Bratsos Sosipatros P, Demir Zilan, Mee John B, Groves Richard W, Tull Thomas J
St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Clin Exp Dermatol. 2024 Dec 23;50(1):97-103. doi: 10.1093/ced/llae283.
Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous disease that is mediated by antibodies that bind collagen VII. The treatment of EBA can be challenging and the use of multiple immunomodulatory drugs is often required. Rituximab has been reported to be an effective treatment for recalcitrant EBA, although the evidence base for this treatment is limited to case reports and case series.
This study aimed to evaluate the efficacy of rituximab for the treatment of patients with EBA.
Patients with EBA who were treated with rituximab were identified by searching electronic medical records. The diagnostic criteria for EBA were mechanobullous skin lesions and/or mucosal ulceration, indirect immunofluorescence localizing to the base of salt-split skin, and positive collagen VII antibodies. Clinical disease activity, collagen VII antibody levels and serum immunoglobulin levels were recorded at each follow-up visit over a 600-day period. Treatment responses were classified as follows: complete remission (CR) was defined as the absence of new or established lesions on minimal therapy for 2 months, partial remission (PR) was defined as transient lesions that heal within 1 week on minimal therapy, and active disease was defined as the development of new lesions.
In total, 14 patients with EBA were treated with rituximab. CR or PR was observed in 11 patients, and the duration of response varied between 4 and 24 months. A reduction in collagen VII antibody levels was observed in all patients. No significant adverse events were reported.
Rituximab is a safe and effective treatment for patients with recalcitrant EBA, although there was significant heterogeneity in the disease response.
获得性大疱性表皮松解症（EBA）是一种慢性黏膜皮肤疾病，由结合Ⅶ型胶原的抗体介导。EBA的治疗可能具有挑战性，通常需要使用多种免疫调节药物。据报道，利妥昔单抗是治疗顽固性EBA的有效药物，尽管该治疗的证据基础仅限于病例报告和病例系列。
本研究旨在评估利妥昔单抗治疗EBA患者的疗效。
通过检索电子病历识别接受利妥昔单抗治疗的EBA患者。EBA的诊断标准为机械性大疱性皮肤病变和/或黏膜溃疡、间接免疫荧光定位于盐裂皮肤的基底以及Ⅶ型胶原抗体阳性。在600天的随访期间，每次随访记录临床疾病活动度、Ⅶ型胶原抗体水平和血清免疫球蛋白水平。治疗反应分类如下：完全缓解（CR）定义为在最小治疗量下2个月无新的或已有的病变，部分缓解（PR）定义为在最小治疗量下1周内愈合的短暂性病变，活动性疾病定义为出现新病变。
共有14例EBA患者接受了利妥昔单抗治疗。11例患者观察到CR或PR，反应持续时间在4至24个月之间。所有患者的Ⅶ型胶原抗体水平均有所降低。未报告明显不良事件。
利妥昔单抗是治疗顽固性EBA患者的一种安全有效的药物，尽管疾病反应存在显著异质性。
