Surgical Management of Hand Contractures and Pseudosyndactyly in Dystrophic Epidermolysis Bullosa

The term epidermolysis bullosa refers to a group of disorders characterized by skin blistering following minor trauma. The hands, because of constant use during normal daily activity, are especially exposed to blistering, with secondary scarring leading to pseudosyndactyly, adduction contracture of the thumb, and flexion or extension contracture of the fingers. The standard surgical approach for the correction of these deformities is based on the incisional release of pseudosyndactyly and contractures, and split skin grafting of the secondary wounds. A simplified approach without skin grafting is presented in this paper. Four children (8 hands) underwent 25 operations over two decades. The results, in terms of postoperative healing and recurrence, were comparable with those of the standard approach involving skin grafting.