title: Bullous pemphigoid associated with acquired hemophilia a: a rare association of autoimmune disease.
abstract: BACKGROUND
  Acquired hemophilia (AH) is a rare autoimmune disease with an annual incidence of one per million and has a mortality rate of up to 22%. It is caused by the development of autoantibodies against factor VIII. Approximately half of the reported cases are associated with autoimmune disorders, pregnancy, malignancies, and adverse drug reactions. Autoimmune diseases are the most frequently associated disorders and include rheumatoid arthritis, systemic lupus erythematosus, cryoglobulinemia, pemphigus vulgaris, and bullous pemphigoid. There are a few reports of acquired hemophilia and bullous pemphigoid in the literature.
  
  
  METHODS
  We report a 73-year-old male who presented with cutaneous blistering, upper gastrointestinal bleeding, and hemoptysis. He later developed right flank pain secondary to a retroperitoneal hematoma. He had a prolonged partial thromboplastin time, a low factor VIII level, and a high factor VIII inhibitor level, all consistent with acquired hemophilia. Skin biopsies were diagnostic for bullous pemphigoid.
  
  
  RESULTS
  He was treated successfully with prednisone, cyclophosphamide, rituximab, and intravenous immunoglobulin.
authors.forename: Mohammed I
authors.initials: MI
authors.lastname: Aljasser
authors.forename: Chris
authors.initials: C
authors.lastname: Sladden
authors.forename: Richard I
authors.initials: RI
authors.lastname: Crawford
authors.forename: Sheila
authors.initials: S
authors.lastname: Au
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology / Q000473:pathology*; D006467:Hemophilia A / Q000097:blood / Q000276:immunology* / Q000473:pathology; D006801:Humans; D008297:Male; D010391:Pemphigoid, Bullous / Q000097:blood / Q000276:immunology* / Q000473:pathology