A Case of Primary Antiphospholipid Antibody Syndrome with Widespread Livedo and Arterial Infarction of the Right Median Nerve Treated by Immunoadsorption Therapy

Abstract

An 18-year-old female patient with a one-year history of right arm numbness presented with a six-month history of livedo on her bilateral dorsal pedis and pretibial regions. A biopsy specimen from an area of livedo showed thrombosis of the small vessels of the deeper dermis. Based on the typical clinical and histopathologic features in addition to the existence of lupus anticoaglant and deep vein thrombosis, she was diagnosed with primary antiphospholipid antibody syndrome and treated with platelet aggregation inhibitors and warfarin. However, the livedo expanded to her trunk and arms, and median nerve paralysis suddenly occurred due to arterial infarction. Immunoadsorption therapy was implemented four times, and oral predonisolone was administered simultaneously. In order to prevent rebound autoantibody production, methylprednisolone pulse therapy was performed. After immunoadsorption therapy, the lupus anticoaglant became negative, and her paralysis improved.