title: [Polymorphic eruption of pregnancy and acquired hemophilia A].
abstract: BACKGROUND
  Acquired haemophilia A (AHA) is a rare and serious disease, and instances of association with skin diseases have been described. We report a case of postpartum AHA associated with atypical polymorphic eruption of pregnancy (PEP).
  
  
  METHODS
  Following delivery of her second child, a 27-year-old woman developed a generalised pruritic erythematous papular and vesicular rash in plaques. The diagnosis of pemphigoid gestationis was ruled out on the basis of negative immunopathology results and a diagnosis of PEP was made. Lengthening of activated cephalin time was observed, without correction by addition of control plasma, and prothrombin time was normal. AHA was confirmed by the very low levels of factor VIII and the presence of antifactor VIII antibody. The patient was given intravenous activated recombinant factor VII for epistaxis and gingival bleeding, followed by an infusion of polyvalent immunoglobulins and systemic corticosteroids. Both diseases regressed within a few weeks.
  
  
  CONCLUSIONS
  This case is original in terms of the atypical presentation of AHA associated with severe PEP. AHA was associated with the presence of antifactor VIII Ab. Although the disease generally occurs alone, it has already been reported during pregnancy and the postpartum period, and in association with various forms of dermatosis, including bullous pemphigoid, although to our knowledge, never in association with PEP or pemphigoid gestationis. However, neither the underlying mechanisms of this association of PEP and AHA, which was probably not a chance occurrence, nor the risks of relapse of these conditions during subsequent pregnancies have been elucidated.
authors.affiliation: Hôpital Robert-Debré, CHU de Reims, France. julie.tollhupp@wanadoo.fr
authors.forename: J
authors.initials: J
authors.lastname: Journet-Tollhupp
authors.forename: T
authors.initials: T
authors.lastname: Tchen
authors.forename: V
authors.initials: V
authors.lastname: Remy-Leroux
authors.forename: N
authors.initials: N
authors.lastname: Hezard
authors.forename: F
authors.initials: F
authors.lastname: Grange
authors.forename: P
authors.initials: P
authors.lastname: Bernard
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003029:Coagulants / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D011247:Pregnancy; D011644:Puerperal Disorders / Q000175:diagnosis* / Q000188:drug therapy; D011994:Recombinant Proteins / Q000627:therapeutic use; D017444:Skin Diseases, Papulosquamous / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D000305:Adrenal Cortex Hormones; D003029:Coagulants; D016756:Immunoglobulins, Intravenous; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa