[Hand management in recessive dystrophic bullous epydermolysis].

PURPOSE: Recessive distrofic epidermolysis bullosa creates severe hand deformities with disabling functional limitations. Hand surgeon should perform surgery when deformity inibits function, in order to restore the pinch.

MATERIALS AND METHOD: We present our experience on 44 patients and 58 operated hands, with the following schema: hand degloving, grafting of the first web and intraoperative dynamic splinting.

RESULTS

In 30 patient with an 8 years follow up, 25 had had good or excellent results, and the 5 remaining shows early recurrence.

CONCLUSION

Association of a correct surgical approach and adequate intra and post-operative rehabilitation improve hand function and a slow down inevitable recurrence.