Abstract

Epidermolysis bullosa (EB) is a heterogeneous group of genetically determined skin fragility disorders in which minor trauma leads to blister formation on the skin. One of the most severe forms is Hallopeau-Siemens recessive dystrophic EB which main cause of mortality is squamous cell carcinoma (SCC). Exeresis of SCC leads to a difficult problem about treating the surgical wound. Most of the time, achieving a split-thickness skin graft on these severely affected EB patients is either too difficult or gives poor quality results. In some cases, flaps could be performed but they represent a too aggressive solution. However cutaneous pinch grafting is really adapted to the healing of these wounds. We have reported 4 cases of patients with recessive dystrophic EB complicated with SCC from 30 mm to 270 mm. After surgical excision of these SCC, dressings have been applied until obtaining a good enough floor for achieving a skin graft. The four patients have been treated by cutaneous pinch grafting. Airway management and monitoring have required particular precautions for avoiding anaesthetic related morbidity. We have noticed no adverse effect. A complete healing of good quality has been obtained in all cases (3 to 16 months of follow-up). No recurrence of SCC has been noticed and donors sites have had got a good healing. Relating to the healing of wounds after exeresis of SCC in case of dystrophic EB, cutaneous pinch grafting represents the most reliable solution with a minimum of physical traumatism.

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