Epidermolysis bullosa pruriginosa responding to dupilumab

Abstract

EB: epidermolysis bullosa EBP: epidermolysis bullosa pruriginosa IL: interleukin INTRODUCTION 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. EB pruriginosa (EBP), a subtype of EB, is characterized by numerous hypertrophic, intensely pruritic nodules and plaques primarily on the distal extremities. The chronic, recalcitrant nature of EBP can be debilitating and can reduce a patient’s quality of life. We present a case of EBP showing rapid clinical response to dupilumab after multiple failed alternative treatments, a response which has been sustained for 9 months thus far.