Acquired Hemophilia Associated with Autoimmune Bullous Diseases: A Report of Two Cases and a Review of the Literature
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- Makita Shinichi
- Department of Hematology, Kitasato University School of Medicine, Japan
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- Aoki Takumi
- Department of Hematology, Kitasato University School of Medicine, Japan
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- Watarai Akira
- Department of Dermatology, Kitasato University School of Medicine, Japan
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- Aida Azusa
- Department of Hematology, Kitasato University School of Medicine, Japan
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- Katayama Takuji
- Department of Hematology, Kitasato University School of Medicine, Japan
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- Danbara Mikio
- Department of Hematology, Kitasato University School of Medicine, Japan
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- Higashihara Masaaki
- Department of Hematology, Kitasato University School of Medicine, Japan
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- Miyazaki Koji
- Department of Hematology, Kitasato University School of Medicine, Japan
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抄録
Acquired hemophilia (AHA) is a relatively rare and life-threatening disease caused by autoantibodies against factor VIII. Autoimmune bullous diseases (ABD) are also caused by autoantibodies against specific skin proteins. We herein report two cases of AHA associated with ABD. These coincidences are extremely rare, and only 14 documented cases have been reported previously. We further analyzed the properties of the autoantibodies in our patients. The epitopes were the A2 domain in patient 1, and both the A2 domain and the light chain in patient 2. Their isoforms were predominantly IgG4. Cross-reactivity could not be demonstrated. An accumulation of cases is required to unveil the pathogenesis of AHA.<br>
収録刊行物
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- Internal Medicine
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Internal Medicine 52 (7), 807-810, 2013
一般社団法人 日本内科学会