Sneddon's syndrome
Sneddon's syndrome[1] (also known as "Idiopathic livedo reticularis with cerebrovascular accidents")[1] is a form of arteriopathy characterized by several symptoms, including:
It is named for Ian Bruce Sneddon.[2][3]
In 1965 Dr. Sneddon first reported 6 patients with a distinct skin rash and cerebrovascular accidents (strokes). Currently, Sneddon's Syndrome is understood as a progressive, noninflammatory arteriopathy leading to the characteristic skin condition and to cerebrovascular problems including stroke, transient ischemic attack (TIA), severe but transient neurological symptoms thought to be caused by cerebral vasospasm, and early onset dementia. Progressive compromise of arterial linings in Sneddon's produces clotting, for which high dose warfarin is most commonly prescribed, and can also cause the development of systemic arterial plaque when cholesterol levels are normal. In the skin, Sneddon's Syndrome manifests as either Livedo Reticularis, a bluish-purple, netlike mottling of the skin, or Livedo Racemosca, which involves larger, less organized patches of bluish-purple mottling of the skin. Both are generally found first in the extremities, both worsen in cold and either may occur without Sneddon's Syndrome or any other systemic disease. Sneddon's Syndrome most often becomes apparent 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. Sneddon's Syndrome was formerly understood to be a variety of the autoimmune disease Antiphospholipid Syndrome, though it has been reclassified as a noninflammatory cerebrovascular disease in which patients test positive for antiphospholipid antibodies in 40-60% of cases. Sneddon's has been known to occur in families and may be inherited in an autosomal dominant fashion. There are no diagnostic tests on which all Sneddon's patients will have abnormal results, though brain MRI and skin biopsy are very often abnormal.
See also
References
External links
- Schellong S, Weissenborn K, Niedermeyer J, Wollenhaupt J, Sosada M, Ehrenheim C, Lubach D (1997). "Classification of Sneddon's syndrome.". Vasa 26 (3): 215–21. PMID 9286155.