Acquired haemophilia A in a patient with pemphigus vulgaris: A case report on a rare coexistence

Acquired haemophilia A is a rare disease, occurs in patients with no family history of haemophilia. In contrast to congenital haemophilia, acquired haemophilia A is mediated by antibodies causing inactivation or increased clearance of factor VIII leading to abnormal bleeding manifestation. It can be associated with many underlying conditions including autoimmune diseases. Herein we report a case of a 64 year old male with underlying pemphigus vulgaris presented with a psoas haematoma. Coagulation profile, factor assay and inhibitor assay were confirmed the diagnosis of acquired haemophilia A. The patient was not responding to first line therapy, later started on rituximab and achieved remission successfully. This is a rare coexistence of acquired haemophilia A with pemphigus vulgaris with few reported cases worldwide. We believe this case report highlights the importance of suspecting an acquired bleeding disorder in a patient with underlying autoimmune disease, present with a recent onset of abnormal bleeding. Even though the bleeding in acquired haemophilia A could be life threatening, early intervention with haemostatic agents and eradication therapy would be lifesaving.