Generalized atrophic benign epidermolysis bullosa--poor prognosis associated with chronic renal failure.
Generalized atrophic benign epidermolysis bullosa (GABEB) is an autosomal recessive form of junctional epidermolysis bullosa, with milder clinical features than the Herlitz subtype. A 25-year-old man presented with the clinical and histological findings of GABEB. At the initial visit, laboratory tests revealed that he also had chronic renal failure (CRF). Usually, GABEB has a good prognosis. However, in this case, the patient had CRF as an associated complication. He died of an intracranial haemorrhage combined with sepsis after 3 weeks of hospitalization. This case suggests that renal complications can occur in this relatively mild form of epidermolysis bullosa, and may contribute to morbidity and premature mortality.
Woo
H J
HJ
Department of Dermatology, College of Medicine, The Catholic University of Korea, and Department of Dermatology, College of Medicine, Yonsei University, Young Dong Hospital, Seoul, Korea.
Lee
J H
JH
Kim
S C
SC
Kim
C W
CW
Kim
T Y
TY
D000328:Adult; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D008297:Male; D011379:Prognosis