[Hand management in recessive dystrophic bullous epydermolysis] [Il trattamento della mano nel paziente affetto da epidermolisi bollosa distrofica recessiva]

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. 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. In 30 patient with an 8 years follow up, 25 had had good or excellent results, and the 5 remaining shows early recurrence. Association of a correct surgical approach and adequate intra and post-operative rehabilitation improve hand function and a slow down inevitable recurrence.