Association of dominant dystrophic epidermolysis bullosa with end-stage renal failure and hearing loss

A 9-year-old boy with dominant dystrophic epidermolysis bullosa had hematuria. At the age of 14 he had proteinuria and at the age of 19 he had developed end-stage renal failure. Histopathological diagnosis of glomerular lesions was not made. During the course of his illness, high-frequency sensorineural hearing impairment developed and he became completely deaf. The hearing loss was thought to be caused by fradiomycin ototoxicity, as fradiomycin ointment had been used to treat secondary infections of the skin lesions since he was an infant. To our knowledge, an association of dominant dystrophic epidermolysis bullosa with end-stage renal failure and ototoxicity due to fradiomycin ointment have not been reported previously. Received: August 25, 1998 / Accepted: October 22, 1998