A 63-year-old woman with atherosclerotic peripheral vascular disease and the lupus anticoagulant developed ischemia of the right lower extremity, requiring progressive amputations. Pathologic specimens revealed inflammatory vasculitis in multiple arteries. Her serum showed anticardiolipin antibodies in high titer. Treatment with high-dose corticosteroids reversed the ischemic process. In patients with antiphospholipid antibodies, thrombosis is the most common pathologic finding associated with cutaneous lesions and/or gangrene. Vasculitis, although uncommon, is known to occur and may respond to corticosteroid therapy.A wide variety of clinical manifestations associated with antiphospholipid antibodies (aPL) have now been reported (1). Peripheral vascular disease with cutaneous manifestations such as ulcers and gangrene is a common association. In most instances, it has been believed that this is due to ischemia from thrombosis, with vasculitis playing little or no role. We
13210.Submitted for publication July 1, 1991 ; accepted in revised form December 3, 1991, present the case of a patient with complications arising from peripheral vascular disease. She was found to have aPL and an active inflammatory vasculitis. She responded well to corticosteroid therapy. The literature on the pathology of cutaneous lesions and gangrene in the aPL syndrome and systemic lupus erythematosus (SLE) is reviewed.
CASE REPORTThe patient, a 63-year-old white woman, was referred for evaluation of nonhealing surgical wounds. Pertinent medical history included a false-positive VDRL test result during a routine pregnancy evaluation at age 28. She was treated with penicillin but later, was told that the test was positive because she had lupus. At that time, she was asymptomatic, and she remained so for the next 30 years.In 1986, testing prior to a hysterectomy revealed a prolonged partial thromboplastin time (PTT) of 62.4 seconds (control 21.5-31.5) and a prothrombin time (PT) of 15.2 seconds (control 10.6-12.3). Mixture of equal parts of the patient's plasma and control plasma did not fully correct the PTT, and the dilute Russell viper venom time was prolonged, at 42.1 seconds, with phospholipid reagent. Clotting corrected fully when platelets were substituted for the phospholipid reagent. These features were consistent with the presence of a lupus anticoagulant (LAC). She underwent hysterectomy, and later, vaginal hernia repair, without complications.In 1988, she developed thigh and buttock claudication. In August 1989, shortly after a hospital admission for unstable angina, she developed signs of ischemia in her right foot. Arteriography showed severe atherosclerotic lesions of the abdominal aorta, with stenosis, narrowing, andor lesions of numerous branches including the inferior mesenteric artery and both iliac arteries. She was treated with anticoagulants, but gangrene ensued, and she underwent surgical amputation of the right first, second, and third toes. Because of poor wound healing and infection, a transmetatarsal
