Sneddon syndrome

David S Liebeskind MD (Dr. Liebeskind of the University of California in Los Angeles received consulting fees from Stryker and Medtronic.)
Steven R Levine MD, editor. (Dr. Levine of the SUNY Health Science Center at Brooklyn has received honorariums from Genentech for service on a scientific advisory committee and a research grant from Genentech as a principal investigator.)
Originally released April 10, 1995; last updated January 16, 2017; expires January 16, 2020

Overview

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.

Key points

 

• 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.

 

Antiphospholipid antibodies have been associated with a significant number of cases of Sneddon syndrome, although absence or fluctuating titers may also occur.

 

• 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 (Sneddon 1965). 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 (Champion and Rook 1960). 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 (Ehrmann 1907).

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 (Bruyn et al 1987; Stockhammer et al 1993).

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