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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