Oral manifestations of Plummer-Vinson syndrome … Samad A et al Journal of International Oral Health 2015; 7(3):68-71
Received: 04th August 2014 Accepted: 02nd November 2014 Conflict of Interest: None Case Report
Source of Support: Nil
Oral Manifestations of Plummer-Vinson Syndrome: A Classic Report with Literature Review
Abdul Samad1, N Mohan2 , R V Suresh Balaji3, Dominic Augustine4, Shankar Gouda Patil5
Contributors: British laryngologists published their findings independently
1
Assistant Professor, Department of Oral Medicine & Radiology, in 1919.1
Vivekananda Dental College for Women, Tiruchengode,
Namakkal, Tamil Nadu, India; 2Professor and Head, Department PVS has become increasingly rare with the availability of iron
of Oral Medicine & Radiology, Vinayaka Missions Sankarachariyar supplements, but nonetheless it should be suspected in cases
Dental College & Hospital, Ariyanoor, Salem, Tamil Nadu, India;
of iron deficiency and dysphagia. The syndrome consists of
3
Senior Resident, Department of Oral Medicine & Radiology, Sri
Muthukumaran Medical College Hospital & Research Institute, glossitis, atrophic oral mucosa, dysphagia, and anemia. Post-
Chennai, Tamil Nadu, India; 4Assistant Professor, Department of menopausal women are commonly affected. Other presenting
Oral and Maxillofacial Pathology, Bangalore Institute of Dental symptoms may include fissuring at the angles of the mouth and
Sciences & Hospital, Lakkasandra, Bengaluru, Karnataka, India; glossodynia. PVS is a potentially malignant disorder with the risk
5
Associate Professor, Department of Oral and Maxillofacial of squamous cell carcinoma of the esophagus due to the post
Pathology, MS Ramaiah Dental College & Hospital, MS Ramaiah cricoidal webs that are formed.2 This article presents a classical
Educational Campus, Bengaluru, Karnataka, India. case of PVS with review of literature with dental implications.
Correspondence:
Dr. Samad A. Department of Oral Medicine & Radiology, Case Report
Vivekananda Dental College for Women, Tiruchengode, Namakkal,
A 20-year-old female reported to the dental outpatient
Tamil Nadu, India. Email:
[email protected]How to cite the article: department with a complaint of difficulty in swallowing for
Samad A, Mohan N, Balaji RV, Augustine D, Patil SG. Oral the past 6 months. Her medical history was non-contributory.
manifestations of Plummer-Vinson syndrome: A classic report with On eliciting her personal history, it was found that she had
literature review. J Int Oral Health 2015;7(3):68-71. increased blood loss during menstruation.
Abstract:
Plummer-Vinson syndrome (PVS) is a triad of microcytic On examination, she appeared to be poorly built and
hypochromic anemia, atrophic glossitis, and esophageal webs or moderately nourished. Extraoral examination revealed pallor
strictures. It is one of the syndromes associated with iron deficiency of the palpebral conjunctiva (Figure 1a) and koilonychia in all
anemia. Symptoms resulting from anemia predominates the
her fingers (Figure 1b).
clinical picture, apart from the additional features such as glossitis,
angular cheilitis, and dysphagia. Dysphagia is main clinical feature
Intra oral examination showed the presence of ulcers at the
of PVS. PVS carries an increased risk of development of squamous
cell carcinoma of esophagus and pharynx. A classic case report angle of mouth with pigmented spots on the tongue (Figure
of PVS with clinical features, oral manifestations, malignant 2a) she had a restricted mouth opening of 25 mm. Mucosal
potential, differential diagnosis, investigation, dental implication, examination revealed pallor of the labial, buccal, palatal
and treatment is discussed here with the literature review from the mucosa, retromolar area, and tongue. Marked mucosal
dentist’s point of view. The article carries a message that dental pigmentation was observed in the buccal and labial mucosa
surgeons have to be familiar with the oral manifestations of anemia (Figure 2b).
and be able to suspect PVS to aid in early diagnosis and prompt
treatment. Hematological investigations revealed her hemoglobin count
Key Words: Dysphagia, iron deficiency anemia, Plummer-Vinson to be only 4 mg/dl, examination of the peripheral blood smear
syndrome showed the distinctive picture of microcytic hypochromic
anemia (Figure 3).
Introduction
Plummer-Vinson syndrome (PVS) is known by several names Radiograph examination consisted of barium swallow test
such as Paterson-Kelly syndrome, Paterson-Brown Kelly which revealed the constriction of esophagus (Figure 4a).
syndrome and sideropenic dysphagia. PVS was first described Endoscopy confirmed the presence of esophageal web (post
by Paterson and Kelly in 1919. It is named after Henry Stanley cricoids web 17-18 cm) and also a ruptured web (Figure 4b).
Plummer (1874-1936) and Porter Paisley Vinson (1890-1959) The instrument did not pass through at the level of the web. On
who were physicians of the Mayo Clinic. Donald Ross Paterson correlating the clinical history, examination and investigations
(1863-1939) and Adam Brown-Kelly (1865-1941), both performed a final diagnosis of PVS was concluded.
68
Oral manifestations of Plummer-Vinson syndrome … Samad A et al Journal of International Oral Health 2015; 7(3):68-71
a b
Figure 1: (a) Extra oral view showing a pale conjunctiva.
(b) Koilonychia evident in nails.
Figure 3: Peripheral blood smear showing microcytic
hypochromic anemia (Leishman’s Stain at ×100 magnification).
a b
Figure 2: (a) Atrophy of tongue and angular cheilitis.
(b) Marked intraoral pigmentation.
The patient is currently managed by iron supplementation in
the form a loading dose of Fe dextran give intramuscular with an
oral maintenance dose of ferrous sulfate 325 mg (60 mg of iron)
given thrice daily. Mechanical dilation of the post cricoidal
webs through endoscopy is being carried out on a regular basis,
and the patient has reported reduction in dysphagia. a b
Figure 4: (a) Barium swallow – lateral view. (b) Barium
She is under constant follow-up and review along with a well-
swallow showing esophageal web.
maintained high vitamin supplemented diet since the post
cricoidal webs have a premalignant potential.
may be the contributory factors of this syndrome. Myasthenic
changes occur in muscles involved in the swallowing
Discussion
mechanism due to the depletion of iron-dependent oxidative
Anemia is a disordered process in which the rate of red cell
enzymes. Atrophy of the esophageal mucosa and formation of
production fails to match the rate of destruction, which
webs is seen as mucosal complications. These changes were
results in a reduction of hemoglobin concentration. The
evident in the present case.
etiopathogenesis of anemia revolves around increased
physiological demand, pathological blood loss and inadequate An autoimmune mechanism is certainly associated as the
iron intake.3 Food sources rich in iron are the main source, and syndrome is seen in association with rheumatoid arthritis,
iron is recycled by the destruction of senescent red blood cells. thyroiditis, celiac disease, and pernicious anemia. Other factors
Iron is important in the body for maturation and to maintain such as nutritional deficiencies, genetic predisposition are
the integrity of the epithelium. thought to play roles in the causation of this disease.
Iron deficiency anemia is caused mainly by the lack of iron Clinical features
and anemia develops when there is inadequate iron for The main clinical features of PVS are dysphagia, iron
hemoglobin synthesis. Peripheral causes include blood loss, deficiency anemia with upper esophageal webs. Majority of
hemolysis, and hypersplenism. Females in reproductive age the patients are women in the fourth to sixth decade of life,
group, menstruation, pregnancy, nutritional deficiency, blood the syndrome has also been described in adolescents and
loss, and malabsorption are all known predisposing factors. children.
Etiopathogenesis The dysphagia usually presents intermittently or progressively
Etiopathogenesis of PVS is uncertain. But iron deficiency, over the years and is usually painless. It is also characterized by
malnutrition, genetic predisposition or autoimmune processes enlargement of the spleen and thyroid.
69
Oral manifestations of Plummer-Vinson syndrome … Samad A et al Journal of International Oral Health 2015; 7(3):68-71
Epithelial changes include koilonychia or spoon shaped nails, Implications of anemia in dentistry
atrophic glossitis, angular stomatitis, dryness, and atrophic 1. Mucosal disease
changes in the conjunctiva along with the formation of post • Glossitis
• Angular stomatitis
cricoidal esophageal webs. All these classic findings were found
• Recurrent aphthae.
in the present case. 2. Infections like candidiasis
3. Risks from general anesthesia
Oral manifestations include stomatitis, angular cheilitis, glossitis • Shortage of oxygen can be dangerous.
with different degree of atrophy of fungiform and filiform
papilla, recurrent aphthous stomatitis, pale oral mucosa, oral Management
candidiasis, erythematous mucositis, and a burning mouth. The Correction of etiology is the mainstay of treatment. Iron
atrophy of filiform papillae are most susceptible to nutritional supplementation in the form of ferrous sulfate orally can
deficiency and disappear first, followed by fungiform papillae, be given or parenteral doses of iron in severe cases. Iron
regeneration occurs in reverse order but vallate and foliate supplementation alone can resolve dysphagia in many patients.
papillae on the posterior third are spared.
Mechanical dilation of webs and strictures are done by
Diagnosis endoscopic dilatation in single or multiple sessions depending
The diagnosis of iron deficiency anemia relies on clinical on the severity of the web, in which wire-guided bougies or
history with questions about dietary intake, and the presence balloons are used to disrupt and rupture the fibrous tissue of
of blood in stools (which may be a sign of hemorrhoids. In the stricture.7
women, a careful inquiry about the duration of periods.3
Clinically, the patient will present with pallor. For tight or highly constricted webs which prevent the passage
of the endoscope a fluoroscopic guidance is usually required.
Hematological investigations revealed an iron deficiency Endoscopic balloon dilation becomes difficult when the webs
anemia with decreased values of hematocrit, hemoglobin, are located proximally, but the same can be performed under
serum iron, mean corpuscular volume, and increased total fluoroscopic guidance.8
iron binding capacity. A pathognomonic picture of microcytic
hypochromic anemia is seen. A similar picture was seen in the Other successful means of disrupting an esophageal web are by
present case. ND:YAG laser therapy or needle-knife electro incision which
is seldom used.
In PVS, radiographic examination of the pharynx will show the
presence of esophageal webs2 along with other manifestations Patient should be kept on a high vitamin diet to maintain
like koilonychia. integrity and maturative potential of the oral epithelium since
PVS is a potentially malignant disorder. Regular follow-up
Differential diagnosis is mandatory. It is wise to perform an upper gastrointestinal
It includes all the possible causes of dysphagia like malignant endoscopy annually.
tumors, benign strictures, diverticula, achalasia, spastic motility
disorders, scleroderma, gastroesophageal reflux disease, Conclusion
neuromuscular and skeletal muscle disorders.4 Iron deficiency is a common cause of anemia. Severe iron
deficiency anemia with koilonychia and dysphagia are the
Malignant potential features of PVS. PVS is considered to be a premalignant
PVS is associated with a high risk of development of squamous condition, and dental surgeons have to be familiar with the oral
cell carcinoma of esophagus and pharynx. A post cricoid web manifestations of anemia and should be able to suspect PVS.
is a rare complication of iron deficiency anemia. Early diagnosis is of utmost importance for a better prognosis. A
case of PVS with barium swallow performed has been described
The mechanism explained is that the anemia causes epithelial here with the literature review.
atrophy, changes in cell kinetics, and decreases the repair
capacity of the mucosa. This allows the carcinogens and References
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of the patients with PVS, has presented with esophageal or carcinoma and carcinoma of the tongue. Am J Gastroenterol
pharyngeal cancer.6 2003;98(5):1208-9.
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Büyükberber M, et al. Plummer-Vinson syndrome and heralded by postcricoid carcinoma. Am J Otolaryngol
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