A 47-year-old female was admitted for severe pain of 1 month's duration in the third and fourth toes of the right foot, culminating in gangrene. Laboratory findings revealed liver enzyme abnormalities, and anti-mitochondrial, anti-phospholipid and antinuclear and doubtful anti-DNA antibodies. Systemic lupus erythematosus (SLE) was excluded on clinical grounds after a 6-year follow-up. Therefore, a diagnosis was made of the primary antiphospholipid syndrome, complicated by microvasculopathy, and associated with primary biliary cirrhosis.
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