Abstract

A 49-year-old man presented with a 10-year history of painful lesions on the buttocks. They developed as fluid-filled blisters that ruptured spontaneously and developed into erosions and thickened plaques.  The patient recieved intermittent medical care; he was treated with prednisone, minocycline and topical clobetasol, with limited response. Despite treatment, he had  pain and drainage and developed new lesions around the mouth and on the legs. Physical examination revealed large, well-demarcated, violaceous, indurated plaques covering the buttocks and lower back, studded with foci of pustules. Examination of the oral mucosa revealed cobblestoning of the tongue and angular cheilitis with verrucous plaques on bilateral oral commissures. All 20 nails were dystrophic. Skin biopsy revealed epidermal hyperplasia with acanthosis, papillomatosis, and intraepidermal collections of neutrophils and eosinophils. There were subtle areas of focal suprabasilar acantholysis within the epidermis. In the dermis, a perivascular and interstitial inflammatory infiltrate was observed, with predominance of eosinophils and plasma cells. Direct immunofluorescence studies showed intercellular deposition of IgG and C3 within the epidermis, confirming the diagnosis of pemphigus vegetans. Additional laboratory work-up was notable for positive hepatitis C antibodies.We report this case to highlight the broad range of clinical findings in chronic, uncontrolled pemphigus vegetans, the importance of clinicopathologic correlation and direct immunofluorescence studies in autoimmune bullous diseases, and the relevance of additional work-up for related conditions such as hepatitis C and underlying malignancies.