Abstract
Sneddon’s syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa(LR). The Orpha number for SS is ORPHA820. It has been estimated that the incidence of SS is 4 per 1 million per annum in general population and generally occurs in women between the ages of 20 and 42 years. LR may precede the onset of stroke by years and the trunk and/or buttocks are involved in nearly all patients. The cerebrovascular manifestations are mostly secondary to ischemia (transient ischemic attacks and cerebral infarct). Other neurological symptoms range from headache, cerebral hemorrhage, seizures, cognitive and psychiatric disturbances. The involved internal organs include heart, kidney, and eyes. Histological findings of skin are characteristic and the involved vessels are small to medium-sized arteries at the border of dermis to subcutis with a distinct histopathological time course. The main diagnostic criteria are general LR with typical histopathological findings on skin biopsy and focal neurological deficits. The pathogenesis is related to hypercoagulable state and intrinsic small-vessel vasculopathy. The optimal management remains an unsolved problem and long-term anticoagulation have been recommended for cerebral ischemic events based on the presumed pathogenesis. There are controversial results in treatment of SS with immunomodulatory agents. The aim of this review is to comprehensively discuss this disease.
Highlights
Definition Sneddon’s syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa (LR)
Three forms of the syndrome were described by Schellong et al.: primary, where no causative factor could be identified, autoimmune with antiphospholipid antibodies or coexisting systemic lupus erythematosus (SLE) or lupus-like disease and a thrombophilic form [4]
SS is a clinically syndrome that probably stems from a number of acquired or congenital hemostatic abnormalities which preferentially involves the cerebral and cutaneous vascular beds
Summary
Definition Sneddon’s syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the combination of cerebrovascular disease with livedo racemosa (LR). The principal pathologic findings in the case reported by Hilton DA et al were multiple small cortical infarcts associated with occlusion of medium sized arteries and prominent focal smooth muscle hyperplasia of smaller arterial vessels [55]. Either clinically audible or detected on echocardiogram, are frequently observed, and their total prevalence is similar in both groups This raises the possibility that the embolic mechanism plays an important role in the occurrence of some neurologic and even skin manifestations of SS [63]. Francès et al found that antiplatelet therapy was less effective in aPL-positive than in aPL-negative patients Among the former, the number of recurrent cerebral events per year of follow-up was 0.5 with antiplatelet therapy and 0.06 with anticoagulation. Future research should focus on identifying potential subgroups of patients who may be good responders
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