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Sneddon syndrome in Adult
Other Resources UpToDate PubMed

Sneddon syndrome in Adult

Contributors: Anne Seidler MD, MBA, Art Papier MD
Other Resources UpToDate PubMed


Sneddon syndrome, also known as idiopathic livedo reticularis with cerebrovascular accidents, is an uncommon (and likely underdiagnosed) syndrome that consists of ischemic strokes and generalized livedo racemosa. The etiology is unknown, but some cases have been reported as inherited in an autosomal-dominant fashion.

Sneddon syndrome predominantly occurs in young women, and the first cerebral ischemic event is usually before the age of 45. The key cutaneous feature is a persistent livedo reticularis, often in a livedo racemosa pattern (form of livedo reticularis consisting of a large branching pattern, usually on the trunk, limbs, and proximal extremities).

Many individuals with Sneddon syndrome have antiphospholipid antibodies. The prevalence has been reported to be from 0% to 85%. Regardless of whether patients have antiphospholipid antibodies, individuals with Sneddon syndrome have identical occlusive arteriopathy and endothelial damage. Up to 35% will have antiendothelial cell antibodies.

Clinical features:
  • Livedo racemosa – often precedes the onset of focal neurologic symptoms.
  • Headache and vertigo – most frequent symptoms; may precede livedo racemosa by several years, but are nonspecific.
  • Ischemic strokes – most symptoms are from middle cerebral artery infarcts: hemiparesis, sensory alterations, and aphasia. Less commonly, there may be dysarthria, visual field deficits, or sudden spontaneous falls.
  • Transient ischemic attacks
  • Vascular dementia
  • Secondary symptoms and other organ manifestations include arterial hypertension (65%), valvulopathy (61%), ischemic heart disease, ocular symptoms, renal involvement (50% to 70%), gastrointestinal symptoms, recurrent deep vein thrombosis, and fetal loss.
The prognosis is poor. Mortality has been reported to be 9.5% over an observation period of 6.2 years.


M30.8 – Other conditions related to polyarteritis nodosa

238776001 – Sneddon syndrome

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Last Updated:01/24/2022
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Sneddon syndrome in Adult
A medical illustration showing key findings of Sneddon syndrome : Headache, Antiphospholipid antibodies present, Aphasia, Trunk, Vertigo, Visual field defect, BP increased, Arms, Legs
Clinical image of Sneddon syndrome - imageId=4342337. Click to open in gallery.  caption: 'Dusky retiform patches (livedo racemosa) on the thigh.'
Dusky retiform patches (livedo racemosa) on the thigh.
Copyright © 2024 VisualDx®. All rights reserved.