Rituximab in the Treatment of Epidermolysis Bullosa Acquisita: A Systematic Review of the Literature.

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disorder characterized by blistering of the skin and mucous membranes. Current pathophysiology implicates autoantibodies targeting type VII collagen, which serves as a crucial component of anchoring fibrils that attach the epidermis to the dermis. Management of EBA remains challenging and relies on a combination of anti-inflammatory, immunosuppressive, and immunomodulating agents. Despite treatment, EBA is often chronic and relapsing, thus significantly impacting patients' quality of life.13 In this report, we aimed to systematically review the literature to provide an overview of the effectiveness of pharmacotherapy in treating cases of EBA with rituximab (RTX), specifically. We explore the efficacy of RTX as immunomodulator monotherapy and also its use in combination with other agents. A total of 51 patients were included over 20 studies and all studies were either case reports/series or retrospective chart reviews. The most utilized adjuvant therapies were glucocorticoids (n = 29) in the forms of methylprednisolone (MP), prednisolone (PL), and prednisone (P). RTX use in combination with PL had the best overall result; this regimen resulted in 15.7% (n = 8) of patients having clinical remission (CR) and 9.8% (n = 5) having well-controlled (WC) disease. Notably, the review elucidated that 4 patients had treatment with RTX alone and 100% of them achieved either CR or partial remission/control (PR/C). Our review highlights the relative efficacy of RTX in combination therapy and as monotherapy for the treatment of this rare condition, but more powerful research in the form of randomized clinical trials is imperative in order to obtain a more comprehensive understanding of its utility in the treatment of EBA. J Drugs Dermatol. 2025;24(4):387-393. doi:10.36849/JDD.8483.