Livedoid Vasculopathy: A Review of 5 Clinical Cases

Detalhes bibliográficos
Autor(a) principal: Santos, Rui Pedro
Data de Publicação: 2018
Outros Autores: Ferreira, Olga, Brito, Celeste
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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spelling 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
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url https://doi.org/10.29021/spdv.76.3.916
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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
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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
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