“…In rare cases, LR appears after neurological symptoms [ 18 ]. In addition to LR, some patients have signs of Raynaud’s phenomenon involving hands and feet and some have widespread cutaneous, mottled-purple discoloration on the body which had been diagnosed as systemic angiomatosis [ 19 , 20 ]. Figure 1 (two cases of SS ) a, diffused livedo racemosa (asterisks); b, FLAIR/T2 MRI brain scan showing multiple ischemic lesions (white arrows) and cortical atrophy (red arrows); c, skin biopsy showed intra-capillary (thin arrow) and parietal (thick arrow) widespread thrombosis; scale bar 100μm (Case1) and 50 μm (Case2).…”
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.
“…In rare cases, LR appears after neurological symptoms [ 18 ]. In addition to LR, some patients have signs of Raynaud’s phenomenon involving hands and feet and some have widespread cutaneous, mottled-purple discoloration on the body which had been diagnosed as systemic angiomatosis [ 19 , 20 ]. Figure 1 (two cases of SS ) a, diffused livedo racemosa (asterisks); b, FLAIR/T2 MRI brain scan showing multiple ischemic lesions (white arrows) and cortical atrophy (red arrows); c, skin biopsy showed intra-capillary (thin arrow) and parietal (thick arrow) widespread thrombosis; scale bar 100μm (Case1) and 50 μm (Case2).…”
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.
The study objective is to demonstrate a rare cause of recurrent acute cerebrovascular diseases in a young patient – Sneddon syndrome. The patient revealed gene polymorphism: homozygous 4G/5G in the plasminogen activator inhibitor-1 (PAI-1) gene, C807T in the glycoprotein I gene (GPIa), T1565C in the glycoprotein III gene (GPIIIa), G1639A in the vitamin K epoxide reductase gene (VKORC1), increased homocysteine, which were risk factors for thrombosis.
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