A case of Sneddon's syndrome with positive ANA and anti-cardiolipin antibodies: primary anti-phospholipid syndrome?
A 22-year-old woman developed ulcerative lesions on the lower extremities which usually exacerbated during the summer. Histological analysis revealed a micro-thrombotic lesion in the deep dermis without inflammatory cell infiltration or fibrinoid degeneration of blood vessels. Magnetic resonance imaging revealed multiple cerebral infarctions. Abnormal laboratory findings included an elevated anti-cardiolipin antibody titer and positive speckled pattern ANA (x80), but without other manifestations or signs of SLE. FACS analysis revealed that the patient's serum reacted with ethanol fixed endothelial cells in addition to keratinocytes and peripheral blood neutrophils. This case was thought to be livedo reticularis and cerebral thrombotic lesions (Sneddon's syndrome) associated with atrophie blanche or livedo(id) vasculitis and may be one clinical subset of primary anti-phospholipid syndrome.
Otoyama
K
K
Department of Dermatology, Kitasato University School of Medicine, Sagamihara, Japan.
Katayama
I
I
Suzuki
Y
Y
Tone
T
T
Nishioka
K
K
Nishiyama
S
S
D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis*; D001323:Autoantibodies / Q000032:analysis*; D002308:Cardiolipins / Q000276:immunology*; D002544:Cerebral Infarction / Q000276:immunology*; D005260:Female; D006801:Humans; D002542:Intracranial Embolism and Thrombosis; D010743:Phospholipids / Q000276:immunology; D012871:Skin Diseases / Q000276:immunology*; D013577:Syndrome
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D002308:Cardiolipins; D010743:Phospholipids