Sneddon’s Syndrome: Generalized Livedo reticularis and Cerebrovascular Disease

Abstract

We report two cases of Sneedon’s syndrome. Both cases had widespread livedo reticularis with repeated cerebrovascular accidents without persistent neurological deficit. In one case, hemostatic examination revealed an imbalance of plas-minogen activator-inhibitor values, possibly related to the thrombogenic propensity of the syndrome. Treatment with acetylsalicylic acid led to normalization of hemostatic parameters and resulted in a symptom-free period of more than 10 months. The importance of hemostatic screening in patients with Sneddon’s syndrome is discussed.

Livedo reticularis: An update

“…Sneddon's syndrome is a rare entity characterized by LR and cerebrovascular lesions. 21 There is significant similarity between the antiphospholipid syndrome and Sneddon's syndrome and the exact relationship of Sneddon's syndrome to SLE and the aPL syndrome is uncertain. Francés and Piette 22 describe looking at this relationship as a continuum of disease, with Sneddon's syndrome joining diverse clinicobiologic entities ranging from aPL-negative to SLE-related cases, with primary antiphospholipid syndrome in-between.…”

The Mystery of Sneddon Syndrome: Relationship with Antiphospholipid Syndrome and Systemic Lupus Erythematosus

“…Various abnormalities have been reported in isolated cases: activated protein C resistance [31], platelet aggregability [32], increased -thromboglobulin levels [33], modifications of the ratio tissue plasminogen activator/inhibitor [34] and familial deficiency in antithrombin III [35]. None of these abnormalities have been confirmed in large series, and the presence of aPL remains the most reliable explanation for the thrombotic process, but it accounts for a subset of SNS patients only.…”

Sneddon's Syndrome

“…We identified 97 case series and reports with 426 individuals with Sneddon's syndrome. In 42 case reports and 23 case series, containing a total of 208 individuals, the presence or absence of headache is not mentioned (15–64). The female to male ratio for this group is 3 : 1.…”