Yan Tian-Meng, He Chun-Xia, Hua Bao-Lai, Li Li, Jin Hong-Zhong, Liu Yue-Hua, Zuo Ya-Gang
Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
J Dermatol. 2017 Jan;44(1):76-79. doi: 10.1111/1346-8138.13546. Epub 2016 Aug 11.
Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. These EBA may be related to the pathogenesis of AHA. In this study, we analyzed the clinical features in the two Chinese cases of EBA coexisting with AHA, and found esophageal hemorrhage and hematemesis were the main symptoms of both patients. Cyclosporin, prednisone and lamivudine effectively control EBA with AHA and hepatitis B. The dose of cyclosporin should be more than 4 mg/kg per day and the period of treatment should be longer than 5 months to reduce the risk of EBA co-occurring with AHA.
获得性大疱性表皮松解症(EBA)是一种罕见的慢性表皮下大疱性自身免疫性疾病。获得性血友病A(AHA)的发病率较低,因此EBA与AHA并存极为罕见。我们在此描述了1例EBA与AHA并存的病例以及1例EBA与AHA和乙型肝炎并存的病例。这些EBA可能与AHA的发病机制有关。在本研究中,我们分析了2例中国EBA与AHA并存病例的临床特征,发现食管出血和呕血是两名患者的主要症状。环孢素、泼尼松和拉米夫定可有效控制合并AHA和乙型肝炎的EBA。环孢素的剂量应每天超过4mg/kg,治疗时间应超过5个月,以降低EBA与AHA同时发生的风险。