Surgical treatment of familial benign chronic pemphigus

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Abstract
Familial benign chronic pemphigus (Hailey and Hailey disease) is a rare, recalcitrant, often disabling genodermatosis that may not respond to conservative dermatologic therapy. Five patients with intertriginous familial benign chronic pemphigus underwent excision and split-thickness skin grafting. All were men whose duration of disease ranged from 1-38 yr. Follow-up evaluations ranging from 10 mo. to 9 yr. revealed no recurrence in graft sites in 3 patients, mild recurrence in 1 patient after 8 yr, and 1 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 4 patients and a satisfactory improvement in life-style.