Treatment of Dystrophic Epidermolysis Bullosa Pruriginosa: A Systematic Review of Clinical Outcomes after Initiation of Dupilumab Therapy

Abstract

Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr), a highly pruritic subtype of dystrophic epidermolysis bullosa (DEB), can substantially impact patients’ quality of life due to symptom severity. This review features 10 patients diagnosed with DEB-Pr and a history of insufficient symptom relief following anti-inflammatory treatment. However, after initiation of dupilumab therapy, these patients exhibited marked clinical improvements in pruritic and cutaneous symptoms. Interestingly, one study showed an increase in type VII collagen following dupilumab therapy. These findings highlight the influence of T helper 2 (Th2)-mediated immunity in the pathogenesis of itch in DEB-Pr and dupilumab’s potential in the treatment of refractory pruritus.