Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid

Abstract

Sixty-seven patients with histologic and immunologic features of bullous pemphigold (BP) were evaluated. Eleven patients had a localized blistering disease that was predominantly confined to one area of the body, most commonly the lower extremities. Two patients displayed a dapsone-responsive blistering disease that was characterized by a flexural distribution of ten to 20 1-cm or less, intensely pruitic, subepidermal bullae and linear IgA basement membrane zone deposition. Two patients had a chronic recalcitrant generalized scarring, hyperkeratotic, subepidermal blistering eruption that demonstrated serologic and direct immunofluorescence (IF) findings of BP. One patient displayed grouped small vesicles surmounted on an erythematous base; the clinical diagnosis was dermatitis herpetiformis, but direct IF examination of the biopsy specimen showed features of BP. One patient with epidermolysis bullosa acquisita had serologic and direct IF features suggestive of BP.