Recessive dystrophic epidermolysis bullosa--management of hand deformities

Scarring in recessive epidermolysis bullosa results in disability through hand deformities. Surgical treatment is complicated by technical and anaesthesiological problems due to the fragility of the skin and mucosa. In its dystrophic form epidermolysis bullosa leads to pseudosyndactyly and finger and palm contracture resulting in a "closed hand". Surgical treatment consists of resecting the contractures and opening the inter-digit webs, the uncovered areas healing under a close tulle gras dressing. Our technique differs from techniques which have already been reported. The dressing is changed only once a week, reducing the number of anaesthetics and allowing the child to attend our Day Hospital. Healing is complete within five weeks. Prevention of recurrence avoids the necessity of further surgery before five years of age. Post-operative open-hand splinting is well tolerated, delaying further contracture. Surgery is undertaken sometimes under general anaesthesia but more often under regional anaesthesia. We report the results of our experience.