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Generalized atrophic benign epidermolysis bullosa – poor prognosis associated with chronic renal failure
Author(s) -
Woo H. J.,
Lee J. H.,
Kim S. C.,
Kim C. W.,
Kim T. Y.
Publication year - 2000
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1046/j.1365-2230.2000.00617.x
Subject(s) - medicine , epidermolysis bullosa , junctional epidermolysis bullosa (veterinary medicine) , sepsis , complication , dermatology , surgery , laminin , genetics , biology , cell
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.