Sneddon reported patients with a combination of ischemic cerebrovascular disease and livedo reticularis, sometimes presenting at a young age. Some cases have been linked to antiphospholipid antibodies, while others have not.
Features:
(1) presence of livedo reticularis, which is typically widespread
Some patients have evidence of antiphospholipid antibodies, qualifying the patient for antiphospholipid syndrome. These patients may show additional features of APS (miscarriage, valvular heart disease, migraine, etc)
Some patients do not show antiphospholipid antibodies. These may represent:
(1) a distinct subset with a vasculopathy affecting small arteries and/or thromboembolic phenomenon
(2) a failure to test for all types of antiphospholipid antibodies, such as anti-beta-glycoprotein I antibody
(3) a level of antiphospholipid antibody below the detection limit for the assay
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