Sneddon’s syndrome (SS) is an arteriopathy, associated with transient neurological symptoms or stroke.
Sneddon’s syndrome generally manifests with stroke or severe, transient neurological symptoms, and a skin rash-livedo reticularis, livedo racemosa.
There is a bluish-purple, netlike mottling of the skin.
Sneddon’s syndrome may instead present with livedo racemosa, which involves larger, less organized patches of bluish-purple mottling of the skin.
Both are generally found first in the extremities.
Skin lesions worsen in cold.
Sneddon’s syndrome can be characterized by: transient amnesia, transient aphasia, palsy, headaches, hypertension, transient ischemic attacks (TIA), stroke, coronary disease and dementia.
Sneddon’s syndrome skin manifestations may precede the neurologic symptoms by years.
Sneddon’s syndrome is a progressive, noninflammatory arteriopathy with the characteristic skin condition and to cerebrovascular problems, including stroke, transient ischemic attack (TIA), severe but transient neurological symptoms.
SS is thought to be associated cerebral vasospasm, coronary disease and early-onset dementia.
Progressive compromise of arterial linings in Sneddon’s produces thrombosis, for which oral anticoagulants are commonly prescribed.
SS can also cause the development of systemic arterial plaque when cholesterol levels are normal
No diagnostic tests exists.
The diagnosis is based on a detailed history and physical examination.
About 40-60% of patients with the syndrome test positive for antiphospholipid antibodies.
Sneddon’s patients are generally treated with oral anticoagulants, and most will experience significant relief of symptoms after several months.
It is a rare condition that is usually misdiagnosed.
It occurs in families and may be inherited in an autosomal dominant fashion.
It is most often found in women in their thirties, though cases do occur in men and in children.
Generally, livedo precedes cerebrovascular involvement by roughly ten years, and many years of cerebrovascular involvement precede the development of dementia, when it occurs.