Livedo vasculitis is characterized by recurrent livedo reticularis of lower extremities and the histopathological findings of segmental hyalinizing vasculitis in the skin. We report a case of a 26-year-old female who manifested mononeuritis multiplex 7 years after the onset of livedo vasculitis. She showed sensori-motor disturbances in the right median and ulnar nerves and sensory deficits of the bilateral peroneal nerves. Sural nerve biopsy revealed a remarkable loss of large and small myelinated fibers and a few vasculitic changes. Steroids therapy was effective for these neurological symptoms. But paroxysmal numbness appeared later recurrently in the regions of affected nerves with painful ulcerations in the right leg. Laboratory tests indicated increased levels of serum thrombin-antithrombin complex (TAT), and antithrombotic drugs (argatroban) remarkably ameliorated the recurrent symptoms and skin lesions. These findings suggest that the pathogenesis of livedo vasculitis might be related to alterations of the blood coagulation system.

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