Article
Epidermolysis bullosa pruriginosa: further clarification of the phenotype.
Pediatric dermatology - 1 Jan 2000
Brick Katherine, Hand Jennifer L, Frankel Amy S, Siegel Dawn H, Thomas Kelly B, El-Azhary Rokea, Krol Alfons
Abstract excerpt
A defect in type VII collagen causes dystrophic epidermolysis bullosa (DEB). The pruriginosa variant (DEB-Pr) is unique because its initial presentation may be delayed until adolescence or adulthood, and its predominant feature is scarring and pruritus without the characteristic skin fragility of DEB. We describe three families with multiple affected members in which DEB-Pr shows an autosomal-dominant inheritance...
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