Epidermolysis bullosa simplex (EBS) EBS subtype synonyms: localized EBS = EBS, Weber-Cockayne; EBS, Dowling-Meara (EBS herpetiformis) Are You Confident of the Diagnosis? Epidermolysis bullosa (EB) simplex, like all types of inherited epidermolysis bullosa, is characterized by the presence of mechanically fragile skin and recurrent blisters, erosions, and crusts. At least 17 subtypes of EBS have…
Are You Confident of the Diagnosis? What you should be alert for in the history Inherited dystrophic epidermolysis bullosa (DEB, including dominant [DDEB] and recessive [RDEB] subtypes) should be considered in any patient with a history of blistering present at or shortly after the time of birth, especially if there is a family history of…
Are You Confident of the Diagnosis? Key historical features The diagnosis of inherited junctional epidermolysis bullosa (EB) should be considered in any newborn or child with mechanically fragile skin and blisters. There are currently eight phenotypically and genotypically distinct JEB subtypes, three of which typically have relatively localized skin involvement. Junctional EB should be considered…
