Acquired Haemophilia Associated with Urticarial Vasculitis

摘要

Acquired haemophilia (AHA) is a rare autoimmune disorder caused by circulating autoantibodies that inhibit the activity of factor VIII (FVIII). Acquired inhibitors against FVIII are rarely seen, with a reported incidence of approximately 1 case per million/year. Clinical conditions and contexts associated with AHA include autoimmune diseases, lymphoproliferative malignancies, drug treatment, pregnancy and infections. An association with urticarial vasculitis is even more rare. Here, we report a case of a 59-year-old woman presenting with cutaneous and muscle haematomas secondary to AHA in association with urticarial vasculitis, who was successfully treated with factor eight inhibitor bypassing activity (FEIBA) and prednisolone.