[Acantholysis and eosinophilic spongiosis: pemphigus herpetiformis. Successful retinoid therapy]

Abstract

Pemphigus herpetiformis is an unusual variant of the pemphigus group, showing acantholytic bulla formation together with marked eosinophilic spongiosis. The clinical picture shows erythematous and pruritic papulovesicular bullous lesions affecting the skin and the oral mucosa. It most closely resembles dermatitis herpetiformis or bullous pemphigoid. In contrast, the presence of acantholysis and of antiepithelial antibodies indicate pemphigus. Three patients, all women, showing this unusual variant of pemphigus, were seen during the last 3 years, a fourth is reported here. One patient responded well to dapsone; the three others did not. Oral treatment with aromatic retinoid (Ro 10-9359) was clearly effective in the last patient, leading to full remission of the skin lesions in a few days. The oral mucosa improved only partly. Discontinuation of the aromatic retinoid treatment and replacement of the drug by placebo was followed by a relapse both of mucosal and skin lesions. Aromatic retinoid possibly inhibits eosinophilic migration into the epidermis and impedes acantholysis associated with this phenomenon.