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Sneddon's Syndrome

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Synonyms: Ehrmann-Sneddon syndrome

Sneddon's syndrome is a non-inflammatory arteriopathy in which livedo reticularis is associated with cerebrovascular disease.1,2 It is slowly progressive and often unrecognised,3 and may be seen in patients with an autoimmune disorder (e.g. antiphospholipid syndrome or SLE).

Epidemiology
  • 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),2 further studies have failed to demonstrate any family history in most affected patients.3
Presentation
  • The condition develops slowly over a variable time frame.
  • Non-specific 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.
Associated diseases
Investigations
  • Anti-phospholipid and anti-prothrombin antibodies have been associated in some but not all cases.
  • Neuroimaging, including MRI, histological, immunohistochemical and ultrasound evaluation may be useful for diagnosis.5
  • Skin biopsy specimens reveal inflammatory changes of small- to medium-sized arteries followed by subendothelial proliferation and fibrosis.3
Management
  • In patients without any prior history of thromboembolism, minimise risk factors (stop smoking and/or combined oral contraceptives, control any hypertension, treat any hyperlipidaemia).
  • Consider prophylaxis with low dose aspirin (clopidogrel if unable to take aspirin), particularly in patients with antiphospholipid or anti-prothrombin antibodies.6 It may be appropriate for all patients but more research is needed.7
  • 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).7
Prognosis
  • 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.3


Document references
  1. Sneddon IB; Cerebro-vascular lesions and livedo reticularis. Br J Dermatol. 1965 Apr;77:180-5.
  2. Sneddon syndrome, Online Mendelian Inheritance in Man (OMIM)
  3. Zelger B, Sepp N, Stockhammer G, et al; Sneddon's syndrome. A long-term follow-up of 21 patients. Arch Dermatol. 1993 Apr;129(4):437-47. [abstract]
  4. Schellong SM, Weissenborn K, Niedermeyer J, et al; Classification of Sneddon's syndrome. Vasa. 1997 Aug;26(3):215-21. [abstract]
  5. 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. [abstract]
  6. Belilos E, Carsons S; Antiphospholipid Syndrome, eMedicine (Aug 2007).
  7. 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. [abstract]

Internet and further reading Acknowledgements EMIS is grateful to Dr Colin Tidy for writing this article and to Dr Huw Thomas for earlier versions. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2008.
DocID: 1313
Document Version: 22
DocRef: bgp2164
Last Updated: 30 Jul 2008
Review Date: 30 Jul 2010

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