2005 Volume 115 Issue 10 Pages 1481-1486
A 15-year-old boy had had a painful ulcer with pigmentation on his left lower leg from May of 2004. He consulted our hospital in August. His laboratory findings showed thrombocytopenia, prolonged APTT, positive antiphospholipid antibody, and positive lupus anticoagulant, all of which still remained positive after six weeks. In addition, his anti-SS-A/Ro antibody titer was 303.6 U/ml (ELISA), but his anti-SS-B/La antibody was negative. Histopathological examination of the lesion showed lymphocytic infiltration around the arterioles and venules and thrombosis in the subcutaneous fat tissue. The findings of chest computerized tomography (CT), lung perfusion, and ventilation scintigraphy showed a pulmonary infarction. Thrombosis in the inferior vena cava was revealed in the abdominal CT image. A diagnosis of antiphospholipid antibody syndrome (APS) was made from these findings. Treatment with 30 mg/day prednisolone was effective, resulting in disappearance of the skin ulcer. The dose of prednisolone was tapered off within 11 weeks. He has also been treated with warfarin, and the dose was determined by monitoring with an international normalized ratio. In addition, a temporary inferior vena cava filter was inserted. We reviewed the literature of child cases with primary APS. There were more cases with cerebral ischemic lesions than cases with pulmonary infarction in pediatric primary APS cases compared to adult cases.
