Department of Immunodermatology, Medical University of Warsaw, Warsaw, Poland.
Front Immunol. 2023 Jul 12;14:1214011. doi: 10.3389/fimmu.2023.1214011. eCollection 2023.
Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen. Standard therapy for EBA includes a combination of systemic corticosteroids and dapsone; however, severe cases may require advanced treatment. The current article reports on four EBA cases in which biologics: infliximab, rituximab (Rtx), and intravenous immunoglobulin (IVIG) were applied. All patients fulfilled the clinical and immunological criteria of EBA: they presented tense blisters healing with atrophic scars on the skin on traumatized areas and in mucous membranes. The diagnosis of EBA was established using numerous techniques: direct and indirect immunofluorescence, salt split skin, ELISA, Fluorescence Overlay Antigen Mapping using Laser Scanning Confocal Microscopy. Since all the patients did not achieve long-term remission on standard treatment (prednisone, dapsone) due to ineffectiveness or side effects of drugs, they eventually were treated with biologics leading to extraordinary skin improvement and stopping the disease for 1-3 years. Biologics in all patients were tolerated very well. No side effects were observed during application as well as multi-month follow-up. The presented cases provide a premise that biological drugs can be a valuable component of EBA therapy.
获得性大疱性表皮松解症（EBA）是一种慢性、复发性自身免疫性表皮下大疱性疾病，其特征是存在针对 VII 型胶原 - 基底膜带抗原的自身抗体。EBA 的标准治疗包括全身性皮质类固醇和氨苯砜的联合治疗；然而，严重的病例可能需要先进的治疗。本文报告了 4 例 EBA 病例，其中使用了生物制剂：英夫利昔单抗、利妥昔单抗（RTX）和静脉注射免疫球蛋白（IVIG）。所有患者均符合 EBA 的临床和免疫学标准：他们表现为紧张性水疱，在创伤部位和粘膜上的皮肤上愈合后留下萎缩性瘢痕。EBA 的诊断是通过多种技术确立的：直接和间接免疫荧光、盐裂皮肤、ELISA、使用激光扫描共聚焦显微镜的荧光叠加抗原映射。由于所有患者由于药物无效或副作用，在标准治疗（泼尼松、氨苯砜）下未获得长期缓解，最终接受生物制剂治疗，导致皮肤显著改善，并使疾病停止 1-3 年。所有患者对生物制剂的耐受性都非常好。在应用过程中和数月的随访中均未观察到副作用。所报告的病例提供了一个前提，即生物药物可以成为 EBA 治疗的有价值的组成部分。
