PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW
Autor(a) principal: | |
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Data de Publicação: | 2014 |
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.71.4.207 |
Resumo: | Introduction: The cutaneous mucinoses are rare disorders in which an abnormal amount of mucin accumulates in the skin. They may be primary or secondary and associated conditions include paraproteinemia, diabetes mellitus, thyroid and autoimmune diseases. In order to favor its clinical and pathological recognition, the primary cutaneous mucinoses diagnosed in our department are reviewed and characterized.Material and methods: A retrospective study from January 2000 to December 2012 of patients with primary cutaneous mucinoses was performed. The demographic, clinical and histopathological features were evaluated and a review of the literature was performed.Results: Fifteen cases were diagnosed in 8 women and 7 men. The mucinoses that have been found were lichen myxedematosus (7/15), reticular erythematous mucinosis (3/15), scleredema (3/15) and pretibial myxedema (2/15). Within the first, it was distinguished the discrete papular form (3/7) acral persistent papular mucinosis (2/7), nodular (1/7) and atypical form (1/7). An association was found with hyperthyroidism (in cases of pretibial myxedema) and primary biliary cirrhosis, autoimmune hepatitis and multiple myeloma (in 2 cases of scleredema).Conclusions: The limited number of cases is explained by the rarity of these conditions. Based on the results, an anatomical and clinical review was performed with a focus on the type, distribution and evolution of cutaneous lesions, well as the presence or absence of any systemic manifestations or pathological associations. Histologically, for the differential diagnosis, the most important features are the distribution of mucin, the degree of fibroblast proliferation, morphology of collagen fibers, the number of elastic fibers and the presence or absence of fibrosis. |
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PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEWMUCINOSES CUTÂNEAS PRIMÁRIAS – REVISÃO CLÍNICOPATOLÓGICAMucinosisfollicular/pathologyMucinsAlcian blueFibroblastsMucinoses cutâneasMucinaAzul de AlcianFibroblastosFibras de ColagénioIntroduction: The cutaneous mucinoses are rare disorders in which an abnormal amount of mucin accumulates in the skin. They may be primary or secondary and associated conditions include paraproteinemia, diabetes mellitus, thyroid and autoimmune diseases. In order to favor its clinical and pathological recognition, the primary cutaneous mucinoses diagnosed in our department are reviewed and characterized.Material and methods: A retrospective study from January 2000 to December 2012 of patients with primary cutaneous mucinoses was performed. The demographic, clinical and histopathological features were evaluated and a review of the literature was performed.Results: Fifteen cases were diagnosed in 8 women and 7 men. The mucinoses that have been found were lichen myxedematosus (7/15), reticular erythematous mucinosis (3/15), scleredema (3/15) and pretibial myxedema (2/15). Within the first, it was distinguished the discrete papular form (3/7) acral persistent papular mucinosis (2/7), nodular (1/7) and atypical form (1/7). An association was found with hyperthyroidism (in cases of pretibial myxedema) and primary biliary cirrhosis, autoimmune hepatitis and multiple myeloma (in 2 cases of scleredema).Conclusions: The limited number of cases is explained by the rarity of these conditions. Based on the results, an anatomical and clinical review was performed with a focus on the type, distribution and evolution of cutaneous lesions, well as the presence or absence of any systemic manifestations or pathological associations. Histologically, for the differential diagnosis, the most important features are the distribution of mucin, the degree of fibroblast proliferation, morphology of collagen fibers, the number of elastic fibers and the presence or absence of fibrosis.Introdução: As mucinoses cutâneas são doenças raras, caracterizadas pela deposição excessiva de mucina na pele. Podem ser primárias ou secundárias e associarem-se a paraproteinémia, diabetes mellitus, doenças tiroideias ou auto-imunes. O objectivo deste estudo consistiu em rever e caracterizar as diferentes mucinoses cutâneas primárias diagnosticadas no nosso Serviço visando facilitar o seu reconhecimento clínico e patológico.Material e Métodos: Realizou-se estudo retrospectivo dos exames histopatológicos dos doentes com diagnóstico de mucinose primária, no período compreendido entre Janeiro de 2000 e Dezembro de 2012. Foram avaliados dados demográficos, clínicos e histológicos e realizada revisão da literatura.Resultados: Foram diagnosticados 15 casos de mucinoses em 8 mulheres e 7 homens. As mucinoses diagnosticadas foram: líquen mixedematoso (7/15), mucinose eritematosa reticular (3/15), escleredema (3/15) e mixedema pré-tibial (2/15). Dentro da primeira distinguiram-se as formas papulosa discreta (3/7), mucinose papular acral persistente (2/7), nodular (1/7) e atípica (1/7). Encontrou-se associação com hipertiroidismo (nos casos de mixedema prétibial) e cirrose biliar primária, hepatite auto-imune e mieloma múltiplo (em 2 dos casos de escleredema).Conclusões: O número limitado de casos diagnosticados explica-se pela raridade destas condições. Com base nos resultados obtidos realizou-se revisão anátomo-clínica com especial ênfase para o tipo de lesão, distribuição e evolução, assim como a presença ou ausência de manifestações sistémicas ou associações patológicas. Em relação à histopatologia os dados fundamentais para o diagnóstico diferencial são o padrão de distribuição da mucina, grau de proliferação fibroblástica, morfologia das fibras de colagénio, número de fibras elásticas e presença ou ausência de fibrose.Sociedade Portuguesa de Dermatologia e Venereologia2014-07-06T00:00:00Zjournal articleinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://doi.org/10.29021/spdv.71.4.207oai:ojs.revista.spdv.com.pt:article/207Journal of the Portuguese Society of Dermatology and Venereology; Vol 71 No 4 (2013): Outubro - Dezembro; 467-475Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 71 n. 4 (2013): Outubro - Dezembro; 467-4752182-24092182-2395reponame: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/207https://doi.org/10.29021/spdv.71.4.207https://revista.spdv.com.pt/index.php/spdv/article/view/207/191Alves, JoãoMatos, DiogoCapitão-Mor, Manuelainfo:eu-repo/semantics/openAccess2022-10-06T12:34:45Zoai:ojs.revista.spdv.com.pt:article/207Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:10:45.681975Repositório 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 |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW MUCINOSES CUTÂNEAS PRIMÁRIAS – REVISÃO CLÍNICOPATOLÓGICA |
title |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW |
spellingShingle |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW Alves, João Mucinosis follicular/pathology Mucins Alcian blue Fibroblasts Mucinoses cutâneas Mucina Azul de Alcian Fibroblastos Fibras de Colagénio |
title_short |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW |
title_full |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW |
title_fullStr |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW |
title_full_unstemmed |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW |
title_sort |
PRIMARY CUTANEOUS MUCINOSES - A CLINICOPATHOLOGICAL REVIEW |
author |
Alves, João |
author_facet |
Alves, João Matos, Diogo Capitão-Mor, Manuela |
author_role |
author |
author2 |
Matos, Diogo Capitão-Mor, Manuela |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Alves, João Matos, Diogo Capitão-Mor, Manuela |
dc.subject.por.fl_str_mv |
Mucinosis follicular/pathology Mucins Alcian blue Fibroblasts Mucinoses cutâneas Mucina Azul de Alcian Fibroblastos Fibras de Colagénio |
topic |
Mucinosis follicular/pathology Mucins Alcian blue Fibroblasts Mucinoses cutâneas Mucina Azul de Alcian Fibroblastos Fibras de Colagénio |
description |
Introduction: The cutaneous mucinoses are rare disorders in which an abnormal amount of mucin accumulates in the skin. They may be primary or secondary and associated conditions include paraproteinemia, diabetes mellitus, thyroid and autoimmune diseases. In order to favor its clinical and pathological recognition, the primary cutaneous mucinoses diagnosed in our department are reviewed and characterized.Material and methods: A retrospective study from January 2000 to December 2012 of patients with primary cutaneous mucinoses was performed. The demographic, clinical and histopathological features were evaluated and a review of the literature was performed.Results: Fifteen cases were diagnosed in 8 women and 7 men. The mucinoses that have been found were lichen myxedematosus (7/15), reticular erythematous mucinosis (3/15), scleredema (3/15) and pretibial myxedema (2/15). Within the first, it was distinguished the discrete papular form (3/7) acral persistent papular mucinosis (2/7), nodular (1/7) and atypical form (1/7). An association was found with hyperthyroidism (in cases of pretibial myxedema) and primary biliary cirrhosis, autoimmune hepatitis and multiple myeloma (in 2 cases of scleredema).Conclusions: The limited number of cases is explained by the rarity of these conditions. Based on the results, an anatomical and clinical review was performed with a focus on the type, distribution and evolution of cutaneous lesions, well as the presence or absence of any systemic manifestations or pathological associations. Histologically, for the differential diagnosis, the most important features are the distribution of mucin, the degree of fibroblast proliferation, morphology of collagen fibers, the number of elastic fibers and the presence or absence of fibrosis. |
publishDate |
2014 |
dc.date.none.fl_str_mv |
2014-07-06T00:00:00Z |
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.71.4.207 oai:ojs.revista.spdv.com.pt:article/207 |
url |
https://doi.org/10.29021/spdv.71.4.207 |
identifier_str_mv |
oai:ojs.revista.spdv.com.pt:article/207 |
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/207 https://doi.org/10.29021/spdv.71.4.207 https://revista.spdv.com.pt/index.php/spdv/article/view/207/191 |
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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 71 No 4 (2013): Outubro - Dezembro; 467-475 Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 71 n. 4 (2013): Outubro - Dezembro; 467-475 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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