Department of Clinical Immunology, Royal Perth Hospital, Perth, WA 6000, Australia.
Department of Clinical Immunology, Royal Perth Hospital, Perth, WA 6000, Australia; Department of Immunology, PathWest Laboratory Medicine, Queen Elizabeth II Medical Centre, WA 6009, Australia; Medical School, University of Western Australia, Perth, WA 6009, Australia.
Clin Immunol. 2019 Jan;198:54-56. doi: 10.1016/j.clim.2018.12.015. Epub 2018 Dec 14.
Bullous pemphigoid (BP) is a blistering dermopathy and a prototypic antibody-mediated autoimmune disease. Detection of IgG autoantibodies against hemidesmosomal proteins BP180 and/or BP230 are diagnostic and levels can correlate with disease activity. Therapies include corticosteroids and oral immunosuppressants, while intravenous immunoglobulin and rituximab are reserved for treatment resistant cases. Here we describe a patient with severe BP which was refractory to standard first line therapy, intravenous immunoglobulin and rituximab induced depletion of peripheral B cells. Use of the monoclonal anti-IgE antibody omalizumab resulted in rapid resolution of blistering despite ongoing high levels of anti-skin IgG antibodies. To our knowledge this is the first case of BP responsive to omalizumab after failure of rituximab to be reported. This case adds to emerging data on omalizumab as a novel BP treatment as well as providing new evidence of an independent role for autoreactive IgE-mediated inflammation in the formation of BP skin lesions.
大疱性类天疱疮（BP）是一种水疱性皮肤病，也是一种典型的抗体介导的自身免疫性疾病。检测针对半桥粒蛋白 BP180 和/或 BP230 的 IgG 自身抗体具有诊断意义，且其水平可与疾病活动度相关。治疗方法包括皮质类固醇和口服免疫抑制剂，而静脉注射免疫球蛋白和利妥昔单抗则保留用于治疗抵抗的病例。在这里，我们描述了一例严重的 BP 患者，该患者对标准一线治疗、静脉注射免疫球蛋白和利妥昔单抗诱导的外周 B 细胞耗竭均无反应。尽管持续存在高水平的抗皮肤 IgG 抗体，但使用单克隆抗 IgE 抗体奥马珠单抗可迅速缓解水疱。据我们所知，这是在利妥昔单抗治疗失败后，首例对奥马珠单抗有反应的 BP 病例。该病例增加了奥马珠单抗作为一种新型 BP 治疗方法的新数据，并提供了自身反应性 IgE 介导的炎症在 BP 皮肤损伤形成中具有独立作用的新证据。
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