Publication:

Autologous skin micro-grafts and allogenic skin allograft for mitten hand deformity treatment in epidermolysis bullosa

Background In the genetic skin blistering disorder dystrophic epidermolysis bullosa recurrent blisters and erosions on mechanically stressed areas, as the hands, result in subsequent scarring and development of pseudosyndactylies and contractures. In its most severe form, all fingers are constrained into a fist of scarred (“mitten-hand”-deformity). No causative therapy exists and only a surgical release of the fingers is possible. Hereby, the development of large skin defects is inevitable; the lack of healthy autologous skin for grafting remains a difficult challenge.

Methods We propose a new technique: It adopts a treatment strategy from the therapy of large extent burns and consists of a combination of autogenic and allogenic skin grafting.

Results We present a beneficial outcome in 18 mitten hand deformities treated with this technique. In all patients, the initial functional outcome was improved. Nearly all patients got a considerably widened grip span and durable skin conditions post-operatively. Mean follow-up was 3 years. Typically, recurrence of the scarring occurred from 6 months to 2 years after operation.

Conclusions This is the first report on the use of the combination of autogenous pinch skin grafts with allogenic (parental) skin overlay for skin defects in EB. It provides undisturbed wound healing under a “biological dressing”, furthermore, a temporary take of the parental skin was visible. Defects were permanently covered with skin of increased mechanical stability, hand function improved remarkably. However, long term follow-up showed beginning recurrence in most of the patients comparable to recurrence rates described in the literature.