Bullous pemphigoid and acquired haemophilia

Introduction. The association of bullous pemphigoid and acquired haemophilia is reported. Case-report. A 74 year-old man developed a bullous pemphigoid after decreasing corticotherapy, ecchymosis and haematomas revealing a high level of acquired anti-VIII antibodies (110 Bethesda UB units; TCA 98 s). Immunosuppressive treatment (cyclosporine, prednisone, azathioprine and bolus of cyclophosphamide) did not stop the disease. Perfusion of recombinant factor Vila, human immunoglobulins and prednisone-azathioprine association permitted clinical and biological remission. Discussion. Acquired haemophilia is idiopathic half the time. It can appear in autoimmune diseases. Mortality is high. Only 4 cases of association with bullous pemphigoid have been reported in the literature. At the haemorrhagic phase, porcine factor VIII or more recombinant activated factor VII with human immunoglobulins are necessary. Immunosuppressive treatment is used to decrease production of anti-factor VIII antibodies.