of the patient can be taken.
After that impression of bite of suspect should
be taken. Tracing can be made from the positive
cast of a bite impression, inking the cutting edge
of front teeth and transferring these to transparent
sheets, which can then be laid over the photograph
to determine correspondence.

CLASSIFICATION OF SKIN WOUNDS

Abrasion—It is superficial type of injury only skin

part is involved, i.e. both epidermis and dermis. It
heals without the scar formation. It is of following
Figure 37.13: Cast is matched with previous mark types.
• Graze—Abrasion caused by friction between
skin and hard surface.
catalytic hardeners. Less satisfactory substances
• Scratch—It is caused by pointed ends of nails,
are water based pastes, such as plaster of Paris,
pins, needles etc. It is localized linear abrasion.
which are poured when wet and allowed to dry
• Imprint abrasion or pattern abrasion—It gives the
before removal. But they have a disadvantage of
pattern of object on the skin. For example, teeth
potential damage to the actual bite. After autopsy,
bite.
it is also possible for the whole area of the skin
carrying the bite to be removed and preserved in Bruise or contusion—Subdermal-extravasation of
formalin for future examination. blood from rupture of subcutaneous vessels and
capillaries of skin and organs.
Matching the Bite Mark with the Suspect
Laceration—Wound must be produced with a
Dentition
blunt object that penetrates the skin to
The teeth of those who are either suspected by the subcutaneous tissue.
police or who had access to the victims should be
Incised wound—Must be caused by sharp edged
examined. In most jurisdictions, it is vital that fully
object like knife. In incised wound length is more
informed consent should be obtained from the
as compared to width and depth.
persons beforehand.
Any refusal must be a bar to any further action. Stab wound—It is caused by pointed end of sharp
When children are concerned, usually in the edged or blunt object. Depth is more as compared
setting of child abuse, the consent of fully to width and length.
informed parents or guardian must be obtained. Contusion and abrasion are described in two
Then dentition is examined for number of teeth, dimensions, i.e. length and width.
missing teeth, complete or partial denture, Laceration, incised wound and stab wound
occlusion, broken teeth, irregular teeth and described in three dimensions.
 Appendix

I Classifications

I. DEVELOPMENTAL DISTURBANCES OF ORAL AND • Ankyloglossia

PARAORAL STRUCTURES • Cleft tongue
• Fissure tongue
Developmental Disturbances of Jaws • Median rhomboid glossitis
• Benign migratory glossitis
• Agnathia
• Hairy tongue
• Micrognathia
• Lingual varices
• Macrognathia
• Lingual thyroid nodule.
• Facial hemihypertrophy
• Facial hemiatrophy
• Abnormalities of dental arch relations. Developmental Disturbances of Oral Lymphoid
Tissue
Developmental Disturbances of Lips and Palate • Reactive lymphoid aggregates
• Congenital lips and commissural pits and fistula • Lymphoid hamartoma
• Double lip • Angiolymphoid hyperplasia with eosinophils
• Cleft lip and palate • Lymphoepithelial cyst.
• Cheilitis glandularis
• Chelitis granulomatosa Developmental Disturbances of Salivary Glands
• Hereditary intestinal polyposis syndrome
• Aplasia
• Labial and oral melanotic macule.
• Xerostomia
• Hyperplasia of palatal gland
Developmental Disturbances of Oral Mucosa • Atresia
• Fordyces granules • Aberrancy
• Focal epithelial hyperplasia (Heck’s disease). • Developmental mandibular salivary gland
depression.
Developmental Disturbances of Gingiva
Developmental Disturbances of Size of Teeth
• Fibromatosis gingivae
• Retrocuspid papilla. • Microdontia
• Macrodontia.
Developmental Disturbances of the Tongue
Development Disturbances in Shape of Teeth
• Microglossia
• Macroglossia • Gemination
744 Textbook of Oral Pathology

• Fusion Alteration in shape

• Concrescence
• Gemination
• Dilaceration
• Fusion
• Talon’s cusp
• Dilacerations
• Dense in dente
• Dens invaginatus
• Dense evaginatus
• Dens evaginatus
• Taurodontism
• Taurodontism
• Supernumerary roots.
• Concrescence
• Supernumerary roots
Developmental Disturbances in Number of Teeth • Enamel pearls
• Oligodontia • Attrition, abrasion, erosion.
• Anodontia
• Supernumerary teeth Alteration in Number
• Predeciduous dentition
• Anodontia
• Post permanent dentition.
– total
– partial
Developmental Disturbances in Structure of Teeth • Impaction of teeth
• Amelogenesis imperfecta • Supernumerary teeth
• Dentinogenesis imperfecta
• Dentin dysplasia Defects of Enamel
• Regional odontodysplasia
• Environmental defects of enamel
• Dentin hypocalcification.
• Amelogenesis imperfecta.
Disturbances of Growth (Eruption) of Teeth
Defect of Enamel and Dentin
• Premature eruption
• Regional odontodysplasia
• Delayed eruption
• Multiple unerupted teeth
• Embedded and impacted teeth Abnormalities of Dental Pulp
• Ankylosed deciduous teeth. • Pulp calcification
• Internal resorption
Fissural (inclusion, developmental) Cyst of Oral • External resorption.
Region
• Median maxillary cyst Alteration in Color
• Median palatal cyst • Exogenous stains
• Globulomaxillary cyst • Endogenous stains.
• Median mandibular cyst
• Nasoalveolar cyst
III. CAUSES OF IMMOBILITY OF MANDIBLE
• Palatal cyst of the neonate
• Thyroglossal tract cyst 1. Traumatic
• Benign cervical lymphoepithelial cyst
• Dermoid and epidermoid cysts. A. Extra-articular
• Blow or fall
II. ABNORMALITIES OF TEETH • Surgical extractions and fracture mandible
• Keloids following burns
Alteration in Size
• Improper inf. Alveolar nerve block injection
• Microdontia • Masseter muscle/spheno-mandibular ligament
• Macrodontia. calcification.
 Classifications 745

B. Intraarticular 6. Congenital (Extraarticular)

• Forceps delivery and followed by ankylosis Ankylosis of maxilla to mandible (unilateral) (as
• TMJ fracture reported by burkitt).
• Upward and backward dislocation
• Acute forward dislocation leading to fination 7. Miscellaneous (Extraarticular only)
of condyl beyond articular eminence-mouth
remains wide open with immobility of • Fusion of coronoid process to zygoma
mandible. • Submucous fibrosis
• Scleroderma.
2. Infections
8. Syndromes
A. Extraarticular • Syndrome of TMJ (oral syndrome by Gorlin)
• Pericoronitis • Inability to fully open the mouth with
• Dental infections/osteomyelitis/cellulitis pseudocompilodactily (coronoid enlargement)
• Parotid infections
• Otitis media and mastoiditis IV. CONDITION PRODUCING MACROCHELIA (LIP
• Tonsilitis ENLARGEMENT AND SWELLING)
• Oral ulcers
• Diphtheria 1. Traumatic
• Tetanus A. Acute
• Infected granulomatous of bone (T.B, syphilis,
• Blow or face, friction and surgical interference
actinomycosis)
• Insect bite increased filariasis
• Granuloma inguinale(opening of mouth limi-
ted no TMJ mobility)–due to scar tissue.
B. Chronic

B. Intraarticular • Mouth breathing

• Self inflicted chronic injuries, e.g. lip sucking
• Osteomyelitis of TMJ (local cause or through and biting,glass blowing and pipe smoking, etc.
blood)
• Arthritis (rheumatoid, osteoarthritis, 2. Inflammatory
rheumatoid fever).
Non specific
3. Neoplastic A. Odontogenic
• Collateral edema from dental infections (H/O
Any tumor intra/extraarticular/exostosis of con- pain first, swelling afterwards
dyle and coronoid • Acute furuncle in nose or lip
• Carbuncle or abscess due to mucous gland or
4. Osteoradionecrosis (Both Intra and/or hair follicle infection
Extraarticular) • Infected traumatic lesions
• Chronic macrochelia following repeated attacks
5. Systemic (All Extra Articular) of inflammations.
• Tetany (hypothyroidism-fallen blood calcium)
• Poisoning (strychnine) Specific
• Epilepsy • Tuberculoma or sarcoidosis
• Hystenia • Syphilis-gumma diffuse type
• Physical, chemical, electrical or mental shock • Actinomycosis
or extreme fear • Molluscum contagiosum-viral
• Brain tumors and hemorrhage in medulla – radiation cheilitis
oblongata. – cystic mucous extravasation cyst, mucocele.
746 Textbook of Oral Pathology

3. Neoplastic b. Malignant
• fibrosarcoma
A. Epithelial • neurofibrosarcoma
a. Benign • lymphosarcoma
• Surface epithelium • angiosarcoma
- papilloma • rhabdomyosarcoma
- melanoma • kaposi’s sarcoma in AIDS.
• Glandular epithelium-adenoma (canalicular/
monomorphic) 4. Allergic
- pleomorphic adenoma
• Local
- mucoepidermoid tumor.
• General (urticaria, angioneurotic edema).
b. Malignant
• Surface epithelium
- squamous cell carcinoma (primary and 5. Congenital
metastatic) • Congenital macrochelia
- malignant melanoma • Congenital double lip.
- basal cell carcinoma.
• Glandular 6. Miscellaneous and Syndromes
- malignant pleomorphic adenoma
- mucoepidermoid tumor • Amyloidosis
- adenocarcinoma. • Keratoacanthoma
• Chelitic glandularis
B. Connective tissue • Papillomatosis in Acanthosis nigricans and
adenocarcinoma
a. Benign • Tuberous sclerosis
• fibroma • Hereditary haemorrhagic telangiectasis
• neurofibroma • Lipoidosis
• lipoma • Idiopathic fibro-edema
• hemangiona • Melkerson Rosentheal syndrome
• lymphangioma • Cheilitis granulomatosis
• granular cell myoblastoma • Crohn’s disease (macrochelia) (polystomatitis
• plasma cytoma. vegetans).

V. CLASSIFICATION OF TUMOR

Tissue of origin Benign Malignant

Epithelial tumors Squamous epithelium Squamous cell papilloma Squamous cell carcinoma
Transitional epithelial Transitional cell papilloma Transitional cell carcinoma
Glandular epithelium Adenoma Adenocarcinoma
Basal cell layer ---- Basal cell carcinoma
Neuroectodermal Nevus Melanoma
Hepatocyte Liver cell adenoma Hepatocellular carcinoma
Mesenchymal Adipose tissue Lipoma Liposarcoma
Adult fibrous tissue Fibroma Fibrosarcoma
Embryonic fibrous tissue Myxoma Myxosarcoma
Contd...
 Classifications 747

Contd...
Tissue of origin Benign Malignant
Cartilage Chondroma Chondrosarcoma
Bone Osteoma Osteosarcoma
Synovium Benign synovioma Synovial sarcoma
Skeletal muscle Rhabdomyoma Rhabdomyosarcoma
Smooth muscle Leiomyoma Leiomyosarcoma
Mesothelium Mesothelioma
Blood vessels Hemangioma Angiosarcoma
Lymph vessels Lymphangioma Lymphangiosarcoma
Glomus cell Glomus tumor
Meninges Meningioma Invasive meningioma
Hemopoietic cell Leukemia
Lymphoid tissue Malignant lymphoma
Nerve sheath Neurilemmoma Neurogenic sarcoma
Nerve cell Ganglioneuroma Neuroblastoma
Mixed tumors Salivary gland Pleomorphic adenoma Malignant salivary gland tumor
Tumor of more than Totipotent cells in gonads Mature teratoma Immature teratoma
one germ cell layer or in embryonal rests

VI. PRECANCEROUS LESION AND CONDITION • Adenosquamous carcinoma

• Undifferentiated carcinoma.
1. Pre-cancerous lesion
• Leukoplakia 2. Benign Lesions Capable of Microscopically
• Erythroplakia Resembling Oral Squamous Cell Carcinoma and
• Palatal lesion associated with reverse smoking Oral Verrucous Carcinoma
• Verrucous hyperplasia • Papillary hyperplasia
• Carcinoma in situ. • Granular cell tumor
• Discoid lupus erythematosus
2. Pre-cancerous condition • Median rhomboid glossitis
• Keratoacanthoma
• Oral submucous fibrosis • Necrotizing sialometaplasia
• Sideropenic anemia • Juxta oral organ of chievitz
• Erosive lichen planus • Chronic hyperplastic candidiasis
• Discoid lupus erythematosus • Verruciform xanthoma
• Dyskeratosis congenita. • Verruca vulgaris
• Condyloma acuminata.
VII. HISTOLOGICAL CLASSIFICATION OF CANCER
AND PRECANCER OF THE ORAL MUCOSA 3. Precancerous Lesions (Clinical Classification)
(HISTOLOGICAL TYPING OF CANCER AND • Leukoplakia
PRECANCER (WHO 1997) • Erythroplakia
• Palatal keratosis associated with reverse smoking.
1. Carcinomas
• Squamous cell carcinoma 4. Precancerous Lesions (Histological
• Verrucous carcinoma Classification)
• Basaloid squamous cell carcinoma • Squamous epithelial dysplasia
• Adenoid squamous cell carcinoma • Squamous cell carcinoma in situ
• Spindle cell carcinoma • Solar keratosis
748 Textbook of Oral Pathology

5. Benign Lesions Capable of Resembling Oral 4. Cystic

Precancerous Lesions A. Odontogenic
• White lesions resembling leukoplakia • Primordial (keratocyst)
• Red lesions resembling erythroplakia • Dentigerous
• Focal epithelial hyperplasia • Dental (periapical)
• Reactive and regenerative atypia. • Residual
• Gingival cyst of adults and children
6. Precancerous Conditions
B. Non odontogenic
• Sideropenic dysphagia
• Lichen planus • Nasoplasmic duct cyst or incisive canal cyst
• Oral submucus fibrosis • Median palatine cyst
• Syphilis • Palatine papilla cyst
• Discoid lupus erythematosus • Globulo mammillary cyst
• Xeroderma pigmentosum • Median alveolar cyst
• Epidermolysis bullosa. • Mucocele of palatal glands.

VIII. PRIMARY AND SECONDARY SWELLINGS OF 5. Neoplastic

HARD AND SOFT PALATE INCLUDING GINGIVA
A. Odontogenic
1. Traumatic 1. Epithelial
• Hematoma • Ameloblastoma
• Postoperative and post injection swellings • Adenomatoid odontogenic tumor
• Denture granuloma and pyogenic granuloma • Calcifying epithelial odontogenic tumor
• Fibrous epulis • Squamous odontogenic tumor
• Peripheral giant cell reparative granuloma 2. Mesenchymal
• Aneurysmal bone cyst • Odontogenic myxoma
• Traumatic bone cyst. • Odontogenic fibroma.
3. Mixed
• Odontoameloblastoma
2. Osteoradionecrosis
• Ameloblastic fibro-odontoma
• Osteoradionecrosis • Complex odontome
• Chemical necrosis • Compound odontome.
• Paraformaldehyde
• Noma B. Osteogenic
1. Benign
3. Inflammatory • Torus palatinus
A. Nonspecific • Osteoma
• Chondroma
• Periapical infections • Solitary plasma cell myeloma.
i. acute 2. Malignant
ii. chronic • Osteogenic sarcoma/chondrosarcoma
• Gingival abscess • Multiple myeloma
• Osteomyelitis
• Quinsy (peritonsillar abscess) C. Neurogenic
• Neurofibroma
B. Specific
• Schwannoma
• Tuberculoma • Chordoma
• Gumma • Olfactory
 Classifications 749

D. Muscle Tissue I. Epithelial

1. Benign 1. Benign
• Leiomyoma • Papilloma (single and multiple)
• Rhabdomyoma • Melanoma
• Granular cell myoblastoma. • Benign melanotic tumor in infantile jaws
2. Malignant 2. Malignant
• Rhabdomyosarcoma-(Soft palate only). • Squamous cell carcinoma
• Malignant melanoma.
E. Vascular
1. Benign J. Metastatic
• Hemangioma 1. Carcinoma
• Angioneuroma • Hypernephroma.
• Hemangiopericytoma.
2. Malignant K. Drugs
• Hemangioendothelioma and/or angiosar- • Dilantin enlargement of sialometaplasia
coma
• Kaposi’s sarcoma L. Systemic
• Ewing’s tumor.
Vitamin C deficiency
F. Lymphoid Tissue
M. Blood Dyscrasis
1. Benign
• Lymphangioma • Leukemia
2. Malignant • Agranulocytosis
• Lymphoblastoma or lymphosarcoma • Angioneurotic edema (soft palate).
• Reticulum cell sarcoma
• Lymphoepithelioma or transitional cell N. Endocrine and Metabolic
carcinoma • Pregnancy tumor
• Hodgkin’s lymphoma. • Hyperparathyroidism
• Fibrous dysplasia
G. Glandular Tissue • Paget’s disease
• Marble bone disease
1. Benign
• Histiocytosis X.
• Adenoma (soft palate)
• Pleomorphic adenoma
O. Allergic (usually soft palate)
• Mucoepidermoid tumor.
2. Malignant • Local
• Malignant pleomorphic adenoma • General
• Mucoepidermoid tumor
• Transitional cell carcinoma IX. SWELLING OF FLOOR OF THE MOUTH
• Adenoid cystic carcinoma
• Adenocarcinoma. Traumatic
• Hematoma
H. Fibrous Tissue
• Embedding of foreign body (foreign body gra-
1. Benign nuloma)
• Fibroma • Denture granuloma.
• Ossifying fibroma
• Gingival fibromatosis Inflammatory
• Myxoma and fibromyxoma.
A. Odontogenic
2. Malignant
• Fibrosarcoma. B. Non Odontogenic
750 Textbook of Oral Pathology

C. Glandular and ductal origin ii. Retention cyst

• Ranula.
• Swellings of Wharton’s duct
• Stone in Wharton’s duct
• Mikulicz disease Neoplastic
• Swelling of sublingual and submandibular sali-
vary gland. A. Epithelial

a. Benign
D. Non odontogenic.non glandular origin i. Surface epithelium
• Acute infections following trauma. • Papilloma
• Melanoma.
X. NON ODONTOGENIC NEOPLASMS OF JAWS ii. Glandular
• Monomorphic adenoma
1. Malignant • Pleomorphic adenoma
• Oxyphilic adenoma.
• Osteosarcoma b. Malignant
• Juxtacortical osteosarcoma i. Surface epithelium
• Parosteal osteosarcoma • Squamous cell carcinoma
• Periosteal osteosarcoma • Malignant melanoma.
• Chondrosarcoma ii. Glandular
• Mesenchymal chondrosarcoma • Mucoepidermoid tumor
• Ewing sarcoma • Lymphoepithelioma
• Burkitt’s lymphoma • Adenocarcinoma
• Plasma cell neoplasms • Malignant pleomorphic adenoma.
• Multiple myeloma
• Solitary plasmacytoma of bone
• Metastatic carcinoma. B. Connective Tissue

i. Benign
2. Benign • Hemangioma
• Ossifying fibroma – capillary
• Fibrous dysplasia – cavernous
• Osteoblastoma • Hemangiopericytoma
• Osteoid osteoma • Endothelioma
• Chondroma • Lymphangioma
• Osteoma • Neurofibroma
• Central giant cell granuloma – single
• Giant cell tumor – multiple
• Hemangioma of bone • Neurilemmoma
• Idiopathic histiocytosis (Langerhans cell disease) • Plasmacytoma
• Tori and exostosis • Rhabdomyoma
• Coronoid hyperplasia. • Leiomyoma
• Lipoma
• Fibroma
XI. POST RADIATION SWELLINGS
• Granular cell myoblastoma.
Cystic swellings ii. Malignant
• Fibrosarcoma
i. Congenital and developmental • Reticulum cell sarcoma
• Sublingual dermoid cyst • Lymphosarcoma
• Thyroglossal duct cyst • Rhabdomyosarcoma
• Lymphangiogenic • Hemangioendothelioma
• Brachial cyst. • Kaposi’s sarcoma (AIDS).
 Classifications 751

Allergic 2. Pathological
A. Local A. Inflammatory Condition
B. General (Angioneurotic edema)
• Foreign body type
Syndromes • Langerhans type
• Hereditary hemorrhagic telangiectasis • Aschoff`s cells
• Lipoidosis • Touton type.
• Sturge–Weber disease.
B. Neoplastic Condition
XII. SALIVARY GLAND DISEASE AND TUMORS
• Osteoclastic
Inflammatory • Reed Sternberg
• Atypical
A. Acute • Giant cell fibroblast.
• Viral: Mumps
• Bacterial: (staphylococcus, streptococcus,gram XIV. CLASSIFICATION OF GIANT CELL LESIONS
negative microorganism)
a. Acute suppurative parotitis 1. Physiological
b. Post surgical
c. Terminal debilitation. • Root resorption of deciduous teeth

B. Chronic 2. Traumatic
• Tuberculosis
• Actinomycosis • Traumatic granuloma or granuloma faciale
• Sarcoidosis. • Peripheral and central giant cell granuloma
• Epulis fissuratum
Systemic or Metabolic • Pyogenic granuloma
• Chronic occlusal trauma
• Obesity
• Internal resorption causing periapical
• Hypertension
granuloma
• Diabetes mellitus
• Root resorption from pressure trauma
• Malnutrition
• Healing of sockets after extraction.
• Alcoholism and liver disease.

Autoimmune Diseases 3. Infection

• Sjogren’s syndrome • Syphilis
• Mikulicz disease. • Yaws
• Bejel
Local Disturbances
• Tuberculosis
• Sialothiasis • Leprosy
• Sialoangiectasis • Sarcoidosis
• Trauma, foreign body, fistula – Melkerson Rosenthal syndrome
• Parotid lymphadenopathy – Heerfordt’s syndrome.
• Cysts, ranula • Plasmocytosis (plasma cell granuloma)
• Local duct obstructions (mucous plugs, congenital). • Blastomycosis
• Histoplasmosis
XIII. CLASSIFICATION OF GIANT CELLS • Candidiasis
• Actinomycosis
1. Physiological
• Aspergillosis
• Osteoblast • Spirotrichosis
• Trophoblast • Mucormycosis
• Megakaryocyte. • Rhinosporidosis.
752 Textbook of Oral Pathology

XV. CLASSIFICATION OF FIBRO OSSEOUS LESION 2. Non Giant Cell Group (Pure Fibro Osseous
Lesions)
Based on histology findings of fibrosis and mineralized
tissue formation, a large number of lesions are present • Fibrous dysplasia
in these grouped as: • Osteitis deformans (paget’s disease)
• Ossifying and cementifying fibroma
1. Periapical lesions • Periapical cemental dysplasia (Cementoma)
• Focal sclerosing osteomyelitis.
• Periapical cemental dysplasia
• Cementifying fibroma XVII. METABOLIC AND GENETIC JAW DISEASE
• Cementoblastoma
• Gigantiform cementoma. 1. Metabolic Conditions
• Paget’s disease
2. Medullary Bone Origin
• Hyperthyroidism
• Ossifying fibroma • Hypophosphatasia
• Paget’s disease • Infantile cortical hyperostosis
• Fibrous dysplasia • Phantom bone disease
• Cherubism • Acromegaly
• Aneurysmal bone cyst • Fibrous dysplasia.
• Hyperparathyroidism
• Central giant cell granuloma 2. Genetic Abnormalities
• Sclerosing osteomyelitis (focal and diffuse)
• Garre’s osteomyelitis • Cherubism
• Giant cell tumor. • Osteopetrosis
• Osteogensis imperfecta
• Cleidocranial dysplasia
3. Malignant Tumors
• Crouzon’s syndrome
• Osteosarcoma • Treacher Collins syndrome (Mandibulofacial
• Chondrosarcoma dysostosis)
• Fibrosarcoma. • Pierre Robin syndrome
• Marfan syndrome
4. Fibro Osseous Epulides • Ehlers Danlos syndrome.

• Pyogenic granuloma XVIII. ULCERATION OF ORAL CAVITY

• Giant cell reparative granuloma
• Giant cell epulis Traumatic
• Fibroma
• Fibroma epulis • Physical
• Fibroepithelial polyp or papilloma • Resorption of periapical area around a primary
• Fibro osseous epulis tooth
• Ossifying fibroma • Rough and badly fitting restoration
• Angiofibroma. • Ulcers due to tooth present at birth and sharp
edges
• From splints, forced injection ulcers on palate
XVI. BENIGN FIBRO OSSEOUS LESION • Electric burn due to live wire
• Post filling ulcer on frenum of tongue
1. Giant Cell Group
• Epileptic ulcers
• Central giant cell granuloma • Thermal change
• Brown’s tumor • Hot liquid
• Aneurysmal bone cyst • Refrigerant local anesthetics
• Cherubism. • Chemical
 Classifications 753

• Drugs, acid, aspirin, arsenic, bismuth, lead • Ischemic lingual necrosis

• Actinic cheilosis on lips • Wegener’s granulomatosis
• Radiation and laser radiation. • Midline lethal granuloma.

Infections D. Degenerative and Related Disorder

• Amyloidosis
• Non specific (streptococcus and staphylococcus)
• Oral submucous fibrosis
• Non specific infected ulcers primarily traumatic
• Senile solar elastosis.
• Specific (bacilli and cocci)
• Aphthous ulcer and periadentis mucosa necrotic E. Pigmentation
recurrens or major aphthae
• Tuberculosis • Racial pigmentation
• Diphtheria • Endocrinopathy
• Tularemia (rabbit fever) • Addison disease
• Granuloma inguinale • Albert syndrome
• Leprosy • Bronze diabetes
• Gonorrheal • Anemia.
• Pneumonia
Typical Histological Feature of Few Lesions
• Ulcers of oral cavity in infections with bacillus.
Typical features Name of disease
XIX. MUCOCUTANEOUS DISORDER Starry sky Burkitt’s lymphoma, infective
mononucleosis, polycythemia
I. Genodermatoses Rushton bodies Dentigerous cyst
Reed Stenberg cell Hodgkin’s disease
• White sponge nevus Dropping off effect Junctional nevus
• Darier’s disease Saw tooth appearance Lichen planus
• Peutz Jeghers syndrome Antoni type A and type Neurilemmoma
• Dyskeratosis congenita B tissue
• Hereditary benign intraepithelial dyskeratosis Honey comb or swiss
cheese pattern Adenoid cystic carcinoma of
• Pachyonychia congenita salivary gland
• Hyalinosis cutis et mucosa oris Picket fence or Primordial cyst
• Pseudoxanthoma elasticum. tombstone
Liesegang ring Calcifying epithelial odon-
II. Non Infective Disease togenic tumor
Safety pin appearance Granuloma inguinale
A. Vesicular Lipschutz bodies Herpes simples infection
Anitschkow cell Aphthous ulcer, sickle cell an-
• Erythema multiforme
emia, megaloblastic anemia
• Pemphigus and iron deficiency anemia
• Benign mucus membrane pemphigoid Henderson Paterson Molluscum contagiosum
• Bullous pemphigoid inclusion
• Epidermolysis bullosa Dilapidated brick wall Familial benign chronic
• Bullous lichen planus. effect pemphigus
Cartwheel or checker- Multiple myeloma
board appearance
B. Non Vesicular Disease
Tobacco cells or cell Hereditary benign intra
• Lichen planus in cells epithelial dyskeratosis
• Benign migratory glossitis. Lava flowing around Dentin dysplasia (shield
boulder’ type I)
C. Collagen Disorder Signet ring appearance Liposarcoma
Cart wheel or storiform Malignant fibrous histiocy-
• Lupus erythematous toma
• Scleroderma Tadpole like Fibrosarcoma
• Polyarteritis nodosa appearance
• Vasculitis Cigar like appearance Leiomyoma
754 Textbook of Oral Pathology

Appendix
Differential Diagnosis
II of Lesion

Differential diagnosis of most common lesion of oral cavity

Disease Etiology Types Clinical features Pathological features Management

Microdontia Pituitary True generalized— Affected teeth are Crown and bridge
dwarfism, Down’s all the teeth are maxillary lateral should be given
syndrome, smaller than incisors and 3rd
congenital heart normal. molars.
disease. progeria. Relative Peg shaped laterals
generalized— (mesial and distal
normal or slightly sides converges or
smaller than taper incisally).
normal teeth; are
present in jaws
that are somewhat
larger than normal.
Localized—it
involves only
single tooth.

Macrodontia Pituitary True generalized – Crowding, Orthodontic

(megadontia) gigantism, facial all the teeth are malocclusion, treatment
hemi-hypertrophy, larger than normal. impaction of teeth.
angioma of face, Relative generalized
and genetic —normal teeth
component. present in smaller
jaw
Localized—one or
more large teeth
exist

Fusion Hereditary Complete (before Esthetic problems, Esthetic recovery

calcification) periodontal
Incomplete (after complication
calcification)

Contd...
 Differential Diagnosis of Lesion 755

Contd...
Disease Etiology Types Clinical features Pathological features Management
Concrescence Traumatic injury, Union with
crowding of teeth, cementum,
hypercementosis extraction of teeth
Dilacerations Trauma during should be done
development carefully
Gemination Hereditary and Sharp bend in root,
familial tendency. may cause
problems during
extraction
Commonly
affected teeth are
deciduous
mandibular
incisors and
permanent
maxillary incisors.
Bifid crown on
single root.
Common pulp
canals and either
single or partially
divided pulp
chambers. Crown
is wider. Enamel
or dentin of crown
hypoplastic or
hypocalcified.

Taurodontism Failure of Hertwig Hypotaurodont Bull like teeth, No treatment is

root sheath to Hypertaurodont elongated pulp required
invaginate chamber,
associated with
Klinefelter
syndrome

Dens in dente Defect in Coronal type Mild type Restoration of

(dens invaginatus) morphological (occur in crown) (accenuted lingual defect
development Radicular type pit area),
(occur in root) intermediated
form (small pear
shaped
invagination)
producing tooth in
tooth appearance
Extreme form (it
extends beyond
pulp chamber)

Dens invaginatus Elongation and Globe shaped or Minor reduction of

proliferation of nipple shaped cusp to maintain
inner epithelium protuberance, pulp occlusal harmony
exposure due to
trauma lead to
pain, infection

Contd...
756 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
Talon cusp T shaped Preventive care
projection called as taken by
eagle Talon. performing
Associated with endodontic
Rubinstein-Taybi treatment
syndrome

Amelogenesis Hereditary, Hypoplastic Chalky white color Hypoplastic (lack Composite veneer
imperfecta involves only Hypomaturation teeth, open contact of differentiation crown should be
ectodermal Hypocalcification point, severe of ameloblasts, no given
component abrasion, cheesy matrix formation),
enamel, snow Hypocalcification
capped teeth (defective matrix
formation and
subnormal mineral
deposition),
Hypomaturation
(alteration in
enamel rod and
rod sheath
structure)

Dentinogenesis Autosomal Type I (with Amber like Enamel is normal, Metal and ceramic
imperfecta dominant mode osteogenesis appearance, mantle dentin crown
imperfecta) translucent teeth, (narrow zone of
Type II (without multiple pulp dentin beneath
osteogenesis exposure, teeth not enamel), less
imperfecta) sensitive dentinal tubules,
Type III large area of a
(brandywine type) tubular dentin,
pulp chamber are No treatment is
obliterated with a required
tubular dentin.

Dentin dysplasia Autosomal Radicular Mobility of teeth, Coronal and

(root less teeth) dominant Coronal bluish or brownish radicular dentin
translucency at comprised of
cervical area, tubular and
enamel does not osteodentin,
chip off appear as lava
flowing around the
boulders. Normal
and abnormal
dentin is well
demarcated

Regional Local ischemic Abnormal pulp, Dentin is thin and Extraction of teeth
odontodysplasia changes during teeth deformed, globular, irregular followed by
(ghost teeth) odontogenesis soft leathery tubules, and wide fabrication of
appearance and predentin layer, prosthesis
yellowish brown in tiny droplet
color calcification

Contd...
 Differential Diagnosis of Lesion 757

Contd...
Disease Etiology Types Clinical features Pathological features Management
Agnathia Congenital Absence of --------- ----
(hypognathous) mandible or
maxilla,
rudimentary
tongue, absence of
ear,

Agenesis Congenital Absence of some -------

part of mandible or
maxilla, absence of
condyle

Micrognathia Congenital heart Apparent type Midfacial -------- Surgical approach

dis, Pierre Robin True type deformity, ------------ to correct
syndrome, in (congenital or interference in micrognathia
acquired mouth acquired) feeding, speech
breathing, problems,
ankylosis malocclusion

Macrognathia Paget’s disease, Gummy smile Resection followed

(megagnathia) fibrous dysplasia, Mandibular by orthodontic
pituitary gigantism prognathisms, treatment
prominent chin
button, steep
mandibular angle

Facial Hormonal Thick skin, No treatment

hemihypertrophy imbalance, hypertrichosis,
incomplete excessive secretion,
twinning and unilateral
lymphatic enlargement
abnormalities

Facial hemiatrophy Trauma, heredity, Vertical furrow or No effective

infection line in midline, treatment
peripheral nerve howling of cheek,
dysfunction delayed tooth
eruption

Cleft lip and cleft Hereditary, Unilateral Difficult Obturators,

palate nutritional factors, incomplete, breastfeeding, surgical correction
defective vascular unilateral upper anterior
supply, traumatic complete, bilateral misplaced, middle
stress, Streeter’s complete cleft, ear infection,
fetal dysplasia difficult in speech,

Double lip Hereditary Congenital or Cupid bow, Excision for

acquired associated with cosmetic region
Ascher’s syndrome

Peutz-Jeghers Autosomal Café au lait spot, Oral macular Surgical treatment

syndrome dominant trait intestinal lesion exhibits for intestinal
polyposis, and excessive polyps
intussusceptions accumulation of Steroid injection
melanin granules
in basal cell layer
Contd...
758 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
Cheilitis granulo- Allergic origin. Diffuse, nodular, Granulomatous and surgical
matosa Hypersensitivity to painless, firm inflammation with excision
bacterial toxins growth of lip. chronic
Associated with inflammatory cells,
Melkersson- Langhans types of
Rosenthal multinucleated
syndrome giant cells

Fordyce granules Hereditary, normal Multiple, small, The peripheral No treatment is

variation discrete milia like cells are flat and required
yellowish lesion. darkly stained and
Confluent plaque inner cells are lipid
are also present rich and pale. The
duct may show
keratin plugging

Heck disease (focal HPV type 13 and Small Hyperparakeratosis Self-regressing

epithelial 32 pedunculated, with extensive lesion
hyperplasia) polypoid or acanthosis,
nodular soft tissue koilocytes, fusion
growth. Size 1 to 5 of rete pegs,
mm. increase mitotic
activity.
Degeneration of
the basal layer.

White sponge Autosomal Bilateral, Marked thickening No treatment is

nevus dominant with asymptomatic, of epithelium with required
incomplete deeply folded hyperparakeratinization,
penetrance white or gray acanthosis and
lesion. Spongy spongiosis.
consistency, Intracellular
sometime ragged edema, pyknotic
white area. nuclei. Basket
wave appearance

Fissured tongue Congenital, genetic Small furrows or No needed

defect, vitamin grooves on the
deficiency, dorsal surface of
tongue. Discomfort
due to food
accumulation.
Associated with
Melkersson-
Rosenthal
syndrome

Median Rhomboid Persistence of Elongated Mild to severe par- Antifungal agents,

glossitis tubercular impair, erythematous akeratosis, thinning antiseptic gargles.
infection due patch, diamond or of suprapapillary
Candida albicans lozenge shaped epithelium, acan-
area of devoid thosis, elongation of
papillae rete pegs, neutro-
philic infiltration,

Contd...
 Differential Diagnosis of Lesion 759

Contd...
Disease Etiology Types Clinical features Pathological features Management
Geographic tongue Family history, Multiple irregular, Hyperparakerati- Heavy dose of
emotional stress well demarcated nization of vitamins should be
patchy epithelium, with given
erythematous loss of filiform
areas with papillae.
desquamation of Intercellular
filiform papillae. edema, spongiotic
Periphery abscess (neutrophil
surrounded by polymorphs)
yellowish-white
serpiginous line

Lingual thyroid Endodermal down Nodular exophytic It composed of Surgical excision

nodule growth at the site mass, smooth normal mature should be done
of foramina cecum cystic thyroid tissue.
swelling,dysphonia, Colloid
dysphagia and degeneration
dyspnoea

Thyroglossal duct Remnant of Asymptomatic Linear stratified Surgical excision

cyst embryonic mobile swelling in squamous along with tract
thyroglossal tract midline of neck, epithelium or
dome shaped, transitional
compressible, epithelium,

Aberrancy Lingual It exhibits normal

mandibular salivary gland
salivary gland lobules and ducts
depression,
gingival salivary
gland choristoma

Papilloma HPV can be Slow growing, Multiple finger like Surgical excision of
responsible cauliflower like projection, the lesion
growth with finger vascular
like projection. connective tissue,
Base is sessile. few inflammatory
White color and cells,
firm in hyperkeratosis and
consistency, it can acanthosis
occur in down
syndrome or
Cowden’s
syndrome

Keratoacanthoma Sun exposed Well Thick hyper- Surgical excision

superficial circumscribed, keratinized with should be carried
epithelium of the elevated parakeratin out
sebaceous ducts umblicated crater plugging,
like lesion with pseudoepithelio-
central depression, matous hyper-
margin rolled and plasia, spinous cell
sharply delineated layer is thick

Contd...
760 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
Intradermal Raised flat area on Clusters or nests of Surgical excision
(intramucosal) skin, with dark nevus cells should be done
nevus common brown color,. confined to
mole Asymptomatic connective tissue,
spindle shaped
cells, multinucle-
ated giant cells,
some cells are
pigmented

Junctional nevus Asymptomatic Focal areas of Surgical excisions

brown or black proliferating nevus should be carried
macule cells, cluster of out
cells present at
apex of epithelial
rete pegs

Compound nevus Pigmented papule Presence of nevus Surgical excision

or macules over cells distributed in should be carried
the hard palate or basal layer of out
the gingiva epithelium as well
as adjacent
connective tissue

Blue nevus Dome shaped, Cells are elongated Surgical excision

dark blue papule, bipolar, and with
flat pigmented spindle shaped. histopathological
macule over the Fusiform dendritic evaluation
skin of mucous cells
membrane

Fibroma Excessive Small, well Proliferation of Surgical excision

proliferation of circumscribed, slow fibroblasts cells, should be carried
fibroblast cells, growing, nodular cells are spindle out
with synthesis of growth, pedun- shaped, bundles of
collagen culated, surface collagen, thin
smooth, soft to firm epithelium and
in consistency flattening of rete
pegs

Ossifying fibroma Reactive Peripheral type Peripheral – small Peripheral— Surgical excision
proliferation of Central type painless, lobulated diffuse sheets of should be carried
periodontal or swelling, sessile, proliferating out
periosteal tissue hard to firm on fibroblast with
palpation plump monomor-
Central – bony phic nuclei,
hard swelling, hypercellular
expansion and reactive tissue,
distortion of the osteoids may be
cortical plate. present
Disfigurement of Central – whorled
face pattern of
fibroblastic stroma
Contd...
 Differential Diagnosis of Lesion 761

Contd...
Disease Etiology Types Clinical features Pathological features Management
with presence of
collagen fiber.
Irregular calcified
masses seen in
later stage

Peripheral giant Connective tissue Small, exophytic, Epithelium Surgical excision

cell granuloma of the periosteum well circumscribed ulcerated with should be done
of jaw bone or pedunculated areas of
from periodontal lesion, painless, hemorrhage,
ligament tissue firm lobulated, proliferating
dark red color, fibroblasts,
bleeding from multinucleated
surface giant cells, spindle
shape cells,
intercellular edema
with inflammatory
cell infiltrate

Central giant cell Reactive lesion Small bony had Fibrovascular Surgical excision
granuloma swelling, connective tissue should be carried
expansion of stroma, proliferat- out
cortical plate, vital ing spindle shaped
teeth, some lesion stomal cells and
may cause interlacing
perforation of collagen fibers,
cortical plate multiple multi-
nucleated giant
cells which contain
5 to 20 nuclei,
small foci of
osteoids of oven
bone is present

Myxoma It is origin from Rare lesion and Loose textured Radical surgery is
primitive firm and nodular tissue containing recommended as it
mesenchyme growth of varying delicate reticulin is aggressive lesion
size fiber and mucoid
material, stellate
shaped cells,
Lipoma Origin from Well defined, soft Proliferating Surgical excision
adipose tissue movable lump, mature fat cells should be carried
painless, yellow in with loose areolar out
color and smooth tissue stroma,
overlying surface round cells,
vacuolated with
centrally placed
nuclei, lobules of
fat cells separated
by fibrous tissue
Hemangioma Vascular tissue Cavernous Raised, Capillary – small Injection of
origin, Capillary Port- multinodular, red, endothelial line sclerosing agent
wine stain Central blue or purple capillaries in the should be given

Contd...
762 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
lesion, blanches on lesion, cells are
compression, single layered,
compressibility cells spindle
test positive, shaped, plump
Central type – Cavernous – large
painful expansile irregularly shaped
jaw swelling, dilated endothelial
affected bone sinuses contain
pulsatile, loosening large aggregates of
of teeth and erythrocytes,
anesthesia of skin single layer of
and mucous flattened
membrane endothelial cells
line, area of
hemorrhage

Lymphangioma Proliferation of Painless nodule, Proliferating, thin May regress

lymphocytes may be diffuse walled, lymphatic spontaneously,
with lighter color, vessels, lined by persistent lesion
crepitant sound, plump endothelial should go surgical
tongue. Lymphan- cells, multiple excisions
gioma may papillomatous
produce nodule on the
macroglossia surface

Osteoma Neoplasms of Compact osteoma Nodular, Dense cortical Surgical excision

osseous tissue cancellous osteoma exophytic, bony bone with distinct
hard growth, lamellar pattern,
expansion of cortical bone
cortical plate, sclerotic and
gardener avascular, reduce
syndrome present marrow spaces
with osteoma
Osteoblastoma Painful, small in Small area of Surgical excision
size, expansion osteoblastic
and distortion of activity followed
cortical plates, by mature osteoid
calcified, nidus
consist of
interlacing
meshwork of bony
trabeculae,
vascular
connective tissue
stroma, numerous
osteoblasts are
present
Chondroma Cartilaginous Slow enhancing Well defined Surgical excision
tissue origin pain bony hard lobules of hyaline should be done
swelling, cartilage, mature
expansion and chondrocytes, cells
distortion of the are round or oval
cortical plate, in shape,

Contd...
 Differential Diagnosis of Lesion 763

Contd...
Disease Etiology Types Clinical features Pathological features Management
Leiomyoma Neoplasm of Slow growing, Spindle shaped Surgical excision
smooth muscle painless, smooth muscle done with
cells submucosal cells, cells surrounding
nodules, surface is arranged in fascicle normal tissue
smooth, yellowish or stream line
in color, firm, fashion, cigar
encapsulated, shaped appearance

Rhabdomyoma Neoplasm of Slow growing, Sharply outline, Surgical excision is

striated muscles well unencapsulated done
circumscribed, mass of round or
painless mass, oval striated
deep seated muscle cells,
multiple vacuoles
in the cell
neoplasm,
irregular cross
striation, cell
nuclei are vesicular

Neurilemmoma Derived from Slow enlarging, Proliferating Surgical excision

(Schwannoma) Schwann cells, well spindle shaped should be done
neuroectodermal circumscribed, neoplastic
origin painless nodule, Schwann cells with
smooth firm, elongated nuclei,
exophytic, tender Antoni A tissue
to palpation, (parallel rows of
central lesion palisading nuclei
present well of Schwann cells)
demarcated bony Antoni B tissue
hard lesion (disorderly
arranged cells and
fibers band)
Verrocay bodies

Neurofibroma Autosomal Submucosal mass, Numerous Surgical excision

dominant multilobulated proliferating should be done
hereditary surface, expansion, spindle shaped
condition pain Paresthesia, cells resembling
soft tissue lesion fibroblast,
present as freely haphazard
movable nodule, arrangement,
flabby masses, café delicate collagen
au lait spot fiber, café au lait
spot revels basilar
melanosis
Neuroectodermal Derived from Swelling, Pigmented cells Surgical excision
tumor of infancy primitive neural expansion, and non with through
crest distortion, facial pigmented cells, curettage
assymetry, surface open nucleus,
is brown or black flattened or cubical
pigmentation, slow in shape with pale
growing nuclei,

Contd...
764 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
un-pigmented cells
occur in cluster,
and surrounded by
pigmented cells

Pleomorphic Slow growing, Proliferation of Surgical excision

adenoma well delineated glandular, should be carried
exophytic growth, basophilic, out
surface smooth, epithelial cells in
lobulated, painless, the form of diffuse
soft and rubbery sheets or clusters,
consistency, polygonal shape
glandular
epithelium cells
arranged in
clumps or
interlacing strand,
squamous
metaplasia, there is
myxoid area,
chondroid area.

Monomorphic Proliferation of Basal cell adenoma Basal cell – Basal type – Surgical excision of
adenoma single epithelial Canalicular encapsulated, granular epithelial the lesion
cell type adenoma movable lesion less cells in the forms
than 3 cm. lesion of oval shaped
are firm nests, outer layer is
Canalicular – small cubiodal and inner
painless, movable layer is uniform
encapsulated Canalicular –
lesion covered by anatomizing
smooth intact network of
epithelium cuboidal and
columnar cells
which give
impression of
multiple intercon-
necting canals , the
connective tissue
stroma is myxoma-
tous and com-
posed of eosino-
philic hypocellular
mucoid matrix
Warthin tumor Consist of Slow enlarging, Multiple cystic Simple surgical
(oncocytoma) oncocytes well spaces lined by excision is done
circumscribed, soft pseudostratified
painless swelling, columnar
well encapsulated epithelial cells,
movable lesion, cells are arranged
compressible and in double layer
doughy feeling pattern, cystic
lumen filled with

Contd...
 Differential Diagnosis of Lesion 765

Contd...
Disease Etiology Types Clinical features Pathological features Management
homogenous
eosinophilic
material

Sq cell carcinoma Tobacco, betel nut, White or red Well differentiated Surgical treatment
(epidermoid alcohol, actinic variegated path, (resembles the cells and radiotherapy
carcinoma) radiation, herpes exophytic invasive of squamous in some cases
simplex, ulcer, induration epithelium, keratin should be given
immunosuppressant around periphery, pearls), moderately
and genetic factors painful due to differentiated
secondary (more dysplastic
infection, little or no keratin
pathological and greater
fracture can occur number of mitotic
in extensive cases cells division),
poorly
differentiated (no
keratin, no
resemblance to
cells of stratified
squamous
epithelium, mitotic
division rate is
high)

Basal cell Exposure to Slow growing, Neoplastic Surgical excision

carcinoma (rodent sunlight elevated lesion proliferation of or electrocautery
ulcer) which develops baseloid epithelial along with
central crust, with cells in solid radiotherapy
rolled border, it island, cells are
invades and columnar in shape,
destroys the palisaded
adjacent tissue arrangement,
intercellular bridge
absent,

Verrucous Tobacco chewing Slow growing Papillary surface LASER therapy

carcinoma and snuff dipping exophytic covered by thick
habits papillary growth layer of
with white pebbly parakeratin,
surface. Multiple acanthotic rete
rugae like folds ridges, parakeratin
with deep clefts, plugging, pushing
regional lymph margin, connective
nodes enlarged tissue intense
and tender inflammatory
infiltration
Malignant Arise from Hutchinson’s Macular Excessive Radical surgery
melanoma melanocytes freckle type pigmented focal proliferation of with prophylactic
superficial lesion which grow neoplastic neck dissection
spreading type rapidly to results melanocytes,
Invasive type in large painful extensive cellular
diffuse mass, pleomorphism,

Contd...
766 Textbook of Oral Pathology

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Disease Etiology Types Clinical features Pathological features Management
surface ulceration cells are round,
is common, small polyhedral and
satellite lesion multinucleated,
numerous mitotic
activity

Spindle cell Pain, ulceration, Spindle shape Surgical excisions

carcinoma swelling, fleshy epithelial cells, is the most
and polypoid nuclear hyperchro- effective treatment
growth pattern matism, cellular
pleomorphism,
increased mitosis,
tumor giant cells,
little or no keratin
formation,
inflammatory cell
infiltration

Fibrosarcoma Neoplasm of Fast enlarging, rise Proliferation of Radical surgical

fibroblast to large, painful, spindle shaped, excision and
bulky, lobulated malignant chemotherapy
fleshy mass, fibroblast cells. It should be given
surface ulcerated has tadpole like
due to trauma appearance,
streaming fashion
arrangement,
abnormal mitotic
activity in the form
of biradiate or tri-
radiate
Malignant fibrous Arises from Enlarging, Proliferating Wide surgical
histiocytoma undifferentiated exophytic polyhedral or oval excision with
mesenchymal cells lobulated and shaped, malignant radiotherapy and
ulcerated, fleshy histiocytes, cart chemotherapy
appearance, pain, wheel or storiform
hemorrhage, pattern, increased
paresthesia, gross mitotic activity,
facial cellular pleomor-
disfigurement phism, giant cells
are present
Liposarcoma Cell along the Rapidly growing, Cellular lesion Surgery and
adipose tissue line painful, contain foamy, fat radiotherapy
submucosal containing
masses, lobulated malignant
lesion, firm in lipoblasts cells,
consistency, signet net
appearance
(vacuolated
cytoplasm) cells
poorly differenti-
ated round cells,
irregularly shaped
giant cells

Contd...
 Differential Diagnosis of Lesion 767

Contd...
Disease Etiology Types Clinical features Pathological features Management
Hemangioendot- Mesenchymal Fast enlarging, Neoplastic Surgical exicision
helioma tissue origin localized, painful, proliferation of and radiotherapy
nodular swelling, malignant should be done
surface ulceration, endothelial cells.
paresthesia, The cells are
anesthesia, pleomorphic, large
bleeding upon polyhedral or
slight trauma, slightly flattened,
expansile lesion, nuclei are round,
increased
abnormal mitosis
Kaposi’s sarcoma Arises from It can be endemic It can be presented Patch stage Treated by
endothelial cells of Rarely can be in patch, plaque, (dilated, irregular, radiotherapy and
the blood epidemic nodular form. blood vessels, chemotherapy
capillaries, Nodule can be lined by normal
triggering factors multiple endothelial cells),
are HIV infection, plaque stage
immunosuppressant (dilated jagged
and environment vascular channels
factors lined by spindle
type cells) nodular
stage (slit like
spacing containing
RBCs)
Ewing’s sarcoma Lesion arise from Moderate fever, Proliferating, Radiotherapy,
endothelial cells or leukocytosis, rapid packed round cells chemotherapy and
undifferentiated swelling severe which have surgery is
reticuloendothelial pain, paresthesia, monotonous round recommended
cells surface ulceration, or oval nuclei,
hyperchromatic
cells arranged in
diffuse pattern,
cells are round,
increased mitotic
activity
Chondrosarcoma Neoplasm of Primary Painless facial Can be well Wide surgical
cartilage tissue, Secondary asymmetry, later differentiated like excision should be
may be cause by pain, tenderness, benign or poorly done
Paget disease, anesthesia or differentiated,
Ollier’s disease paresthesia in the spindle shaped
benign region, extensive malignant cells,
chondroma. local tissue binuclear cells,
destruction,
expansion of bone,
nasal obstruction
Osteosarcoma Arises from bone, Medullary Fast enlarging Actively Combination
may arise from osteosarcoma painful swelling proliferating therapy is given in
pre-existing periosteal causing expansion, spindle shaped, the form of
Paget’s diease, osteosarcoma, and distortion, oval angular radiotherapy, and
fibrous dysplasia, parosteal facial deformity, malignant chemotherapy
osteochondroma osteosarcoma, soft displacement of osteoblast cells
and chronic tissue teeth, numbness of with cellular
osteomyelitis osteosarcoma lip, overlying skin stroma, cellular
Contd...
768 Textbook of Oral Pathology

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Disease Etiology Types Clinical features Pathological features Management
inflamed, pleomorphism,
ulceration, increased mitosis,
hemorrhage, multiple area of
pathological osteoid bone
fracture within fibrous
stroma
Non-Hodgkins Neoplasms of cells Fever, night Proliferation of Chemotherapy
lymphoma of lymphoid tissue sweats, malaise, malignant should be given
anorexia, weight lymphocytes,
loss, generalized cellular
lymphadenopathy, pleomorphism,
abdominal pain, cells small in size,
fast enlarging Nodular pattern –
exophytic growth, tend to aggregate
lymph nodes firm, in large cluster
rubbery, overlying Diffuse pattern –
surface is red and monotonous
inflamed, proliferating tumor
cells within
connective tissues

Burkitt’s Cause by Epstein Rapid painless, Proliferation of Chemotherapy

lymphoma Barr virus, loosening of teeth, lymphocytes, should be given
fast enlarging large syncytial
expansile swelling, appearance
overlying mucosa (nucleus
is ulcerated, surrounded by
paraplegia, cytoplasm), mitotic
perforation of activity is
cortical plate, abundant, starry
sky appearance
(macrophages with
clear cytoplasm
scatter uniformly
throughout the
tumor)
Hodgkin Neoplasm of Persistent Malignant Chemotherapy
lymphoma lymphocytes generalized lymphoid cells, should be given
lymphadenopathy, non-neoplastic
lymph nodes are inflammatory cells,
firm rubbery, Reed-Sternberg
generalized giant cells (tow
weakness, pain mirror image
low grade fever, nuclei), Owl
edema of appearance It can
extremities, be of lymphocyte
hepatosplenomegaly, prominent, mixed
oral lesion present cellularity,
as submucosal lymphocytes
swelling with depletion and
ulceration nodular sclerosis

Contd...
 Differential Diagnosis of Lesion 769

Contd...
Disease Etiology Types Clinical features Pathological features Management
Multiple myeloma Neoplasm of Severe deep bone Sheets of closely Chemotherapy
plasma pain, gradual bone packed, round or
loss, increased oval cells, resemble
susceptibility to plasma cells,
infection, nausea, cartwheel or
vomiting, anemia, checkerboard
jaw lesion fast pattern (eccentri-
enlarging, painful cally placed nuclei
swelling, egg shell exhibits chroma-
cracking, tin), mitotic figure
perforation of may be high,
cortex, binucleated or
multinucleated
cells
Rhabdomyosar- Striated muscle Embryonal Rapidly growing Embryonal (small Surgery,
coma origin, Alveolar lesion, swelling, round cells with radiotherapy and
Pleomorphic pain, extensive monotonous chemotherapy
damage, hyperchromatic should be given
indurated, fixed nuclei) Alveolar
and ulcerated (round cells
pattern similar to
lung alveoli)
Pleomorphic
(primitive muscle
formation,
prominent nuclei)
Adenoid cystic Arises from Slow enlarging Small darkly Wide surgical
carcinoma glandular growth, surface staining, polygonal excision should be
(cylindroma) epithelium of ulceration, mass or cuboidal cells of carried out
salivary gland below the ear, pain uniform size, Swiss
is seen fixation and cheese pattern
induration of (double layer of
tumor paresthesia tumor cells
may be seen arranged in duct
like pattern,
containing
Eosinophilic
coagulum at
center), cribriform
pattern,
neurotrophism
(spread via
perineural or
intranueral spaces
Mucoepidermoid Slow growing, It contain mucus Surgical excision
tumor painless swelling secreting,
having cystic epidermoid and
feeling, in some intermediated
cases rapid types of cells, it
growth, pain can be well
hemorrhage, differentiated (no
ulceration, cellular

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770 Textbook of Oral Pathology

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Disease Etiology Types Clinical features Pathological features Management
paresthesia occurs. pleomorphism)
Bony expansion, Poorly
facial nerve differentiated
paralysis, (cellular
pleomorphism,
pushing front)

Leukoplakia Tobacco, alcohol, Homogenous Non Homogenous Hyperorthokerati- Stoppage of habit,

candidiasis, homogenous (white patch nization, cryosurgery,
dietary deficiency, Cryptogenic having smooth or hyperparakerati- vitamin A,
syphilis, viral, Speckled or corrugated surface nization, antioxidant
hormonal nodular Ulcerative with irregular acanthosis, nuclear therapy
imbalance, chronic margin) Ulcerative hyperchromatism,
irritation, actinic (mixed red and cellular
radiation, white lesion with pleomorphism,
galvanism small keratotic poikilocarynosis,
nodules) Nodular dyskeratosis,
(maximum risk for enlarged nucleoli,
malignant dropshaped rete
transformation) pegs, baseloid
appearance,
Candidial hyphae,
Carcinoma in situ Severe stage of White plaque, Hyperkeratosis, on Surgical excision
epithelial ulcerated, eroded, surface of lesion, should be carried
dysplasia, or reddened area, keratin pearl out
resemble formation, loss of
leukoplakia or orientation, loss of
erythroplakia, polarity, basement
membrane intact,
Erythroplakia Heavy use of Homogenous Small, extensive, Features of Deep and wide
tobacco, alcohol Erythroleukoplakia red velvety lesion invasive surgical excision
Speckled with well defined epidermoid
margin, carcinoma, chronic
inflmamtory cell
infiltration,
reduction in
keratin production
and increase in
vascularity
Leukoedema Normal variant Diffuse translu- Thickening of No treatment
cent, grayish, epithelium with
white area, filmy mild parakeratosis,
appearance, enlarged spinous
wrinkled appear- cells with pyknotic
ance, when nuclei, rete peg are
stretched mucosal broad,
condition
disappear
Stomatitis nicotina Tobacco Red palatal Hyperparakeratosis, Complete
mucosa, few red acanthosis, ductal stoppage of oral
and dot like areas epithelium exhibits habits
surrounded by squamous

Contd...
 Differential Diagnosis of Lesion 771

Contd...
Disease Etiology Types Clinical features Pathological features Management
white keratotic metaplasia,
ring, ulceration can atrophic changes,
be seen, inflammatory cell
infiltration,
Oral, submucous Betel nut, red Burning sensation, Hyperkeratomized Intralesional
fibrosis chillies, nutritional vesicle atrophic injection of steroid,
deficiency, inflammatory epithelium, enzyme, systemic
immunological reaction flattening and steroid, injection of
factors, genetic xerostomia or shortening of rete placental extract
factors excessive pegs, cellular
salivation, atypia, nuclear
stiffening of oral pleomorphism,
mucosa, leathery increases mitosis,
feeling, trismus, basilar
blanched mucosa, hyperplasia, blood
difficulty in vessels dilated and
deglutition congested,
perivascular
fibrosis
Lichen planus Psychological Reticular Erosive Wickham striae Hyperorthokerati- Steroids, dapsone
stress, Plaque Atrophic present Reticular – nization, therapy
autoimmune Bullous raised thin white hyperparakerati-
reaction, radiating line, non- nization,
elevated, burning thickening of
sensation erosive – granular cell layer,
mixture of saw tooth
erythematous appearance,
ulcerated and liquefaction
white degeneration,
pseudomembranous civatte bodies
area, pain and (round ovoid
burning plaque – amorphous
raises or flattened eosinophilic
white area, bodies) are seen
atrophic – smooth
poorly defined
erythema area.
Bullous – large
vesicle are seen
Sialolithiasis Unknown stimuli Submandibular Stone is acellular, Can be removed
gland more amorphous, outer by digital
involved, margin aggregates manipulation,
intermittent pain microbial colonies, lithotripsy
which can be ductal lining
severe. Affected changed into
gland enlarged, stratified
stone can be squamous
palpated, epithelium,
Necrotizing Unknown etiology, Deep seated Absence of Lesion heal
sialometaplasia infraction of tissue, punched out epithelium, spontaneously
ulceration on hard necrotic debris,
or soft palate, coagulation of

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772 Textbook of Oral Pathology

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Disease Etiology Types Clinical features Pathological features Management
presence of grey necrosis, distended
granular lobules, cells, basophilic
numbness or nuclei, accumula-
burning pain, tion of mucin in
zone of necrosis,
Bacterial Streptococcus Acute Chronic Acute – sudden Chronic – acinar Antibiotics therapy
sialadenitis pyogenes, onset of painful atrophy of salivary
Staphylococcus swelling, fever, gland with
aureus redness of subsequent
overlying skin, fibrosis, dilatation
trismus, difficulty of the ductal
in swallowing, system,
parotid papilla hyperplasia of the
inflamed Chronic ductal epithelium,
– recurrent periductal fibrosis,
tendered swelling, chronic
duct orifice inflammatory cell
inflamed, infiltration
decreased salivary
flow
Mumps (endemic Cause by Parotid gland is Antiviral drugs
parotitis) paramyxovirus affected, eversion
of ear lobe, acute
pain during
salivation,
recurrent
exudation
Mikulicz’s disease Autoimmune Unilateral or Benign infiltration Steroid should be
disease, defective bilateral diffuse of lymphocytes, given
cell-mediated swelling. Soft myoepithelial
immunity, swelling, movable, islands.
fever, URI, Obliteration of
xerostomia, lumen of duct due
to proliferating
epithelial cells,
eosinophilic
material
Sjögren’s Immune mediated Primary (sicca Xerostomia, Infiltration of Steroids,
syndrome chronic syndrome) – no xerophthalmia and lymphocytes in antibiotics,
inflammatory other disease arthralgia. Severe intralobular ducts antifungal
response, presence Secondary – with tiredness, of salivary gland,
of serum arthritis disturbed taste atrophy of salivary
antinuclear sensation, dry gland acini,
antibodies, mucosa, red and hyperplasia of
atrophic tongue ductal epithelium,
mucosa, cobble myoepithelial
stone appearance islands,
(fissuring and
lobulation of the
surface)

Contd...
 Differential Diagnosis of Lesion 773

Contd...
Disease Etiology Types Clinical features Pathological features Management
Sialosis Hormonal Parotid gland Hypertrophy of Elimination of
disturbance, involved little serous acinar cells, causative organism
malnutrition, liver pain, discomfort, secretary granules
cirrhosis, chronic in cytoplasm and
alcoholism, lipomatosis may
diabetes occur
Ameloblastoma Arises from Peripheral type Slow growing, Plexiform – Enucleation should
odontogenic Admantinoma of painless, ovoid, continuous be carried out
epithelium. It may long bone fusiform bony anastomosing
be predispose by Mural hard swelling, strands, fish net
trauma, infection, ameloblastoma expansion and pattern, columnar
previous distortion of cell. Follicular –
inflammation cortex, gross facial discrete follicle,
asymmetry, egg island of fibrous
shell cracking, continuous strand,
pathological microcyst
fracture, formation.
Acanthomatous –
keratin pearls,
squamous
metaplasia.
Granular
cells – stellate
reticulum like cells
swollen with
coarse Eosinophilic
granules. Basal cell
– cuboidal shaped
in narrow strand
Adenomatoid Reduce enamel Maxillary anterior Odontogenic cells Surgical
odontogenic epithelium region, slow in duct like pattern enucleation should
tumors enlarging, bony within stroma be done
hard swelling, giving
elevation of upper adenomatoid
lip, expansion of appearance, small
jaw foci of calcification
is seen, solid nests
or rosette patterns
Calcifying Cells of stratum Mandible > Closely packed, Surgical
epithelial intermedium maxilla, Slow polyhedral cells, enucleation should
odontogenic tumor enlarging painless cribriform be done
(pindborg tumors) swelling, arrangement,
expansion, hyalinized stroma ,
distortion of oval shaped nuclei,
cortical plate, hyperchromatic
displacement of nuclei, prominent
teeth intercellular
bridges and
distinct cell
boundaries,
Lisegang ring
(calcified masses),
clear cells
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774 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
Squamous Remnants of Maxillary incisor- Irregularly shaped, Surgical
odontogenic tumor dental lamina, cells canine area, islands of well enucleation should
rests of Malassez, painless swelling, differentiated be done
and basal layer of mobility of teeth, squamous
oral epithelium local tenderness epithelium in
fibrous connective
tissue stroma,
round or oval
shaped islands,
microcyst
formation,
calcification is seen
Ameloblastic Mandibular Epithelial and Surgical excision
fibroma premolar-molar mesenchymal cells should be carried
area, slow are present, out
growing, painless, multiple sharply
distortion of defined strands or
cortical plate, islands bordered
displacement of by tall columnar
teeth, facial cells, cell free zone
asymmetry of hyaline
connective tissue
in epithelial
component,
Odontoma Occur after his- Complex – Complex – anterior Presence of Surgical
todifferentiation disorganized maxilla. Com- encapsulated mass enucleation should
but before dental tissue. pound – posterior of denticles, be done
morpho- Compound – mandible, small complex odontome
differentiation discrete tooth like asymptomatic presented as
structure lesion, expansion irregularly
of cortex, displaced arranged dental
teeth, tissue,
Odontogenic Derived from Peripheral type – Peripheral – slow Peripheral – mass Surgical excision
fibroma connective tissue extra-osseousely growing, of dense should be carried
of odontogenic Central – arises exophytic, well connective tissue out
epithelium within the jawbone circumscribed with spindle
growth, firm in shaped fibroblast,
consistency, surface epithelium
painless, slender rete pegs
interdental lesion project in CT, clear
cause separation of cells Central – thin
teeth Central – strands
slow growing, odontogenic, clear
painless swelling, cells, areas of
displacement of spherical or diffuse
teeth, cortical calcification and
expansion contain giant cells
Odontogenic Derived from Slow growing, Widely separated Surgical excision
Myxoma dental papilla or painless swelling, undifferentiated should be carried
follicular displacement of spindle or angular out
mesenchymal regional teeth, or stellate shaped
expansion of bone cells. Focal areas of
Contd...
 Differential Diagnosis of Lesion 775

Contd...
Disease Etiology Types Clinical features Pathological features Management
delicate immature
collagen fibrilar
strands, blood
vessels exhibits
hyalinization at
periphery
Periapical Mandibular Initial stage - No treatment is
cemental dysplasia anterior teeth, cemental tissue at required
females are apex of involved
affected, and replaced by
asymptomatic fibrous connective
small and tissue,
multiple, teeth are Cementoblastic
vital, radiologically stage—small
detected amorphous masses
immature
cementum Mature
stage – entire
fibrous tissue
replaced by
mature cemental
tissue
Familial Hamartomatous Slow growing Loose vascular Surgical osseous
gigantiform malformation of painless expansile tissue stroma, recontouring
cementoma cementum forming jaw swelling, delicate collagen
tissue autosomal multiple lesion fiber,
dominant trait seen , facial monomorphic
asymmetry can fibroblasts,
also occur acellular
cementum, ovoid
calcification
Cementoblastoma Arises from Mandible Large mass of Surgical excision
cementoblast >maxilla, slow amorphous should be carried
growing bony hard cemental tissue out
swelling, with presence of
expansion of reversel line, soft
cortical plate, and vascular
intermittent pain, connective tissue
dull sound when stroma, cemento-
tooth percussed, blasts or cemento-
clast are present,
multinucleated
cells are present
Odontogenic Intraosseous lesion Large swelling Islands or strands Radical surgical
carcinoma with expansion of of clear cells, cells excision should be
cortical plate, are glycogen rich, carried out
mobility of teeth non-clear cells
resembles dental
lamina, epithelial
tissue surrounded
by zone of
myxomatous tissue

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776 Textbook of Oral Pathology

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Disease Etiology Types Clinical features Pathological features Management
Odontogenic Arise from Initially Cystic cavity lined Surgical
keratocyst remnant of the asymptomatic, by keratinized enucleation or
dental lamina, large lesion stratified epithe- marsupialization
developing tooth produce facial lium, lining of the cyst can be
germ, basal layer asymmetry, pain epithelium is flat, carried out
of oral epithelium mobility and V shaped rete pegs
displacement of formation, there is
teeth, bony para-keratiniza-
expansion, tion, basophilic
paresthesia, nuclei, reverse
pathological polarity, daughter
fracture can occur, cyst or satellite
associated with cyst present
nevoid basal cell (multiple small
nevus syndrome micro cyst) present
Dentigerous cyst Cells of reduced Mandible Cystic cavity line Marsupialization
enamel epithelium >maxilla, of odontogenic should be carried
asymptomatic, epithelium, stroma out
slowly enlarging contain young
bony hard swelling fibroblast cells
occur, expansion of separated by
bone, Crepitus ground substance
sensation, facial rich in collagen
asymmetry, bundles, cystic
paresthesia and epithelium is flat,
pathological low columnar,
fracture may occur some cases bud
like proliferation
called mural
proliferation is seen
Radicular cyst Inflammatory Non-vital tooth, Cystic cavity line Root canal
origin asymptomatic and by non-keratinized treatment with
detected on stratified apical curettage
radiograph, squamous can be done. In
expansion occur in epithelium, larger lesion
larger lesion, pain localized area of excision should be
if secondarily increased cell done
infected, proliferation,
cholesterol cleft
(small ribbon
shaped cleft like
space) arcading
pattern can be
seen.
Inflammatory cell
infiltration
Eruption cyst Accumulation of Small fluctuant Ghost cells within No treatment is
fluid in follicular swelling on the lumen of cyst needed
space alveolar ridge,
eruption
hematoma occur
due to mastication,

Contd...
 Differential Diagnosis of Lesion 777

Contd...
Disease Etiology Types Clinical features Pathological features Management
Lateral periodontal Asymptomatic, Small cystic cavity Surgical excision
cyst small painless soft lined by should be carried
tissue swelling can connective tissue out
occur, mucosa is wall, on inner
pale in color, vital aspect by non-
tooth, keratinized
stratified
squamous
epithelium.
Thickening of
lining epithelium,
cells have pyknotic
nuclei
Gingival cyst of Cyst of dental Multiple Small keratin fill Not needed
new- born lamina (along asymptomatic, cystic cavity line
alveolar ridge, small discrete by flattened
odontogenic white nodule, It epithelium
origin) Epstein undergo
pearls (along spontaneous
midpalatine raphe) regression
Bohn’s nodule (at
junction of hard
and soft palate)
Gingival cyst of Arise from Firm compressible, Cystic cavity lined Surgical excisions
adult gingival soft tissue, fluid filled by a thin should be carried
cell rest of dental swelling, well epithelium made out
lamina circumscribed, up of flat or
smooth surface cuboidal cells.
and bluish or pyknotic nuclei
normal in color with perinuclear
cytoplastic
vacuoles

Sialoodontogenic Remnant of dental Slow enlarging Line by thin Surgical

cyst lamina asymptomatic squamous enucleation should
growth in epithelium with be carried out
mandibular focal area of
anterior region thickening,
microcyst is also
found,
organization of
glandular elements
may results in
acinar like cluster
Botyroid Well defined, Multiple cystic Surgical excision
odontogenic cyst painless expansile cavities separated should be carried
jaw lesion from one another out
by fibrous septa.
Line by cuboidal
or squamous
epithelium

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778 Textbook of Oral Pathology

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Disease Etiology Types Clinical features Pathological features Management
Calcifying Bony hard Cystic cavity lined Surgical excisions
epithelial extensive swelling by odontogenic should be carried
odontogenic cyst of the jaw, keratinized out
expansion and epithelium, cells
distortion of are columnar or
cortical pates, cuboidal, ghost
tooth vital, cells are
perforation of the eosinophilic,
cortex satellite microcyst
Paradental cyst Inflammatory in Seen in Cystic cavity line Surgical excision
origin, cell rests of mandibular third by hyperplastic, should be carried
Malassez or molar and tooth non-keratinized out
reduced enamel has history of squamous
epithelium pericoronitis epithelium, intense
inflammatory
reaction
Globulomaxillary Proliferation of Asymptomatic, Cystic cavity line Surgical removal
cyst epithelium along pain when by stratified or should be done
the line of fusion secondary pseudo- stratified
between maxilla infected, small ciliated columnar
and premaxilla swelling in canine epithelium chronic
region and tooth is inflammatory cell
vital infiltration
Nasolabial cyst Lower part of Small painless swel- Cystic lumen Surgical excision
(Kelstadt’s cyst) embryonic ling of upper lip, supported by should be done
nasolacrimal duct obliteration of connective tissue
nasolabial fold, can wall, line by
project into floor of pseudo-stratified
nose, ciliated columnar
epithelium,
infolding of cystic
lining
Nasopalatine duct Proliferation and Small painful Cystic lined Surgical excision
cyst cystic degeneration swelling in midline ciliated columnar
of epithelial of anterior part of or non- keratinized
remnants after hard palate, stratified
closure of pressure sensation squamous
embryonic on floor of the epithelium, mucus
nasopalatine duct nose, displacement secretory cells,
of root of central presence of
incisor, salty pigment, presence
discharged, of neurovascular
fluctuation can be bundles,
done
Traumatic bone Pseudocyst Painful bony hard Cystic cavity Surgical
cyst swelling, surrounded by exploration should
paresthesia of lip, loose vascular be done
expansion of connective tissue
cortical plate, wall, no epithelial
displacement of lining, CT stroma
teeth, vital teeth is made of fibrous
tissue

Contd...
 Differential Diagnosis of Lesion 779

Contd...
Disease Etiology Types Clinical features Pathological features Management
Aneurysmal bone Enlarging diffuse, Multiple blood Surgical curettage
cyst firm swelling, filled spaces line should be done
facial asymmetry, by spindle shaped
swelling pulsatile, cells or flat
egg shell cracking, endothelial cells,
pathological epithelial absent,
fracture, profuse cystic spaces
bleeding, separated by loose
paresthesia, connective tissue
difficulty in wall, multiple
opening mouth multinucleated
giant cells
Mucocele Accumulation of Mucous retention Lower lip Mucus retention – Surgical excision
saliva due to cyst involved, small small cystic cavity should be carried
obstruction Mucous raised lesion and filled with mucous out
extravasations cyst bluish swelling, line by flattened
round to oval epithelial cells
shaped smooth Mucous
fluctuant extravasations –
cystic cavity
surrounded by
compressed
connective tissue
wall. Mucus
stroma
Ranula Obstruction of the Soft fluctuant Large mucous Surgical excision
duct, compression unilateral swelling filled area should be carried
of duct, in floor of mouth, surrounded by out
perforation of duct bluish translucent connective tissue
appearance like wall, mucous filled
frog belly area
Dermoid cyst Remnant of Painless swelling Cystic cavity lined Surgical excision
embryonic skin with doughy by orthrokera- should be carried
consistency, tinized stratified out
elevation of squamous
tongue, midline epithelium
location, exhibiting hair
follicle, sebaceous
gland, desqua-
mated keratin, cyst
capsule consist of
narrow zone of
compressed
connective tissue
Focal reversible Chronic carious Tooth sensitive to Acute inflamma- Elimination of
pulpitis lesion, stimuli of thermal changes, tory reaction in causative factor,
short duration, pain of short odontoblastic pulp capping
chemical irritation, duration, vitality regions, dilatation should be done
severe attrition or test is positive of pulpal blood
abrasion vessels, edema in
pulp with
infiltration by

Contd...
780 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
polymorphonuclear
leukocytes,
thrombosis of
pulpal blood
vessels,
Acute pulpitis Caries reaching Tooth is extremely Severe edema with Direct pulp
pulp, pulp sensitive, vasodilatation, capping, drainages
exposure by cavity lacinating pain, dense infiltration of pus, root canal
preparation , percussion test of polymorpho- treatment should
trauma to teeth, positive, history of nuclear leukocytes, be done
chemical irritation, night pain, pain destruction of
cracked tooth subsided after odontoblasts cells
syndrome, drainage at pulp dentin
established. border, microab-
scess formation
Chronic pulpitis Same as acute Intermittent dull or Cellular infiltration Extraction of tooth
pulpitis throbbing pain, by lymphocytes, or root canal
tooth is less plasma cells and treatment should
sensitive to pain as macrophages, be done
compared to acute blood capillaries
pulpitis are prominent,
fibroblastic
activity, formation
of collagen
bundles
Pulp polyp Intense Small pinkish red, Numerous Root canal
(chronic proliferation of lobulated mass proliferating treatment
hyperplastic pulpal connective protruding from fibroblasts and
pulpitis tissue due to low pulp chamber. young blood
grade infection Large open carious capillaries,
cavity, lesion bleed inflammatory cell
profusely upon infiltration, by
provocation, tooth plasma cells and
is painless lymphocytes,
stratified
squamous
epithelial lining
Acute apical Results of Moderate pain, Restoration of
periodontitis extension of pulpal sensitivity, tooth with proper
inflammation extrusion of tooth, antibiotics
severe pain, coverage
tenderness
positive,
Periapical Response to Percussion test Granulation tissue Root canal
granuloma infection, occlusal positive, mild pain, mass consist of treatment with
trauma discomfort, proliferating apicotomy
fibroblasts,
endothelial cells,
immature blood
capillaries, chronic

Contd...
 Differential Diagnosis of Lesion 781

Contd...
Disease Etiology Types Clinical features Pathological features Management
inflammatory cells,
cholesterol cleft,
foam cells
Osteomyelitis Infection of dental Acute suppurative Acute suppurative Acute suppurative Antibiotics,
pulp, infected Acute – severe pain, – bone marrow sequestrectomy,
granuloma, subperiosteal diffuse large undergo analgesic,
infected cyst, Chronic swelling, excessive liquefaction, hyperbaric oxygen
compound suppurative salivation, bad thrombosis of therapy should be
fracture, post Chronic diffuse breath, multiple blood vessels, given
radiation sclerosing sinus, paresthesia acute
secondary Chronic focal of lip. Chronic inflammatory cell
infection sclerosing suppurative – pain infiltration,
is mild and dull, Brodie’s abscess
jaw swelling, sinus can be seen.
formation. Chronic
Focal sclerosing – suppurative –
asymptomatic, accumulation of
tooth with large exudates and pus,
carious lesion. lymphocytes,
Diffuse sclerosing plasma cells,
– asymptomatic, macrophages,
vague pain, foul osteoblastic and
taste, acute osteoblastic
exacerbation may activity producing
occur producing reversal line. Focal
mild pain and sclerosing –
fistula tract presence of dense
formation mass, without
marrow tissue,
fibrotic marrow.
Diffuse sclerosing
– formation of
dense irregular
bone with
hypocellular
fibrous stroma,
reversal line and
resting line is seen
Garre’s Low grade Carious non-vital Multiple osteoid, Elimination of
osteomyelitis infection or trauma tooth (lower first primitive bony causative agents
molar), slight tissue, osteoblastic with extraction of
tenderness or activity is involved tooth
vague pain can be prominent,
present marrow spaces
contain patchy
area of chronic
inflammatory cell
infiltration
Cellulitis Virulent bacteria Large diffuse, Fibrin and serum Antibiotics and
like Streptococcus painful swelling fluid in tissue, removal of
pyogenes and over the face or separation of primary factors
bacteroides, neck with facial periosteum and

Contd...
782 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
osteomyelitis, asymmetry, muscle, acute
infected post- swelling is firm, inflammatory cell
extraction wound brawny, fever, infiltration,
chill, regional
lymphadenopathy,
trismus,
Ludwig’s angina Causative Large diffuse Acute High dose of
organism board like swelling inflammatory cell antibiotics
hemolytic in floor with infiltration
streptococci brawny
Periapical, induration,
pericoronal or elevation of
periodontal tongue, no pitting
infection, gunshot on pressure,
injury, speech difficulty,
osteomyelitis

Hand-Schuller- Replacement of Exophthalmos, Multiple large Surgery and

Christian disease marrow by diabetes insipidus, vacuolated foam chemotherapy
macrophages skins rashes, otitis cells, small non- should be given
media, facial vaculolated cells,
asymmetry,
ulceration and
necrosis of oral
mucosa, loosing of
teeth and halitosis
Eosinophils Fever malaise, Numerous Surgical curettage
granuloma headache and proliferating
anorexia, localized histiocytes, in
pain, tenderness, diffuse sheets,
gingival soft tissue eosinophils is seen,
swelling multinucleated
giant cell present
Letterer-Siwe Hepatosplenomegaly, Marked Poor prognosis
disease lymphadenopathy, proliferation of
ecchymosis of skin, non-lipidized
mucosal histiocytes
ulceration, gingival
hyperplasia
Hyper- Adenoma Primary Fatigue, weakness, Osteoclastic Excision of
parathyroidism hyperplasia of Secondary polyuria, thirst, resorption of bony parathyroid tumor
parathyroid gland depression, loss of trabeculae, areas of
memory, peptic excessive
ulcer, bone pain, hemorrhage and
loosening of teeth hemosiderin
and fracture of jaw pigmentation,
bone brown tumor
(tissue takes brown
color), multiple
multinucleated
giant cells

Contd...
 Differential Diagnosis of Lesion 783

Contd...
Disease Etiology Types Clinical features Pathological features Management
Paget’s disease Inflammatory Deep aching bone Osteoclastic bone Administration of
reaction, with bilateral resorption, bone calcitonin, surgery
circulatory symmetrical replaced by highly in severe cases
disturbance, swelling, vascularized
defective deformity of bone cellular connective
connective tissue in stress bearing tissue, bone
mechanism, area, headache, marrow replaced
autoimmune deafness, blindness by fibrous stroma,
disorders occur due to reversal and
narrowing of skull resting line, mosaic
foramina, bowing pattern seen in the
of leg, waddling bone, chronic
gait, diastema, inflammatory cells
loosening of teeth, and dilated blood
difficulty in lip capillaries
closure,
pathological
fracture of bone
Fibrous dysplasia Can be Monostotic Slow enlarging, Highly cellular, Growth cease
developmental or Polyostotic (Jaffe’s painless, unilateral proliferating, well after puberty,
cause by liver type and swelling, facial vascularized surgical
damage, glandular Albright’s asymmetry, fibrilar connective recontouring
dysfunctions, syndrome) expansion and tissue, spindle
trauma gradual distortion shaped fibroblasts
of cortical plate, arranged in
displacement of whorled pattern.
teeth, Chinese latter
malocclusion, in pattern seen,
Albright’s spheroidal areas of
syndrome café-au calcification, lesion
lait spot, and other blends with
endocrine surrounding
abnormalities can normal bone.
be seen Remodeling of
woven bone to
lamellar bone
Cherubism It can occur due to Bilateral Pain-less Whorled pattern Self limiting
latent symmetric cellular connective disease
hyperparathyroidism, swelling, giving tissue stroma,
hormone rise to chubby face, proliferating
dependent eyes raised to fibroblasts,
neoplasm, trauma, heaven look, multinucleated
familial pattern increases cheek giant cells,
fullness, widening eosinophilic
of alveolar ridge, perivascular
lymphadenopathy, cuffing of collagen
premature fibers, extravasated
exfoliation of teeth, RBC, hemosiderin
pigments,
Osteogenesis Defective matrix Neonatal lethal Multiple fracture Thinning of cortex, No treatment is
imperfecta formation, cross type of bone, immature woven possible
linking of adjacent Sever non lethal generalized body bone, short, thin,
molecule type Moderate and deformity, fragile bony

Contd...
784 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management
deforming type dwarfed stature, trabeculae,
Mild and non blue sclera, increased
deforming type deafness, deafness, osteoblasts,
defective heart osteoclasts
valves
Cleidocranial Hereditary Absence of No treatment is
dysplasia autosomal clavicle, shoulder possible
dominant trait can meet in
midline, nose is
flat, wide,
hypoplastic
maxilla, delayed
closure of
fontanels, high and
narrow arched
palate
Osteopetrosis Genetic defect Autosomal Increased Dense and sclerotic No treatment is
(marble bone dominant tendency of bone with required
disease) Autosomal development compensatory
recessive severe remodeling.
osteomyelitis, Medullary cavity is
anemia, small and little
thrombocytopenia, amount of marrow
leucopenia, tissue. Osteoblast
deafness, are present
blindness, facial
paralysis, frontal
bossing, lone
bones shortened,
spontaneous
hematoma
Infantile cortical Rapidly bilateral Edema and No treatment is
hyperostosis symmetrical thickening of required
mandibular periosteum with
swelling, deep apposition of many
seated tendered thin bony
soft tissue trabeculae parallel
swelling, to each other
dysphagia,
pseudo-paralysis
anemia occurs.
Massive osteolysis Replacement of Progress rapidly, Foci of resorption, Radiation therapy
bone by fibrous pain in bone, bone is replaced by can be given
tissue pathological fibro-vascular
fracture occur, connective tissue
facial asymmetry showing chronic
inflammatory cell
infiltration
Osteoarthritis Degenerative Clicking sounds Vertical or Analgesics, anti-
disease, aging while opening and horizontal crack on inflammatory
process or trauma closing the articular drugs should be
movements. cartilage, cartilage given
Contd...
 Differential Diagnosis of Lesion 785

Contd...
Disease Etiology Types Clinical features Pathological features Management
Limitation of less elastic,
movement. Pain elevation of disc
surface called
lipping,
degeneration of
chondrocytes
Psoriasis Genetically Painless, dry white Atrophy with Not specific
determined scaly patches, Hyperparakeratosis,
patch are well absence if granular
circumscribed, cell layer, clubbing
erythematous, of rete pegs,
sterile pustule, intraepithelial
Auspitz’s sign microabscess
(tiny bleeding formation (monro
point) oral cavity abscess), increased
lesion are well mitotic activity,
defined, grayish mild lymphocyte
white or yellowish cell infiltration
patches
Erythema Can be Rapidly Acanthosis, intra Topical and
multiforme precipitated by developing or intercellular systemic steroid
tuberculosis, erythematous edema and therapy is given
herpes simplex, macules, papules, necrosis of the
infectious bulls eye or target epithelium, sub
mononucleosis, lesion (concentric epithelial
hyperimmune erythematous connective tissue
reaction rings separated by shows edema and
ring of near perivascular
normal color on infiltration of
skin), vesicle are lymphocytes and
eroded or macrophages
ulcerated bleed
profusely, foul
smell in mouth
White sponge Hereditary Asymptomatic Mild to moderate Self regressing
nevus white folded areas hyper-para
in the mucosa, oral keratosis,
lesion are soft and acanthosis,
spongy and intercellular
peculiar opalescent edema, vacuolated
hue, surface show cells in the spinous
area of cell layer having
desquamation pyknotic nuclei,
mild inflammatory
cell infiltration
Pemphigus Autoimmune P vulgaris P vulgaris – Suprabasilar split Steroid
mechanism P vegetans rapidely occur due to Antibiotics to
P foliaceous developing formation of control infection
P erythematosus vesibel, rupture vesicle or bullae,
bullae are painful, basal layer of row
bleeds profusely, of tomb stones,

Contd...
786 Textbook of Oral Pathology

Contd...
Disease Etiology Types Clinical features Pathological features Management

Nikolsky’s sign loss of intercellular

positive, orally bridges, disruption
bleb like blister are of prickle cells,
seen Tzank cells are
P vegetans – found, acanthosis
papillomatous in spinous cell
hyperplasia layer
following rupture,
cerebriform tongue

Cicatricial Mild erosion or Extracellular Systemic steroid

pemphigoid desquamation edema, therapy
gingival tissue, vacuolation in
erythematous basement
lesion, mucosal membrane zone,
bullae are tense, formation of
tough, after subepithelial
rupture painful vesicles or bullae,
eroded or inflammatory cell
ulcerated area infiltration present,
Nikolsky’s sign is blood vessels
positive. Heal by dilated
scar formation,
corneal ulceration,
scaring results in
adhesion in
conjunctiva
Bullous Ulceration and Same as cicatricial Same as above
pemphigoid heal without type
scarring, skin
lesion as red
eczematous plaque

Epidermolysis Acquired type is EB simplex Multiple vesicle Destruction of Systemic steroid

bullosa caused by multiple EB dystrophic and bullae on basal or suprabasal therapy
myeloma, diabetes Junctional EB pressure area, after layers of the oral
mellitus, TB, EB acquista rupture raw epithelium,
amyloidosis painful ulcers resulting in
which heal with formation of
scarring, nail vesicle or bullae
sheds, decreased
mouth opening
due to scarring,
delayed eruption
and increased
periodontal
disease

Contd...
 Differential Diagnosis of Lesion 787

Contd...
Disease Etiology Types Clinical features Pathological features Management

Lupus Autoantibodies Systemic LE SLE – skin lesion SLE – atrophy with Systemic steroid
erythematosus and immune Discoid LE in butterfly hyperkeratinization, therapy should be
complex configuration over liquefactive given
malar region, degeneration,
itching, burning, edema of
hyperpigmentation, subepithelial
loss of hair, connective tissue,
xerostomia, lymphocyte
mucosal petechiae infiltration
DLE – elevated red fibrinoid
purple macule, degeneration
covered by yellow DLE – hyperpara-
or gray scale, keratinization,
butterfly atrophic
distribution, carpet epithelium, keratin
track appearance, plugging,
enlarge at acanthosis,
periphery pain and pseudoepithe-
burning of oral liomatous
mucosa hyperplasia,
perivascular
lymphocytes
infiltration

Tabular presentation of tooth tissue loss

Term Definition Clinical Appearance
Attrition Loss by wear of surface of tooth or • Matching wear on occluding surfaces.
restoration caused by tooth to tooth • Shiny facets on amalgam contact.
contact during mastication or • Enamel and dentin wear at the same rate.
parafunction • Possible fracture of cusps or restorations.
• Usually located at cervical areas of teeth.
Abrasion Loss by wear of dental tissue caused by • Lesions are more wide than deep.
abrasion by foreign substance (e.g., • Premolars and cuspids are commonly affected.
toothbrush, dentifrice) • Broad concavities within smooth surface enamel.
Erosion Progressive loss of hard dental tissue by • Cupping of occlusal surfaces, (incisal grooving) with dentin
chemical processes not involving exposure.
bacterial action • Increased incisal translucency, wear on non-occluding
surfaces and raised amalgam restorations.
• Clean, non-tarnished appearance of amalgams.
• Loss of surface characteristics of enamel in young children.
• Preservation of enamel "cuff" in gingival crevice is
common.
• Hypersensitivity and pulp exposure in deciduous teeth.
Abfraction Loss of tooth surface at the cervical areas • Affects buccal/labial cervical areas of teeth.
of teeth caused by tensile and • Deep, narrow V-shaped notch.
compressive forces during tooth flexure • Commonly affects single tooth with excursive interferences
or eccentric occlusal loads.
788 Textbook of Oral Pathology

Important bacterial infections

Lesion and causative Clinical features Pathognomic / Characteristic oral Diagnosis
organism features
Scarlet fever Tonsillitis, Pharyngitis, fever, vomiting, Stomatitis scarletina Culture
B-hemolytic skin rash, bright scarlet on 2nd or 3rd Tonsillitis with grayish exudates Dick test
streptococci day due to injury to endothelium and “Strawberry tongue” and “raspberry
dilatation of blood capillaries. tongue”
Diphtheria Skin and mucous membrane lesions. Leathery, grayish white “pseudo- Staining by Alberts
Corynebacterium Mucous membrane lesions are found on membrane”. It is formed by dead stain and Ponder’s stain
diphtheriae larynx, pharynx and tonsils.,conjunctiva. epithelial cells, coagulated fibrin and and Neisser’s stain.
Hoarseness of voice, respiratory stridor purulent exudates. “Bull neck” Swelling Culture on Loeffler’s
and dyspnea may lead to airway of neck due to striking cervical serum. Tellirite agar is
obstruction in children. lymphadenopathy. selective media.
Eleck’s test Schick test
Tuberculosis Persistent cough with or without Oral tuberculous ulcers, more commonly Staining by Ziehl-
Mycobacterium haemoptysis, gradual weight loss, found on tongue, are irregular superficial Neelsen stain Culture
tuberculosis cervical, axillary and mediastinal or deep painful ulcers which tend to by Lowenstein-Jensen
Acid fast bacilli lymphadenopathy. Tuberculous increase in size. Tuberculoma- media, Loefller,s se-
lymphadenitis (Scrofulla), painfull Tuberculous periapical granuloma rum slope. Tuberculin
lymph nodes, with perforations and test (Mantoux test)
crustations, Cold abscess. Lupus
vulgaris is primary tuberculosis of skin
especially of face leading to formation
of papular nodules which may ulcerate.
Miliary tuberculosis occurs due to
lymphatic dissemination.
Leprosy It affects skin, peripheral nerves upper Lepromatous nodules tongue lips and Slit skin smears stained
Mycobacterium respiratory tract. Hypopigmented hard palate. Gingival hyperplasia with with Z-N stain. Lep-
leprae patches with partial or complete loss of loosening of tooth. romin test
sensation. Two types tuberculoid
(Skin)and lepromatous (Nerves)types-It
produces disfigurement like loss of
fingers, toes, (claw toe) Nasal
depression. May cause sudden death.
Actinomycosis Cervicofacial is Most common. It Involvement of maxilla and mandible Grams staining, Cul-
Actinomycosis involves face neck tongue and may lead to actin my osteomyelitis. Pus ture of sulphur gran-
isreali, neaslundi, mandible. Organisms may enter oral contains sulphur granules. When ules
viscosus, mucosa producing swelling and stained with gram stain they show “sun
odontolyticus and induration of the tissues. It produces ray appearance”.
proprionica abscess which opens onto skin surface.
Other areas involved are pulmonary
and abdominal cavity.
Tetanus It enters the wound and produce toxins Lock jaw-Spasm of masseter muscle Grams staining Cul-
Clostridium tetani Tetanospasmin in anaerobic conditions. leads to trismus. Dysphagia, laryngeal ture in Robertsons’s
It affects the synapse of motor spasms may lead to asphyxia. Risus cooked meat medium
interneurons causing sever muscle sardonicus or Grimace-sustained and blood agar. Direct
spasms. Opisthotonus-Arched back contraction of facial muscles. immunofluorescence.
due to contraction of back muscles.

Contd...
 Differential Diagnosis of Lesion 789

Contd...
Lesion and causative Clinical features Pathognomic / Characteristic oral Diagnosis
organism features
Syphilis Primary- (3day to 3 months) Chancre. Primary-Chancre on lips, palate, Direct examination by
Treponema A lesion that develops at site of gingival and tonsils Secondary-Mucous dark field microscopy
pallidum inoculation. Secondary- (6 weeks after patches (Snail track ulcers)-Multiple Culture not possible
primary) Skin-Macular papular grayish white plaques overlying over an Wasserman’s test,
painless lesions Painless macules and ulcerated surface. Seen on tongue, VDRL test, Kahn test,
papules Tertiary- Gumma is focal gingival and buccal mucosa. Tertiary Fluorescent trepone-
granulomatous lesion with central syphilis-Gumma of tongue and palate mal antibody absorp-
necrosis. CVS and CNS involvement is and produce a deep painless ulcer and tion test and T.pallidum
seen Congenital-Transmission of palatal perforation. Syphilitic glossitis hemagglutination as-
infection to child by mother Frontal Congenital: Short maxilla High arched say.
bossing, saddle nose, irregular palate, Hutchinson’s triad-Hypoplasia of
thickening of supraclavicular joint incisor and molar teeth (Mulberry molar,
(Hogoumenaki’s Sign) Saber shin Moon’s molar, Fournier teeth, Screw
driver shaped incisors) Eight nerve
deafness, and interstitial keratitis of eyes

Important viral infections

Infection Incubation Clinical features Oral manifestations
Herpes simplex 2-26 days Herpetic eczema, herpetic conjunctivits, herpetic Herpetic stomatitis herpes labialis,
Primary and meningoencephalitis and disseminated herpes vesicular lesions rupture and form
secondary simplex of the newborn. Herpetic whitlow. shallow ragged extremely painful
Prodromal symptoms include fever headache ulcer covered by grey membrane
Bodyache surrounded by erythematous
halo. Heal spontaneously with in
7-14 days without scar.
Herpangina 2-10 days Sore throat cough, low grade fever, rhinorrhoea tonsillar small vesicles that
Coxsackie group A rupture to form crops of ulcers.
virus Causes dysphagia.
Measles 8-12 days Acute contagious dermatropic infection Koplick’s spots- These occur 2-3
affecting children. Fever, cough, conjunctivitis, days of cutaneous rashes. They are
photophobia, lacrymation and eruptive lesions small irregular spots bluish white
of skin and mucous membranes. Lesions are tiny specks surrounded by bright mar-
red macules or papules which coalesce to form gins. Palatal pharyngeal petechiae,
large lesions. gingival ulceration congestion of
throat may occur.
Chickenpox 2 weeks It is a primary varicella zoster infection. It is Small blister like lesions on buccal
Varicella zoster acute, extremely contagious disease occurring mucosa tongue gingival and
in children. It is characterized by exanthematous palate. Lesions rupture to form
vesicular rash with headache, fever anorexia. eroded ulcers.
Lesions occur on trunk face and extremities.
Lesion rupture to form a superficial crust and
heal by desquamation.
Varicella Zoster Reactivation of Extremely painful lesion due to inflammation Extremely painful, unilateral
chickenpox is of dorsal root ganglia. Fever pain tenderness vesicles which form ulcers are
called as zoster along the course of involved sensory nerves. It found on buccal mucosa tongue
is characteristically dermatomic and unilateral uvula and pharynx and larynx.
in distribution.
Mumps 14-18 days It is an acute contagious viral infection of children. Unilateral and bilateral swellings
Epidemic parotitis of the salivary glands usually parotid glands. Swellings are usually rubbery in
consistency producing pain on mastication. Fever, pain below the ear. Duct of
parotid gland becomes puffy and red.
790 Textbook of Oral Pathology

Comparison of different forms of gingivitis

Form of gingivitis Clinical features Contributory factors Significance
Marginal gingivitis Erythema, edema, tenderness None beyond the poor oral hygiene Superficial, resolve quickly
limited to the marginal gingiva and relatively short duration of the with plaque formation with
and interdental papillae; younger process no permanent defect
patient
Hyperplastic gingi- Bulbous, edematous enlarge- Cause by poor oral hygiene, hormonal Deeper, hyperplastic
vitis ment of gingiva, may be focal or or medication response resolve with plaque
generalized; ‘boggy’ and pocket removal but enlargement
depth is increase persist
Chronic gingivitis Pale, fibrotic appearance of Chronic inflammation produce a Inflammation of the
gingiva, loss of stippling, edema, recurring cycle of active inflammation crevicular pockets can be
exudate and hemorrhage from and reparative fibrosis controlled by plaque removal
the sulcular surface on probing, if pockets can be kept clean
increase pocket depth
Desquamative gin- Erythematous and atrophic ap- Autoimmune conditions such as Topical corticosteroid appli-
givitis pearance without enlargement; lichen planus and cicatrical pemphig- cation during episode of in-
pain and sloughing of surface oid crease severity usually re-
epithelium are characteristic quired to maintain symptom-
atic control
ANUG Severe pain, fetid odor, punched Compromise host resistance to Rapid response to improved
out papilla, pseudomembranous infection status of the host, antibiotics
ulceration, exudate and therapy and superficial
erythema; usually most sever in debridment of the tissue
anterior region
Pregnancy Indistinguishable from hyper- Pregnancy, puberty, hormonal Improved hygiene and oral
gingivitis plastic gingivitis, except that then fluctuation may be contributory prophylaxis usually yields
erythematous enlargement is gen- significant improvement by
eralized and the patient history minimizing the inflammatory
component of the process
Hemorrhagic Edematous enlargement and Associate with bleeding disorders, Diagnostic sign of the caus-
gingivitis erythema may be dramatic, but scurvy, leukemia and hemopoietic ative condition; improvment
the consistent feature is dramatic suppression; may become severe is usually dramatic following
hemorrhage after even slight resolution of systemic condi-
pressure on the tissue tion
Uremic gingivitis Painful, erythematous gingiva as Associated with renal failure Often persist to some degree
well as odor of ammonia and despite local plaque control
excessive salivation
Idiopathic Evidence of inflammation persist Failure to respond to normally Indicates the need for addi-
gingivitis following eliminations of plaque effective treatment usually suggest tional diagnostic evaluation
and calculus, as well as improved diminished host resistance or other to identify the underlying
hygiene contributory systemic disease that has contributory condition
not been diagnosed
 Differential Diagnosis of Lesion 791

Enamel pathology

A. Developmental C. Enamel caries

• Amelogenesis imperfecta • Pit and fissure
• Dens invaginatus • Smooth surface
• Enameloma • Caries at cementoenamel junction
B. Environmental pathology D. Pigmentation
• Attrition • Endogenous
• Abrasion • Exogenous
• Erosion E. Enameloma

Dentin pathology

A. Developmental C. Neoplastic
• Dentinogenesis imperfecta • Dentinoma
• Dentinal dysplasia • Odontoma
• Regional odontodysplasia D. Regressive changes
• Dentin hypocalcification • Secondary dentin
B. Dentinal caries • Dentinal sclerosis

Leukopenia
I. Infections • Aleukemic leukemia
A. Bacterial • Agranulocytosis
• Typhoid III. Chemical agent
• Paratyphoid fever A. Agents commonly producing leukopenia in all patient
• Brucellosis if given in sufficient dose
• Tularemia (early) • Mustards (sulfur and nitrogen mustards)
B. Viral and rickettsial • Urethane
• Influenza • Busulfan
• Measles • Benzene
• Rubella • Antimetabolites
• Chicken pox B. Agents occasionally associated with leukopenia
• Infectious hepatitis apparently as result of individual sensitivity
• Colorado tick fever • Analgesics, sedative and anti-inflammatory
• Dengue • Antithyroid drug
• Yellow fever • Anticonvulsant
C. Protozoal • Sulfonamides
• Malaria • Antihistamine
• Relapsing fever • Antimicrobial agents
• Kala azar • Tranquilizers
D. Any overwhelming infection IV. Physical agents
• Miliary tuberculosis • X-ray radiation and radioactive substance
• Septicemia V. Anaphylactic shock and early stages reaction of foreign
II. Hemopoietic disorders protein
• Gaucher’s disease VI. Disease of unknown etiology
• Pernicious anemia • Liver cirrhosis
• Aplastic anemia • Disseminated erythematosus
• Chronic hypochromic anemia • Cyclic neutropenia
792 Textbook of Oral Pathology

Basophilia
I. Blood disorders III. Infection
• Chronic myelocytic leukemia • Chronic inflammation of accessory tissue
• Chronic anemia • Small pox
• Hodgkin’s disease • Chicken pox
II. Splenectomy IV. Myxedema
V. After injection of foreign proteins
VI. Some cases of nephrosis

Neutrophilia

A. Acute infection D. Acute hemorrhage

• Coccal E. Acute hemolysis
• Bacilli F. Malignant tumor of
• Fungi • Gastrointestinal tract
• Spirochetes • Liver
• Virus • Bone marrow
• Rheumatic fever G. Blood disorders
• Diphtheria • Myelocytic leukemia
• Small pox • Polycythemia
B. Inflammatory • Myelofibrosis
• Coronary thrombosis • Myeloid metaplasia
• Gout • Chronic idiopathic neutropenia
• Collagen vascular disease • Hereditary neutrophilia
• Burns H. Miscellaneous
• Hypersensitivity reaction • Physiologic in the newborn
C. Intoxication • During labor
• Uremia • After repeated vomiting
• Diabetes acidosis • Convulsion
• Poisoning by chemical and drugs like • Paroxysmal tachycardia
lead, mercury, digitalis, insect venoms, • After epinephrine injection
black widow spider

Eosinophilia
A. Allergic • Hodgkin’s disease
• Bronchial asthma • Pernicious anemia
• Urticaria E. Infection
• Angioneurotic edema • Scarlet fever
• Hay fever • Chorea
• Allergic rhinitis • Erythema multiforme
• Drug sensitivity F. Malignant disease of any type
B. Skin disease G. Following irradiation
• Pemphigus H. Miscellaneous
• Demits herpetiformis • Pulmonary infiltration with eosinophilia
• Bullous pemphigoid • Tropical eosinophilia
C. Parasitic infection • Polyarteritis nodosa
• Trichinosis • Rheumatoid arthritis
• Echinococcosis disease • Sarcoidosis
D. Blood disorders • Certain poison
• Chronic myelocytic leukemia I. Inherited
• Polycythemia Vera J. Idiopathic
 Differential Diagnosis of Lesion 793

Lymphocytosis
A. Acute infection C. Lymphocytic leukemia
• Infectious mononucleosis D. Lymphosarcoma
• Acute infectious lymphocytosis E. Heavy chain disease
• Infectious hepatitis F. Hemopoietic disorders
B. Chronic infection • Neutropenia
• Tuberculosis • Exanthema
• Secondary and congenital syphilis
• Undulant fever

Monocytosis
A. Bacterial infection • Hodgkin’s disease
• Tuberculosis • Multiple myeloma
• Subacute bacterial endocarditis D. Lipid storage disease
• Syphilis • Gaucher disease
• Brucellosis E. Malignant neoplasm
• Typhoid • Carcinoma of ovary, breast and stomach
B. Protozoan and rickettsial infection F. Collagen vascular disease
• Malaria • Lupus erythematosus
• Rocky Mountain spotted fever • Rheumatoid arthritis
• Typhus G. Granulomatous disease
• Kala azar • Sarcoidosis
• Trypanosomiasis • Ulcerative colitis
• Oriental sore • Regional arteritis
C. Blood disorders H. Chronic high dose steroid therapy
• Lymphoma
• Leukemia

Peripheral plasmocytosis

I. Infection B. Antitoxins
A. Viral • Equine tetanus
• Rubella • Equine diphtheria
• Rubeola III. Neoplasm
• Varicella A. Hematological
• Infectious mononucleosis • Plasma cell leukemia
B. Bacterial • Chronic lymphocytic leukemia
• Streptococcal B. Non hematological
• Diplococcal • Breast
• Syphilis • Prostate
• Tuberculosis IV. Miscellaneous
C. Protozoal • Transfusion
• Malaria • Hyper-immunization
• Trichinosis • Trauma
II. Serum sickness
A. Drugs
• Penicillin
• Sulfisoxazole
794 Textbook of Oral Pathology

Causes and mechanism of vitamin D deficiency

Predisposing factors Mechanism
Dietary lack of meat and dairy product Low levels of vitamin D in the diet
Lack of adequate exposure to ultraviolet light Failure of vitamin D precursor synthesis in the skin
Gastric intestinal disease or chronic liver disease Malabsorption of vitamin D and calcium
Aluminum toxicity and biphosphonates Direct inhibition of bone mineralization
Administration of anticonvulsant drug like phenobarbitone These drug enhance liver enzyme activity which
result in increase breakdown of vitamin D to biological inert product
Chronic renal failure Reduced conversion of 25(OH)D3 to 1,25 (OH)2 D3
Hypophosphatemia rickets (X linked dominant) Inherited defect in renal tubular phosphate
reabsorption leading to hypophosphatemia
Hypophosphatasia (autosomal recessive) Defect mutation in bone alkaline phosphatase which
cause inhibition of bone mineralization at the calcification front

Differentiating features of ossifying fibroma of fibrous dysplasia F.D

Features ossifying fibroma fibrous dysplasia
Age 3rd & 4th decades 1st & 2nd decades
Gender predilection females Equal
Location Body of mandible Maxilla
Radiography Well defined margin Poorly defined margin
Lesion shape Roughly nodular or spherical Fusiform or elliptical
 Appendix Histology and
Histopathological
III Diagrams of Oral Tissues

NORMAL HISTOLOGICAL DIAGRAM

Figure AP.1: Dental and vestibular lamina Figure AP.3: Early tooth development

Figure AP.2: Development of dental lamina Figure AP.4: Bud stage

796 Textbook of Oral Pathology

Figure AP.5: Cap stage Figure AP.7: Enamel cord

Figure AP.6: Enamel knot Figure AP.8: Bell stage

Figure AP.9: Advanced bell stage

 Histology and Histopathological Diagrams of Oral Tissues 797

Figure AP.12: Gingiva

Figure AP.10: Hertwig’s epithelial root sheath

Figure AP.13: Taste buds

Figure AP.11: Fordyce’s granules Figure AP.14: Circumvallate, fungiform and filiform
papillae
798 Textbook of Oral Pathology

Figure AP.15: Histopathological diagram of the normal lip Figure AP.18: Principal fibers of periodontal ligament

Figure AP.16: Normal pulp

Figure AP.19: Serous salivary gland

Figure AP.17: Periodontal ligament Figure AP.20: Ducts of salivary gland

 Histology and Histopathological Diagrams of Oral Tissues 799

Figure AP.24: Key hole pattern of enamel

Figure AP.21: Mucous salivary gland

Figure AP.25: Dentin

Figure AP.22: Mixed salivary gland

Figure AP.23: Enamel Figure AP.26: Intra and intertubular dentin

800 Textbook of Oral Pathology

Figure AP.30: Acellular cementum

Figure AP.27: Interglobular dentin

Figure AP.28: Dentinoenamel junction Figure AP.31: Cementoenamel junction gap joint

Figure AP.29: Cellular cementum Figure AP.32: Cementoenamel junction overlapping joint
 Histology and Histopathological Diagrams of Oral Tissues 801

Figure AP.33: Cementoenamel junction butt joint

Figure AP.36: Enamel niche

Figure AP.34: Dead tract Figure AP.37: Enamel spindles tufts and lamellae

Figure AP.35: Epithelial cell rests of Malassez Figure AP.38: Epithelial cell rest
802 Textbook of Oral Pathology

Figure AP.41: Nerve ending in human periodontal

Figure AP.39: Internal dental epithelium ligament

Figure AP.40: Buccal mucosa Figure AP.42: Nerve innervations

 Histology and Histopathological Diagrams of Oral Tissues 803

Figure AP.43: Non-keratinized epithelium Figure AP.45: Orthokeratinized epithelium

Figure AP.44: Soft palate Figure AP.46: Tomes’ granular layer

Figure AP.47: Alveolar bone

804 Textbook of Oral Pathology

HISTOPATHOLOGICAL DIAGRAMS OF ORAL

LESIONS

Figure AP.51: Junctional nevus

Figure AP.48: Squamous papilloma

Figure AP.52: Compound nevi

Figure AP.49: Keratoacanthoma

Figure AP.50: Intradermal nevus Figure AP.53: Fibroma

 Histology and Histopathological Diagrams of Oral Tissues 805

Figure AP.54: Giant cell fibroma Figure AP.57: Cancellous osteoma

Figure AP.55: Lipoma Figure AP.58: Osteoma

Figure AP.56: Chondroma Figure AP.59: Osteoblastoma

806 Textbook of Oral Pathology

Figure AP.60: Leukoplakia Figure AP.63: Severe epithelial dysplasia

Figure AP.61: Mild epithelial dysplasia Figure AP.64: Leukoedema

Figure AP.62: Moderate epithelial dysplasia Figure AP.65: Mild oral submucous fibrosis
 Histology and Histopathological Diagrams of Oral Tissues 807

Figure AP.66: Moderate oral submucous fibrosis Figure AP.69: Poorly differentiated sq cell carcinoma

Figure AP.67: Severe oral submucous fibrosis Figure AP.70: Basal cell carcinoma

Figure AP.68: Moderately differentiated sq cell carcinoma Figure AP.71: Verrucous carcinoma
808 Textbook of Oral Pathology

Figure AP.72: Fibrosarcoma Figure AP.75: Mesenchymal chondrosarcoma

Figure AP.73: Liposarcoma Figure AP.76: Osteosarcoma

Figure AP.74: Chondrosarcoma Figure AP.77: Neurofibrosarcoma

 Histology and Histopathological Diagrams of Oral Tissues 809

Figure AP.78: Leiomyosarcoma Figure AP.81: Plexiform ameloblastoma

Figure AP.79: Multiple myeloma Figure AP.82: Acanthomatous ameloblastoma

Figure AP.80: Hodgkin’s lymphoma Figure AP.83: Granular ameloblastoma

810 Textbook of Oral Pathology

Figure AP.84: Baseloid ameloblastoma Figure AP.87: Unicystic ameloblastoma

Figure AP.85: Peripheral ameloblastoma Figure AP.88: Ameloblastic fibroma

Figure AP.86: Mural ameloblastoma Figure AP.89: Complex odontome

 Histology and Histopathological Diagrams of Oral Tissues 811

Figure AP.90: Odontogenic fibroma simple type Figure AP.93: Benign cementoblastoma

Figure AP.91: Odontogenic fibroma WHO type Figure AP.94: Periapical cemental dysplasia

Figure AP.92: Odontogenic myxoma Figure AP.95: Dentigerous cyst

812 Textbook of Oral Pathology

Figure AP.96: Odontogenic keratocyst Figure AP.99: Lateral periodontal cyst

Figure AP.97: Daughter cyst present in odontogenic Figure AP.100: Aneurysmal bone cyst
keratocyst aneurysmal bone cyst

Figure AP.98: Orthokeratinization seen in OKC Figure AP.101: Radicular cyst

 Histology and Histopathological Diagrams of Oral Tissues 813

Figure AP.102: Cholesterol cleft Figure AP.105: Aneurysmal bone cyst

Figure AP.103: Nasopalatine duct cyst Figure AP.106: Epidermoid cyst

Figure AP.104: Traumatic bone cyst Figure AP.107: Smooth surface caries
814 Textbook of Oral Pathology

Figure AP.108: Dentinal caries Figure AP.111: Extravasation mucocele

Figure AP.109: Pit and fissure caries Figure AP.112: Retention of mucocele

Figure AP.110: Congenital epulis Figure AP.113: Pleomorphic adenoma

 Histology and Histopathological Diagrams of Oral Tissues 815

Figure AP.114: Different pattern of pleomorphic adenoma

Figure AP.115: Canalicular adenoma Figure AP.116: Warthin’s tumor

816 Textbook of Oral Pathology

Figure AP.117: Oncocytoma Figure AP.120: Adenoid cystic carcinoma cribriform type

Figure AP.118: Mucoepidermoid carcinoma Figure AP.121: Adenoid cystic carcinoma solid types

Figure AP.119: Adenoid cystic carcinoma tubular type Figure AP.122: Acinic cell carcinoma
 Histology and Histopathological Diagrams of Oral Tissues 817

Figure AP.123: Adenocarcinoma showing solid lobular Figure AP.126: Tubular and papillary cystic area
area

Figure AP.124: Adenocarcinoma showing cribriform Figure AP.127: Necrotizing sialometaplasia

areas

Figure AP.125: Adenocarcinoma showing strands Figure AP.128: Pulp stone

of perineural whorls
818 Textbook of Oral Pathology

Figure AP.129: Tubercular ulcer Figure AP.132: Candidiasis

Figure AP.130: Tuberculosis granuloma Figure AP.133: Fibrous dysplasia

Figure AP.131: Actinomycosis Figure AP.134: Central giant cell granuloma

 Histology and Histopathological Diagrams of Oral Tissues 819

Figure AP.135: Paget’s disease Figure AP.138: Peripheral ossifying fibroma

Figure AP.136: Florid osseous dysplasia Figure AP.139: Pemphigus

Figure AP.137: Ossifying fibroma Figure AP.140: Mucous membrane pemphigoid

 Glossary

GLOSSARY • Actinic cheilitis—when this atrophic tissue abrades

to ulcer, it is called as actinic cheilitis.
• Aberration—it is a variation from the normal form • Acanthosis—this condition is characterized by
or course. widening and thickening of stratum spinosum.
• Aberrancy—it is defined as that situation in which • Acantholysis—it is the pathological separation of
a tissue develops at a site where it is not normally epidermal or epithelial cells by breakdown of
found. desmosomes in stratum spinosum (seen in
• Ablation—it is removal of a part by excision or pemphigus).
amputation. • Acquired—relating to something not of genetic
• Abnormal—it is not normal, deviating in some origin but resulting from outside influence.
from the usual structure, position or state. • Acrocephalic—it is a highly arched or pointed skull.
• Abrasion—it is the wearing away of a structure or • Acute—having severe symptoms and a short
substance by mechanical means such as scrubbing course.
or grinding.
• Adduction—drawing in towards the center or to
• Abrasive—it is a substance which contains an median line, as opposed to abduction.
abrasive which tends to erode the surface.
• Adenomatosis oris—it is the swelling of the mucous
• Abfraction—loss of tooth surface at the cervical glands of the lips with no inflammation or
areas of teeth, caused by tensile and compressive secretion.
forces during tooth flexure; cervical erosive lesions
that can not be attributed to any particular cause. • Adrenodontia—it is a morphological indication of
over activity of adrenal glands characterized by
• Abscess—an abscess is a localized collection of pus large pointed canines and teeth with occlusal
surrounded by an area of inflamed tissue in which surfaces showing brown discoloration.
hyperemia and infiltration of leucocytes is marked.
• Aerodontia—it is that branch of dentistry concerned
• Actinic keratosis—it is a pre-malignant squamous cell with the care and treatment of dental conditions
lesion resulting from long-term exposure to solar
caused by high altitude flying.
radiation and may be found on the vermilion border
of lip as well as other sun exposed skin surfaces. • Afferent nerve—it refers to any nerve transmitting
impulse from the periphery to the center.
• Actinic elastosis—it is a lesion on the labial mucosa
exposed to sun. A white area of atrophic epithelium • Ageusia—it is the loss or absence of sense of taste.
develops with underlying scarring of the lamina • Aglossostomia—it is the congenital absence of the
propria. tongue and of mouth opening.
822 Textbook of Oral Pathology

• Agranulocytosis—a marked decrease in the • Anosmia—it is the absence of sense of smell.

number of granulocytes, particularly neutrophils. • Antagonist—it is any tissue that acts against or in
• Allelograft—a graft using material not derived opposition to another tissue.
from a donor or from animal sources, e.g. • Anaplasia—it is the reversion of the same type of
synthetic resins, stainless steel alloy. cells from a more highly differentiated to a less
• Allergen—a substance capable of inducing highly differentiated type.
hypersensitivity or an allergic reaction. • Antibody—it is any one of the class of substances
• Allergy—it is hypersensitivity to any normally produced in the body as a reaction to a specific
harmless substance resulting in an exaggerated antigen and with which, it reacts in some
or abnormal reaction. observable way to produce a specific effect such
• Allograft—it is a graft derived from a donor of the as inactivation, agglutination, and/or flocculation.
same species but genetically dissimilar. • Antibiotics—these are substances produced by
• Amniocentesis—it is diagnostic procedure in which microorganisms which suppress the growth or kill
a small amount of amniotic fluid is withdrawn other microorganisms at a very low concentration.
from amniotic sac, a membrane surrounding the • Antidote—it is an agent used to counteract or
fetus in uterus, to detect fetal defects. prevent the action of poisons.
• Amalgam tattoo—oral soft tissue discolorations due • Antigen—it is any substance that when introduced
to amalgam; most common pigmentation of the into the body, excites the formation of specific
oral cavity. antibodies.
• Amelogenesis—the formation of the enamel portion • Angioma—a tumor made up of blood or lymph
of the tooth. vessels.
• Analgesia—it is relief from pain or insensitibilty to • Ankyloglossia—extensive adhesion of the tongue to
pain. the floor of the mouth or the lingual aspect of the
• Analogous—having similar properties anterior portion of the mandible caused by a short
• Anesthesia—it is the general loss of all sensations lingual frenum.
or feelings. • Apertognathia (open bite)—a condition in which the
• Anemia—it is an abnormal reduction in the anterior or the posterior teeth of the mandible
number of circulating red blood cells, the quantity can not be brought into occlusion with antagonist
of hemoglobin and the volume of packed red cells teeth of maxilla.
in a given unit of blood. • Aponeuroses—these are collagenous sheets or
• Anaphylaxis—it is an antigen-antibody reaction ribbons that resemble flat, broad tendons. It may
produced by the parenteral injection of an antigen cover the surface of the muscle and assist in
causing hypersensitivity. attaching superficial muscles or separate the
structures.
• Anastomosis—it is a communication between two
vessels. • Aplasia—absence of an organ or organ’s part due
to failure of development of the embryonic tissue
• Anachoresis—if the bacteria circulating in the
of origin.
bloodstream settle in areas of inflammation or of
lowered resistance in the pulp and produce pulpitis, • Arteriosclerosis—a condition characterized by loss
abscess or necrosis, the phenomenon is referred of elasticity and thickening of artery walls.
to as anachoresis. • Atrophy—it is a reduction in size of tissue or of an
• Anomaly—deviation or irregularity as compared organ due to decrease in the size or number of its
with the normal. constituent cells.
• Anorexia—it is the lack of appetite. • Atresia—it is the congenital occlusion or absence
of one or two major salivary gland ducts.
• Anomalad—it is a malformation together with its
subsequently derived structural changes; the • Atypical—irregular, not conformable to the type.
primary defect setting off a series of secondary or • Attrition—it is the physiologic wearing away of
even tertiary events resulting in multiple anomalies. tooth material as a result of tooth to tooth contact.
 Glossary 823

• Auscultation—listening to the sound produced • Benign—not malignant; favourable for recovery.

within the body with the help of a stethoscope. • Bicameral abscess—it is an abscess which contains
• Autogenous—it is produced within the body itself. two chambers.
It is self- generated. • Biopsy—it is the gross and microscopic
• Autograft—it is a graft taken from one of the examination of tissue or cells removed from living
patient’s body and transplanted to another part patients for the purpose of diagnosis or prognosis
in the same individual. of the disease or the confirmation of the normal
• Autoantibody—an antibody that reacts against an condition.
antigenic constituent of the person's own tissues. • Blanching—to take the color out of and make
• Autoimmune disease—a disease characterized by white.
tissue injury caused by a humoral or cell-mediated • Bleb—it is a bulla or other skin blister filled with
immune response against constituents of the blood or serous fluid.
body's own tissues.
• Blind abscess—it is the one having no fistulous
• Autoimmunity—immune-mediated destruction of tracts.
the body's own cells and tissues; immunity against
• Blister—it is a vesicle caused by localized
self.
accumulation of fluid beneath the skin.
• Autosomes—the non-sex chromosomes that are
• Blood—it is the red fluid in the vessels of the
identical for men and women.
circulating system which conveys oxygen and
• Autoinoculation—to inoculate with a pathogen nutritive materials to the tissue and removes
such as a virus from one's own body. carbon dioxide and waste matter.
• Bacteremia—It refers to the circulation of bacteria • Blood pressure —it is the pressure exerted by the
in the blood. blood on the artery walls and is dependent on the
• Bacteria—these are microscopic unicellular force of heart action, the elasticity of the vessel
vegetative organisms having a single walls, capillary resistance and the volume and
chromosome, no nuclear envelope and a rigid cell viscosity of blood.
wall. They may be seen as rods, cocci or filaments • Blood transfusion—the intravenous administration
and divide by binary fission. of blood to help replenish excess blood loss due to
• Bacteriostatic—it is any agent that inhibits the hemorrhage or otherwise, is known as blood
growth and multiplication of bacteria. transfusion.
• Baelz’s disease—it is a disease characterized by the • Boil—it is a localized skin abscess usually at the
presence of painless papules on the labial mucous site of a hair follicle.
membrane. (Cheilitis glandularis- superficial • Bosselated—having a knob like protrusion or
suppurative type). bosses.
• Ballooning degeneration—it is characterized by the
• Bowen’s disease—It is a localized intra-epidermoid
isolation of a cell from its neighbors, especially in
carcinoma that may progress to invasive
the lower layers of the epidermis, the withdrawing
carcinoma over many years.
of its prickles after intra- cytoplasmic edema and
vacuolization and the amitotic division of its nucleus • Bradycardia—it is an abnormal slowness of the
so as to form multinucleated giant cells. heart and pulse rate.
• Bay cyst—apical cyst which have a direct • Bradyglossia—it is an abnormal slowness of speech,
connection with apical foramen have been termed due to difficulty in tongue movements.
as ‘bay cyst’. • Bradypnea—it is an abnormal slowness of
• Bednar’ aphthae—two ulcers appearing respiration.
symmetrically one on either side of the midline of • Bruise—it is a superficial injury, caused by a blow
the hard palate in infants, thought to be caused by with no laceration but with discoloration of the
the nipple or by thumb sucking or sucking hard skin and subcutaneous tissue produced by an
object. accumulation of blood.
824 Textbook of Oral Pathology

• Bruxism—it can be defined as the involuntary, • Callus—the mesh of fibrous bony tissue
unconscious, and excessive grinding, tapping or surrounding and uniting the bone ends after
clenching of teeth or it is defined as non-functional fracture. It is later replaced by hard bone.
grinding or gnashing of the teeth, usually during • Camper’s line—it is the line extending from the
sleep. external auditory meatus to a point below the nasal
• Buccal bifurcation cyst—a cyst of uncertain origin point and is also called as facial line.
found primarily on the distal or facial aspect of a
• Cancellous—having a lattice like spongy structure;
vital mandibular third molar, consisting of
applied to bone tissue.
intensely inflamed connective tissue and epithelial
lining. • Canker—it is an ulceration especially of the mouth
and lips and it is also called as aphthous stomatitis.
• Bullae—it is an elevated blister like lesion
containing clear fluid and is bigger than 1cm in • Capillary—these are one of the very fine thread
diameter. like blood vessels connecting the veins and
• Burrows—these are short, linear, straight or arteries.
sinuous lines in the skin. • Carbuncle—it is a staphylococcal infection of the
• Burn—it is the injury resulting from the application sweat glands or hair follicles causing inflammation
of excessive heat, electric current, friction and of the surrounding subcutaneous tissue and
caustics to skin or mucous membrane. discharging pus through several openings, finally
• Carcinogenesis—carcinogenesis or oncogenesis or sloughing away.
tumorogenesis means induction of a tumor agent • Carcinosarcoma—it is a mixed tumor containing
which can induce tumor. The tumor agents are characteristics of both carcinoma and sarcoma.
called as carcinogens. • Cariology—it is the scientific study of dental caries,
• Carabelli’s cusp—it is an accessory lingual cusp its causes, prevention and treatment.
located on mesiopalatine cusp of maxillary second • Carrier—the individual who continues to harbor
primary molars and 1st, 2nd and 3rd permanent infectious agent either following recovery from
molars. the illness it induced.
• Capsule—compressed fibrous connective tissue • Cartilage—it is a form of elastic, non-vascular
around a benign neoplasm separating it from connective tissue attached to articular bone surfaces
surrounding tissues. and also forming some parts of the skeleton.
• Carcinoma—a malignant growth made up of
• Catabolism—it is the process of breakdown of
epithelial cells that are capable of infiltration and
complex compounds by the body.
metastasis.
• Catarrh—it is the inflammation of the mucous
• Caries—demineralization of inorganic and
membranes, especially those of nose and throat,
dissolution of organic part of the tooth surface
with discharge of mucus.
caused by bacteria.
• Carcinoma in situ—it is a histopathological • Causalgia—it is a burning sensation arising after
diagnosis defined as a proliferation of basal trauma to a sensory nerve.
epithelial cells from the basement membrane to • Cellulitis—cellulitis may be defined as a non-
the surface, with almost all of the cells manifesting suppurative inflammation of the subcutaneous
cytologic atypia. Immediate maturation into a tissue extending along the connective tissue planes
superficial keratin layer is possible, but no invasion and across the intercellular spaces.
into the underlying connective tissues can be seen. • Cell—it is one of the minute masses of protoplasm,
• Calcareous—relating to or containing calcium or containing a nucleus which forms the basis of all
calcium salts; chalky. animal and plant structure.
• Calcification—it is the deposition in organic tissue • Cementicle—it is a small calcareous body
of calcium salts causing hardening. developing in the periodontal membrane.
• Calcinosis—it is a condition characterized by either • Cell-mediated immunity—it is the type of immunity
localized or generalized deposition of calcium salts in which the predominant role is played by T
in nodules in the soft tissues. lymphocytes.
 Glossary 825

• Central—in oral pathology, it is the lesion • Cold abscess—it is a slow developing tuberculous
occurring within bone. abscess generally about a bone or joint and with
little inflammation.
• Centromere—the constricted portion of the
chromosome that divides the short arms from • Collar stud abscess—it is a superficial abscess
the long arms. connected by a sinus tract to a larger deep abscess.
• Complement system—This consists of a group of
• Chief complaint—it is the patient’s response to the
serum proteins which by series of reactions
dentist’s question. produce and release by products whose functions
• Cheilitis—it is the inflammation of lip. are to initiate an inflammatory reaction, to regulate
• Chemoprophylaxis—it is the use of chemical drugs and enhance phagocytic function and attack the
in the prevention of disease. bacterial cell membrane.
• Congenital—present at or before birth but not
• Chemotherapy—it is the treatment of a disease by
necessarily inherited.
chemicals which affect pathogenic organisms
without harming the patient or it is the treatment • Coalesce—it is a term used to denote to fusion or
union of separated parts.
of malignant neoplasia by chemical means.
• Consanguinity—blood relationship. In genetics, the
• Cheesy—lesion’s texture is similar to curd of cheese.
term is generally used to describe marriages
• Chemotaxis—taxis or movement in response to among close relatives.
chemical stimulation. • Corrugated—having a surface that appears wrinkled.
• Chromatin—a general term used to refer to the • Cotton wool—confluent radiopacities.
material (DNA) that forms the chromosomes. • Coarctation—it is narrowing or constriction,
• Chronic—persisting over a long time; when applied especially to blood vessels.
applied to a disease, chronic means that there has • Col—it is a depression in an interdental papilla
been little change or extremely slow progression between the two peaks, one on each side of the
over a long period. contact area.
• Chills—it is cold sensation with shivering, often • Coma—it is a state of complete unconsciousness
characteristic of onset of fever. from which a patient can not be aroused, even by
• Chloroma—it is a condition characterized by determined external stimulation.
multiple myeloid tumors of greenish color, • Commensal—it is an organism that lives on or
affecting particularly the face and skull, and within another organism, to its own advantage
associated with blood picture of leukemia. and without being detrimental to the host.
• Commissure—it is the point of union between
• Choriostoma—it refers to excessive amount of
similar parts or bodies.
normal tissue that is present in abnormal location.
• Concretion—it refers to any hardened or solidified
• Chondromalacia—it is a condition characterized by mass in the tissue.
abnormal softness of the cartilage.
• Counter irritation—it refers to the deliberate
• Ciliated—having hair like processes or fringe of hair. production of superficial irritation in order to mask
• Circulation—it is the movement or flow in a circle, or relive an existing irritation or pain.
retracing its course repeatedly, applied especially • Concrescence—it is a form of fusion that occurs
to the flow of blood through the body. after the root and other major parts of the
involved teeth are formed or when the roots of
• Cleft lip—it is a birth defect that results in a
two or more teeth are united by cementum, below
unilateral or bilateral opening in the upper lip the cementoenamel junction.
between the mouth and the nose.
• Craniomalacia—it refers to a condition characterized
• Cleft palate—cleft palate is a birth defect by softness of bones of the skull, usually seen in
characterized by an opening in the roof of the infants.
mouth caused by lack of tissue development. • Crater—it is a localized depression, usually circular,
• Coagulation—when blood is shed, it loses its fluidity with raised edge or rim.
in few minutes and sets into a semisolid jelly. This • Crust—dry products of exudation from lesions
is called as coagulation or clotting. occurring on skin and lips.
826 Textbook of Oral Pathology

• Crepitations—it refers to a crackling noise occurring • Debridment—it is the removal of dead tissue and
in the joint when affected by certain disease. foreign matter from a wound.
• Cryosurgery—it is the use of extreme cold for • Degeneration—it refers to the gradual deterioration
surgical destruction of tissue. of tissue with loss of function and chemical changes
• Cryotherapy—it is the treatment of disease with within the tissue.
use of extreme cold. • Dentistry—it is a branch of medicine concerned
• Cryptogenic leukoplakia—in a small proportion of with oral and dental diseases and their prevention
cases of leukoplakia, no underlying cause has been and treatment and with oral prosthesis.
found. Such lesions are termed as idiopathic or • Desmosomes—the term desmosomes refers to the
cryptogenic leukoplakia. structures forming the site of contact between
• Culture—it is the growth of microorganisms in an adjacent cells, especially epithelial cells.
artificial medium. • Desquamation—it refers to the peeling off of the
• Curettage—it refers to the removal of foreign outer layer of epithelium.
matter from the walls of a bony cavity or from • Dental fluorosis—a condition of enamel hypoplasia
the root surface. characterized by white chalky spots or brown
• Cyst—cyst is a pathological cavity which may or staining and pitting of teeth due to an increased
may not be lined by epithelium and consists of level of fluoride; affecting enamel matrix
fluid, semi-fluid or gaseous content (but not by formation and calcification by impairment of
pus) and surrounded by connective tissue capsule. ameloblastic function.
True cyst is a pathologic cavity always lined by
• Dentigerous cyst—an odontogenic cyst that
epithelium usually containing fluid or semi-solid
surrounds the crown of an impacted tooth; caused
material.
by fluid accumulation between the reduced
• Cytology—it is the scientific study of cell. enamel epithelium and enamel surface, resulting
• Cytopathic—pertaining to or characterized by in a cyst.
pathologic changes in cells. • Deoxyribonucleic acid (DNA)—a substance
• Cyanosis—it is the bluish discoloration of the skin composed of a double chain of polynucleotide;
and mucous membranes, often due to deficient both chains coiled around a central axis form a
oxygenation of the blood. double helix. DNA is the basic genetic code or
• Dental Kinesiology—it is the study of motion and template for amino acid formation.
function of jaws and oral musculature; the • Dermoid cyst—a cyst of midline of the upper neck
accompanying neurological, vascular and other or the anterior floor of the mouth of young
supporting system network and the impact of patients, derived from remnants of embryonic
those muscle functions and neurological dynamics skin; consisting of a lumen lined by a keratinizing
have on dental and systemic health. stratified squamous epithelium and containing one
• Developmental anomalies—malformation or defects or more skin appendages such as hair, sweat or
resulting from disturbance of growth and sebaceous glands.
development are known as developmental
• Diffuse—used in the description of a lesion; when
anomalies.
borders of the lesion are not well defined and it is
• Dens in dente—it is also called as dens invaginatus. not possible to detect the exact parameters of the
Infolding of the outer surface of the tooth into lesion, then this term is used.
interior. It is a developmental variation which is
• Diploid—having two sets of chromosomes; the
thought to arise as a result of invagination in the
surface of tooth crown before calcification occurs. normal constitution of somatic cells.
• Dens evaginatus—dens evaginatus is a develop- • Diagnosis—it is the determination of the nature or
mental condition that appears clinically as an cause of the disease.
accessory cusp or globules of enamel on occlusal • Differential diagnosis—the list of similar clinical
surface between buccal and lingual cusp of picture, according to probable identity of condition
premolars. at hand, is the differential diagnosis.
 Glossary 827

• Dimorphic anemia—it is a condition in iron • Drug—it is any medicinal substance.

deficiency and folic acid deficiency anemia can • Dyskinesia—it is defined as a impairment of
occur concomitantly. voluntary motions, causing movements that are
• Diploe—the spongy layer of bone position between incomplete or only partial.
the inner and outer layers of compact bone. • Dysesthesia—it refers to the impairment of feeling
• Diverticuli—they are small pouches or out pocket or sensations; a condition in which a normal
of the ductal system of one of the major salivary stimulus produces disagreeable sensations.
glands. • Dyskeratosis—this lesion shows abnormal
• Disinfection—this is the process by which orientation in development of epithelial cells.
pathogenic microorganisms are removed from • Dysodontiasis—it refers to the painful, difficult or
the surface, without removing bacterial spores. delayed eruption of the teeth.
• Dilacerations—it refers to angulations or sharp • Dysostosis—it refers to the congenital defective
bends or curves in the root and crown of the teeth. bone formation.
• Disease—it is the departure from the average • Dysphagia—an experience of having great difficulty
anatomical structure or is an abnormal degree of in swallowing.
failure of physiological function or some reduction • Dystrophic calcification—pathologic calcification that
in psychological efficiency, due to either adversity occurs in degenerating and dead tissue.
in the genetic endowment of the individual or
misuse of his free will or to adverse factors in the • Dysplasia—it refers to the abnormal formation or
environment in which he lives or some development.
combination of these factors or It is defined as • Dyspnea—it is a shortness of breath.
loss of ease. • Ecchymosis—it refers to the diffuse extravasation
• Distomolar—found in the molar region frequently of blood into the tissues. Larger purpuric lesions
located distal to 3rd molar. are called as ecchymoses.
• Discrete—separate. composed of separate parts, • Ectoderm—the outermost of the three primary
not joined or blended. germ layers of the embryo, from which are
developed the epidermis, the external sense
• Dislocation—it is the displacement of any part from
organs and the oral and anal mucous membranes.
its normal position, especially in the cases of bone
and joints. • Edema—it is accumulation of excess fluid in the
intercellular tissue spaces or body cavities.
• Direct fracture—it refers to the fracture that occurs
at the site of blow. • Electrocautery—it is cauterization by low voltage
current producing burn like tissue repair, but with
• DLE (discoid lupus erythematous)—it is a
no control over the extent or quality of tissue
circumscribed slightly elevated white patch that
destruction.
may be surrounded by a red telangiectic halo.
• Electrodesiccation—it refers to the deeply penetrat-
• Dose—it is one measured portion of any medicine
ing tissue dehydration produced by the insertion
which is to be taken one at a time.
of electrodes into the tissue.
• Dominant—in genetics, a trait or characteristic that • Empirical therapy—with serious infections, it is often
is manifested when it is carried by only one of a necessary to begin antibiotic therapy before
pair of homologous chromosomes. culture result is available, this is called as empirical
• Dorsal—directed towards or situated on the back therapy which is directed towards organisms
surface (opposite of ventral). which are most likely to have caused that infection.
• Dry abscess—it is an abscess that disperses without • Embedded teeth—those teeth which are unerupted
bursting or coming to a head. usually because of lack of eruptive force.
• Drainage—it is the gradual removal of fluid from • Embolism—it refers to the sudden blockage of
a cavity or wound. blood vessels by a clot or other obstruction within
• Dressing—it is a medicament used to promote the blood stream, causing failure of circulation.
wound healing or as a covering for a wound, used • Empyema—it is the accumulation of pus in a body
for protection or to assist healing. cavity or a hollow organ.
828 Textbook of Oral Pathology

• Enamel pearls, nodules, or droplets—pearls or • Eruption—the act of appearing, or pushing

droplets described as small buttons or nodules of through, as of teeth coming through the gums or
enamel usually about 1 mm or 2 mm in diameter a visible skin lesion occurring in disease.
that form on the root or at the bifurcation of multi- • Erythema—it is the redness in the skin either diffuse
rooted teeth. or patchy, caused by congestion of the
• Embryonic—pertaining to the earliest stage of subcutaneous capillaries.
development of an organism. • Erythematous—it characterized by a redness of the
• Emigration—the passage of white blood cells tissue due to engorgement of the capillaries in the
through the endothelium and walls of small blood region.
vessels. • Erythroplastic—it is characterized by a reddish
• Endotoxin—they are heat stable phospholipid- appearance. This term implies abnormal tissue
polysaccharide-protein complex contained as a proliferation in the reddish area.
structural part of the cell of many gram negative • Erythrocyte—one of the red cells found in blood
bacterias and released by disintegration of the cells. which carries oxygen and is produced by the bone
• Enanthema—it is an eruption occurring on a marrow.
mucous surface or on any surface within the body • Erythroplakia—the term is applied to any area of
as opposed to exanthema. reddened velvety textured mucosa that can not
• Endemic—prevalent in a particular region. be identified on the basis of clinical and
• Endosteal—it is within the bone. histopathological examination as a cause of
inflammation or any other disease process.
• Endothelium—it refers to the membrane lining the
heart and blood vessels. • Erythrodontia—there is deposition of porphyrins
in dentin and to a lesser extent in the enamel which
• Engorgement—it refers to the excess of blood in
imparts red or brown color to the deciduous and
any part of the body or it is the localized
permanent teeth and known as erythrodontia.
congestion or distension.
• Erosion—it is a shallow crater in the epithelial surface
• Enostosis—it is a localized morbid bone growth
that appears on clinical examination as a very
arising within the bone cavity.
shallow erythematous area with only superficial
• Enucleate—the word enucleate means to remove changes. Or a moist red lesion often caused by
an organ or part, or a circumscribed, space filling rupture in vesicles and bullae as well as trauma.
lesion entirely, i.e. from its outer sheath or
covering. • Erosion (teeth)—it is loss of tooth substance due to
chemical process that does not involve bacterial
• Endodermal—pertaining to the innermost of the activity.
three primitive germ layers of an embryo.
Endodermal structures include the epithelium • Erythroplasia—these are painless erythematous
pharynx, respiratory tract (except the nose) and eruptions, popular or macular in nature, affecting
digestive tract. the mucous membrane.
• Epidemic—affecting large number of people within • Eschar—it is a dry slough, the result of burning or
an area or region. due to contact with a corrosive agent.
• Epidemiology—it is that branch of science concerned • Etiology—the study or theory of the factors that
with the study of a disease or condition through cause disease and their introduction to the host.
its frequency and distribution. • Eversion—a turning outward or a state being
• Epithelium—it is a thin cellular layer covering or turned outwards.
lining the organs and tissues of the body. • Excrescence—it refers to an abnormal growth
• Eponym—the name of an organ, syndrome, protruding from body or plant.
disease etc. that contains or is derived from a • Exacerbation—it refers to an increase in the severity
proper name. of a disease or any symptoms.
• Epulis—any tumor of the gums; more especially • Examination—it refers to investigations carried out
either a fibrous or a giant cell tumor. for diagnostic purpose.
 Glossary 829

• Exanthema—it is an eruptive fever. • Fenestrate—to pierce with one or more holes,

• Excoriation—it is the superficial loss of surface skin sometimes used on the walls of bony defect in an
or a graze. attempt to stimulate repair.
• Excursion—it refers to any movement of a • Fenestration—it refers to a surgical procedure by
movable part from a resting position during the which one or more holes are pierced in hard tissue.
performance of some functions. • Fever—it refers to an abnormal increase in body
• Exfoliation—it is the peeling off in layers or in scales. temperature.
• Exophthalmos—it refers to the abnormal protrusion • Final diagnosis—it is statement with which precise
of the eyeball. diagnosis has been made on the basis of all
required observation, identification of definitive
• Exophytic—it refers to a word relating to
symptoms and the pathological report and patient
something growing outwards, used for tumor
response to therapy.
projecting above the normal surface contours or
it refers to any pathological growth that project • Fibrocemento-osseous lesions—it is a skeletal
above the normal contours of the oral surface. disorder in which bone is replaced by fibrous tissue
which in turn is replaced by mineralized tissue.
• Expansile—capable of being extended or
expanded. • Fissure—it is a linear often crusted, tender, painful
• Expressivity—in genetics, the degree of clinical defect in continuity of the skin, occurring usually
manifestation of a trait or characteristic. at the mucocutaneous junctions and at sites where
there is considerable elasticity of the skin.
• Exostosis—it is a bony swelling developing on the
bone surface or on a tooth root. • Fistula—it is communicating tract between two
epithelial surfaces which is lined by granulation
• Exotoxin—it refers to a toxic secretion of bacterial tissue which is subsequently epithelized.
cells which cause damage in sites distant from the
focus of infections or they are heat labile proteins • Fibrosis—there is an abnormal formation of
which are secreted by certain bacteria and diffuse fibrous tissue.
readily into surrounding tissue. • Fluctuant—a wavelike motion felt on palpating a
• Extravasation—it is the escape of fluid from vessels cavity with non-rigid walls, especially one
into the surrounding tissue. containing fluid.
• Extrinsic—having its origin outside and separated • Fluoride mottling—a condition of enamel
from a body, organ or part. hypoplasia characterized by white chalky spots
or brown staining and pitting of teeth due to an
• Exudate—the matter that passes out into adjacent increased level of fluoride affecting enamel matrix
tissues through vessel walls in inflammation. formation and calcification by impairment of
• Facies—the appearance of the face. ameloblastic function.
• Factitial injuries—these are accidentally self- • Focus of infection—it refers to a circumscribed area
induced injuries on the basis of habits with of tissue, which is infected with exogenous
frequent psychological backgrounds. pathogenic microorganisms and which is usually
• Favorable fracture—if the fracture line runs in such located near a mucous or cutaneous surface.
a manner that the associated muscle tends to hold • Focal infection—it refers to metastasis from the
the fragments together, the fracture is described focus of infection of organisms or their products
as favorable. that are capable of injuring tissue.
• Facet—it is a small abraded area on a bone or on • Foramen—a small hole in a bone through which
tooth surface. passes either blood vessels or nerves or both.
• Familial—relating to a family, or affecting several • Focal osteitis—a condition sometimes occurring
of its members. after tooth extraction, particularly after traumatic
• Fascia—it is the layer of areolar tissue beneath the extraction, resulting in a dry appearance of the
skin or the layer of areolar tissue investing the exposed bone in the socket, due to disintegration
muscles, nerves and other organs. or loss of the blood clot.
830 Textbook of Oral Pathology

• Foreign body granuloma—a reaction to foreign • Granulation tissue—it is the reparative tissue that
materials that are too large to be ingested by either is formed on the surface of wound having pink,
microphages (PMNs) or macrophages. soft, granular appearance showing histologically
• Frenal tag—a redundant piece of mucosal tissue new small blood vessel and fibroblast.
that projects from the maxillary labial frenum. • Green stick bone fracture—it is a fracture in which
• Fusion—It is also called as synodontia. It one side of bone is broken and the other side is
represents the embryonic union of normally bent but intact.
separated tooth germs. • Ground glass—fine radiopaque spots in radiolucent
• Fulguration—it refers to the superficial tissue background.
dehydration produced by a surgical electrode held • Gustatory—the sense of taste or the act of tasting.
slightly away from the tissue, causing sparking. • Hamartomas—it is a tumor like malformation of
• Galvanism—the production of an electric current oral tissues, developmental in origin with tissue
caused when two dissimilar metals used as being native to the site.
restorations in the mouth come into contact, this • Habit—it is a tendency toward an act or an act that
can cause discomfort and even pain. has become a repeated performance, relatively
• Gangrene—it is the necrosis of tissue due to failure fixed, constant, easy to perform and almost
of the arterial blood supply caused by injury or automatic.
disease. • Hemoglobinopathies—these are a group of
• Gelation—the process of change of a colloid from hereditary disorders characterized by the presence
a sol to a gel. of structurally abnormal hemoglobin.
• Gerodontia—it is that branch of dentistry which • Hereditary disease—they are apparent at birth but
deals with the care of old people. some may not become evident for years.
• Gemination—It refers to the process whereby single • Hemoptysis—the presence of blood in the sputum
tooth germ invaginates resulting in incomplete caused by bleeding in the upper respiratory tract
formation of two teeth that may appear as a bifid or the lungs.
crown on a single root. • Hemorrhage—it refers to the internal or external
• Genetic heterogeneity—having more than one loss of blood due to injury or other damage to
inheritance pattern. blood vessels.
• Ghost teeth—a developmental disturbance of • Hemidesmosome—it refers to a structure found on
several adjacent teeth in which the enamel and the basal surface of an epithelial cell, the attachment
dentin are thin and irregular and fail to adequately site between the cell and the underlying membrane.
mineralize; surrounding soft tissue is hyperplastic • Heredity—it refers to the transmission of a
and contains focal accumulations of spherical characteristic from parent to child or to later
calcifications and odontogenic rests. generation.
• Gingivosis—it refers to the any degenerative • Heterotrophic—it is a term used relating to
condition affecting the gingiva. organisms which require a complex source of
carbon for nourishment and growth.
• Gland—an organ that produces secretions.
• Hematoma—it is large clot resulting from blood
• Glossodynia—it refers to the burning or painful
released into the tissue form a ruptured or injured
condition of the tongue.
blood vessel.
• Gomphosis—it is the firm attachment of two bones
• Healing—it is repair and replacement of dead or
without a movable joint.
damaged cells by healthy cells.
• Gorham’s disease—in this condition a large portion • Histology—it refers to the study of the anatomy
of bone disappears without any apparent cause. and physiology of tissue and cells using
• Granuloma—a tumor composed of granulation microscopic technique.
tissue. • Holistic—it refers to an approach to treatment that
• Granulomatosis—it refers to the development of takes into consideration the whole person, not
multiple granuloma. just the disease or condition.
 Glossary 831

• Homologous—having the same or corresponding • Implant—the word implant means to insert into
structure or position but not necessary similar in the body or to graft as in plastic surgery.
function. • Infection—it is a clinicopathological entity-
• Horner’s teeth—incisor teeth with horizontal involving invasion of the body by pathologic
grooves cause by enamel deficiency. microorganisms and the reaction of tissues to
• Hypodontia—it refers to the absence of one or more microorganism and their toxins.
teeth. • Inspection—it refers to an examination of the
• Hypertrophy—it refers to the enlargement caused affected part of the body.
by an increase in size of cells. • Internal derangement—it can be defined as
• Hydrocyst—it refers to a cyst whose contents are malrelationship of the meniscus to the condylar
watery in nature. head and articular eminence where an alteration
of its attachment allows the meniscus to assume
• Hyperplasia—it refers to the enlargement caused an abnormal position.
by increase in number of cells.
• Inflammatory collateral cyst—it is a cyst which arises
• Hypoplasia—it is the failure of full development of in the periodontium of an erupted tooth as a result
an organ or tissue. of inflammatory process in the periodontal pocket.
• Hydrostomia—it refers to a condition characterized • Involucrum—small section of necrotic bone may
by constant dribbling from the mouth. be completely lysed, while a large one may get
• Hygroma—it refers to a swelling caused by fluid localized, and get separated and form shell of new
surrounding an inflamed bursa, or distending a bone called involucrum by a bed of granulation
sac or cyst. tissue or a sheath particularly new bone sheath
• Hypertension—exceptionally high tension that forms about sequestration.
especially abnormally high blood pressure. • Indirect fracture—fracture site distant from where
• Hypnosis—it refers to a sleep or a trance state, the actual blow takes place, usually seen on
especially one induced artificially by verbal contralateral side.
suggestions or concentration upon some object. • In vitro—within glass referring to observations
• Hypsodont—having teeth with long crowns and made in a test tube or culture dish as opposed to
short roots seen in herbivorous animals. in vivo.
• Hydropic degeneration—it refers to replacement of • In vivo—within a living organism.
the nuclei of stratum basal by clear space due to • Indentation—it refers to the condition of being
edema and degeneration of cells. serrated or notched.
• Iatrogenic diseases—theses are the diseases • Induced—brought on by an outside agent or is
produced by the action of a doctor or due to artificially produced.
medical treatment. • Induration—it refers to the state of being hard or
• Idiopathic—it is any spontaneous or primary the process of becoming hard.
disease with no apparent external cause. • Inflammation—it is the reaction of living tissue to
• Idiosyncrasy—it refers to a reaction to a particular injury.
drug in therapeutic doses in a manner not • Infarction—it is a localized area of ischemic necrosis
necessarily related to its pharmacological in an organ or tissue resulting from sudden
properties. reduction of either its arterial supply or venous
• Impacted teeth—they are those prevented from drainage.
erupting by some physical barriers in the eruption • Inflation—it refers to the distension with gas
path. especially air.
• Immunity—It is the resistance exhibited by the host • Inostosis—it refers to the process by which bony
towards injury caused by microorganisms and tissue is reformed to replace tissue that has been
their products. destroyed.
• Impermeable—not permitting passage especially of • Insidious—unperceived coming on gradually and
fluids. stealthily.
832 Textbook of Oral Pathology

• Intermittent—occurring at intervals with periods • Lateral—away from midline

of cessation. • Ludwig’s angina—this condition may be defined
• Intubation—it refers to the introduction of a tube as an overwhelming rapidly spreading septic
through the mouth or the nose to allow air, gas or cellulitis involving submandibular, submental and
vapor to pass into the lungs. sublingual space bilaterally.
• Iontophoresis—it refers to the therapeutic treatment • Lymph—it refers to the clear fluid found in the
by electrical introduction of ions into the body lymphatics vessels.
tissue. • Lymphadenitis—it refers to the inflammation of the
• Ischemia—it refers to the deficiency in the blood lymph nodes.
supply to a part or an organ which may be due to • Macule—well circumscribed flat lesion that is
constriction, contraction or blocking of the arteries. noticeable due to the change from the normal skin
• Isograft—it refers to a graft derived from one color to red may be due to inflammation or
member of a pair of monozygotic twins and pigmented due to presence of melanin
transplanted to the other. hemosiderin or other drugs.
• Jaw winking—it refers to a movement of the lower • Maceration—it refers to the softening of a substance
jaw causing an involuntary movement of the by soaking in a liquid.
eyelids. • Malocclusion—it refers to any deviation from the
• Joint—the place of connection between two bones, normal occlusion of the teeth resulting in impaired
allowing of more or less movement an functions.
articulation. • Marrow—it refers to the soft tissue canal and
• Keloid—it refers to a fibrous hyperplastic scar interstices of bones.
growth on the skin. • Marsupialization—it refers to an operation for the
• Kernicterus—staining of brain tissue cause by evacuation of a cyst and the suturing of its walls
accumulation of unconjugated bilirubin in the to the edges of the wound.
brain. • Metastasis—it is defined as spread of tumor by
• Knitting—it refers to the process of repair of a invasion in such a way that discontinuous
bone fracture. secondary tumor mass/masses arc formed at the
• Lain’s disease—burning of the tongue and the soft site of lodgment.
tissue of the mouth due to electrogalvanism • Metaplasia—it is a reversible change in which one
caused by the use of dissimilar metals in dental adult cell type is replaced by another adult cell
restoration. type.
• Lancinating—it is the term used to describe • Mesiodens—it refers to supernumerary tooth
shooting, tearing or sharply cutting type of pain. located at or near the midline in the incisal region
• Leukoplakia—a white patch or plaque that can not of maxilla between the central incisors.
be scraped off and can not be characterized • Medicine—it refers to the study and treatment of
clinically or pathologically as any other disease, diseases especially treatment without recourse to
which is more than 5 mm. surgery or any drug used for the treatment of the
• Lesion—a wound or injury or a patch of disease disease.
on the skin. A morbid change in tissue function. • Metabolism—it refers to the physical and chemical
• Lichen planus—relatively common dermatitis changes in the tissue by which a living body is
occurs on skin and oral mucus membrane and maintained and energy generated.
refers to a lace-like pattern produced by symbolic • Mitosis—it is the indirect division of cells, a typical
algal and fungal colonies on the surface of rocks method of cell reproduction.
in nature. • Mucocele—it is a term used to describe swelling
• Lipomatosis—it refers to excessive localized caused by pooling of saliva at the site of injured
accumulation of fats in the tissues. minor salivary gland.
• Localized—lesion or condition happening within • Muscle—it is a contractile organ by means of which
one small area. movement is produced in an animal organism.
 Glossary 833

• Muscle spasm—it refers to a sudden involuntary • Oral submucus fibrosis—an insidious chronic disease
contraction of the muscle or group of muscles affecting any part of the oral cavity and sometimes
attended by pain and interference with function. the pharynx, proceeded by and/or associated with
• Mucus plug—these are incompletely mineralized vesicle formation, it is always associated with
sialoliths. juxtaepithelial inflammatory reaction followed by
fibroelastic change of the lamina propria, with
• Natal teeth—these are teeth which are observed in
epithelial atrophy leading to stiffness of the oral
the oral cavities at birth.
mucosa and causing trismus and inability to eat.
• Narcosis—a state of profound unconsciousness or
• Oral medicine—it is that area of dental practice
stupor produced by drugs.
which deals with diagnosis and treatment of oral
• Nausea—a feeling of sickness or a tendency to vomit. disease by non-surgical means, which may be
• Necrosis—it the sum of the morphologic changes localized in the oral cavity or which may be oral
that follow cell death in a living tissue or organs. manifestation of systemic disease and those phases
• Neonatal teeth—these are teeth which erupt during of dental practice concerned with diagnosis and
the first 30 days of life. treatment of medically compromised patients.
• Neoplasia—It is an abnormal mass of tissue, the • Organ—it refers to any separate part of the body
growth of which exceeds and is un-coordinate with having a specific function.
that of normal tissue and persists in the same • Organism—it refers any individual plant or animal
excessive manner after cessation of stimuli which or an organized body of living cells.
evoke the changes. • Osteomyelitis—it is an inflammation of bone
• Neurotropic—attracted to or having an affinity for marrow that produce clinically apparent pus and
nervous tissue. secondarily affect the calcified component or it is
• Nevus—it is circumscribed new growth of skin or an infection of bone that involves all three
oral mucosa of congenital origin, presenting as components periosteum, cortex, and marrow or
small, elevated, or flat pigmented lesion. it may defined as an inflammatory condition of
the bone that begins as an infection of medullary
• Nodules—this lesion is present deep in the dermis
cavity and the haversian system which extends to
and epidermis and can be moved easily over them.
involve the periosteum of the affected area.
• Nociceptive—relating to any pain producing • Papules—these are solid lesions raised above the
stimulus, or to pain receptor nerves. skin surface that are smaller than 1 cm in diameter.
• Nosology—it refers to the science of classification • Pathogenecity—it is the ability of microbial species
of disease. to produce disease.
• Numbness—partial or total loss of sensation which • Paramolar—it is a supernumerary molar usually
may be deliberately induced as in cases of local small and rudimentary which is situated buccally
anesthesia or it may be pathological. or lingually to one of maxillary molars or inter-
• Nutrition—it refers to the process by which food proximally between 1st, 2nd and 3rd maxillary
is assimilated. molars.
• Ointment—it refers to a fatty semisolid substance • Palpation—it refers to feeling of the affected part
used as a base for local medicaments for external by hand.
application. • Pain—it refers to the distressing or unpleasant
• Oligodontia—it is agenesis of a few numbers of sensation transmitted by a sensory nerve usually
teeth. indicative of injury or of disease.
• Oncology—it refers to the study of neoplasm. • Palliation—it refers to the act of alleviating or
• Operation—anything performed, especially any affording relief without curing.
procedure by a surgeon, either with instruments • Paresthesia—the term refers to perverted
or by hand. sensation like burning, prickling or crawling
• Oroantral opening—the accidental opening in the sensation of the skin.
floor of maxillary sinus during dental extraction is • Parageusia—it refers to an unpleasant taste in the
called as oroantral opening. mouth.
834 Textbook of Oral Pathology

• Parakeratosis—it refers to any abnormality of the gingival tissue extends into the underlying
stratum corneum of the epidermis, which may be alveolar bone and there is loss of attachment.
associated with inflammation of the prickle cell • Periodontosis—chronic non-inflammatory
layers causing defective formation of keratin and destruction of periodontal ligament and the
characterized by the persistence nuclei. associated alveolar bone.
• Paralysis—it is the loss or impairment of muscle • Phoenix abscess—it an acute exacerbation of a
function or of sensation due to nerve injury or chronic or suppurative apical periodontitis.
destruction of neurons.
• Phlegmon—acute inflammation of the subcutane-
• Pararhizoclasia—it is the inflammatory ulcerative ous connective tissue.
destruction of the deep layers of tissue and the
alveolar process about the root of a tooth. • Pit—it is defined as hollow fovea or indent blind
tracts lined with epithelium.
• Parenteral—descriptive of methods of drug
administration other than by the alimentary canal. • Pilation—it is a hair like fracture found in cranial
bones.
• Parodontal—near or next to a tooth sometimes used
as synonymous with periodontal. • Plasmapheresis—it is a method of increasing the
number of blood cells in the blood count. From
• Parrot tongue—a horny, dry tongue which can not the blood plasma is skimmed out simply on
be protruded, seen in typhus and low fever is standing and remaining concentrate is reinfused
called as parrot tongue. into the patient.
• Pathogen—any agent that produces or is able to • Plaque—it is a solid raised lesion that is over 1 cm
produce disease. in diameter.
• Parulis—it is mass of granulation tissue which
• Pleomorphic—the word pleomorphic means
covers the opening of a sinus.
occurring in several distinct shapes.
• Pathogenesis—the development of disease from
• Pleurodont—having teeth attached to the side of a
its inception to the appearance of characteristic
bony socket or to the side of the jaw.
symptoms or lesions.
• Plexus—a plexus of nerves or a network of blood
• Pathognomonic—characteristic of one specific
or lymphatic vessels.
disease or pathological condition as distinct from
any others. • Pocket—it is an abnormal space developing
between the tooth root and the gums.
• Pathology—that branch of medicine which is
concerned with the structural and functional • Poison—any substance that when absorbed into
changes caused by disease. the system of a living body is liable to cause injury
and to endanger life.
• Peridens—supernumerary teeth that are erupted
ectopically either buccally or lingually to the • Poikiloderma—it refers to a combination of atrophy,
normal arch referred to as peridens. telangiectasia and pigmentary changes.
• Petechiae—purpuric lesions 1 to 2 cms in diameter. • Polylophodont—these are teeth with multiridged
crowns.
• Permeation—the spreading or extension through
tissues or organs, used especially of malignant • Precancerous lesion—morphologically altered
tumors extending by continuous growth through tissue in which cancer is more likely to occur than
the lymphatics. its normal counterpart.
• Pericemental abscess—a parodental abscess not • Pre-cancerous condition—it is a generalized state
arising from a diseased pulp or an extension of associated with a significantly increased risk of
the periodontal pocket. cancer.
• Percussion—listening to the tapping note with a • Premedication—the administration of drugs or
finger placed on the affected part or in cases of sedatives before treatment, to help in patient
teeth with the help of handle of the probe. management especially with nervous patient.
• Periodontitis—it is name given to periodontal • Prescribe—to write instruction for the preparation,
disease when the superficial inflammation in the composition and administration of a medicine.
 Glossary 835

• Prevalence—the number of cases of a disease at • Pyemia—generalized septicemia caused by

any given time in any given place. pyogenic microorganism in the blood stream and
• Priestley’s mass—a green or brown stain on the marked by the formation of multiple abscesses.
anterior teeth of the young or where reduced • Ranula—the term ranula is used for a mucocele
enamel epithelium remains over the enamel. occurring in the floor of mouth in association with
• Procheilia—it is the condition in which one lip ducts of the submandibular or sublingual glands.
protrudes forwards of its normal position. • Radiology—it is that branch of health sciences
• Prognosis—it is the prediction of the course, dealing with radioactive substances and radiant
duration, and termination of the disease and the energy and with the diagnosis and treatment of
likelihood of its response to therapy. disease by means of both ionizing (X-rays) and
non-ionizing (ultrasound) radiations.
• Prosthesis—the word prosthesis is used for a
manufactured appliance used to take the place of • Radiolucent—offering little resistance to X-rays in
a natural part or to correct a congenital radiography; almost transparent.
abnormality or it may be defined as an appliance • Rash—it refers to a temporary cutaneous eruption.
which replaces lost or congenitally missing tissue. • Recrudescence—the return of symptoms or the
• Proteolysis—the process of digestion of proteins recurrence of the disease after a temporary
and its conversion by enzymes into peptones, remission.
proteoses, etc. • Recurrence—the return of symptoms or of a
• Protuberance—the word protuberances refers to disease after a period of remission.
a swelling, eminence or knob of the tissue. • Regurgitation—the return of undigested or partially
• Pseudomembrane—it refers to a false membrane, a digested food from the stomach or esophagus to
skin like layer formed by fibrinous exudates the mouth or of fluid or semifluid to the nose.
containing leukocytes and bacteria. • Reticular—relating to net or net like structure.
• Pseudoepitheliomatous hyperplasia—in this • Regeneration—it is replacement of injured tissue
conditions the retepegs extend far downward, by parenchymal cells of the same types.
usually accompanied by acanthosis. The cells are • Retrogenia—it refers to a condition in which chin is
normal in size, shape and chromaticity. set back in relation to the rest of the facial skeleton.
• Psychosomatic—relating to the mind and the body; • Rh hump—In the deciduous 1st molar crown a
particularly relating to the interdependence of characteristic ring like defect may be seen which
mental processes and bodily function. is called as Rh hump.
• Pustule—it refers to a raised lesion containing • Rhinorrhea—it refers to any discharge of fluid from
purulent material. the nose.
• Purpura—it refers to reddish to purple flat lesion • Rhizotomy—a surgical division of either a tooth
caused by blood extravasated from a vessel root or a nerve root.
leaking into subcutaneous tissue.
• Root dehiscence—it is a pathological condition in
• Pulsation—it is the rhythmic throb or beating as which the vestibular surface of the tooth root is
that of the heart. exposed to the oral cavity over some or all of the
• Pulse—the expansion and contraction of an artery apical two-thirds of its length.
due to increased tension of its walls following • Rudiment—it refers to an organ or part either
contraction of the heart and subsequent relaxation. imperfectly developed or at an early stage of
• Pus—it is a liquid usually yellowish in color development.
formed in certain infection and composed of tissue • Rubber jaw—in this condition it is possible to mold
fluid containing bacteria and leukocytes. the shape of the jaw with the fingers, but teeth
• Putrefaction—the decomposition of organic matter will resume its position when the pressure is
through the action of microorganisms, resulting released.
in the production of various solid and liquid • Satellite abscess—it is a secondary abscess arising
compounds and gases giving off a foul odor. from and situated near a primary abscess.
836 Textbook of Oral Pathology

• Saburra—it refers to a foul condition of the mouth • Sign—it defined as any change in the body or its
and teeth or of the stomach due to food debris. function which is perceptible to a trained observer
• Saucerization—it is the wide and shallow and may indicate a specific disease.
depression occurring about a wound or bone • Systemic lupus erythematous (SLE)—it is
cavity as in osteomyelitis. characterized by the presence of abnormal serum
• Sclerosis—it refers to hardening of vessels or part antibodies and immune complexes.
applied particularly to arteries and to proliferation • Slough—it refers to the necrotizing tissue that
of connective tissue in the nervous system as a scales or peels off in ulcerative conditions.
result of degeneration. • Spongiosis—this term is used to signify intercellular
• Scale—loosened imperfectly cornified parakeratotic edema of the epithelium, in which intercellular
superficial layer of skin that is shed as fine, brawny, bridges of the stratum spinosum become more
dirty white, yellowish keratinous dust or large prominent.
pearly white flakes. • Stagnation—it refers to the cessation of flow of
• Sequestra—small pieces of necrotic bone which are any circulating fluid in the body.
avascular and which harbor microorganisms are
• Sterilization—it is the process of destruction of the
known as sequestra.
microbial life from an article or surface inclusive
• Serum—if blood is allowed to clot an amber of bacterial spores.
colored liquid which remains after separation of
• Sterile abscess—it refers to an abscess containing
the clot is known as serum.
no microorganisms.
• Septicemia—the word septicemia implies a
• Stenosis—it is the constriction or narrowing of an
overwhelming bacterial proliferation and release
aperture canal or duct.
of toxins in the blood.
• Stimulus—it refers to any agent or impulse that
• Shock—it is state of inadequate perfusion of all
excites or promotes a functional reaction.
cells and tissues, which at first leads to reversible
hypoxic injury, but if sufficiently protracted or • Stippled—having a mottled or spotted appearance
grave, to irreversible cell and organ injury and with light and dark patches.
sometimes to the death of the patient. • Stomatology—the medical speciality concerned with
• Sickle cell anemia—in homozygous individuals the the mouth and its diseases sometimes used
whole of HbA (hemoglobin A) is replaced by HbS synonymously with dentistry.
(hemoglobin S i.e. an abnormal hemoglobin) and • Striation—it is a stripe or streak or a series of stripes
this is known as sickle cell disease. or streaks.
• Sickle cell trait—in heterozygous individuals only • Stricture—it is an abnormal contraction of any
50% of HbA is replaced by HbS and this is known aperture or vessels.
as sickle cell trait.
• Stridor—it is a harsh whistling sound produced
• Sinus—it is a blind tract leading from the surface by the respiratory system.
down to the tissue which is lined by granulation
• Superficially invasive (microinvasive) squamous cell
tissue or which may be epithelized.
carcinoma—a histopathological diagnosis of a
• Sialorrhea (ptyalism)—an increased salivary routine squamous cell carcinoma, usually well
secretion is termed as sialorrhea or ptyalism. differentiated, which has invaded only slightly into
• Sialolithiasis—it is the formation of calcific the underlying connective tissues.
concretions within parenchyma or ductal system • Subluxation (hypermobility)—it is the unilateral or
of major or minor salivary glands. bilateral positioning of the condyle anterior to the
• Sinusitis—inflammation of mucosa of paranasal articular eminence, with repositioning to normal
sinuses is referred to as sinusitis. When maxillary to accomplish normal physiologic activity.
sinus is involved it is called as maxillary sinusitis. • Subscription—it is a part of a prescription
• Sialoschesis—it is the suppression of the secretion containing direction for the preparation and
of the salivary glands. compounding of the ingredients of a medicine.
 Glossary 837

• Suzanne’s gland—an oral mucous gland found in • Taurodontism—body of tooth is enlarge at the
the alveolo-lingual sulcus near the midline. expense of root. It is characterized by clinical and
• Symbiosis—it is the intimate association of two anatomical crown of normal shape and size, an
organism of different species. elongated body and short root with a
longitudinally enlarged pulp chamber.
• Symptoms—any indication of the presence or
course of a disease either by functional or other • Tablet—it is a small solid disc containing one dose
changes occurring in the patient. of a drug.
• Syncope—it is a transient loss of consciousness • Tapir mouth—it is a condition characterized by loose
caused by cerebral hypoxia or changes in cerebral thickened lips and caused by atrophy of the
blood flow. orbicularis oris muscle.
• Syndesmosis—it is the joining of two bone surfaces • Taste—the perception of flavor, a sensation
by the interposition of connective tissue which produced by stimulation of the gustatory nerve
forms an interosseous membrane. endings in the tongue with a soluble substance.
• Syndrome—a complex of symptoms, occurring • Telangiectasia—it is the dilatation of the capillaries
together, which characterize one disease or lesion. and small arteries forming types of angiomas.
• Talon’s cusp—it projects lingually from the
• Teratoma—it is true neoplasm made up of a number
cingulum area of maxillary and mandibular teeth
of different types of tissue which are not native to
or it is anomalous hyperplasia of cingulum on the
the are in which the tumor occurs.
lingual of maxillary and mandibular incisor
resulting in the formation of a supernumerary • Thalassemia—It is an inherited impairment of
cusp. hemoglobin.
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 Index

A Acute periapical abscess 430 Albers-Schonberg disease 617

Acute pulpitis 422 Albright’s syndrome 606, 710
Abbe condenser 4 Acute subperiosteal osteomyelitis Aldrich syndrome 601
Aberrancy 245 439 Aleukemia 234
Abfraction 117 Acute suppurative osteomyelitis Allergic sialadenitis 252
Abrasion 116, 740 438 Alpha thalassemia 588, 589
Abscess 429 Acute suppurative sialadenitis 253 Alum hematoxylin 32
Acanthomatous ameloblastoma Acute posthemorrhagic anemia Alveolar soft part sarcoma 228
292 592 Amalgam tattoo 378
Acanthosis nigricans 633, 728 Adamantine epithelioma 288 Ameloblastic carcinoma 314
Accessory duct 246 Adamantinoblastoma 288 Ameloblastic fibroma 305
Achondroplasia Adamantinohemangiomas 293 Ameloblastic fibroodontoma 307
Achromatic condenser 6 Adamantinoma 288 Ameloblastic fibrosarcoma 316
Acidogenic theory 402 Adamantinoma of long bone 296 Ameloblastic hemangiomas 293
Acidophilic adenoma 268 Addison anemia 587 Ameloblastic odontoma 309
Acinic cell adenoma 274 Addison’s disease 680 Ameloblastic odontosarcoma 317
Acinic cell carcinoma 274 Adenoacanthoma 218 Ameloblastic sarcoma 316
Acquired myotonia 691 Adenocystic carcinoma 272 Ameloblastoma 288
Acquired nevi 138 Adenoid cystic carcinoma 272 Amelogenesis imperfecta 102
Acrocephalysyndactyly 700 Adenoid squamous cell carcinoma Amniotic cavity 55
Acrodermatitis enteropathica 651 218 Amputation neuroma 159
Acrodynia 380 Adenolymphoma 265 Amyloid disease 644
Acromegaly 671 Adenomatoid odontogenic tumor Amyloidosis 644
Acroosteolysis 702 300 Amyotrophic lateral sclerosis 566,
Actinic cheilitis 579 Adrenal insufficiency 681 688
Actinic elastosis 582 Adrenogenital syndrome 681, 725 Anatomy of tongue 549
Actinic keratosis 579 Adult rickets 665 Anderson syndrome 702
Actinic lichen planus 189 Adult’s periodontitis 537 Aneurin 653
Actinomycosis 475 Advanced bell stage 283 Aneurysm varix 158
Acute alveolar abscess 430 African jaw lymphoma 232 Aneurysmal bone cyst 357
Acute atrophic candidiasis 510 Age assessment 734 Angiod streaks 639
Acute bacterial sialadenitis 253 Agenesis 124 Angioreticuloendothelioma 501
Acute exacerbation of chronic Aggressive fibromatosis 142 Angular cheilitis 498, 578
lesion 431 Aging pemphigus 628 Angular cheilosis 578
Acute febrile neutrophilic Aglossia 554 Angular diaphragm 13
dermatosis 722 Aglossia adactylia syndrome 717 Anhydrotic ectodermal dysplasia
Acute herpetic gingivostomatitis Agnathia 123 715
484 Agranulocytic angina 597 Ankyloglossia 555
Acute leukemia 235 Agranulocytopenia 597 Ankyloglossum superius
Acute lymphonodular pharyngitis AIDS 493 syndrome 556
490 AIDS related complex 495 Ankylosed teeth 113
Acute necrotizing ulcerative AJC staging 82 Ankylosis 544
gingivitis 527 Alban test 44 Annular lichen planus 189, 190
844 Textbook of Oral Pathology
Anodontia 99 Basal cell ameloblastoma 292 Blue rubber bleb nevus syndrome
Anterior alveolar cyst 355 Basal cell carcinoma 213 723
Anterior median lingual cyst 363 Basal cell epithelioma 213 Blueing 32
Anti-aging vitamin 667 Basal cell nevus syndrome 727 Boeck’s sarcoid 394
Antibiotic vitamin 661 Basal cell of oral epithelium 285 Bohn’s nodule 339
Antibiotics sore mouth 510 Baseloid mixed cell tumor 272 Botryoid odontogenic cyst 338
Apert’s syndrome 700 Batsakis theory 262 Bowen disease 184
Aphthous pharyngitis 490 B-complex vitamins 653 Bowen syndrome 702
Aphthous stomatitis 389 Beauty vitamin 655 Brachial cyst of parotid 265
Apical periodontal cyst 342 Branchial cleft cyst 361
Beckwith-Wiedemann syndrome
Aplanatic condenser 5 Branchioma 261
716, 729
Aplasia of salivary gland 245 Brandywine type dentinogenesis
Behcet syndrome 391, 724
Aplastic anemia 591 imperfecta 108
Bell stage 283
Arachnodactyly 619 Brazilian pemphigus 626
ARC 495 Bell’s palsy 685 Brittle bone 618
Argyll Robertson pupil 465 Bender ulcer 370 Broder’s classification system 81
Argyria 381 Benign cementoblastoma 312 Bronchial candidiasis -513
Argyrosis 381 Benign lymphoepithelial lesion Bruise 740
Arhinencephaly 131 256 Brushfiel’s spots 701
Arrested caries 417 Benign metastasizing mixed tumor Bruxism 374
Arsenism 382 277 Bruxomania 374
Arterial hemangioma 154 Benign migratory glossitis 560 Buccal caries 412
Arteriovenous fistula 157 Benign mucous membrane Buccolingual masticatory
Arteriovenous malformation 157 pemphigoid 628 syndrome 567
Arteriovenous shunt 157 Beriberi 654 Bud stage 283
Artifacts of histologic section 26 Bernard Soulier syndrome 601 Buffalo hump 681
Asboe-Hansen sign 625 Besnier-Boeck-Schaumann’s Buffer capacity test 45
Ascher’s syndrome 573, 714 disease 394 Bull’s eye 623
Ascorbic acid 661 Beta thalassemia 588, 589 Bullous lichen planus 190
Aspergillosis 520 Biermer’s anemia 587 Bullous pemphigoid 628
Aspiration biopsy 39 Bifid crown 92 Burkitt’s lymphoma 232
Atresia 246 Bifid rib syndrome 714 Burning mouth syndrome 717
Atrophic lichen planus 189
Bifid tongue 556
Attrition 114 C
Bilaminar disc 55
Atypical periodontal disease 503
Biologic carcinogen 76
Auric stomatitis 382 Caffey’s disease 617
Biology of tumor growth 78
Auriculotemporal nerve syndrome Caffey-Silverman syndrome 617,
686 Biopsy 36
702
Auriculotemporal syndrome 719 Biotin 658
Calcific degeneration 427
Auspitz’s sign 635 Bismuthism 378 Calcifying epithelial odontogenic
Autoimmune theory 408 Bite marks 735 cyst 340
Bite marks in human skin 737 Calcifying epithelial odontogenic
B Bite marks on foodstuff 736 tumor 298
Blans syndrome 723 Calcinosis cutis 692
Bacteremia 458 Blast cell leukemia 234 Calcinosis universalis 692
Bacterial meningitis 456 Blastomycosis 516 Callus formation 87
Baelz’s disease 577 Bloch-Sulzberger syndrome 633, Canalicular adenoma 265
Bartholin duct 244 709 Cancellous osteoma 148
Basal cell adenocarcinoma 277 Blue domed cyst 325 Cancrum oris 477
Basal cell adenoma 264 Blue nevus 139 Candida associated lesion 511
 Index 845
Candidal balanitis 513 Cerebrocostomandibular syn- Chronic hemorrhagic osteomyelitis
Candidal endocarditis 515 drome 702 610
Candidal granuloma 514 Cerebrohepatorenal syndrome 702 Chronic hyperplastic candidiasis
Candidal leukoplakia 510 Cervical caries 412 510
Candidal meningitis 515 Chapping of the lips 582 Chronic hyperplastic pulpitis 424
Candidal onychia 512 Charcot triad 689 Chronic idiopathic neutropenia
Candidal paronychia 512 Chediak-Higashi syndrome 599
Candidal septicemia 514 598, 722 Chronic inflammatory gingival
Candidal vulvovaginitis 513 Cheilitis glandularis 576 enlargement 531
Candidiasis 498, 507 Cheilitis glandularis apostematosa Chronic inflammatory periodonti-
Candidiasis with major immuno- 577 tis 537
logical defect 514 Cheilitis granulomatosa 577 Chronic localized mucocutaneous
Candidosis 507 Chemical burn 372 candidiasis 514
Cannon’s disease 640 Chemical carcinogen 75 Chronic lymphatic leukemia 238
Cap stage 283 Chemical oxidation 32 Chronic mucocutaneous candidi-
Carabelli cusp 467 Chemical theory 29, 401 asis 513
Carcinogenesis 73 Chemistry of stain 28 Chronic mucocutaneous candidi-
Carcinoma ex odontogenic cyst 316 Chemoparasitic theory 402 asis in association with thy-
Carcinoma ex pleomorphic Cherubism 608 moma 514
adenoma 275 Chickenpox 487 Chronic myeloid leukemia 237
Carcinoma in situ 183 Child abuse 735 Chronic post hemorrhagic anemia
Carcinoma of buccal mucosa 209 Chip munk facies 589 593
Carcinoma of floor of mouth 208 Chloroma 236 Chronic pulpitis 423
Carcinoma of labial mucosa 210 Choline 662 Chronic sclerosing osteomyelitis
Carcinoma of lip 581 Chondroblastoma 145 613
Carcinoma of maxillary sinus 211 Chondrodystrophia fetalis 620 Chronic sclerosing sialadenitis 254
Carcinoma of palate 210 Chondroectodermal dysplasia 131, Chronic superficial glossitis 568
Carcinosarcoma 218, 277 700, 708 Chronic superficial interstitial
Caries activity test 43 Chondrogenic sarcoma 221 glossitis 466
Cat scratch disease 481 Chondroma 144 Chronic suppurative osteomyelitis
Cat scratch fever 481 Chondromyxoid fibroma 146 440
Causes of pulpal inflammation 420 Chondrosarcoma 221 Cicatricial pemphigoid 628
Cavernous hemangioma 153 Chorea minor 566 Cigarette smoker lip lesion 185
Cavernous sinus thrombosis 457 Choristoma 71 CIN grading 81
Celiac disease 652 Christmas disease 603 Cirsoid aneurysm 158
Cell rest of serrae 284 Christ-Siemes-Fouraine syndrome Classification of bite marks 736
Cellulitis 449 715 Classification of stain 29
Cemental hyperplasia 121 Chromatic aberration 8 Cleaning of eyepiece 18
Cementicles 122 Chronic adult’s periodontitis 537 Clear cell ameloblastoma 293
Cementifying fibroma 614 Chronic alveolar abscess 432 Clear cell odontogenic carcinoma
Cemento-ossifying fibroma 614 Chronic atrophic candidiasis 511 316
Central giant cell granuloma Chronic bacterial sialadenitis 253 Clear cell odontogenic tumor 316
165, 610 Chronic diffuse mucocutaneous Clearing 23
Central mandibular carcinoma 315 candidiasis 514 Cleft formation 574
Central mucoepidermoid carci- Chronic disseminated histiocytosis Cleft lip 573
noma 272 X 646 Cleft palate 573
Central papillary atrophy of Chronic familial mucocutaneous Cleft tongue 556
tongue 559 candidiasis 514 Cleidocranial dysostosis 127
Central squamous cell carcinoma Chronic granulomatosis disease Cleidocranial dysplasia 127, 700
315 395 Clonus 691
846 Textbook of Oral Pathology
Cluster headache 719 Craniofacial fibrous dysplasia 607 Dentin dysplasia 109
Coccidiodal granuloma 518 Craniotabes 665 Dentin hypocalcification 110
Coccidioidomycosis 518 Crenated tongue 562 Dentinal sclerosis 118
Codman’s tumor 145 CREST syndrome 715 Dentinoameloblastoma 294
Coecal plate 57 Cretinism 674 Dentinogenesis imperfecta 108
Cold abscess 473 Critical illumination 4 Denture injury tumor 382
Cold sore 484 Crocodile tear 686 Denture stomatitis 511
Collagen disorders 396 Crouzon syndrome 128, 701 Depapillation of the tongue 563
Color codes 7 Cryptococcosis 517 Deparaffinization 35
Comedo necrosis 278 Cryptogenic leukoplakia 174 Dermatitis herpetiformis 631
Commissural pits 572 Curry-Hall syndrome 707 Dermatitis medicamentosa 387
Compact osteoma 148 Cushing’s syndrome 615, 681, 725 Dermatitis venenata 387
Compensating eyepiece 10 Cutis hyperelastica 634 Dermatomyositis 565, 692
Complex odontoma 308 Cutis rhomdoidalis 582 Dermato-polyneuritis 380
Compound microscope 3 Cyanocobalamin 659 Dermoid cyst 359
Compound nevi 138 Cyclic neutropenia 596 Desert fever 518
Compound odontoma 308 Cylindroma 272 Desmoplastic ameloblastoma 294
Concrescence 93 Cyst lining 285 Desmoplastic fibroma 142
Condenser 4 Cystic hygroma 157, 363 Desquamative gingivitis 529
Condensing osteitis 433 Cysticercosis cellulosae 365 Development of mandible 60
Condyloma acuminatum 492 Cytology 41 Development of maxillary sinus 61
Condyloma lata 465 Cytomegalovirus inclusion disease Development of tongue 59
Confocal microscope 15 252 Development of tooth 282
Congenital epulis 533 Cytomegalovirus infection 493, Developmental salivary gland
Congenital erythrokeratoderma 499 depression 245
708 Dewar test 45
Congenital facial diplegia 717 D Di George syndrome 515
Congenital fistulas 572 Diabetes mellitus 678
Congenital leukokeratosis 640 Dardick theory 262 Diffuse calcification 427
Congenital lip pits 572 Darier’s disease 638 Diffuse lipoma 146
Congenital myotonia 691 Darier-White disease 638 Diffuse neonatal hemangiomatosis
Congenital nevi 138 Dark field microscopy 11 723
Congenital syphilis 466 Darling disease 515 Dilaceration 95
Congenital teeth 102 Decalcification method -21 Dilated composite odontome 95
Construction of microscope 3 Decubitus ulcer 370 Diphtheria 478
Contact allergy 387 Degenerative arthritis 541 Direct staining 30
Contact cheilitis 579 Dehydration 22 Disappearing bone 621
Continuum concept 304 Delayed eruption 112 Discoid lupus erythematosus 202
Control of dental caries 417 Dens evaginatus 97 Discrete pulp stone 426
Contusion 740 Dens invaginatus 95 Disseminated juvenile fibrous
Cooley’s anemia 588 Dense bone island 152 dysplasia 608
Coronal dens invaginatus 96 Dense in dente 95 Disseminated sclerosis 689
Cotton roll burns 372 Dental comparison 731 Distomolar 93, 101
Cowden syndrome 715 Dental fluorosis 106 Diverticuli 246
Coxsackie’s virus infection 490 Dental follicle 283 DNA oncogenic virus 77
Cracked tooth syndrome 428 Dental lamina cyst of newborn 339 DNA profiling 734
Cranial arteritis 564 Dental lamina formation 282 Dolichocephaly 707
Craniocleidodysostosis 127 Dental sac 283 Donovanosis 480
Cranioectodermal dysplasia 707 Denticles 426 Double demonstration eyepiece 10
Craniofacial dysostosis 128, 701 Dentigerous cyst 323 Double lip 573
 Index 847
Down syndrome 619, 701 Endothelial myeloma 224 Excrescence 184
Drug allergy 387 Endothelioma 261 Exfoliative cheilitis 500, 580
Drug idiosyncrasy 387 Enostosis 152 Exfoliative cytology 42
Drug-induced cheilitis 581 Enucleation 368 Exocrine gland 242
Drug-induced osteoporosis 616 Enzyme histochemistry 48 Exostosis 152
Drug sensitivity 387 Eosin 33 Exostosis of root 121
Dry beriberi 655 Eosinophilic granuloma 647 External resorption 119
Dubreuilh’s elastoma 582 Eosinophilic granuloma of tongue Extraosseous ameloblastoma 295
Ductal papillomas 269 563 Extravasation cyst 355
Duhring-Brocq disease 631 Epidermoid carcinoma 206, 277 Eyepiece 9
Dukes AJC staging 82 Epidermoid cyst 359
Dyskeratosis congenita 201, 715 Epidermolysis bullosa 630 F
Dyskinesia 566 Epidermolysis bullosa dystrophic
Dystrophic myotonia 690 dominant 630 Facial hemiatrophy 126
Dystrophic myotonica 690 Epidermolysis bullosa dystrophic Facial hemihypertrophy -125
recessive 630 Facial paralysis 685
E Epidermolysis bullosa latalis 630 Facial space infection 454
Epidermolysis bullosa simplex 630 Factor V deficiency 603
Ecchondroma 145 Epigenetic theory 78 Factors affecting healing 84
Echinococcosis 364 Epithelial myoepithelial carcinoma Factors affecting staining 31
Ectodermal dysplasia 100 277 False gemination 93
Ectopic eruption 114 Epithelial odontome 288 Familial benign chronic pemphi-
Eczematous cheilitis 579 Epithelioma 206 gus 629
Edward syndrome 701 Epitheloid cell nevus 138 Familial fibrous dysplasia of the
Ehlers Danlos syndrome 634, 705 Epstein pearl 339 jaws 608
Electrical burns 371 Epulis fissuratum 382 Familial hypophosphatemia 666
Electron microscope 4, 16 Epulis granulomatosum 384 Familial multilocular cystic disease
Elephantiasis gingivae 532 Erosion 116 of the jaws 608
Elephantiasis neuromatosa 161 Erosion interdigitalis -512 Familial osteodysplasia 702
ELISA 504 Erosive lichen planus 193 Familial pancytopenia 713
Ellis-Van Creveld syndrome 131, Eruption cyst 329 Familial panmyelophthisis 713
700, 708 Eruption hematoma 329 Familial thrombasthenia 602
Embedded teeth 112 Eruption of teeth 110 Fanconi’s syndrome 713
Embedding 23 Eruption sequestration 113 Fat soluble vitamins 662
Embryonal Rhabdomyosarcoma Erysipelas 462 Fetal deciduous teeth 102
228 Erythema multiforme 623 Fever blister 484
Enamel cord 283 Erythema nodosum leprosum 471 Fibroadamanblastoma 305
Enamel droplet 98 Erythematous candidiasis 498 Fibrocementoma 313
Enamel hypoplasia 105 Erythremia 595 Fibrocystic disease 605
Enamel knot 283 Erythroblast 55 Fibroma 140
Enamel nodule 98 Erythroblastic anemia 588 Fibroma molluscum 160
Enamel pearl 98 Erythroblastosis fetalis 593 Fibromatosis gingivae 532
Enameloma 98 Erythrodermic psoriasis 635 Fibro-osteodystrophy 605
Encapsulated lipoma 146 Erythroleukoplakia 182 Fibrosarcoma 219
Encephalofacial angiomatosis 724 Erythroplakia 181 Fibrous adamantinoma 305
Enchondroma 145, 261 Erythroplasia of Queyrat 181 Fibrous dysplasia 605
Enclavoma 261 Erythropoietic porphyria 594, 645 Fibrous epulis 142
Endocrinopathy candidiasis Evaginated odontome 97 Fibrous histiocytoma 142
syndrome 514 Ewing sarcoma 224 Fibrous hyperplasia 382
Endodermal layer 56 Excisional biopsy 38 Fibroxanthoma 142
848 Textbook of Oral Pathology
Fine needle aspiration cytology 43 Gastro-intestinal candidiasis 513 Glucose 6 phosphatase deficiency
Fissured tongue 559 Gaucher disease 648 anemia 594
Fixation 21 Gemination 92 Gonorrhea 467
Fixation in histochemistry 47 General Adaptation syndrome 725 Gorham syndrome 621
Floor of mouth 67 General paresis 465 Gorlin-Goltz syndrome 366, 714,
Florid osseous dysplasia 613 Generalized cortical hyperostosis 727
Flow cytometry 51 621 Gorlin cyst 340
Fluorescence microscopy 15 Generalized neurological disease Gorlin sign 557
Fluorescent microscope 4 566 Gougerat-Sjogren’s syndrome 710
Fluoride toxicity 620 Generalized septicemia 451 Gout 547
Fly-catcher’s tongue or bon-bon Genetic theory 78 Grading of dysplasia 178
sign 567 Geniculate neuralgia 684 Grading of oral submucus fibrosis
Focal epithelial hyperplasia 132 Genital wart 492 199
Focal infection 459 Geographic tongue 560 Grading of tumor 80
Focal length 6 Geotrichosis 519 Graham little syndrome 187
Focal melanosis 131 Gestant odontome 95 Granular ameloblastoma 292
Focal osteitis fibrosa 605 Ghost teeth 109 Granular cell myoblastoma 163
Focal osteoporotic bone marrow Giant cell epulis 165 Granular cell schwannoma 163
defect 130 Giant cell fibroma 143 Granular cell tumor 163
Focal reversible pulpitis 422 Giant cell granuloma 165 Granulocytopenia 597
Folacin 658 Giant cell reparative granuloma 610 Granuloma inguinale 480
Folate 658 Giant cell tumor 164 Granuloma pyogenicum 167, 535
Foliate papillitis 563 Giant osteoid osteoma 149 Granuloma venereum 480
Folic acid 658 Gigantiform cementoma 613 Granulomatous cheilitis 577
Folic acid deficiency 565 Gigantism 671 Graze 740
Follicular ameloblastoma 290 Gilles de la Tourette’s syndrome Ground section 20
Follicular cyst 323 566 Gubernacular dentis 285
Follicular lichen planus 189 Gingival abscess 530 Gumma 465
Foot and mouth disease 491 Gingival cyst of adults 336, 338 Gustatory sweating 686, 719
Fordyce granule 132 Gingival cyst of newborn 338 Guttate lichen planus 190
Fosdick acid dissolution test 45 Gingival enlargement due to drug Guttate psoriasis 635
Fournier molar 99 534
Fracture of teeth 377 Gingival inflammation 525 H
Franceschetti syndrome 129 Gingival salivary gland choristoma
Frey’s syndrome 686, 719 245 Habitual cheek biting 369
Friedrich disease 125 Gingivitis 525 Hailey-Hailey disease 629
Frontonasal dysplasia 703 Glandular cheilitis 576 Hair lip 575
Frontonasal process 58 Glandular fever 598 Hairy leukemia 239
Frost bite of lip 372 Glandular odontogenic cyst 338 Hairy leukoplakia 500
Frozen section 24 Glanzmann’s disease 602 Hairy tongue 561
Frozen section biopsy 40 Globodontia 99 Hajadu-Cheney syndrome 702,
Fusion 93 Globulomaxillary cyst 353 715
Glomangioma 158 Hallopeau type of pemphigus 626
G Glomus tumor 158 Hamartoma 71, 134
Glossalgia 565 Hand-Schuller-Christian disease
Ganglioneuroma 161 Glossitis areata exfoliativa 560 646
Gangrenous stomatitis 477 Erythema migrans 560 Hand foot and mouth disease 490
GAPO 708 Glossodynia 565 Hansen disease 468
Gardner’s syndrome 712 Glossoplegia 567 Harrison grooves 665
Garre’s osteomyelitis 441 Glossopyrosis 565 Healing cyst 348
 Index 849
Healing of extraction wounds 86 Hidebound disease 397 Identification of edentulous bodies
Healing of fracture 87 Histiocytosis X 646 733
Healing of osteogranated implants Histiocytosis Y 136 Idiopathic bone cavity 355
88 Histochemical technique 47 Idiopathic leukoplakia 174
Heck disease 132 Histoplasmosis 515 Idiopathic steatorrhea 652
Heerfordt’s syndrome 257, 711 History of microscope 3 Idiopathic thrombocytopenic
Hemangioameloblastoma 293 Hodgkin lymphoma 229 purpura 503, 599
Hemangioma 152 Homogenous leukoplakia 175 Immature teeth 102
Hemangiopericytoma 158 Homozygous β-thalassemia 588 Immunofluorescent technique 50
Hematoxylin 32 Hormonal carcinogen 76 Immunohistochemical method 48
Hematoxylin and eosin 31 Hormonal tumor 169 Impacted teeth 112
Hemifacial microstomia 126 Horner’s syndrome 720 Impetigo contagiosa 461
Hemimaxillofacial dysplasia 127 Host defense mechanism 444 Impetigo vulgaris 461
Hemoglobin Bart disease 588 Humoral theory 402 Importance of bite marks 735
Hemoglobin H disease 588 Hunter’s glossitis 660 Imprint abrasion 740
Hemophilia A 602 Huntington’s chorea 566 Incised wound 740
Hemophilia B 603 Hurler syndrome 649, 716 Incisional biopsy 39
Hemorrhagic bone cyst 355 Hutchinson-Gilford syndrome 726 Incisive canal cyst 348
Hemorrhagic vitamin 667 Hutchinson triad 467 Incontinentia pigmenti 633, 709
Hepatic porphyria 645 Hutchison incisor 99 Indirect staining 30
Hereditary benign intraepithelial Hutchison sign 488 Infantile cortical hyperostosis 617,
dyskeratosis 641 Hyalinosis cutis et mucosa oris 640 702
Hereditary brown enamel 102 Hybridization method 53 Infantile osteomyelitis 439
Hereditary brown opalescent teeth Hydatid cyst 364 Infectious mononucleosis 598
102 Hydatid disease 364 Infectious stomatitis 484
Hereditary disease of newborn 593 Hydrostatic enlargement theory of Infiltration 23
Hereditary ectodermal dysplasia cyst growth 322 Inflamed dentigerous cyst 327
706 Hypercementosis 121 Inflammatory collateral cyst 347
Hereditary ellpitocytosis 594 Hyperdontia 101 Inflammatory fibrous hyperplasia
Hereditary enamel dysplasia 102 Hypermobility 546 382
Hereditary fibrous dysplasia of the Hyperostosis 152 Inflammatory hyperplasia 167
jaws 608 Hyperparathyroidism 676 Inflammatory papillary hyperpla-
Hereditary fructose intolerance Hyperpituitarism 671 sia 383
650 Hyperplasia 70, 134 Inositol 662
Hereditary hemorrhagic Hyperplasia of salivary gland 246 Interdigital candidiasis -512
telangiectasia 156, 714 Hyperthyroidism 673 Internal resorption 120
Hereditary hypohidrotic (anhy- Hypertrophic lichen planus 189 Interproximal caries 412
drotic) ectodermal dysplasia Hypervitaminosis A 664 Intertriginous candidiasis -513
100, 708 Hypoglycemia 650 Intra-alveolar cyst 353
Hereditary mucoepithelial Hypognathous 123 Intradermal nevi 138
dysplasia 639 Hypoparathyroidism 677 Intraepidermoid carcinoma 184
Hereditary opalescent teeth 108 Hypophosphatasia 651 Intraepithelial carcinoma 183
Hereditary spherocytosis 594 Hypopituitarism 672 Intra-lingual cyst of foregut origin
Hereditary thymic dysplasia 514 Hypoplasia of salivary gland 245 363
Herlitz’s disease 630 Hypothyroidism 674 Intraoral lymphoepithelial cyst 361
Herpangina 490 Intra-osseous biopsy 39
Herpes labialis 484 I Intraosseous CEOT 299
Herpes zoster 488, 499 Inverted ductal papilloma 269
Herpetiform ulcer 389 Iceberg tumor 261 Investigations of a bite 738
Hibernoma 147 Id reaction 511 Iron deficiency anemia 564, 585, 721
850 Textbook of Oral Pathology
Iron hematoxylin 33 Laceration 740 Lipoid proteinosis 640, 650
Irregular dentin 118 Lacrimo-auriculo-dento-digital Lipoma 146
Irreversible pulpitis 424 syndrome 713 Lipomatosis 146
Isolated Darier’s disease 638 Lane tumor 218 Liposarcoma 220
Ivory osteoma 148 Laser capture microdissection 54 Ljubljana classification system 179
Lateral periodontal cyst 337 Lobstein disease 618
J Lateral pterygoid 65 Localized myositis ossificans 687
Lazy leukocyte syndrome 596, 723 Lou Gehrig disease 688
Jadassohn-Lewandowsky syn- Lead poisoning 379 Ludwig angina 450
drome 637 Legend of worm 401 Luekemoid reaction 234
Jaffe type fibrous dysplasia 606 Leiomyoma 162 Lues 462
James Ramsey Hunt syndrome 489 Leiomyosarcoma 227 Lumbar lordosis 665
Jaw - winking syndrome 721 Leishmaniasis 521 Lupus erythematosus 201
Jaw-cyst-bifid rib syndrome 366, Lemmoma 159 Lyell’s disease 624
727 Lentigo maligna melanoma 215 Lymphangioma 156
Johanson-Blizzard syndrome 713, Leong premolar 97 Lymphoepithelial cyst 361
708 Leonine facies 470 Lymphoepithelial cyst of neck 361
Junctional bullous epidermatosis LEOPARD syndrome 708 Lymphoepithelial cyst of parotid
630 Lepromas 471 gland 361
Junctional epidermolysis bullosa Lepromatous leprosy 470 Lymphogranuloma venereum 480
630 Leprosy 468
Junctional nevi 138 Letterer Siwe disease 647 M
Juvenile myxedema 674 Leucosis 234
Juvenile periodontitis 539 Leukemia 234 Macrodontia 91
Juxtacortical osteosarcoma 223 Leukoedema 170 Macroglossia 554
Leukokeratosis nicotina glossi 563 Macrognathia 125
K Leukoplakia 171, 568 Maffucci syndrome 723
Leukoplakia erosiva 175 Magnification 6
Kaposi’s sarcoma 501 Leukoplakia simplex 175 Major aphthae 389
Kawasaki disease 398, 709 Leukoplakia verrucosa 175 Malabsorption syndrome 652
Keratinizing and calcifying Lichen planus 185 Malignant ameloblastoma 313
odontogenic cyst 340 Lichen planus pemphigoides 190 Malignant changes in odontogenic
Keratinizing metaplasia 663 Lichen planus pigmentosus 190 cyst 316
Keratoacanthoma 137 Lichenoid contact dermatitis 190 Malignant fibrous histiocytoma
Keratoameloblastoma 293 Light microscope 4 220
Keratosis follicularis 638 Linea Alba 369 Malignant fibroxanthoma 220
Klestadt’s cyst 352 Linear lichen planus 190 Malignant granular cell
Klinefelter syndrome 98 Lingua dissecta 559 myoblastoma 228
Klippel-Trenaunay syndrome 729 Lingua villosa 561 Malignant granuloma 395
Klippel-Trenaunay-Weber syn- Lingual caries 412 Malignant hemangioendothelioma
drome 718 Lingual cyst 558 225
Koebner phenomenon 187 Lingual paresthesia or dysesthesia Malignant hemangiopericytoma
Korsakoff’s psychosis 655 565 226
Kuttner’s disease 254 Lingual polyp 558 Malignant lymphoma 229
Kwashiorkor disease 643 Lingual thyroid nodule 557 Malignant melanoma 214
Lingual varicosities 556 Malignant pleomorphic adenoma
L Lip ulcers due to caliber persistent 275
artery 582 Malignant schwannoma 227
Laband syndrome 718 Lipid reticuloendothelioses 646 Management of AIDS 505
Labial melanotic macule 131 Lipoblastomatosis 147 Management of osteomyelitis 442
 Index 851
Mandible 63 Mesiodens 93, 101 Morsicatio buccarum 369
Mandibular buccal infected cyst Mesocrine gland 242 Morula 55
347 Mesoderm 57 Motor system disease 688
Mandibular tori 151 Metachromatic staining 31 Mottled enamel 106
Mandibulofacial dysostosis 129, Metallic impregnation 31 Mounting 25
703 Metaplasia 70 Mucinous adenocarcinoma 278
Marasmus 643 Metaplasia in dentigerous cyst 328 Mucocele 248
Marble bone disease 617 Metastasis 80 Mucocutaneous lymph node
Marcus gun phenomenon 721 Metastasizing pleomorphic syndrome 398, 709
Marfan’s syndrome 619, 705 adenoma 277
Mucoepidermoid carcinoma 269
Marfan-Achard syndrome 619 Metastatic carcinoma 212, 277
Mucoepidermoid cyst 338
Marie and Sainton disease 127 Method of preservation of bite
Mucopolysaccharide 716
Marsupialization of cyst 367 marks 736
Mucormycosis 517
Masseter muscle 64 Mibelli’s disease 637
Massive osteolysis 621 Microbiology of dental caries 403 Mucositis 373
Masson’s trichrome 34 Microdontia 91 Mucous salivary gland 242
Matching the bite mark 740 Microglossia 554 Mucous extravasation cyst 249
Mature teeth 102 Micrognathia 124 Mucous patches 464
Maxilla 62 Micrometry 10 Mucous retention cyst 249
Maxillary sinus 67 Midline lethal granuloma 395 Mulberry molar 99, 467
Maxillary sinusitis 452 Midline lethal granulomatous Multicentric oral carcinoma 218
Measles 486 ulceration 395 Multifocal eosinophilic granuloma
Mechanical tube 9 Midline non-healing granuloma 646
Medial pterygoid 65 395 Multilocular cyst 288
Median alveolar cyst 355 Miescher’s disease 714 Multiple endocrine neoplasia
Median anterior maxillary cyst 348 Miescher’s syndrome 577 syndrome 727
Median cleft face syndrome 703 Migraine 720 Multiple enostosis 613
Median mandibular cyst 353 Migraine syndrome 720 Multiple hamartoma syndrome
Median palatine cyst 351 Mikulicz disease 256 715
Median rhomboid glossitis 559 Mild restricted muscular dystro- Multiple myeloma 239
Median rhomboidal glossitis 511 phy 690 Multiple sclerosis 689
Mediastinitis 458 Miliary tubercle 474 Multistep theory 80
Mediterranean anemia 588 Minor aphthae 389
Mumps 252
Megadontia 91 Minor salivary gland tumor 278
Munro’s abscess 636
Megagnathia 125 Mixed odontogenic tumor 304
Mural ameloblastoma 295
Megaloblastic anemia 591 Mobius syndrome 717
Mural growth theory of cyst
Melanoameloblastoma 162, 295 Mohr syndrome 703
Melanotic ameloblastoma 162 Molluscum contagiosum infection growth 322
Melanotic neuroectodermal tumor 492 Muscle of mastication 64
of infancy 162 Molluscum sebaceum 137 Muscular dystrophy 689
Melanotic progonoma 162 Mongolism 619 Myasthenia gravis 691
Melkersson-Rosenthal syndrome Mongoloid appearance 589 Mycosis fungoides 232
578, 714 Monoclonal hypothesis 79 Myeloid sarcoma 610
Melnick Needles syndrome 703 Monomelic fibrous dysplasia 606 Myelomatosis 239
Membrane preformitiva 283 Monomorphic adenoma 264 Myiasis 481
MEN syndrome 727 Monostotic fibrous dysplasia 606 Myoblastic myxoma 163
Mercurialism 380 Moon face 681 Myoepithelioma 268
Mesenchymal chondrosarcoma Moon molar 99, 467 Myositis ossificans 686
222 Morbilli 486 Myotonias 690
Mesenchyme 57 Morphea 397 Myotonic dystrophy 690
852 Textbook of Oral Pathology
Myxadenitis labialis 577 Non-Hodgkin lymphoma 230 Orofacial digital syndrome 715
Myxodema 675 Non-odontogenic hamartoma 71 Orofacial digital syndrome II 703
Myxofibroma 144 Non-lipid reticuloendothelioses Oropharyngeal dysphagia 567
Myxoma 144 646 Osier’s disease 714
Non-opalescent and opalescent Osier-Weber-Rendu disease 714
N teeth 109 Osler’s disease 595
Norton’s syndrome 719 Ossifying fibroma 614
Naegali-Franceschetti-Jadassohn Nosepiece 9 Osteitis deformans 612
syndrome 713 Notochord 56 Osteitis fibrosa localisata 605
Nasoalveolar cyst 352 Numerical aperture 7 Osteoarthritis 541
Nasolabial cyst 352 Nursing bottle caries 416 Osteoarthrosis 541
Nasopalatine cyst 348 Osteoblastoma 149
Nasopalatine duct cyst 348 O Osteochondroma 150
Nasopharyngeal angiofibroma 167 Osteoclastoma 165, 610
Nasopharyngeal cyst 364 Occlusal caries 414 Osteodysplasty 703
Natal teeth 102 Occlusal enamel pearls 97 Osteogenesis imperfecta 396, 618
Necrosis of pulp 428 Oculo-dento-digital dysplasia 713 Osteogenic sarcoma 223
Necrotizing fascitis 458 Odontoameloblastoma 309 Osteoid osteoma 149
Necrotizing sialometaplasia 279 Odontogenesis 282 Osteoma 148
Neonatal teeth 102 Odontogenesis imperfecta 108 Osteomalacia 665
Neoplasm 72, 134 Odontogenic dysplasia 109 Osteomatosis 148
Nerve sheath myxoma 144 Odontogenic fibroma 310 Osteomyelitis 435
Neumann type of pemphigus 626 Odontogenic fibro-myxoma 311 Osteopetrosis 617
Neural cord 57 Odontogenic fibrosarcoma 317 Osteopoikile 152
Neural plate 57 Odontogenic hamartoma 71 Osteopoikilosis 152
Neurilemmoma 159 Odontogenic infection of orbit 457 Osteoporosis 615
Neurinoma 159 Odontogenic keratocyst 330 Osteosarcoma 223
Neuroblastoma 226 Odontogenic myxoma 311 Osteosclerosis 433
Neurofibroma 160 Odontoma 308 Osteosclerosis fragilis generalisata
Neurofibromatosis 160 OFD II 703 617
Neurofibrosarcoma 227 OFD type 1 716 Otodental dysplasia 707
Neurogenic sarcoma 226 Oncocytic carcinoma 278 Out of focus light rays 16
Neuroma 159 Oncocytoma 268 Oxyphilic adenoma 268
Neurosarcoma 226 Oncocytosis 268
Neurosyphilis 465 Optical aberration 7 P
Nevoid basal cell carcinoma 366 Optical maintenance 18
Nevoid basal cell carcinoma Optical path 10 Pachyderma oralis 640
syndrome 714, 727 Oral alimentary tract cyst 363 Pachyonychia congenita 637
Niacin 656 Oral cyst with gastric epithelium Paget’s disease 612
Niacin deficiency 565 363 Palatal caries 412
Nicotinic acid 656 Oral cyst with intestinal epithelium Palatal epithelial hyperplasia 383
Niemann-Pick disease 648 363 Palatal papillomatosis 383
Night grinding 374 Oral epithelial nevi 640 Palatine torus 151
Nikolsky’s sign 625, 629 Oral florid papillomatosis 176 Pantothenic acid 657
Nodular elastoidosis 582 Oral foci of infection 459 PAP smear 41
Nodular fascitis 384 Oral human papilloma virus Papillary cyst adenocarcinoma 278
Nodular leukoplakia 175 infection 499 Papillary cystadenoma
Nodular melanoma 215 Oral melanotic macule 131 lymphomatosum 265
Noma 477 Oral mucosal brush biopsy 40 Papillion-Lefevre syndrome 540, 718
Non-bullous impetigo 461 Oral submucus fibrosis 194 Papilloferous ameloblastoma 293
 Index 853
Papilloma 135 Periodontal abscess 431 Plexiform hemangioma 154
Papular lichen planus 188 Periodontal pockets 536 Plexiform neuroma 161
Paradental cyst 347 Periodontosis 539 PLGA 276
Parahemophilia 603 Periorificial lentiginosis 710 Plicated tongue 559
Paralysis 567 Periosteal osteosarcoma 223 Plumbism 379
Paramedian lip pits 572 Periostitis of jaw 435 Plummer-Vinson syndrome 564,
Paramolar 101 Peripheral ameloblastoma 295 586, 721
Paraneoplastic pemphigus 625 Peripheral CEOT 299 Plunging ranula 251
Para-pemphigus 628 Peripheral giant cell granuloma Pointer eyepiece 10
Parasitic cyst 364 165 Polarized light microscopy 14
Parasitic theory 401 Peripheral giant cell reparative Polycythemia rubra vera 595
Paratrigeminal syndrome 719 granuloma 165 Polycythemia vera 595
Parkes-Weber syndrome 729 Peripheral giant cell tumor 165 Polymerase chain reaction 52, 504
Parosteal osteosarcoma 223 Peripheral vascular disease 565 Polymorphous low grade
Parotid gland 243 Perleche 578 adenocarcinoma 276
Paroxysmal nocturnal Pernicious anemia 564, 587 Polymyositis 692
hemoglobinemia 595 Peterson-Brown-Kelly syndrome Polyostotic fibrous dysplasia -606
Parrot beak 129 586 Polypoid squamous cell carcinoma
Parry Romberg syndrome 126 Peutz-Jeghers syndrome 710 218
Partial harelip 131 Phantom bone 621 Porokeratosis 637
Partsch’s operation 367 Pharynx 69 Porphyria 645
PAS 33 Phase contrast microscopy 12 Port wine stain 154
Patau syndrome 702 Phase shifting ring 13 Post herpetic neuralgia 488
Patent thyroglossal duct cyst 558 Phlebectasia 570 Post menopausal osteoporosis 615
Pathophysiology of infection 448 Phlegemon 449 Post permanent dentition 102
Pattern abrasion 740 Phoenix abscess 431 Post traumatic myositis ossificans
PCR 52 Phosotungestic acid hematoxylin 687
Pellagra 656 33 Postmortem serology 734
Pemphigus 625 Phycomycosis 517 Postrhagadic scarring 466
Pemphigus erythematosus 626 Phyloquinone 667 Pott’s disease 473
Pemphigus foliaceous 626 Physical carcinogen 75 Pre-eruptive caries 417
Pemphigus vegetans 626 Physical theory 30 Precancerous conditions 170
Pemphigus vulgaris 625 Pierre Robin syndrome 619, 704 Precancerous lesion 170
Periapical abscess 429 Pigeon breast 665 Predeciduous dentition 102
Periapical cemental dysplasia 313 Pigmentation of tongue 569 Pregnancy tumor 169, 535
Periapical cyst 342 Pigmented ameloblastoma 162 Preleukoplakia 174
Periapical fibrosarcoma 313 Pigmented cellular nevus 138 Premature eruption 112
Periapical granuloma 433 Pindborg tumor 298 Premature exfoliation 114
Periapical osteitis 429 Pingueculae 648 Premaxilla-maxillary cyst 353
Periapical osteofibrosis 313 Pink disease 380 Primary anemia 587
Periapical scar 434 Pink spot 120 Primary epithelial tumor of the jaw
Pericoronal abscess 530 Pit and fissure caries 414 315
Pericoronal cyst 323 Pituitary ameloblastoma 296 Primary healing 85
Pericoronal dental follicle 284 Pityriasis rosea 632 Primary herpes simplex infection
Pericoronitis 530 Pizza burn 372 484
Peridens 101 Plaque lichen planus 189 Primary intra-alveolar epidermoid
Perineural fibroblastoma 159 Plasma cell cheilitis 581 carcinoma 315
Periodic acid Schiff method 33 Plasmacytoma 241 Primary intraosseous carcinoma
Periodic migranous neuralgia 719 Pleomorphic adenoma 261 315
Periodic neutropenia 596 Plexiform ameloblastoma 291 Primary lymphoma of bone 232
854 Textbook of Oral Pathology
Primary palate formation 58 Pyridoxine 658 Riboflavin 655
Primary reticular cell carcinoma Pyruvate kinase deficiency anemia Rickety rosary 665
232 593 Riga fede disease 102
Primary Sjogren syndrome 254 Riley-Day syndrome 712
Primary syphilis 463 R RNA oncogenic virus 77
Primary thrombocytopenic Robin anomalad 619
purpura 599 Rabbit syndrome 567 Rodent facies 589
Primitive node 56 Rachitic metaphysis 667 Rodent ulcer 213
Primitive streak 56 Radiation carcinogen 75 Romberg hemifacial atrophy 126
Primordial cyst 335 Radiation injury 373 Root caries 415
Pringle-Bourneville syndrome 728 Radiation osteomyelitis 442 Root end cyst 342
Adenoma sebaceum 728 Radicular cyst 342 Root less teeth 109
Procedure in dental identification Radicular dens invaginatus 96 Routine stain 31
732 Radicular dentin dysplasia 109 Rubeola 486
Prochordal plate 56 Ramon syndrome 718 Rubinstein Taybi syndrome 94
Progeria 673, 726 Rampant caries 416 Rutherford syndrome 718
Progressive bulbar palsy 688 Ranula 250
Progressive hemifacial atrophy Rapidly progressive periodontitis S
126 538
Progressive muscular atrophy 688 Reactive lymphoid aggregated 558 Salivary duct carcinoma 278
Progressive myositis ossificans 687 Reader’s syndrome 719 Salivary gland calculus 247
Progressive osteolysis 621 Real image 10 Salivary gland stone 247
Progressive staining 30 Recovery of dental structure 732 Salivary gland virus disease -252
Proliferative periostitis 441 Recurrence herpes labialis 499 Salmon patch 154
Prolymphocytic leukemia 239 Recurrence herpetic infection 486 Sarcoidosis 394
Protein deficiency 643 Recurrence intraoral herpes 486 Scanning electron microscope 17
Proteolysis chelation theory 408 Recurrent bullous eruption of Scarlet fever 477
Proteolytic theory 407 hands and feet 630 Schwannoma 159
Proteomics 54 Recurrent caries 415 Scleroderma 397
Provitamins 653 Recurrent herpes labialis 486 Sclerosing cementum 313
Pseudo-hypoparathyroidism 677 Reductase test 46 Sclerosing osteitis 613
Pseudolipoma 146 Refractory rickets 666 Sclerosing osteomyelitis 440
Pseudomembranous candidiasis Regional odontodysplasia 109 Sclerotic cemental masses 613
498 Regressive staining 30 Scorbutic lattice 662
Pseudosarcomatosus fascitis 384 Reiter syndrome 392, 724 Scratch 740
Pseudoxanthoma elasticum 639 Rendu-Osler-Weber disease 156 Scrofula 473
Psoriasis 634 Reparative lesion 563 Scrotal tongue 559
Pterygoid Levator synkinesis 721 Residual cyst 346 Scurvy 661
Pulp calcification 426 Reticular atrophy of pulp 427 Scurvy bud 661
Pulp degeneration 425 Reticular lichen planus 188 Sebaceous carcinoma 278
Pulp hyperemia 422 Retinal analage tumor 162 Secondary carcinoma 212
Pulp nodules 426 Retinol 663 Secondary dentin 118
Pulp polyp 424 Retrocuspid gingiva 534 Secondary healing 86
Pulp stone 426 Reversible pulpitis 421 Secondary herpetic infection 486
Pulpitis aperta 424 Rhabdomyoma 163 Secondary palate formation 58
Punch biopsy 40 Rhabdomyosarcoma 227 Secondary Sjogren syndrome 254
Purpura hemorrhagic 599 Rheumatoid arthritis 542 Secondary syphilis 464
Pustular psoriasis 635 Rheumatoid sialadenitis 710 Secondary vaccinia 388
Pyogenic granuloma 167 Rhinoscleroma 480 Secondary yolk sac 55
Pyostomatitis vegetans 482 Rhinosporidiosis 520 Sectioning 23
 Index 855
Segmental odontomaxillary Solar cheilosis 579 Streptococcus mutant level in
dysplasia 127 Solar elastosis 582 saliva 45
Self-healing carcinoma 137 Solitary bone cyst 355 Stress lesion 117
Senear-Usher syndrome 626 Solitary labial lentigo 131 Stricture 247
Senile elastosis 582 Solitary myositis 687 Sturge-Weber syndrome 724
Senile osteoporosis 615 Somatomers 58 Subclinical fibrous dysplasia 606
Sentinel tubercle 473 Somites formation 58 Subleukemia 234
Septic theory 401 Special stain 25, 33 Sublingual gland 244
Septicemia 458 Specimen assessing 19 Sublingual keratosis 174
Sequelae of pulpitis 428 Speckled leukoplakia 175 Subluxation 546
Serous cell adenoma 274 Sphenopalatine neuralgia 719 Submandibular gland 244
Seromucous salivary gland 242 Spherical aberration 8 Submerged teeth 113
Serous salivary gland 242 Sphingomyelin lipidosis 648 Sucrose chelation theory 408
7th nerve paralysis 685 Spindle cell carcinoma 218 Sulphur granule 475
Severe generalized muscular Spindle cell nevi 138 Sunshine vitamin 664
dystrophy 689 Spitz nevus 139 Superficial spreading melanoma
Sexual assaults 735 Split papule 465 214
Sharp staging 175 Sporotrichosis 519 Supernumerary root 98
Shingles 488 Spread of infection 447 Supernumerary teeth 101
Shovel shaped incisor 99 Sprue 652 Sutton disease 389
Sialadenoma paipilliferum 269 Squamous cell carcinoma of Swab test 46
Sialadenosis 251 salivary gland 277 Sweet’s syndrome 722
Sialolithiasis 247 Squamous cell carcinoma 206 Swift’s disease 380
Sialoodontogenic cyst 338 Squamous cell carcinoma of tongue Swiss-type agammaglobulinaemia
Sialosis 251 568 514
Sicca syndrome 254, 710 Squamous odontogenic tumor 297 Sympathetic ophthalmoplegia 720
Sickle cell anemia 590 Stab wound 740 Synodontia 93
Sickle cell crisis 590 Stable plaque psoriasis 635 Synovial chondromatosis 547
Sickle cell disease 590 Stages of tooth development 282 Synovial sarcoma 220
Sickle cell trait 590 Staging of leukoplakia 172 Syphilis 462
Simmond’s disease 672 Staging of oral submucous fibrosis Syphilitic rhagades 466
Simple bone cyst 355 199 Systemic candidiasis 514
Simple ductal papilloma 269 Staging of salivary gland tumor Systemic lupus erythematosus 202
Simple microscope 3 261 Systemic sclerosis 397
Sjogren’s syndrome 254, 710 Staging of tumor 80
Skeletal fluorosis 620 Staining 25 T
Slip sign 147 Staining procedure 34
Slowly progressive periodontitis Stem cell leukemia 234 Tabes dorsalis 465
537 Stenosis 247 Talon cusp 94
Small cell carcinoma 278 Stensen’s duct 243 Tardive dyskinesia 567
Smallpox 491 Steven Johnson syndrome Taurodontism 97
Smoker palate 184, 385 624, 708 Tay-Sachs disease 649
Smoker’s tongue 563 Stiff joint 544 Technique for identification in
Smooth surface caries 412 STNMP staging 83 mass disaster 733
Snail track ulcer 465 Stomatitis medicamentosa 387 Teeth 66
Snow capped teeth 104 Stomatitis nicotina 184, 385 Temporalis muscle 65
Snuff dipper lesion 185 Stomatitis nicotina palati 385 Teratoma 166
Snyder test 44 Stomatitis venenata 387 Tertiary dentin 118, 119
Soft mixed odontoma 305 Stomatodynia 565 Tertiary syphilis 465
Soft odontoma 305 Strawberry hemangioma 153 Thalassemia 588
856 Textbook of Oral Pathology
Thalassemia intermedia 588 Trench mouth 527 Varicella 487
Thalassemia major 588 Trichinosis -521 Varicella zoster infection 487
Thalassemia minor 588 Trichodento osseous syndrome 98, Varicose aneurysm 158
Thalassemia trait 588 707, 713 Varicosity 570
Theories of carcinogenesis 78 Trichoonychodental syndrome 707 Variola 491
Theories of cariogenesis 401 Trichrome stain 34 Vascular nevus 152
Theories of cyst enlargement 321 Trigeminal neuralgia 683 Venereal wart 492
Theories of salivary gland tumor Triggers zone 684 Vermicular movements 567
259 Trisomy 14 syndrome 702 Verruca acuminate 492
Theories of staining 29 Trisomy 18 syndrome 701 Verruciform xanthoma 136
Theories of tooth eruption 111 Trisomy 21 701 Verrucous carcinoma 216
Thermal burns 371 Trisomy 21 syndrome 619 Verrucous leukoplakia 175
Thiamine 653 Trisomy syndromes 701 Vesicular-erosive lesion 499
Thomson disease 691 Trophoblast 55 Vestibule 66
Thrombotic thrombocytopenic Tropic ulcer 370 Vestigial cyst 348
purpura 600 Trousseau’s sign 677 Viral culture 504
Thrush 498, 509 True cementoma 312 Virtual image 10
Thymic cyst 364 Truncal obesity 681 Virus theory 79
Thyroglossal duct cyst 362 Tuberculosis 472 Vital staining 30
Thyroid crisis 673 Tuberous sclerosis 718, 728 Vital theory 402
Thyroid storm 673 Tularemia 479 Vitamin A 663
Thyrotoxic osteoporosis 616 Tumor marker 50 Vitamin B1 653
Thyrotoxicosis 673 Twinning 93 Vitamin B12 659
Tic douloureux 683 Type I diabetes mellitus 678 Vitamin B2 655
TNM staging 82 Type II diabetes mellitus 679 Vitamin B3 656
Tobacco pouch mouth 397 Vitamin B5 657
Tocopherol 667 U Vitamin B6 658
Tongue thrusting 557 Vitamin B8 658
Tongue tie 555 Ulcerated granuloma eosino- Vitamin B9 658
Tooth ankylosis 378 philicum diutinum 563 Vitamin C 661
Tooth germ 285 Ulcerated leukoplakia 175 Vitamin D 664
Torulosis 517 Ulcerative lichen planus 190 Vitamin D deficient rickets 665
Torus mandibularis 151 Undifferentiated carcinoma 277 Vitamin D resistant rickets 666
Torus palatinus 151 Unicameral cyst 355 Vitamin E 667
Toxic epidermal necrolysis 624 Unicystic ameloblastoma 295 Vitamin H 658
Transitional cell carcinoma 214 Unifocal eosinophilic granuloma Vitamin K 667
Transmission electron microscope 647 Volkmann’s cheilitis 577
17 Urbach-Wiethe syndrome 640 Von Buchem disease 621
Transparent dentin 118 Uremic stomatitis 384 Von Geison’s method 34
Transposition 113 UV rays 734 Von Hippel -Lindau syndrome 729
Traumatic bone cyst 355 Uveoparotid fever 257, 711 Von Recklinghausen’s disease of
Traumatic granuloma 563 Uveoparotitis 711 skin 160
Traumatic keratosis 384 Von Recklinghausen’s neurofibro-
Traumatic lesion due to sexual V matosis 726
habit 376
Traumatic neuroma 159 Vaccinia autonoculata 388 W
Traumatic ulcer 370 Valley fever 518
Treacher Collins syndrome 129, Van Der Woude’s syndrome 714 Wandering rash 560
703 Vanishing bone 621 Warrensburg’s syndrome 708
Trefoil tongue 557 Vaquez’s disease 595 Warthin tumor 265
 Index 857
Warty dyskeratoma 638 White sponge nevus 640 Z
Water soluble vitamins 653 Wickham striae 187
Waterhouse Friderichsen syn- Winchester syndrome 715 Zellweger syndrome 715
drome 681, 726 Wiskott-Aldrich syndrome 601 Zimmermann Laband syndrome
Weber-Cockayne syndrome 630 Witkop-Von Sallman syndrome 641 718
Wegner granulomatosis 393 Working distance 6 Zinc deficiency 651
Werlhof’s disease 599 Zinsser-Engman-Cole syndrome
Wernicke’s encephalopathy 655 X 201, 715
Western blot method 504 Zona 488
Wet beriberi 654 Xanthomatosis 646 Zycomycosis 517
Wharton duct 244 Xerostomia 500
White folded gingivostomatitis 640 X-ray 733