Xenopoulou Dimitra, Marson Justin W, Asrani Falguni
J Drugs Dermatol. 2025 Apr 1;24(4):387-393. doi: 10.36849/JDD.8483.
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.
获得性大疱性表皮松解症（EBA）是一种罕见的自身免疫性疾病，其特征为皮肤和黏膜出现水疱。目前的病理生理学表明，自身抗体靶向VII型胶原蛋白，VII型胶原蛋白是将表皮与真皮相连的锚定原纤维的关键组成部分。EBA的治疗仍然具有挑战性，依赖于抗炎、免疫抑制和免疫调节药物的联合使用。尽管进行了治疗，EBA通常是慢性复发性疾病，因此严重影响患者的生活质量。在本报告中，我们旨在系统回顾文献，以具体概述利妥昔单抗（RTX）药物治疗EBA病例的有效性。我们探讨了RTX作为免疫调节剂单一疗法的疗效，以及它与其他药物联合使用的情况。20多项研究共纳入了51例患者，所有研究均为病例报告/系列或回顾性病历审查。最常用的辅助治疗药物是糖皮质激素（n = 29），形式为甲泼尼龙（MP）、泼尼松龙（PL）和泼尼松（P）。RTX与PL联合使用总体效果最佳；该方案使15.7%（n = 8）的患者实现临床缓解（CR），9.8%（n = 5）的患者病情得到良好控制（WC）。值得注意的是，该综述表明有4例患者仅接受了RTX治疗，其中100%实现了CR或部分缓解/控制（PR/C）。我们的综述强调了RTX在联合治疗和单一治疗这种罕见疾病中的相对疗效，但必须开展更有力的随机临床试验研究，以便更全面地了解其在EBA治疗中的效用。《药物皮肤病学杂志》。2025年；24(4):387 - 393。doi:10.36849/JDD.8483。
