Sneddon's syndrome
| Sneddon syndrome | |
|---|---|
| Classification and external resources | |
| ICD-10 | M30.8 (ILDS M30.820) |
| OMIM | 182410 |
| DiseasesDB | 12257 |
| MeSH | D018860 |
Sneddon's syndrome[1] (also known as "Idiopathic livedo reticularis with cerebrovascular accidents")[1] is a form of arteriopathy characterized by several symptoms, including:
Signs and symptoms
Sneddon's syndrome manifests with elevated blood pressure (hypertension), a skin rash (livedo reticularis), and cerebrovascular disease. 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.
The neurologic symptoms include: headaches, labile hypertension, transient amnesia, transient aphasia, palsy, transient ischemic attacks (TIA), and stroke.[2] The skin manifestations may precede the neurologic symptoms by years.[2]
Pathogenesis
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.
Diagnosis
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
The treatment of Sneddon's syndrome includes daily aspirin and low dose heparin, which is an anticoagulant.[2]
Epidemiology
Sneddon's syndrome is a rare condition[2] that 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
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.
See also
- Livedoid vasculopathy
- List of cutaneous conditions
References
- ↑ 1.0 1.1 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ 2.0 2.1 2.2 2.3 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.
- ↑ synd/1732 at Who Named It?
- ↑ 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.
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.
| |||||||||||||||||||||||||||||||||||||||||
| |||||||||||||||||||||||||||||||