Acquired Hemophilia A in a Patient with Pemphigus Vulgaris: A Case Report

Abstract

Acquired hemophilia A is a rare disorder in which factor VIII inhibition occurs, resulting in decreased factor VIII levels and sudden subcutaneous or intramuscular hemorrhage. Although the mechanism of inhibition development remains unclear, it is sometimes associated with autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis, suggesting autoimmune involvement. An 80-year-old woman was diagnosed with pemphigus vulgaris after developing oral erosions one and a half months before her initial visit. She was treated with prednisolone 25 mg/day; however, her condition did not improve and high-dose gamma globulin therapy was administered. Her symptoms improved, but, when the dose of prednisolone was reduced to 12.5 mg/day, an intramuscular hematoma was observed in her right upper limb, and she was diagnosed with acquired hemophilia A. This case demonstrates that acquired hemophilia A may also be associated with autoimmune bullous dermatosis. Dermatologists should consider this condition when bleeding symptoms such as purpura and swelling are observed, even if the patient has a good therapeutic response with regards to the bullous disease.