血管炎性ニューロパチーを伴った原発性抗リン脂質抗体症候群の1例(神経内科学教室竹宮敏子教授退任記念特別号)

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We report a rare case of 50-year-old man who had primary antiphospholipid antibody syndrome with vasculitic neuropathy. In March 1997, he noticed sensory disturbances on the right side of his body, including his face. He was diagnosed with cerebral infarction at another hospital. From May to August, he noted painful nodules in both his lower legs, followed by successive sensory disturbances such as dysesthesia in the right foot and then the left foot, numbness in both fingers, dysesthesia in the ulnar side of the right forearm and the lateral side of the right lower leg. He was admitted to the Department of Dermatology Tokyo Wemen's Medical University in October. A skin biopsy revealed necrotizing vasculitis. He was referred to the Department of Neurology for further examination of his sensory disturbances. Laboratory findings revealed positive lupus anticoagulant, a coagulation abnormality, increased thrombocyte functions, and decreased sensory nerve action potentials. A brain MRI showed small infarctions in the left putamen and thalamus. In the lung scintigraphy there were multiple embolisms in both lungs. A sural nerve biopsy revealed multifocal myelinated fiber loss and perivascular inflammatory cell infiltrations in the small epineurial vessels. Although he was positive for antinuclear antibodies, anti-RNP antibodies, and anti ss-DNA antibody, his laboratory or clinical findings were not compatible with any collagen vascular diseases such as systemic lupus erythematodus and mixed connective tissue disease. Consequently, he was diagnosed as having primary antiphospholipid antibody syndrome with associated skin and peripheral nerve vasculitides. A course of oral corticosteroid and warfarin were started and his symptoms were gradually improved. Vasculitis in primary antiphospholipid antibody syndrome is rare. Only ten such cases were reported previously. In such a case, it may develop into collagen disease or Bechet disease and it is necessary to follow it up sufficiently.
東京女子医科大学の論文
2000-12-30

血管炎性ニューロパチーを伴った原発性抗リン脂質抗体症候群の1例(神経内科学教室竹宮敏子教授退任記念特別号)

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