| Classification of Sneddon's syndrome. | |
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MedLine Citation:
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PMID: 9286155 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: The combination of generalized broken ("racemose") livedo and cerebrovascular accidents is referred to as "Sneddon's syndrome". Although several pathogenetic factors have been suggested the aetiology of Sneddon's syndrome is unknown. Furthermore, considerable variability of patient characteristics gives rise to the question whether "Sneddon's syndrome" denotes a homogeneous disease entity at all. We hypothesized that the diagnosis "Sneddon's syndrome" can be broken down into different subgroups according to possible aetiologic factors. PATIENTS AND METHODS: Thirty-two patients with the combination of generalized broken livedo and cerebrovascular accidents were evaluated by clinical examination, routine diagnostic procedures, MRI of the brain, echocardiography, vascular ultrasound, immunologic and haemostaseologic testing. Patient groups were formed, depending on (1) whether or not an additional feature with a possibly aetiologic role for Sneddon's syndrome was present, and (2) which kind of feature it was. RESULTS: In 16 out of 32 patients, diagnostic features with an implication for the pathogenesis of Sneddon's syndrome could be identified. An autoimmune disorder was diagnosed in six patients. A thrombophilic state was detected in six patients. Three patients had preexisting atherosclerosis. One patient suffered from an embolizing atrial myxoma. Extent and kind of cerebral pathology differed between patient groups as did the kind of cardiac involvement. CONCLUSION: Sneddon's syndrome is not a homogeneous disease entity. Patients should be classified as "primary Sneddon's syndrome" if no aetiologic factor can be detected. On clinical grounds, this from differs from several varieties of "secondary Sneddon's syndrome" which occurs mainly as part of an autoimmune disorder or in a thrombophilic state. |
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Authors:
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S M Schellong; K Weissenborn; J Niedermeyer; J Wollenhaupt; M Sosada; C Ehrenheim; D Lubach |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: VASA. Zeitschrift für Gefässkrankheiten. Journal for vascular diseases Volume: 26 ISSN: 0301-1526 ISO Abbreviation: VASA Publication Date: 1997 Aug |
Date Detail:
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Created Date: 1997-10-30 Completed Date: 1997-10-30 Revised Date: 2008-02-13 |
Medline Journal Info:
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Nlm Unique ID: 0317051 Medline TA: Vasa Country: SWITZERLAND |
Other Details:
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Languages: eng Pagination: 215-21 Citation Subset: IM |
Affiliation:
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Department of Angiology, Hannover Medical School. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Antiphospholipid Syndrome / complications, diagnosis Blood Coagulation Tests Cerebrovascular Disorders / classification, diagnosis, etiology Diagnosis, Differential Diagnostic Imaging Female Humans Lupus Erythematosus, Systemic / complications, diagnosis Male Middle Aged Risk Factors Sneddon Syndrome / classification*, diagnosis, etiology |
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