Epidermolysis bullosa pruriginosa (EBP) is a rare subtype of dystrophic epidermolysis bullosa characterized by severe pruritus, blistering, and nodular prurigo-like lesions.The condition is often associated with mutations in the COL7A1 gene, which encodes type VII collagen.Treatment options for EBP are limited, and managing pruritus and skin lesions remains challenging.Janus kinase (JAK) inhibitors, such as upadacitinib, have shown promise in treating inflammatory skin conditions, but their efficacy in EBP has not been well-documented.The patient experienced a significant reduction in pruritus within the first two weeks of treatment, with VAS scores decreasing by 70% at 4 wk.By 12 wk, there was marked improvement in skin lesions, with reduction in blister formation and resolution of nodules.The patient reported substantial improvement in quality of life, with no serious adverse effects observed during the 6-mo follow-up period.