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，是一种原型抗体介导的自身免疫性疾病。针对半融合体蛋白BP180和/或BP230的IgG自身抗体的检测是诊断性的，其水平可与疾病活动相关。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耐药的病例。在这里，我们描述了患有重度BP的患者，该患者对标准的一线治疗，静脉内免疫球蛋白和利妥昔单抗诱导的外周血B细胞耗竭是难治的。尽管持续存在高水平的抗皮肤IgG抗体，单克隆抗IgE抗体omalizumab的使用仍可快速解决水疱。据我们所知，这是首次报告了利妥昔单抗治疗失败后对奥马珠单抗有反应的BP病例。