Livedoid Vasculopathy: A Review of 5 Clinical Cases
Autor(a) principal: | |
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Data de Publicação: | 2018 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://doi.org/10.29021/spdv.76.3.916 |
Resumo: | Livedoid vasculopathy is an unusual thrombo-occlusive dermatosis that manifests with punctiform erythemato-purpuric lesions and extremely painful ulcers, preferably in the lower third of the legs. We report five patients with livedoid vasculopathy, three men and two women, aged between 24 and 54 years, with no relevant personal background. The diagnosis was confirmed by biopsy in four cases. Study of thrombophilias revealed heterozygosity of the MTHFR gene in two cases. Treatment was diversified and included among others, venothropics, anticoagulants, antithrombotics, immunosuppressants and intravenous immunoglobulin. Only one patient has unsatisfactory control of the disease. Livedoid vasculopathy etiopathogenesis remains uncertain. It may occur in the context of hereditary or acquired thrombophilia, autoimmunity or neoplasms. These changes should be excluded, but most cases remain idiopathic, which contributes to the therapeutic difficulty. The authors intend to emphasize the importance of the Dermatologist in the diagnosis of a probably under-recognized disease that causes significant morbidity. |
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Livedoid Vasculopathy: A Review of 5 Clinical CasesVasculopatia Livedóide: Uma Revisão de 5 Casos ClínicosArterial Occlusive DiseasesLeg UlcerLivedo ReticularisSkin UlcerArteriopatias OclusivasLivedo ReticularÚlcera da PeleÚlcera de PernaLivedoid vasculopathy is an unusual thrombo-occlusive dermatosis that manifests with punctiform erythemato-purpuric lesions and extremely painful ulcers, preferably in the lower third of the legs. We report five patients with livedoid vasculopathy, three men and two women, aged between 24 and 54 years, with no relevant personal background. The diagnosis was confirmed by biopsy in four cases. Study of thrombophilias revealed heterozygosity of the MTHFR gene in two cases. Treatment was diversified and included among others, venothropics, anticoagulants, antithrombotics, immunosuppressants and intravenous immunoglobulin. Only one patient has unsatisfactory control of the disease. Livedoid vasculopathy etiopathogenesis remains uncertain. It may occur in the context of hereditary or acquired thrombophilia, autoimmunity or neoplasms. These changes should be excluded, but most cases remain idiopathic, which contributes to the therapeutic difficulty. The authors intend to emphasize the importance of the Dermatologist in the diagnosis of a probably under-recognized disease that causes significant morbidity.A vasculopatia livedóide é uma dermatose trombo-oclusiva incomum que se manifesta com lesões eritemato-purpúricas punctiformes e úlceras extremamente dolorosas, preferencialmente no terço inferior das pernas. Apresentam-se cinco doentes com vasculopatia livedóide, três homens e duas mulheres, com idades entre os 24 e os 54 anos, sem antecedentes pessoais relevantes. O diagnóstico foi confirmado por biópsia em quatro casos. O estudo de trombofilias revelou heterozigotias do gene MTHFR em dois casos. A terapêutica efetuada pelos cinco doentes incluiu venotrópicos, antitrombóticos, anticoagulantes, imunossupressores e imunoglobulina endovenosa. Apenas um doente mantém controlo insatisfatório da doença. A etiopatogenia da vasculopatia livedóide permanece incerta. Pode surgir no contexto de trombofilias hereditárias ou adquiridas, autoimunidade ou neoplasias. Estas alterações deverão ser excluídas, embora grande parte dos casos permaneça idiopática, o que contribui para a dificuldade na abordagem terapêutica. Os autores pretendem salientar a importância do Dermatologista no diagnóstico de uma patologia provavelmente subdiagnosticada, mas que condiciona morbilidade significativa.Sociedade Portuguesa de Dermatologia e Venereologia2018-10-05T00:00:00Zjournal articleinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://doi.org/10.29021/spdv.76.3.916oai:ojs.revista.spdv.com.pt:article/916Journal of the Portuguese Society of Dermatology and Venereology; Vol 76 No 3 (2018): July - September; 287-294Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 76 n. 3 (2018): Julho - Setembro; 287-2942182-24092182-2395reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spdv.com.pt/index.php/spdv/article/view/916https://doi.org/10.29021/spdv.76.3.916https://revista.spdv.com.pt/index.php/spdv/article/view/916/584Santos, Rui PedroFerreira, OlgaBrito, Celesteinfo:eu-repo/semantics/openAccess2022-10-06T12:35:08Zoai:ojs.revista.spdv.com.pt:article/916Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:11:06.900081Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Livedoid Vasculopathy: A Review of 5 Clinical Cases Vasculopatia Livedóide: Uma Revisão de 5 Casos Clínicos |
title |
Livedoid Vasculopathy: A Review of 5 Clinical Cases |
spellingShingle |
Livedoid Vasculopathy: A Review of 5 Clinical Cases Santos, Rui Pedro Arterial Occlusive Diseases Leg Ulcer Livedo Reticularis Skin Ulcer Arteriopatias Oclusivas Livedo Reticular Úlcera da Pele Úlcera de Perna |
title_short |
Livedoid Vasculopathy: A Review of 5 Clinical Cases |
title_full |
Livedoid Vasculopathy: A Review of 5 Clinical Cases |
title_fullStr |
Livedoid Vasculopathy: A Review of 5 Clinical Cases |
title_full_unstemmed |
Livedoid Vasculopathy: A Review of 5 Clinical Cases |
title_sort |
Livedoid Vasculopathy: A Review of 5 Clinical Cases |
author |
Santos, Rui Pedro |
author_facet |
Santos, Rui Pedro Ferreira, Olga Brito, Celeste |
author_role |
author |
author2 |
Ferreira, Olga Brito, Celeste |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Santos, Rui Pedro Ferreira, Olga Brito, Celeste |
dc.subject.por.fl_str_mv |
Arterial Occlusive Diseases Leg Ulcer Livedo Reticularis Skin Ulcer Arteriopatias Oclusivas Livedo Reticular Úlcera da Pele Úlcera de Perna |
topic |
Arterial Occlusive Diseases Leg Ulcer Livedo Reticularis Skin Ulcer Arteriopatias Oclusivas Livedo Reticular Úlcera da Pele Úlcera de Perna |
description |
Livedoid vasculopathy is an unusual thrombo-occlusive dermatosis that manifests with punctiform erythemato-purpuric lesions and extremely painful ulcers, preferably in the lower third of the legs. We report five patients with livedoid vasculopathy, three men and two women, aged between 24 and 54 years, with no relevant personal background. The diagnosis was confirmed by biopsy in four cases. Study of thrombophilias revealed heterozygosity of the MTHFR gene in two cases. Treatment was diversified and included among others, venothropics, anticoagulants, antithrombotics, immunosuppressants and intravenous immunoglobulin. Only one patient has unsatisfactory control of the disease. Livedoid vasculopathy etiopathogenesis remains uncertain. It may occur in the context of hereditary or acquired thrombophilia, autoimmunity or neoplasms. These changes should be excluded, but most cases remain idiopathic, which contributes to the therapeutic difficulty. The authors intend to emphasize the importance of the Dermatologist in the diagnosis of a probably under-recognized disease that causes significant morbidity. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018-10-05T00:00:00Z |
dc.type.driver.fl_str_mv |
journal article info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.29021/spdv.76.3.916 oai:ojs.revista.spdv.com.pt:article/916 |
url |
https://doi.org/10.29021/spdv.76.3.916 |
identifier_str_mv |
oai:ojs.revista.spdv.com.pt:article/916 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://revista.spdv.com.pt/index.php/spdv/article/view/916 https://doi.org/10.29021/spdv.76.3.916 https://revista.spdv.com.pt/index.php/spdv/article/view/916/584 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
dc.source.none.fl_str_mv |
Journal of the Portuguese Society of Dermatology and Venereology; Vol 76 No 3 (2018): July - September; 287-294 Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 76 n. 3 (2018): Julho - Setembro; 287-294 2182-2409 2182-2395 reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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