Surgical restoration of the hand in epidermolysis bullosa.

The extremities are affected by epidermolysis bullosa with blistering, scarring, and joint contractures. The hands are particularly vulnerable because of contact and shearing forces. Repeated cycles of blistering and scarring result in gradual encasement of the hand by an epidermal "cocoon." This article reviews the treatment approaches to loss of motion and independent digital function in the hands of several juvenile patients. All experienced an increase in joint motion and enhanced function as a surgical outcome of epidermal "degloving." Indications for surgery, preoperative planning, and postoperative care are discussed.