Surgical treatment of familial benign chronic pemphigus

Familial benign chronic pemphigus (Hailey and Hailey disease) is a rare, recalcitrant, often disabling genodermatosis that may not respond to conservative dermatologic therapy. We describe herein five patients with intertriginous familial benign chronic pemphigus who underwent excision and split-thickness skin grafting. All were men whose duration of disease ranged from one to 38 years. Follow-up evaluations ranging from ten months to nine years revealed no recurrence in graft sites in three patients, mild recurrence in one patient after eight years, and one death from pulmonary embolus in the postoperative period. The occurrence of familial benign chronic pemphigus around graft edges was a universal, but relatively minor, problem. Surgical excision provided definite relief from an otherwise disabling disease in four of our patients and a satisfactory improvement in life-style.