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Sneddon syndrome
Classification and external resources
Specialty rheumatology
ICD-10 M30.8 (ILDS M30.820)
OMIM 182410
DiseasesDB 12257
MeSH D018860

Sneddon's syndrome[1] is a form of arteriopathy characterized by several symptoms, including:

Signs and symptoms[edit]

Sneddon's syndrome generally manifests with stroke or severe, transient neurological symptoms, and a skin rash (livedo reticularis). Livedo reticularis appears as a bluish-purple, netlike mottling of the skin. Sneddon's syndrome may instead present with 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 can be characterized by: transient amnesia, transient aphasia, palsy, headaches, hypertension, transient ischemic attacks (TIA), stroke,[2] coronary disease and dementia. The skin manifestations may precede the neurologic symptoms by years.[2]

Pathogenesis[edit]

Sneddon's Syndrome is 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, coronary disease 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.

Diagnosis[edit]

There are no diagnostic tests on which all Sneddon's patients will have abnormal results, although brain MRI and skin biopsy are often abnormal. The diagnosis is based on a detailed history and physical examination. About 40-60% of patients with the syndrome test positive for antiphospholipid antibodies.

Treatment[edit]

Sneddon's patients are generally treated with warfarin, maintaining a high INR of 3-4.[1] Because most will experience significant relief of symptoms after several months of consistent INR in this range, treatment with warfarin is often used as a diagnostic tool.

Epidemiology[edit]

Sneddon's syndrome is a rare condition that is usually misdiagnosed.[2] It occurs in families and may be inherited in an autosomal dominant fashion. 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.

History[edit]

It is named for Ian Bruce Sneddon.[3][4] In 1965, Dr. Sneddon first reported 6 patients with a distinct skin rash and cerebrovascular accidents (strokes).

Sneddon's Syndrome was formerly understood to be a type of autoimmune disease called antiphospholipid syndrome, although it has been reclassified as a noninflammatory cerebrovascular disease. It should be considered in patients diagnosed with vasculitis when standard treatments fail.

See also[edit]

[5]== References ==

  1. ^ a b Berlit, Peter. "Sneddon's Syndrome". Orphanet. 
  2. ^ a b c Johnson, Klaus Wolff, Richard Allen (2009). Fitzpatrick's color atlas and synopsis of clinical dermatology. (6th ed. ed.). New York: McGraw-Hill Medical. p. Section 14. ISBN 978-0071599757. 
  3. ^ synd/1732 at Who Named It?
  4. ^ Sneddon IB (April 1965). "Cerebrovascular lesions and livedo reticularis.". Br. J. Dermatol. 77: 180–5. doi:10.1111/j.1365-2133.1965.tb14628.x. PMID 14278790. 
  5. ^ Adams et al. Principles of Neurology, 6th Edition. p. 861. 

External links[edit]

  • 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. 

Original courtesy of Wikipedia: http://en.wikipedia.org/wiki/Sneddon's_syndrome — Please support Wikipedia.
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5 news items

Mad In America

Mad In America
Sat, 18 Apr 2015 10:49:45 -0700

Imagine your life turned upside down with chronic symptoms like fatigue, stroke-like symptoms, brain fog, sharp chest pain, coughing up blood … and doctors can't seem to figure out what is wrong. Then imagine your feelings when one of the doctors ...
 
Washington Post
Mon, 02 Jul 2012 11:44:05 -0700

One night, she said, she stumbled upon a rare, progresssive disorder called Sneddon's syndrome. Named for the physician who first described a cluster of cases in 1965, Sneddon's affects the blood vessels, causing neurological abnormalities including ...

TheBlaze.com

TheBlaze.com
Thu, 20 Feb 2014 10:19:53 -0800

She was eventually diagnosed with Sneddon's syndrome, a disease affecting the arteries. After she started receiving treatment for this syndrome, Mildon said she went from the “point where I was dying” to one where she is functioning. O'Leary has ...
 
Mirror.co.uk
Tue, 22 Nov 2011 16:12:33 -0800

Tests showed Vicky was suffering from a rare and previously undiagnosed condition called Sneddon's syndrome, which means her blood clots more easily, putting her at a higher risk of a stroke. She was also found to have a leaky heart valve. In fact, an ...
 
EmpowHer
Mon, 29 Nov 2010 12:56:09 -0800

Sneddon's syndrome is a rare blood vessel and neurological disease that occurs predominately in women. A livedo reticularis rash appears following a stroke and the person will be at continued risk of developing further stroke events since blood vessels ...
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