Surgical treatment of the hand deformities in dystrophic Epidermolysis bullosa. Case report

Background. Surgical intervention is the mainstay of treatment of the hand deformity in Epidermolysis bullosa. Case report. We describe the surgical therapy of a 4 year old girl sufferin g from this disease and having both hands completely clumped to a fist, bas ed on the consequent release of pseudosynclactyly and contractures of the f ingers with dermolysis, arthrolysis, arterio- and neurolysis. Spontaneous e pithelialization of the skin wounds occurred within 8-10 weeks. This method has the advantage of short operating time, limited trauma and satisfying f unctional results. Discussion. Maintenance of the results depends on the implementation of an adequate postoperative aftercare including passive and active kinetotherapy , gradual mobilisation of the affected joints and thorough skin care.