Literature DB >> 27511892

Coexistence of acquired hemophilia A and epidermolysis bullosa acquisita: Two case reports and published work review.

Tian-Meng Yan1, Chun-Xia He1, Bao-Lai Hua2, Li Li1, Hong-Zhong Jin1, Yue-Hua Liu1, Ya-Gang Zuo1.   

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.
© 2016 Japanese Dermatological Association.

Entities:  

Keywords:  acquired hemophilia A; cyclosporin; epidermolysis bullosa acquisita; hepatitis B; lamivudine

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Year:  2016        PMID: 27511892     DOI: 10.1111/1346-8138.13546

Source DB:  PubMed          Journal:  J Dermatol        ISSN: 0385-2407            Impact factor:   4.005


  2 in total

1.  Successful treatment of acquired hemophilia A associated with immune thrombocytopenia and joint hemarthrosis: A case report and literature review.

Authors:  Fang Wei
Journal:  Medicine (Baltimore)       Date:  2018-09       Impact factor: 1.817

2.  Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients.

Authors:  Hiroaki Iwata; Artem Vorobyev; Hiroshi Koga; Andreas Recke; Detlef Zillikens; Catherine Prost-Squarcioni; Norito Ishii; Takashi Hashimoto; Ralf J Ludwig
Journal:  Orphanet J Rare Dis       Date:  2018-09-04       Impact factor: 4.123

  2 in total

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