THE SURGICAL-MANAGEMENT OF DYSTROPHIC EPIDERMOLYSIS-BULLOSA (EXCLUDING THE HAND)

Fifty patients with Dystrophic Epidermolysis Bullosa (DEB) underwent surgery including release of limb, oral, anal, eye and penile contractures and treatment of chronic skin ulceration or skin tumours. Correction of contractures involves extensive release of skin and underlying tissues, with split skin grafting of secondary defects. Specific regions are discussed. Recurrence is inevitable due to ongoing disease; however, functional improvement is obtained for several years. Management of chronic skin ulceration with split skin grafting has failed to produce long term healing, with local flaps successful but limited by the problem of donor site instability. Nine of the 17 patients over 20 years of age developed squamous cell carcinomas (29 lesions), benign hyperkeratosis (9) or malignant melanoma (1) requiring excision and skin grafting or amputation of digits. Local recurrence was infrequent (3 squamous cell carcinomas), with distant metastatic spread occurring in 1 patient.