Paraneoplastic pemphigus presenting as an unusual bullous eruption.

Abstract

We describe a patient with a widespread annular bullous eruption on the trunk and extremities and involvement of the conjunctivae, oral mucous membranes, and esophagus. Findings of a biopsy specimen of an intraabdominal mass revealed nodular and diffuse well-differentiated lymphoma. Histologic examination of a skin lesion showed acantholysis with bulla formation and keratinocyte necrosis. Direct immunofluorescence testing of a skin lesion showed intercellular deposition of IgG. The patient's serum contained autoantibodies with broad specificity for skin and other epithelia and identified the characteristic bands of paraneoplastic pemphigus at 250, 230, 210, and 190 kd on immunoprecipitation. The patient died despite therapy.