Familial Benign Chronic Pemphigus: Surgical Treatment and Pathogenesis

A patient with recalcitrant familial benign chronic pemphigus was treated with split-thickness grafts to the groin. Postoperative fever and immobilization led to sweating and maceration of the skin, which in turn was followed by widespread clinical disease. This occurrence suggested that in this genodermatosis the susceptible skin is not limited to the neck, axillae, and groin but that hyperhidrosis and subsequent maceration account for this distribution. After surgery, the patient improved but continued to develop occasional cutaneous lesions in the grafted sites following exercise or trauma. We believe that the decreased number of functioning sweat glands (and subsequent decrease in sweating) produced by grafting favorably altered the intertriginous environment and accounted for his clinical improvement.