JUNCTIONAL EPIDERMOLYSIS-BULLOSA - DIAGNOSIS AND MANAGEMENT OF A PATIENT WITH THE HERLITZ VARIANT

A case of Herlitz-type epidermolysis bullosa (EB) is reported. The bab y was born after a normal, full-term pregnancy with blisters on his ex tremities. Over the next several months, progressive skin sloughing in volving 95% of his body surface area developed, including gastrointest inal, laryngeal, tracheobronchial, and corneal involvement. The diagno sis of junctional EB, Herlitz type, was made using clinical, electron microscopic, and immunohistochemical criteria. Despite meticulous woun d care, aggressive nutritional support, and continuous antimicrobial t herapy, the baby died at 9 months of age. This report discusses the pr esentation, complications, and management of this unusual and tragic d isease. Copyright (C) 1998 by W.B. Saunders Company.