Chronic benign familial pemphigus

Ten patients with benign familial chronic pemphigus (BFCP) (Hailey-Hailey disease) were evaluated; semiologic and localization differences were described, and special attention was given to solitary or atypical forms. In all the cases, the diagnosis was confirmed by histopathology; some histopathologic differences and the results achieved by direct immunofluorescence of skin are discussed. Most of the patients responded to the treatment with corticosteroids and antibiotics.