Pemphigus
Vulgaris
Done By : Yosra Moustafa
Table of contents
Similarities
01 About the disease 04 between types 07 Pathophysiology
02 Types of Pemphigus 05 Epidemiology 08 Clinical
Manifestations
Differences between Etiology & Risk
03 types 06 factors 09 Diagnosis
Table of contents
Severity
10 Assessments 13 Complications
11 Treatments 14 Key Points
12 Side effects 15 References
Introduction
Pemphigus comprises a collection of
uncommon autoimmune conditions
characterized by the formation of
blisters on both the skin and mucous
membranes across various bodily
regions, including the mouth, nose,
throat, eyes, and genital area. Among
these, pemphigus vulgaris stands as the
most prevalent form.
Introduction
Pemphigus disorder prompts the
immune system to mistakenly target
the body's own cells, the same way it
defense against foreign pathogens.
Types of Pemphigus
Pemphigus Vulgaris IgA Pemphigus
Pemphigus Foliaceus Paraneoplastic Pemphigus
Differences between types
Autoantibody Targets Clinical Variants Manifestation Locations
Involves mucosal
and/or skin
Autoantibodies attack Variants include
involvement, often
desmoglein 3 or both pemphigus vegetans and
Pemphigus Vulgaris starting in the mouth.
desmoglein 1 and pemphigus
Other mucous
desmoglein 3 herpetiformis.
membrane areas may
also be affected.
Variants include
endemic pemphigus Primarily affects the
Autoantibodies target foliaceus, pemphigus skin, with mucous
Pemphigus Foliaceus
desmoglein 1 erythematosus, and membranes typically
pemphigus spared.
herpetiformis.
Differences between types
Autoantibody Targets Clinical Variants Manifestation Locations
Subtypes include
subcorneal pustular
Typically presents with
dermatosis-type IgA
Autoantibodies attack skin lesions, with
IgA Pemphigus pemphigus and
desmocollin 1 mucous membranes
intraepidermal
usually unaffected.
neutrophilic IgA
dermatosis.
Extensive oral and skin
blistering, often
Autoantibodies weaken
associated with
Paraneoplastic cell adhesion, with No specific clinical
underlying
Pemphigus inflammatory cells variants
malignancies. Also can
infiltrating the skin.
lead to lifethreatening
complications
Similarities between types
Blisters Autoimmune Nature Potential Severity
Severe symptoms,
including pain,
Each disease involves inflammation, and
All four diseases involve autoantibodies attacking potential life-threatening
the formation of blisters specific proteins complications, can
or lesions on the skin or involved in cell occur in all four
mucous membranes. adhesion, leading to diseases, particularly if
blister formation left untreated or if
extensive blistering
occurs.
Prevalence of Pemphigus Vulgaris
70%
of all
pemphigus
cases
worldwide
Epidemiology
Etiology and Risk
Factors
NOT FULLY UNDERSTOOD
It's thought to arise when individuals with a
genetic predisposition encounter certain
environmental factors, like chemicals or
medications. In some instances, discontinuing
exposure to the trigger can lead to resolution of
the condition.
Etiology and Risk
Factors
In pemphigus vulgaris, the immune system
targets and attacks the proteins that hold skin
cells together. This can lead to the destruction
of these proteins, causing the affected cells to
weaken and eventually die. This can lead to
fluid accumulation between these cells and the
formation of blisters.
Some people find that stress and certain foods
such as Garlic, make living with pemphigus
vulgaris more difficult.
Pathophysiology
Pathophysiology
Normal Pemphigus
Vulgaris
Symptoms
Pemphigus vulgaris commonly initiates within the oral
cavity.
- Occurrence of Blisters on skin that appears
otherwise healthy or unaffected.
- Blisters formed are fragile and can break open easily
even with minimal pressure or contact.
- Skin affected can easily come off or detach when
rubbed or touched
- Pain associated with blister formation
Diagnosis : Biopsy
Diagnosis
- Biopsy
- Direct immunofluorescence
staining
- Blood tests
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Assessing the Severity
• PDAI: Pemphigus Disease Area index
• PAAS: Pemphigus Area and Activity Score
• PAS: Pemphigus Activity Score
• ABSDIS: Autoimmune Bullous Skin Disorder Intensity Score
• Saraswat Oral pemphigus scoring
• Pemphigus Vulgaris lesion severity score
• Harman pemphigus grading
• Kumar scoring system
• Mahajan scoring system
Treatments
Treatments
Treatment with Systemic Glucocorticoids
• Systemic glucocorticoids are typically given orally.
• For practical reasons, glucocorticoid therapy often begins before
rituximab therapy.
• Initial signs of clinical improvement related to systemic glucocorticoid
therapy usually become evident within two to three weeks.
• Systemic glucocorticoid therapy typically consists of oral Prednisone (or
Prednisolone). Initial doses range from 0.5 to 1.5 mg/kg per day based
on the disease severity
Treatment with Systemic Glucocorticoids
Dosage Regimen:
Mild Pemphigus : 0.5 mg/kg per day
Moderate to Severe Pemphigus : 1 mg/kg per day
Treatment with Rituximab - IV infusion
• Rituximab is given via IV infusion
• Effects of Rituximab may not appear for 8 to 12 weeks
• The therapeutic regimen for Rituximab involves two
initial infusions followed by periodic maintenance
doses, as needed. Typical initial dosing is as follows:
• Two 1000 mg intravenous infusions separated by two
weeks
• Intravenous Methylprednisolone (100 mg) or an
equivalent glucocorticoid should be given 30 minutes
prior to each Rituximab infusion.
Adverse effects
According to UpToDate:
“ Potential adverse effects of prolonged systemic glucocorticoid
therapy includes hypertension, high cholesterol, diabetes,
osteoporosis, increased risk of infections, stomach ulcers, and aseptic
bone necrosis. These side effects can significantly impact a patient's
health and may even lead to death. Therefore, it's crucial for patients
on long-term glucocorticoid therapy to have regular check-ups and
receive appropriate preventive and therapeutic treatments to manage
these side effects “
Adverse effects
According to UpToDate:
“ the use of additional medications alongside
glucocorticoids to reduce their dosage while still
controlling the disease (glucocorticoid-sparing effect).
Azathioprine and mycophenolate mofetil are the main
drugs used for this purpose in pemphigus treatment.
Although azathioprine has more evidence supporting
its glucocorticoid-sparing effect, mycophenolate
mofetil is often preferred due to its better side effect
profile and ease of use. “
Pemphigus
Vulgaris
Complications
Pemphigus Vulgaris Complications
01 Secondary bacterial infection
02 Fungal infection, especially Candida
03 Viral infections, especially herpes simplex
Pemphigus Vulgaris Complications
04 Nutritional deficiencies due to difficulty eating
Complications of systemic steroids especially
05 infections and osteoporosis
06 Complications of immune suppressive treatments
Notes from the Dr.
1- Treating this condition can take 2 to 5 years or even more.
2- Treatments might lead to serious side effects
3- Even after successful treatment, Pemphigus Vulgaris can still come
back
4- Visit the physician if you notice painful, soft blistering on the skin
or mucous membranes.
5- Treatment will prevent the blisters from spreading and getting
worse.
6- Lifestyle management may be helpful
Notes from the Dr.
7- Drug-induced pemphigus is also recognized and is most often
caused by Penicillamine, ACE inhibitors, Angiotensin receptor
blockers, and Cephalosporins.
8- Pemphigus is sometimes triggered by Cancers, particularly
Lymphomas and Castelman disease (Paraneoplastic pemphigus),
infections or trauma.
9- Even after successful treatment, Pemphigus Vulgaris can still come
back
10- Clinicians or patients should puncture and drain large blisters in a
sterile manner.
11- Wound areas should be kept clean to reduce the risk for infection
Notes from the Dr.
12- Twice daily application of high-potency topical corticosteroid
such as Clobetasol propionate ointment or gel directly to erosions
can be useful adjunct to systemic therapy for persistent, active, hard-
to-treat pemphigus lesions.
13- Avoidance of Spicy, sharp, abrasive, or very hot foods, also dairy
products can exacerbate symptoms.
14- Application of topical anesthetics as needed such as Lidocaine
2% gel
15- Oral hygiene is important
16- Erosions may be covered with antibiotic ointment or a bland
emollient such as Petrolatum with or without a non-adhesive wound
dressing
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Resources
1- Ingold, C. J., Sathe, N. C., & Khan, M. A. (2024, March 1). Pemphigus vulgaris. StatPearls - NCBI
Bookshelf. https://www.ncbi.nlm.nih.gov/books/NBK560860/
2- International Pemphigus & Pemphigoid Foundation. (2020, August 4). Pemphigus - IPPF. IPPF -
. https://www.pemphigus.org/pemphigus/#:~:text=The%20four%20major%20types%20of,pemphigus%20foliaceus
%20is%20more%20prevalent.
3- Kridin, K., & Schmidt, E. (2021). Epidemiology of Pemphigus. JID Innovations, 1(1),
100004. https://doi.org/10.1016/j.xjidi.2021.100004
4- Pemphigus vulgaris. (2019, November 19). Johns Hopkins
Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases/pemphigus-
vulgaris#:~:text=What%20is%20pemphigus%20vulgaris%3F,most%20common%20type%20of%20pemphigus
5- Shibboleth Authentication Request. (n.d.). https://www-uptodate-com.qulib.idm.oclc.org/contents/initial-
management-of-pemphigus-vulgaris-and-pemphigus-
foliaceus?search=pemphigus%20vulgaris&source=search_result&selectedTitle=1%7E53&usage_type=default&display
_rank=1#H806899198
