title: Elevated levels of antibodies against phosphatidylserine/prothrombin complex and/or cardiolipin associated with infection and recurrent purpura in a child: a forme fruste of antiphospholipid syndrome?
abstract: Antiphospholipid syndrome is an autoimmune disorder characterized by the occurrence of venous and arterial thrombosis, as well as morbidity in pregnancy, in the presence of anti-phospholipid antibodies. The diagnosis of antiphospholipid syndrome is usually established based on clinical and laboratory findings by strictly following the 2006 Sapporo classification. However, the diagnosis remains challenging owing to the ongoing debates on the serological criteria. We report a case we describe as forme fruste antiphospholipid syndrome in which these criteria were not fulfilled. Purpura appeared repeatedly in a female infant starting from the age of 6 months and following episodes of upper respiratory infections and vaccinations. The levels of anti-cardiolipin IgG antibodies and anti-phosphatidylserine/prothrombin complex antibodies were elevated in accordance with these events. Histopathological evaluation revealed multiple small vessel thrombi in the dermis and adipose tissue. After 2 weeks of treatment with aspirin and heparin, the cutaneous symptoms subsided. Infection has long been associated with antiphospholipid syndrome, and anti-phosphatidylserine/prothrombin antibodies are considered a new marker for the diagnosis of antiphospholipid syndrome. Forme fruste antiphospholipid syndrome should be considered even if the antiphospholipid syndrome diagnostic criteria are not completely fulfilled, especially in the presence of elevated levels of anti-phosphatidylserine/prothrombin antibodies and known preceding infections.
authors.affiliation: Nippon Medical School, Japan. yuri-kino@nms.ac.jp.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Kinoshita
authors.forename: Nobuko
authors.initials: N
authors.lastname: Mayumi
authors.forename: Motoyuki
authors.initials: M
authors.lastname: Inaba
authors.forename: Touru
authors.initials: T
authors.lastname: Igarashi
authors.forename: Ichigen
authors.initials: I
authors.lastname: Katagiri
authors.forename: Seiji
authors.initials: S
authors.lastname: Kawana
mesh_terms: D017153:Antibodies, Anticardiolipin / Q000276:immunology* / Q000378:metabolism; D016736:Antiphospholipid Syndrome / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D001241:Aspirin / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology / Q000378:metabolism; D001424:Bacterial Infections / Q000276:immunology / Q000503:physiopathology; D001707:Biopsy, Needle; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D010718:Phosphatidylserines / Q000276:immunology* / Q000378:metabolism; D011379:Prognosis; D011516:Prothrombin / Q000276:immunology* / Q000378:metabolism; D011693:Purpura / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D012008:Recurrence; D018570:Risk Assessment; D016896:Treatment Outcome
chemical_list: D017153:Antibodies, Anticardiolipin; D001323:Autoantibodies; D010718:Phosphatidylserines; D011516:Prothrombin; D001241:Aspirin