Bullous pemphigoid and acquired hemophilia

Introduction. Acquired hemophilia is a rare disease. Associated pemphigoid is extremely uncommon. We report a case of acquired hemophilia in the course of this dermatosis illustrating the severity of the disease. Observation. An 83 year-old woman was followed for 3 years for pemphigoid treated with topical corticosteroids following a recent relapse. She was hospitalized for surgical treatment of extensive and painful hematoma of the anterior aspect of the right leg following a fall. Preoperative clotting tests showed an extremely long cephalin coagulation time (> 2 x normal) not corrected by addition of control plasma, with a normal prothrombin time, due to acquired hemophilia type A and the presence of factor VIII inhibitor (17 Bethesda units/ml). The coagulant activity of factor VIII was reduced by 3%. The patient was treated with recombinant human factor VII (NovoSeven (R)) and systemic corticosteroids were subsequently administered at a dose of 1 mg/kg/d to ensure direct action on antibody production. Seven days later, the patient presented intense abdominal pain and extension deficit in the right lower limb. An abdominal-pelvic scan revealed spontaneous hematoma of the right psoas-iliac muscle. Despite replacement therapy with NovoSeven and oral corticosteroids, worsening of the hematoma occurred, complicated by hemorrhagic shock, resulting in death. Discussion. Acquired hemophilia, as revealed by cutaneous-mucosal bleeding, is a rare disease (1 to 4 cases per million subjects) more comonly seen in adults. it is associated with the presence of antibodies directed against factor VIII. Its complications, particularly hemorrhagic, are fatal in 15 to 20% of cases. While acquired hemophilia seems to occur in isolation in one of every two cases, it may be associated with autoimmune diseases, lymphoproliferative syndromes, solid tumors, the post-partum period, or use of certain drugs. In dermatology, acquired hemophilia has been reported in association with pemphigoid (9 cases), in which case the prognosis is consistently very poor, with pemphigus vulgaris (5 cases), more recently with acquired epidermolysis bullosa (3 cases), and finally with mucosal pemphigoid (1 case). Conclusion. Given the severity of associated hemorrhagic accidents, early identification of this clotting disorder is warranted in order to allow initiation of treatment as soon as possible.