Off-Label Use of Double-Dose Beremagene Geperpavec (B-VEC) in the Treatment of Recessive Dystrophic Epidermolysis Bullosa: A Promising Clinical Case.

Abstract

Dystrophic epidermolysis bullosa (DEB) is a rare, inherited blistering skin disease with limited treatment options. Beremagene geperpavec (B-VEC) is a herpes simplex virus type 1-based topical gene therapy that is approved by the Food and Drug Administration (FDA) for the treatment of DEB at a fixed weekly dose. While B-VEC has shown significant promise in treating DEB, few studies have examined off-label use of B-VEC at higher dose volumes. We describe the successful off-label use of a temporarily increased B-VEC dose in a patient with recessive DEB (RDEB) with extensive wounds following an episode of acute urticaria, highlighting the potential utility of higher dose B-VEC in managing patients with extensive wound burden.