Synonym: Ehrmann-Sneddon syndrome
Sneddon's syndrome is a noninflammatory arteriopathy in which livedo reticularis is associated with cerebrovascular disease. It is slowly progressive and may go undiagnosed for some time. It may be seen in patients with an autoimmune disorder, eg antiphospholipid syndrome or systemic lupus erythematosus (SLE).
- Incidence is estimated at four cases per million population per year.
- Although initially thought to have a genetic aetiology (one family cluster displayed autosomal dominant inheritance), further studies have failed to demonstrate any family history in most affected patients.
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- The condition develops slowly over a variable timeframe.
- Nonspecific prodromal symptoms (headache, dizziness) often precede livedo reticularis by several years and this is followed by the development of focal neurological symptoms and then progressive cognitive impairment.
- Involvement of fundi, peripheral nerves, heart, and kidneys is frequent but usually asymptomatic.
- Livedo reticularis is a bluish mottling of skin, usually on the legs, and the appearance may be aggravated by exposure to cold.
- Sneddon's syndrome has been classified as primary if there is no clear cause, or secondary where it occurs as part of an autoimmune disorder, eg polyarteritis nodosa, SLE, or in a thrombophilic state, eg thrombocythaemia.
- Other associations include hypertension, systemic ischaemia (including ischaemic heart disease), heart valve lesions and venous thrombosis.
- Antiphospholipid and antiprothrombin antibodies have been associated in some but not all cases.
- Neuroimaging, including MRI, histological, immunohistochemical and ultrasound evaluation may be useful for diagnosis.
- Skin biopsy specimens reveal inflammatory changes of small- to medium-sized arteries, followed by subendothelial proliferation and fibrosis.
- In patients without any prior history of thromboembolism, minimise risk factors (stop smoking and/or combined oral contraceptives, control any hypertension, and treat any hyperlipidaemia).
- Consider prophylaxis with low-dose aspirin (clopidogrel if unable to take aspirin), particularly in patients with antiphospholipid or antiprothrombin antibodies. It may be appropriate for all patients but more research is needed.
- Patients with a history of thrombosis are treated indefinitely with anticoagulation (with warfarin ± low-dose aspirin).
- Hydroxychloroquine has been used in patients with SLE (it may have intrinsic antithrombotic properties).
- Some symptoms tend to resolve over a very variable time course.
- Prevention of further vascular events with anticoagulation is the key to a good prognosis.
- Hypertension is associated with a more severe course of the disease.
Further reading & references
- Sneddon IB; Cerebro-vascular lesions and livedo reticularis. Br J Dermatol. 1965 Apr;77:180-5.
- Sneddon Syndrome, Online Mendelian Inheritance in Man (OMIM)
- Marinho JL, Piovesan EJ, Leite Neto MP, et al; Clinical, neurovascular and neuropathological features in Sneddon's syndrome. Arq Neuropsiquiatr. 2007 Jun;65(2B):390-5.
- Schellong SM, Weissenborn K, Niedermeyer J, et al; Classification of Sneddon's syndrome. Vasa. 1997 Aug;26(3):215-21.
- Lewandowska E, Wierzba-Bobrowicz T, Wagner T, et al; Sneddon's syndrome as a disorder of small arteries with endothelial cells proliferation: ultrastructural and neuroimaging study. Folia Neuropathol. 2005;43(4):345-54. Folia Neuropathol. 2005;43(4):345-54.
- Legierse CM, Canninga-Van Dijk MR, Bruijnzeel-Koomen CA, et al; Sneddon syndrome and the diagnostic value of skin biopsies - three young patients Eur J Dermatol. 2008 May-Jun;18(3):322-8. Epub 2008 May 13.
- Belilos E et al; Antiphospholipid Syndrome, eMedicine, Aug 2009
- Frances C, Papo T, Wechsler B, et al; Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. Medicine (Baltimore). 1999 Jul;78(4):209-19.
|Original Author: Dr Huw Thomas||Current Version: Dr Hayley Willacy|
|Last Checked: 19/11/2010||Document ID: 1313 Version: 23||© EMIS|
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