Congenital Dystrophic Bullous Epidermolysis is a genetically caused disease. Its pathologico-anatomic characteristic is dermo-epidermal joint-level anchorage fibrils lack. Immediate consequences are bullation and skin detachment in all body areas, mucosae included, both after even slight traumatisms and spontaneously. Particularly, we focus on hands, where clinical picture goes from finger extension deficit to severe 'fist' closed hand. All agree in giving priority to hand opening and finger separation, while disagree on how to cover degloved hand and, above all, how to delay relapses. At present, in fact, relapses are responsible for both surgery and medico-physiatrical failure. A relapse after 4 years is acceptable, but a relapse within 2 years is absolutely insufficient. According to our case-report, average relapse rate was 1,5 years (a very poor rate indeed). On the contrary, our last patient has been treated with a new protocol: degloving, intraoperative splinting with metallic clips traction in P3 and homologous tension splint with calibered elastics, Corticotulle covering and truss; first dressing in day 15, in exposure; in day 30, splint displacement and assisted active kinesis initiation, with rest positioned splint during night; the thermoplastic material splint is constantly adjusted in time.
|Translated title of the contribution||Congenital Bullous Epidermolysis: Our protocol in upper limb total treatment|
|Number of pages||4|
|Journal||Rivista Italiana di Chirurgia Plastica|
|Issue number||1 SUPPL.|
|Publication status||Published - 1993|
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