Refractory Epidermolysis Bullosa Pruriginosa with Rapid Clinical Response to Upadacitinib Treatment

Epidermolysis bullosa pruriginosa (EBP) is a rare subtype of dystrophic EB. Topical corticosteroids, tacrolimus, oral thalidomide, and immunosuppressants are among the treatment options that can be given, but there is no consistently effective treatment. Some case reports have shown positive results in treatment with omalizumab, dupilumab, and Janus kinase inhibitors. We present a 37-year-old female EBP patient who had not responded to multiple previous treatments (systemic steroids, hydroxychloroquine, dapsone, omalizumab, and cyclosporine) but achieved a rapid clinical response to upadacitinib treatment. We are sharing our case to encourage the consideration of upadacitinib as an alternative treatment for refractory EBP cases.