A Case of Familial Benign Chronic Pemphigus / 대한피부과학회지

ABSTRACT

Since the description of Familial Benign Chronic Pemphigus by Hailey-Hailey, several cases of this clinical entity have reported in Korea. Familial Benign Chronic Pemphigus is a rare hereditary acantholytic disorder, transmitted as an irregular autosomal dominant traits, characterized by recurrent eruption of vesicles, bullae and impetiginous lesions mainly on the sides of neck, axillae and groins symmetrically. We presented a case of Familial Benign Chronic Femphigus in a 49 years old woman. She had suffered from these characteristic skin lesions have spread to the axillae, inframammary areas, antecubital fossae, inguinal and perianal area progressively. Moderate itching was associated with skin lesions, which were worse in summer and better in winter. Biopsy findings showed hyperkeratosis, irregular acanthosis, multilocular suprabasal clefts of bullae with extensive acantholysis and villi formation. Two daughtera of the patient has had the same clinical features on the sides of neck since puberty,