Benign Familial Chronic Pemphigus

Abstract

In 1939 Hailey and Hailey¹described 4 patients with an apparently new and previously undescribed skin disorder. They stated that they had not found any report of a cutaneous condition characteristic of their cases. They named the condition "benign familial chronic pemphigus." Benign familial chronic pemphigus is characterized by vesicular plaques on the nape and sides of the neck, the axillae, the groin, and the intercrural, genital, perianal, and periumbilical areas, with occasional spread to the scalp and larger areas of the glabrous skin (Figs. 1, 2 and 3). The term "pemphigus" describes the clinical and histopathologic vesicular quality of the disease, but it should not connote a severe generalized blistering disease as is usually indicated by the term. The primary lesion is a vesicle, arising on apparently normal skin. It enlarges rapidlyto become a flaccid bulla. Nikolsky's sign may be positive. The vesicle ruptures, leaving an eroded base which exudes serum and results in a crust formation that strongly resembles impetigo. The lesions tend to spread peripherally, leaving a dry center and producing