title: Coexistence of acquired hemophilia A and epidermolysis bullosa acquisita: Two case reports and published work review.
abstract: 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.
authors: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Tian-Meng
authors: TM
authors: Yan
authors: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Chun-Xia
authors: CX
authors: He
authors: Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Bao-Lai
authors: BL
authors: Hua
authors: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Li
authors: L
authors: Li
authors: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Hong-Zhong
authors: HZ
authors: Jin
authors: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Yue-Hua
authors: YH
authors: Liu
authors: Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Ya-Gang
authors: YG
authors: Zuo
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D001706:Biopsy; D002681:China; D024143:Collagen Type VII / Q000276:immunology; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use; D016107:Epidermolysis Bullosa Acquisita / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D004935:Esophageal Diseases / Q000209:etiology*; D005169:Factor VIII / Q000276:immunology; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D006396:Hematemesis / Q000209:etiology*; D006467:Hemophilia A / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D006509:Hepatitis B / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D019259:Lamivudine / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D018894:Reverse Transcriptase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D012867:Skin / Q000473:pathology
chemical_list: D001323:Autoantibodies; D024143:Collagen Type VII; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D018894:Reverse Transcriptase Inhibitors; D019259:Lamivudine; D016572:Cyclosporine; D005169:Factor VIII; D011241:Prednisone
keywords: acquired hemophilia A; cyclosporin; epidermolysis bullosa acquisita; hepatitis B; lamivudine