April 2003

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History:
A 60 year-old white female without a significant past medical history presented to her local dermatologist with complaints of progressive denudation of skin for three weeks. Her lesions began as flaccid bulla in her mouth that ruptured and became painful. These lesions next appeared on her face, particularly periorbital, perineum, and, over the course of two weeks, her entire body, sparing her abdomen and feet. She was not taking any medications prior to this onset, and had no evidence of systemic infection. She presented to her local dermatologist and was diagnosed with ulcerative lichen planus. Bullous pemphigoid and pemphigus vulgaris serologies were negative. These lesions did not respond to treatment with corticosteroids and continued to progress. The patient was admitted to Barnes-Jewish Hospital for further evaluation.A biopsy of these lesions was then performed and the pathological diagnosis of pemphigus vulgaris was made. The patient was initiated on CellCept and low dose systemic corticosteroids without benefit.

Physical Examination:
The patient's physical exam was remarkable for an extensive area of denuded skin over the patient's face, neck, back, chest, arms, palms, fingers, perineal region, buttocks, and plantar surfaces of her feet (Figs 1-3). Few flaccid bullae were identified. Some of these lesions had overlying eschar, especially in the periorbital region. She had multiple erythematous ulcerations on the tongue and buccal mucosa. In addition, the patient had a palpable abdominal mass without hepatosplenomegaly.

 

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