Sneddon syndrome is characterized by livedo reticularis and cerebrovascular events. Clinical manifestations may include hypertension and associated coagulopathies. The underlying pathophysiology of this syndrome remains obscure, yet increasing recognition of this entity may further ongoing investigations. In this article, the author summarizes the clinical features and most recent data related to this syndrome.
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• Sneddon syndrome is typically characterized by livedo reticularis, a patchy, netlike, violaceous skin discoloration that typically appears on the trunk and extremities and spares the face.
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• Antiphospholipid antibodies have been associated with a significant number of cases of Sneddon syndrome, although absence or fluctuating titers may also occur.
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• The majority of reported cases of Sneddon syndrome are female and hypertensive, often without evidence of significant renal involvement.
Historical note and terminology
In 1965 British dermatologist I B Sneddon described 6 patients with "cerebrovascular incidents which have been of limited and benign nature" associated with "benign type" hypertension and livedo reticularis (87). The patients ranged in age from 20 to 42 years, with livedo reticularis often preceding the cerebral manifestations by many years. He was unable to identify an underlying disease in these patients, though specifically excluding syphilis, tuberculosis, systemic lupus erythematosus, and polyarteritis nodosa. Although the syndrome of stroke and livedo reticularis is linked in name to Sneddon, a similar idiopathic case of livedo reticularis and cerebral thromboses in a 39-year-old man with biopsy-proven endarteritis obliterans had been published 5 years prior to Sneddon's report (16). The initial association of ischemic stroke and livedo reticularis preceded both these accounts by more than 50 years in a case report describing a patient with syphilis (26).
Different perspectives have arisen in the literature regarding the nomenclature for the dermatologic phenomenon that Sneddon referred to as "livedo reticularis." European authors often use the term "livedo racemosa" to describe this irregular, dark netlike pattern that persists on warming and reserve the term "livedo reticularis" for the diffuse "cutis marmorata" phenomenon, a transient physiologic response to cold usually occurring in children (13; 90).