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

The recessive form of dystrophic epidermolysis bullosa (RDEB) is associated with severe hand deformities characterized by cocooning pseudopolysyndactyly, recurrent ulceration, fibrosis, and adduction contracture of the thumb. In addition, RDEB is associated with an aggressive form of squamous carcinoma, notable for rapid metastases, appearance at an early age, and a relative resistance to radiation or chemotherapy. To date, reported reconstructive treatment has been limited to excision with skin grafting or amputation. We report the successful combined use of tissue expansion and microsurgical free tissue transplantation to reconstruct a patient with RDEB found to have malignancy of the hand. Donor skin was found to expand without blistering, and use of the expander allowed primary donor site closure after microsurgical transplantation 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 develop malignancy.