Generalized atrophic benign epidermolysis bullosa - poor prognosis associated with chronic renal failure

Generalized atrophic benign epidermolysis bullosa (GABEB) is an autosomal r ecessive form of junctional epidermolysis bullosa, with milder clinical fea tures than the Herlitz subtype. A 25-year-old man presented with the clinic al and histological findings of GABEB. At the initial visit, laboratory tes ts revealed that he also had chronic renal failure (CRF). Usually, GABEB ha s a good prognosis. However, in this case, the patient had CRF as an associ ated complication. He died of an intracranial haemorrhage combined with sep sis after 3 weeks of hospitalization. This case suggests that renal complic ations can occur in this relatively mild form of epidermolysis bullosa, and may contribute to morbidity and premature mortality.