Sneddon’s syndrome: a comprehensive review of the literature

@article{Wu2014SneddonsSA,
  title={Sneddon’s syndrome: a comprehensive review of the literature},
  author={Shengjun Wu and Ziqi Xu and Hui Liang},
  journal={Orphanet Journal of Rare Diseases},
  year={2014},
  volume={9}
}
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… Expand
Cognitive and psychiatric changes as first clinical presentation in Sneddon syndrome
TLDR
A case of SS is reported in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis, and antiphospholipid antibodies were borderline. Expand
Sneddon syndrome and non-bacterial thrombotic endocarditis: a clinicopathological study
TLDR
The case of this patient was included within the authors' series of idiopathic SS, though the possibility of primary APSrelated SS cannot entirely be excluded given that, inevitably, at the time of admission blood tests, did not screen anti-cardiolipin and anti-β2GPI antibodies. Expand
Sneddon syndrome: anaesthetic and surgical challenges in cardiac valve surgery
TLDR
All the aspects described in the literature are summarized and the management to take when facing this rare disease is supported, considering the strong association with aPL antibodies and SS. Expand
Sneddon syndrome: a comprehensive clinical review of 53 patients
TLDR
The objective was to present the clinical features of Sneddon syndrome patients and to assess the role of antiphospholipid antibodies (APL) in patients diagnosed with SS, and to find no differences in the main clinical features between APL-positive andAPL-negative patients. Expand
Sneddon Syndrome with the Initial Presentation of Intracranial Hemorrhage: A Case Report
TLDR
A normal woman with negative medical history who, despite the normal ischemic cerebral manifestations, initially presented with intracranial hemorrhage, which progressed to new skin lesions (livedo reticularis) after a few days, and was diagnosed with SS. Expand
Sneddon Syndrome: Livedo Reticularis and Ischemic Stroke
TLDR
Sneddon syndrome(SS) is a rare, progressive vasculopathy with recurrent ischemic attacks, various treatment methods such as anticoagulants, antiaggregants, and immunosuppressions are recommended. Expand
Sneddon ' s syndrome , presented at first with intracranial hemorrhage : A case report
  • 2017
Sneddon's syndrome is characterized by chronic, progressive arteriopathy that causes ischemic stroke and skin lesions. It seems that thrombotic or embolic process in vessels may have role in itsExpand
Sneddon syndrome: under diagnosed disease, complex clinical manifestations and challenging diagnosis. A case-based review
TLDR
A case-based review of the Sneddon Syndrome is reported, illustrating the difficulty in recognizing SS and how the heterogeneity of the disease may be contributing to the difficulty making a distinct diagnosis. Expand
Characteristic imaging features of neurovascular involvement in primary Sneddon’s syndrome: an analysis of 12 cases
TLDR
MRI lesion severity was not correlated with angiographic arteriopathy severity, clinical stage, or presentation symptoms, andBrain MRI has diagnostic value in Sneddon's syndrome. Expand
Sneddon’s Syndrome: clinical case
TLDR
Dermatologic examination showed dusky erythematous to violaceous, irregular, broken circular segments, resulting in a seemingly larger pattern, located on limbs, trunk, and buttocks, exaggerated by the cold and persistent on warming. Expand
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References

SHOWING 1-10 OF 116 REFERENCES
[Sneddon syndrome: 9 cases].
TLDR
Sneddon's syndrome presents with heterogeneous signs related to its complex pathophysiology, and laboratory tests revealed a lupus band in one, anticardilipid antibodies in another and obliterating fibrous endartiritis of the renal arteries in the third. Expand
[Cerebral hemorrhage in Sneddon syndrome].
TLDR
A 49 year old man who, from the age of 23, had had repeated transient or established episodes compatible with ischemic cerebrovascular accidents and died of a cerebral hemorrhage whilst on anticoagulant treatment with Synthron is described. Expand
Sneddon's syndrome: clinical and laboratory analysis of 10 cases.
TLDR
The entire vascular, haematologic, neurologic, and dermatologic systems should be evaluated in patients diagnosed with Sneddon's syndrome. Expand
Livedo and ischemic strokes: diagnostic hints of a rare condition
TLDR
Two patients with clinical and imaging features suggestive of Sneddon’s syndrome, both 65-year-old men, presented with a 3-year history of progressive lower limbs weakness and mixed dysphagia, and coagulation parameters and thrombophilic screening were normal. Expand
Sneddon syndrome associated with Protein S deficiency.
TLDR
A 33-year-old woman with Sneddon syndrome is presented with diffuse livedo racemosa, recurrent cerebrovascular diseases, migraine-type headache, sinus vein thrombosis, and protein S deficiency. Expand
SNEDDON'S SYNDROME
TLDR
It is suggested that anti-2GPi antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with Sneddon syndrome. Expand
Life history of cutaneous vascular lesions in Sneddon's syndrome.
Sneddon's syndrome is a potentially fatal arterio-occlusive disorder characterized by generalized livedo racemosa and cerebrovascular lesions. Skin biopsies often fail to yield diagnostic arterialExpand
Sneddon's Syndrome
TLDR
The histopathological, radiological and serological data in Sneddon's syndrome and migraine underscores the plausibility of an association, and whether livedo reticularis may be a risk marker for stroke in migraineurs is an area for further study. Expand
Sneddon’s syndrome presenting with severe disabling bilateral headache
TLDR
The case of a 64-year-old woman presenting with a 4-year history of a throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom is described, noting the importance of using headache classification to avoid diagnostic errors. Expand
Livedo reticularis and cerebrovascular lesions (Sneddon's syndrome). Clinical, radiological and pathological features in eight cases.
TLDR
It is concluded that Sneddon's syndrome is a new genetic and progressive arteriopathy, occlusive and noninflammatory, involving medium-sized vessels. Expand
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