Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome
The Sneddon’s syndrome is a rare disorder characterized by theoccurrence of cerebrovascular disease associated with livedoreticularis. The antiphospholipid syndrome is the most frequent typeof acquired thrombophilia, defined by the occurrence of thrombosisor pregnancy morbidity in the presence of pe...
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Instituto Israelita de Ensino e Pesquisa Albert Einstein
2012-06-01
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Series: | Einstein (São Paulo) |
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Online Access: | http://apps.einstein.br/revista/arquivos/PDF/2258-230-232.pdf |
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author | Livia Almeida Dutra Pedro Braga-Neto José Luiz Pedroso Orlando Graziani Povoas Barsottini |
author_facet | Livia Almeida Dutra Pedro Braga-Neto José Luiz Pedroso Orlando Graziani Povoas Barsottini |
author_sort | Livia Almeida Dutra |
collection | DOAJ |
description | The Sneddon’s syndrome is a rare disorder characterized by theoccurrence of cerebrovascular disease associated with livedoreticularis. The antiphospholipid syndrome is the most frequent typeof acquired thrombophilia, defined by the occurrence of thrombosisor pregnancy morbidity in the presence of persistently positiveantiphospholipid antibodies. Approximately 80% of Sneddon’ssyndrome patients have an antiphospholipid antibody marker. Theseantibodies may play a pathogenetic role in some cases of Sneddon’ssyndrome, and many authors consider these two syndromes as thesame entity. Although clinical features of antiphospholipid syndromeand Sneddon’s syndrome may overlap, there is a distinction betweenclinical and laboratory evidence suggesting that these two entitiesare different diseases. A recent finding of coagulopathies, includingelevated levels of coagulation factor VII, decreased levels ofprotein S, and activated protein C in Sneddon’s syndrome patientssuggested a possible biological link between the vasculopathy anda primary coagulopathy. Moreover, the clinical course seems to beprogressive in Sneddon’s syndrome patients and includes increaseof disability and cognitive deterioration, more arterial involvement,and the antiphospholipid syndrome shows a more benign course.Both syndromes share clinical and laboratory features, and whetherSneddon’s syndrome represents a spectrum of antiphospholipidsyndrome remains unclear. Sneddon’s syndrome patients have aworse prognosis and may represent a subgroup of patients whodemands more rigorous follow-up. It is important to recognize theSneddon’s syndrome, particularly because stroke episodes may beprevented through appropriate treatment. |
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format | Article |
id | doaj.art-ef83ab0fb7cb48cf91f30470aba60d1b |
institution | Directory Open Access Journal |
issn | 1679-4508 |
language | English |
last_indexed | 2024-12-23T20:05:50Z |
publishDate | 2012-06-01 |
publisher | Instituto Israelita de Ensino e Pesquisa Albert Einstein |
record_format | Article |
series | Einstein (São Paulo) |
spelling | doaj.art-ef83ab0fb7cb48cf91f30470aba60d1b2022-12-21T17:32:58ZengInstituto Israelita de Ensino e Pesquisa Albert EinsteinEinstein (São Paulo)1679-45082012-06-01102230232Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndromeLivia Almeida DutraPedro Braga-NetoJosé Luiz PedrosoOrlando Graziani Povoas BarsottiniThe Sneddon’s syndrome is a rare disorder characterized by theoccurrence of cerebrovascular disease associated with livedoreticularis. The antiphospholipid syndrome is the most frequent typeof acquired thrombophilia, defined by the occurrence of thrombosisor pregnancy morbidity in the presence of persistently positiveantiphospholipid antibodies. Approximately 80% of Sneddon’ssyndrome patients have an antiphospholipid antibody marker. Theseantibodies may play a pathogenetic role in some cases of Sneddon’ssyndrome, and many authors consider these two syndromes as thesame entity. Although clinical features of antiphospholipid syndromeand Sneddon’s syndrome may overlap, there is a distinction betweenclinical and laboratory evidence suggesting that these two entitiesare different diseases. A recent finding of coagulopathies, includingelevated levels of coagulation factor VII, decreased levels ofprotein S, and activated protein C in Sneddon’s syndrome patientssuggested a possible biological link between the vasculopathy anda primary coagulopathy. Moreover, the clinical course seems to beprogressive in Sneddon’s syndrome patients and includes increaseof disability and cognitive deterioration, more arterial involvement,and the antiphospholipid syndrome shows a more benign course.Both syndromes share clinical and laboratory features, and whetherSneddon’s syndrome represents a spectrum of antiphospholipidsyndrome remains unclear. Sneddon’s syndrome patients have aworse prognosis and may represent a subgroup of patients whodemands more rigorous follow-up. It is important to recognize theSneddon’s syndrome, particularly because stroke episodes may beprevented through appropriate treatment.http://apps.einstein.br/revista/arquivos/PDF/2258-230-232.pdfSneddon syndrome/immunologyAntiphospholipid syndrome/ immunologyAntibodiesanticardiolipinAntibodiesantiphospholipidLivedo reticularisStrokeCase reports |
spellingShingle | Livia Almeida Dutra Pedro Braga-Neto José Luiz Pedroso Orlando Graziani Povoas Barsottini Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome Einstein (São Paulo) Sneddon syndrome/immunology Antiphospholipid syndrome/ immunology Antibodies anticardiolipin Antibodies antiphospholipid Livedo reticularis Stroke Case reports |
title | Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome |
title_full | Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome |
title_fullStr | Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome |
title_full_unstemmed | Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome |
title_short | Sneddon’s syndrome: case report and review of its relationship with antiphospholipid syndrome |
title_sort | sneddon s syndrome case report and review of its relationship with antiphospholipid syndrome |
topic | Sneddon syndrome/immunology Antiphospholipid syndrome/ immunology Antibodies anticardiolipin Antibodies antiphospholipid Livedo reticularis Stroke Case reports |
url | http://apps.einstein.br/revista/arquivos/PDF/2258-230-232.pdf |
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