中国麻风皮肤病杂志 ›› 2022, Vol. 38 ›› Issue (2): 110-111.doi: 10.12144/zgmfskin202202110

• 病例报告 • 上一篇    下一篇

大疱性类天疱疮合并获得性血友病一例

车炫霖1,2,3,4,粟娟1,2,3,4,陈明亮1,2,3,4   

  1. 1中南大学湘雅医院皮肤科，湖南长沙，410008;
    2皮肤肿瘤与银屑病湖南省重点实验室，湖南长沙，410008;
    3皮肤健康与疾病湖南省工程研究中心，湖南长沙，410008;
    4国家老年疾病临床医学研究中心，湖南长沙，410008
  • 出版日期:2022-02-15 发布日期:2021-12-20
  • 通讯作者: 粟娟，E-mail: sujuanderm@csu.edu.cn；陈明亮，E-mail: xymingliang@csu.edu.cn

Acquired hemophilia associated with bullous pemphigus: a case report

CHE Xuanlin1,2,3,4, SU Juan1,2,3,4, CHEN Mingliang1,2,3,4   

  1. 1 Department of Dermatology, Xiangya Hospital, Central South University, Changsha 410008, China; 2 Hunan Key Laboratory of Skin Cancer and Psoriasis, Central South University, Changsha 410008, China; 3 Hunan Engineering Research Center of Skin Health and Disease, Central South University, Changsha 410008, China; 4 National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Changsha 410008, China
  • Online:2022-02-15 Published:2021-12-20
  • Contact: SU Juan, E-mail: sujuanderm@csu.edu.cn; CHEN Mingliang, E-mail: xymingliang@csu.edu.cn

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摘要： 获得性血友病A(acquired hemophilia A, AHA)死亡率为15%~42%，约50%左右的AHA为特发性，而另外50%与一些基础疾病有关，如系统性红斑狼疮、肿瘤、药物过敏、皮肤病（如银屑病，大疱性类天疱疮）等。本文报道一例大疱性类天疱疮合并获得性血友病，采用泼尼松、人凝血酶原复合物联合环孢素治疗后取得了显著疗效。

关键词: 大疱性类天疱疮, 获得性血友病, Ⅷ因子抑制剂

Abstract: The mortality rate of acquired hemophilia A (AHA) is 15%-42%, about 50% of AHA is idiopathic, and the other 50% is related to some underlying diseases, such as systemic lupus erythematosus, tumor, drug allergy, skin diseases (such as psoriasis, bullous pemphigoid) and so on. Herein, we report a patient with bullous pemphigoid complicated with AHA, who achieved significant efficacy after comprehensive treatment of oral prednisone and human prothrombin complex, cyclosporine.

Key words: bullous pemphigoid, acquired hemophilia, factor Ⅷ inhibitor