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...

Full description

Bibliographic Details
Main Authors: Livia Almeida Dutra, Pedro Braga-Neto, José Luiz Pedroso, Orlando Graziani Povoas Barsottini
Format: Article
Language:English
Published: Instituto Israelita de Ensino e Pesquisa Albert Einstein 2012-06-01
Series:Einstein (São Paulo)
Subjects:
Online Access:http://apps.einstein.br/revista/arquivos/PDF/2258-230-232.pdf
_version_ 1819262950814253056
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.
first_indexed 2024-12-23T20:05:50Z
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
work_keys_str_mv AT liviaalmeidadutra sneddonssyndromecasereportandreviewofitsrelationshipwithantiphospholipidsyndrome
AT pedrobraganeto sneddonssyndromecasereportandreviewofitsrelationshipwithantiphospholipidsyndrome
AT joseluizpedroso sneddonssyndromecasereportandreviewofitsrelationshipwithantiphospholipidsyndrome
AT orlandograzianipovoasbarsottini sneddonssyndromecasereportandreviewofitsrelationshipwithantiphospholipidsyndrome