title: Acquired hemophilia associated with autoimmune bullous diseases: a report of two cases and a review of the literature.
abstract: 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.
authors.affiliation: Department of Hematology, Kitasato University School of Medicine, Japan.
authors.forename: Shinichi
authors.initials: S
authors.lastname: Makita
authors.forename: Takumi
authors.initials: T
authors.lastname: Aoki
authors.forename: Akira
authors.initials: A
authors.lastname: Watarai
authors.forename: Azusa
authors.initials: A
authors.lastname: Aida
authors.forename: Takuji
authors.initials: T
authors.lastname: Katayama
authors.forename: Mikio
authors.initials: M
authors.lastname: Danbara
authors.forename: Masaaki
authors.initials: M
authors.lastname: Higashihara
authors.forename: Koji
authors.initials: K
authors.lastname: Miyazaki
mesh_terms: D000328:Adult; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications / Q000175:diagnosis; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D010391:Pemphigoid, Bullous / Q000097:blood / Q000150:complications* / Q000175:diagnosis*
chemical_list: D001323:Autoantibodies