ANTIPHOSPHOLIPID ANTIBODY SYNDROME

By Gary M. White, MD

Antiphospholipid Antibody Syndrome Necrotic lesions on the distal fingers.


The antiphospholipid antibodies are a group of circulating autoantibodies directed against negatively charged phospholipid compounds. These antibodies promote thrombosis.

The diagnosis requires that a patient have:

Clinical

Widespread dusky erythema, cutaneous necrosis, leg ulceration, livedo racemosa, splinter hemorrhages, and ecchymosis all occur in the antiphospholipid antibody syndrome (APS). In one review, 84% of patients were women and the average age at presentation was 42 years of age.

Other findings include thrombocytopenia, Coombs' positive hemolytic anemia, valvular heart disease (e.g., valvular thickening, vegetation, and calcification), arthralgias, and elevated LFT's. The disease may be primary or secondary (occurring in association with other diseases, the classic being systemic lupus erythematosus), or as a characteristic familial trait. In one review, 53% of patients had primary disease while 36% were associated with lupus. The primary and secondary forms are similar in clinical expression. Systemic lupus erythematosus (SLE) patients with secondary APS have the signs and symptoms of APS but also satisfy the criteria for SLE. Some patient's disease presents at times of hormonal change, e.g., use of oral contraceptives. A false positive VDRL or a prolonged PTT may suggest the diagnosis. Up to 2% of apparently normal people have detectable antibodies and in 0.2% the titer is high. The risk of thrombosis in these patients is unknown.

Treatment

Referral to a specialist is appropriate.

Additional Pictures

Antiphospholipid Antibody Syndrome Antiphospholipid Antibody Syndrome

References

An adult woman. Actas Dermo-Sifiliograficas

A girl with SLE. Dermatología Argentina

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