Paraneoplastic pemphigus in a burn intensive care unit: case report and review of the literature

Abstract

Paraneoplastic pemphigus is a rare cause of acute diffuse blistering in the adult patient. It commonly presents with subepidermal blistering, epidermal necrosis, and symptoms of mucosal irritation, such as conjunctivitis and vaginal ulceration. Because of its rarity, it is frequently misdiagnosed as Stevens-Johnson syndrome or toxic epidermal necrolysis. In this study, the authors will describe clinical and histologic manifestations of paraneoplastic pemphigus. This case report describes a 45-year-old woman with paraneoplastic pemphigus who was admitted and treated in a burn intensive care unit. Although initially diagnosed with Stevens-Johnson syndrome, the patient had progression of desquamation when potentially offending medications were discontinued. Diffuse adenopathy was noted on examination, and biopsy confirmed a low-grade lymphoma. Paraneoplastic pemphigus is a rare but important cause of acute diffuse blistering in adults. This disorder should be considered in the differential diagnosis of patients with diffuse blistering.

Figure 1

Clinical presentation of patient with acute, diffuse blistering. Images were obtained on hospital day five at our institution.

Figure 2

Histology of Skin Biopsy a. Skin biopsy demonstrating suprabasilar bulla (H&E, 40×). b. Interface reaction reminiscent of erythema multiforme with infiltration by eosinophils (H&E, 200×). c. Suprabasilar cleft with some acantholysis (H&E, 200×).