PR39P TREATMENT OF MITTEN HAND DEFORMITY IN A LONG TERM SURVIVOR OF EPIDERMOLYSIS BULLOSA

Abstract

EB is a recessive condition caused by a defect in collagen 7 production, such that epithelial cells do not adhere adequately to the underlying basement membranes. Minor mechanical trauma and stress forces will cause recurrent blistering of the skin and mucosal surfaces, which heal by scarring.The hand suffers particularly badly, and a characteristic mitten deformity of scar tissue is a common stigmata of the disease.We report a 51 year old survivor of EB with a bilateral mitten hand deformity that had been present since the age of 6 years.Extensive surgical release of the dominant left hand was undertaken, resurfacing the created defects with split skin grafts under regional anaesthesia. Fingers that had been buried for 45 years were straightened and held in place with k wires whilst the grafts were vascularising.Extensive post operative physiotherapy and splinting were undertaken, with functional improvement to the extent that the patient was able to hold a pen and write. Skin graft donor sites healed uneventfully.Extensive release of mitten hand deformity in long term survivors of EB is a worthwhile surgical procedure with good functional results. The problems of surgery and anaesthesia in this condition are discussed. Release of the second hand is planned.