[Pemphigoid and acquired hemophilia].
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.
Lightburn
E
E
Service de Dermatologie, HIA Laveran, 13998 Marseille Armées.
Morand
J J
JJ
Graffin
B
B
Molinier
S
S
Raphenon
G
G
Poullin
P
P
Harlé
J R
JR
Chouc
C
C
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
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D005167:Factor VII; D005169:Factor VIII