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, wit h secondary scarring leading to pseudosyndactyly, adduction contracture of the thumb, and flexion or extension contracture of the fingers. The standar d surgical approach for the correction of these deformities is based on the incisional release of pseudosyndactyly and contractures, and split skin gr afting of the secondary wounds. A simplified approach without skin grafting is presented in this paper. Four children (8 hands) underwent 25 operation s over two decades. The results, in terms of postoperative healing and recu rrence, were comparable with those of the standard approach involving skin grafting.