Oral pemphigus vegetans: A case report
1. Department of Oral Pathology, School of Dentistry, University of São Paulo, São Paulo, Brazil
Pemphigus is a rare vesiculobullous autoimmune disease that exhibits blistering of the skin and oral cavity. It is caused by autoantibodies directed against antigens on the surface of keratinocytes. All forms of pemphigus are associated with the presence of circulating and skin-fixed autoantibodies. Pemphigus vegetans is a rare clinical variant of pemphigus vulgaris and comprises up to 5 percent of all pemphigus cases . In the following we present the oral presentation of pemphigus vegetans. We describe a 33-year-old man who was referred to our clinic complaining about mouth sores, tooth pain, and multiple pustules. During clinical exam we were able to recognize multiple pustules, ulcerated areas on the gingiva, and whitish mucosal plaques. Clinical, histopathological, and direct immunofluorescence findings were compatible with pemphigus vegetans.
A 33-year-old man was referred to Special Care Dentistry Center at the School of Dentistry, University of São Paulo (CAPE-FOUSP) for evaluation and treatment of mouth sores and multiples pustules. Informed signed consent form was given. Medical history revealed constant weight loss and an intermittent history of bloody diarrhea with more than 5 loose bowel movements per day. During oral examination several oral lesions occurring predominantly on the buccal gingiva that included small white lesions resembling pustules and a few ulcerated areas were observed (Figure 1A). An oral mucosal biopsy specimen showed histopathological and direct immunofluorescence findings that were diagnostic for pemphigus vulgaris. Histopathological sections revealed several well-defined foci of suprabasal acantholysis with crusting, a few neutrophils in the overlying space, and mixed lymphocytes and eosinophils (Figure 1C). Direct immunofluorescence showed bright intercellular deposition of IgG in the epidermis. In addition, intercellular C3 staining was verified in the epidermis (Figure 1D). Based on clinical findings, direct immunofluorescence, and histopathological characteristics, a final diagnosis of pemphigus vegetans was established. Indirect serology for pemphigus antibodies was negative. A colonoscopy was performed and the biopsy specimens were described as ulcerative rectocolitis with evidence of previous severe mucosal injury. These changes were compatible with idiopathic inflammatory bowel disease.
The patient was admitted for hospitalization for 21 days. Results of laboratory investigations revealed a severe anemia (hemoglobin 7.0 mg/dl [men: 13.8 to 18.2 mg/dL], Ht 30.7% [48%], platelets 531,000 [150,000 and 450,000], Fe+ 12 g/dl [59 – 158 mg/dl], Ferritin 4 ng/ml [30 – 400 ng/ml], iron saturation 3.75% [20 – 40]). Anemia was treated with oral iron supplements (325 mg) and dietary measures. Daily treatment with 40 mg of prednisone over 50 days effectively healed all mucosal ulcerations (Figure 1B). Once a clinical response was observed, the dose of systemic corticosteroids was tapered gradually to 10 mg daily and eventually discontinued. At two-year follow-up, the patient was asymptomatic without recurrences. After the clearance of the oral lesions, it was possible to verify generalized gingival enlargement compatible with drug-induced gingival growth (Figure 1B).
Pemphigus vegetans is a rare clinical variant of pemphigus vulgaris and oral involvement is very common. It is clinically classified as 1)Neuwmann type with oral lesions that resemble flaccid bullae and small ulcerated areas and 2)Hallopeau type with pustular lesions and a benign course with few relapses [1, 2]. As in pemphigus vulgaris, 50 percent of pemphigus vegetans cases begin in the oral cavity months before skin lesions appearance. Patients initially having cutaneous lesions will ultimately develop oral manifestations. Patients with the Hallopeau type pemphigus often have a relatively benign disease, requiring lower doses of systemic corticosteroids to control the mucocutaneous manifestations and usually have a prolonged remission. The differential diagnosis of oral ulcerations associated with chronic inflammatory bowel disease are granulomatous lesions of Crohn disease, recurrent aphthous stomatitis, or pyostomatitis vegetans . It is particularly important to rule out pyostomatitis vegetans, a disorder that is an acknowledged marker for both ulcerative colitis and Crohn disease. Pemphigus vegetans and pyostomatitis vegetans demonstrate similar clinical and histopathological findings, being distinguished by direct immunofluorescence studies. To establish the diagnosis it is mandatory to identify both histopathology and direct immunofluorescence features of mucosal involvement. Indirect immunofluorescence is positive in up to 80 to 90 percent of the patients and usually is correlated with the severity of the disease .
In conclusion, high dose treatment with systemic corticosteroids was an effective therapy in our pemphigus vegetans patient and produced healing of all oral mucosal ulceration. This improved his food intake and anemia status. The patient continued using the same medications 24 months after being discharged from the hospital without any recurrences.
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