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Update on Approved Therapies for Dystrophic and Junctional Epidermolysis Bullosa
Alberto Pappalardo1, Edward Eid2, Rebecca C Olsen3
1Department of Dermatology, Columbia University Irving Medical Center, New York, New York, USA.
Abstract:
Dystrophic epidermolysis bullosa (DEB) and junctional EB (JEB) are severe, bullous genodermatoses induced by mutations of genes encoding structural skin proteins that disrupt epidermal adhesion. Until recently, treatment was limited to symptomatic care. Since 2022, three therapies - birch triterpenes gel (Filsuvez), beremagene geperpavec-svdt (B-VEC, Vyjuvek), and prademagene zamikeracel (pz-cel, Zevaskyn) - have received regulatory approval, representing the first specific interventions for epidermolysis bullosa. Herein, we provide an overview on their mechanisms of action, efficacy, safety, and clinical implications.
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