Epidermolysis bullosa pruriginosa responding to dupilumab

Abstract

Epidermolysis bullosa (EB) is a group of rare, inherited blistering disorders characterized by deficient dermoepidermal cohesion, resulting in fragile, easily blistered skin. The dystrophic variants are caused by a number of mutations of the COL7A1 gene on human chromosome 3, resulting in absent or dysfunctional collagen VII with resultant dysfunction of the anchoring fibrils below the basal lamina.1 EB pruriginosa (EBP), a subtype of EB, is characterized by numerous hypertrophic, intensely pruritic nodules and plaques primarily on the distal extremities.

Topics & Concepts