Care of the hand in recessive epidermolysis bullosa.

ABSTRACT

Epidermolysis bullosa refers to a group of disorders whose common feature is blistering of the skin. This paper deals specifically with the loss of motion and digital function resulting from the recessive dystrophic type of epidermolysis bullosa in five young patients aged 3 to 11 years. Indications for surgery and preoperative planning are discussed. Special management considerations included skin care, the need for dietary supplements, and a preference for ketamine anesthesia. Epidermal degloving, full release of contractures, the use of split-thickness skin grafts, and immobilization and suspension of the hand by means of a traction bow without the use of other dressings constituted the important operative points. Postoperative treatment emphasizes wound care, splinting, and gradual mobilization of the joints. Long-term use of a splint to provide gentle digital separation helped prevent early recurrence of webbing. All the patients obtained increased joint motion and enhanced hand function as a result of their treatment.