Pemphigus Foliaceus

Abstract

REPORT OF CASES Case 1.— A 63-year-old woman presented with a 6-year history of extensive, crusted plaques on her face, trunk, and neck. She noted exacerbations after exposure to sunlight. A lesional biopsy specimen demonstrated a cleft containing acantholytic cells in the stratum granulosum. Direct immunofluorescence was remarkable for characteristic high epidermal intercellular substance deposits of IgG and C3. Pemphigus antibody was detectable by indirect immunofluorescence at a titer of 1:80. Prednisone therapy (100 mg/d) did not control the skin lesions, and therapy was complicated by severe emotional lability and glaucoma. Intramuscular gold therapy was initiated briefly but discontinued because of hematuria. Azathioprine (150 mg/d) was added to her therapy, and the prednisone dose was gradually tapered to 80 mg, alternating with 20 mg with incomplete disease control. Subsequently, azathioprine therapy was discontinued, and hydroxychloroquine therapy (200 mg twice daily) was initiated. Within 2 months, the frequency of new lesions