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Facts about the skin from DermNet New Zealand Trust. Topic index: A B C D E F G H I J K L M N O P Q R S T U V W X Y Z


Antiphospholipid syndrome

What is antiphospholipid syndrome?

Antiphospholipid syndrome is a disorder characterised by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests).

Primary antiphospholipid syndrome occurs when there is no evidence of associated diseases. Antiphospholipid syndrome in the presence of an underlying disease, usually systemic lupus erythematosus, is called secondary antiphospholipid syndrome.

A similar syndrome in which antiphospholipid antibodies are not detected is called seronegative antiphospholipid-like syndrome.

The name 'lupus anticoagulant' is misleading because not all patients with a positive result have lupus erythematosus, and it is associated with increased blood clotting rather than increased bleeding.

Who gets antiphospholipid syndrome?

Antiphospholipid syndrome occurs more commonly in young to middle-aged adults. However, it has also been found in a child as young as 8 months and in elderly people. Secondary antiphospholipid syndrome appears to be more common in females and is most likely due to the fact that systemic lupus erythematosus and other connective tissue disorders have a female predominance.

Younger patients with a history of deep vein thrombosis (DVT), pulmonary embolism, myocardial infarction, or cerebrovascular accidents (CVA), need to be investigated for antiphospholipid syndrome, particularly if no other risk factors are present.

What are the signs and symptoms of antiphospholipid syndrome?

Signs and symptoms of antiphospholipid syndrome are many and varied but to classify as antiphospholipid syndrome a patient must have at least one of the two following clinical manifestations in addition to the presence of certain laboratory abnormalities.

Livedo
Livedo
Splinter haemorrhages
Splinter haemorrhages
Cutaneous vasculitis
Vasculitis
Cutaneous vasculitis
Antiphospholipid syndrome

Other symptoms, although not part of the classification criteria, include:

A severe form of the disease is called catastrophic antiphospholipid syndrome. This is where blockage of blood vessels in multiple organs may occur over days or weeks. The condition is serious and often lethal.

Diagnosis of antiphospholipid syndrome

Diagnosis of antiphospholipid syndrome is confirmed from laboratory tests that show the presence of antiphospholipid antibodies or abnormalities in phospholipid-dependent tests of blood clotting (coagulation). Patients with suspected antiphospholipid syndrome should be tested for the following:

What treatment is available for antiphospholipid syndrome?

The main aim of treatment is to prevent the clinical manifestations of antiphospholipid syndrome. Risk factors for thrombosis should be identified and removed or corrected, for example, smoking, oral contraceptives, high blood pressure or elevated blood fats. Antiplatelet drugs such as low dose aspirin may be useful.

There is no specific treatment for antiphospholipid syndrome. Signs and symptoms are treated as they occur. For example, a thrombosis or embolism is treated with the anticoagulants heparin and warfarin.

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Author: Vanessa Ngan, staff writer


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