A Case of Generalized Familial Benign Pemphigus

Lee, Sung Yul; Bang, Sung Won; Lee, Jong Suk

Abstract

Familial benign pemphigus(Hailey-Hailey disease) is a rare hereditary dermatosis that begins in the 2nd or 3rd decade of life. The skin lesion is characterized by a localized, recurrent eruption of small vesicles on an erythematous base. During its course there are remissions and exacerbations. It seldom begins in early childhood or after the age of 50. The main treatment modalities are conservative ones. A 59-year-old woman with familial benign chronic pemphigus presented with a 10 year history of generalized pruritic recurrent skin lesions on her neck, axilla, inguinal, antecubital, and trunk area. Physical examination showed moist, macerated, fissured and scaly patches on an erythematous base in the axillae, groins, neck, antecubital, and trunk. A biopsy specimen showed extensive suprabasal separation containing acantholytic cells.