COMBINED TISSUE EXPANSION AND FREE TISSUE TRANSFER FOR RECONSTRUCTIONOF THE HAND IN EPIDERMOLYSIS BULLOSA-ASSOCIATED MALIGNANCY

The recessive form of dystrophic epidermolysis bullosa (RDEB) is assoc iated with severe hand deformities characterized by cocooning pseudopo lysyndactyly, recurrent ulceration, fibrosis, and adduction contractur e of the thumb. In addition, RDEB is associated with an aggressive for m of squamous carcinoma, notable for rapid metastases, appearance at a n early age, and a relative resistance to radiation or chemotherapy. T o date, reported reconstructive treatment has been limited to excision with skin grafting or amputation. We report the successful combined u se of tissue expansion and microsurgical free tissue transplantation t o reconstruct a patient with RDEB found to have malignancy of the hand . Donor skin was found to expand without blistering, and use of the ex pander allowed primary donor site closure after microsurgical transpla ntation of a scapular flap. We recommend wide tumor resection and the aggressive use of contemporary reconstructive methods to maintain hand function and associated quality of life in patients with RDEB who dev elop malignancy.