Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Department of Dermatology, Shunyi Maternal and Children's Hospital of Beijing Children's Hospital, Beijing, China.
Front Immunol. 2023 Mar 8;14:1155181. doi: 10.3389/fimmu.2023.1155181. eCollection 2023.
Bullous pemphigoid is an autoimmune blistering disorder that primarily occurs in elderly patients. Reports indicate that BP coexists with various hematological diseases, including acquired hemophilia A, hypereosinophilic syndrome, aplastic anemia, autoimmune thrombocytopenia, and hematological malignancies. Early identification of these comorbidities contributes to a better control and reduced mortality. This article details the atypical clinical manifestations of BP when associated with hematological diseases, specific diagnostic strategies, underlying mechanistic connections, and possible treatments. Cross-reactivity between autoantibodies and exposed abnormal epitopes, shared cytokines and immune cells, together with genetic susceptibility are the most common connections between BP and hematological diseases. Patients were most often successfully treated with oral steroids combined with medications specifically targeting the hematological disorders. However, the individual comorbidities require specific considerations.
大疱性类天疱疮是一种自身免疫性水疱性疾病,主要发生在老年患者中。有报道称,BP 可与多种血液系统疾病同时存在,包括获得性血友病 A、嗜酸性粒细胞增多综合征、再生障碍性贫血、自身免疫性血小板减少症和血液系统恶性肿瘤。早期识别这些合并症有助于更好地控制疾病并降低死亡率。本文详细介绍了 BP 与血液系统疾病相关时的非典型临床表现、特定的诊断策略、潜在的发病机制联系以及可能的治疗方法。自身抗体与暴露的异常表位、共同的细胞因子和免疫细胞以及遗传易感性是 BP 与血液系统疾病之间最常见的联系。患者通常通过口服类固醇联合针对血液系统疾病的特定药物治疗获得成功,但个体合并症需要具体考虑。