title: [Pemphigoid and acquired hemophilia].
abstract: BACKGROUND
  The association of bullous pemphigoid and acquired haemophilia is reported.
  
  
  METHODS
  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 VIIa, human immunoglobulins and prednisone-azathioprine association permitted clinical and biological remission.
  
  
  CONCLUSIONS
  Acquired haemophilia is idiopathic half the time. It can appear in autoimmmune 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.
authors.affiliation: Service de Dermatologie, HIA Laveran, 13998 Marseille Armées.
authors.forename: E
authors.initials: E
authors.lastname: Lightburn
authors.forename: J J
authors.initials: JJ
authors.lastname: Morand
authors.forename: B
authors.initials: B
authors.lastname: Graffin
authors.forename: S
authors.initials: S
authors.lastname: Molinier
authors.forename: G
authors.initials: G
authors.lastname: Raphenon
authors.forename: P
authors.initials: P
authors.lastname: Poullin
authors.forename: J R
authors.initials: JR
authors.lastname: Harlé
authors.forename: C
authors.initials: C
authors.lastname: Chouc
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood; D005167:Factor VII / Q000627:therapeutic use; D005169:Factor VIII / Q000276:immunology*; D006467:Hemophilia A / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007116:Immunization, Passive; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D010391:Pemphigoid, Bullous / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D005167:Factor VII; D005169:Factor VIII