Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease),
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Anais brasileiros de dermatologia (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962021000600730 |
Resumo: | Abstract Lipoid proteinosis is a rare autosomal recessive disease, characterized by hyaline deposits of PAS-positive material in tissues due to mutations in the ECM1 gene. This study evaluated the ultrastructure of the skin of a 6-year-old child affected by this condition. The light microscopy identified PAS-positive hyaline deposits, which were more intense in the papillary dermis. Scanning electron microscopy of the dermis showed a compact papillary dermis and fibrillar deposits in the middle dermis. Transmission electron microscopy clearly showed the deposition of fibrillar material in the dermis, forming clusters adherent to elastic fibers, between the collagen bundles and the collagen fibers, and also filling up the cytoplasm of dermal fibroblasts. |
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Anais brasileiros de dermatologia (Online) |
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Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease),Lipoid proteinosis of Urbach and WietheMicroscopyelectron, scanningMicroscopy, electron, transmissionSkin diseases, geneticAbstract Lipoid proteinosis is a rare autosomal recessive disease, characterized by hyaline deposits of PAS-positive material in tissues due to mutations in the ECM1 gene. This study evaluated the ultrastructure of the skin of a 6-year-old child affected by this condition. The light microscopy identified PAS-positive hyaline deposits, which were more intense in the papillary dermis. Scanning electron microscopy of the dermis showed a compact papillary dermis and fibrillar deposits in the middle dermis. Transmission electron microscopy clearly showed the deposition of fibrillar material in the dermis, forming clusters adherent to elastic fibers, between the collagen bundles and the collagen fibers, and also filling up the cytoplasm of dermal fibroblasts.Sociedade Brasileira de Dermatologia2021-12-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962021000600730Anais Brasileiros de Dermatologia v.96 n.6 2021reponame:Anais brasileiros de dermatologia (Online)instname:Sociedade Brasileira de Dermatologia (SBD)instacron:SBD10.1016/j.abd.2021.04.010info:eu-repo/semantics/openAccessAlmeida Jr,Hiram Larangeira deRodeghiero,Raphael GoveiaSuzuki,Patrícia Naomi AndoOgawa,Marília Marufujieng2022-01-13T00:00:00Zoai:scielo:S0365-05962021000600730Revistahttp://www.anaisdedermatologia.org.br/https://old.scielo.br/oai/scielo-oai.phpabd@sbd.org.br||revista@sbd.org.br1806-48410365-0596opendoar:2022-01-13T00:00Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)false |
dc.title.none.fl_str_mv |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
title |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
spellingShingle |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), Almeida Jr,Hiram Larangeira de Lipoid proteinosis of Urbach and Wiethe Microscopy electron, scanning Microscopy, electron, transmission Skin diseases, genetic |
title_short |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
title_full |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
title_fullStr |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
title_full_unstemmed |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
title_sort |
Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease), |
author |
Almeida Jr,Hiram Larangeira de |
author_facet |
Almeida Jr,Hiram Larangeira de Rodeghiero,Raphael Goveia Suzuki,Patrícia Naomi Ando Ogawa,Marília Marufuji |
author_role |
author |
author2 |
Rodeghiero,Raphael Goveia Suzuki,Patrícia Naomi Ando Ogawa,Marília Marufuji |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Almeida Jr,Hiram Larangeira de Rodeghiero,Raphael Goveia Suzuki,Patrícia Naomi Ando Ogawa,Marília Marufuji |
dc.subject.por.fl_str_mv |
Lipoid proteinosis of Urbach and Wiethe Microscopy electron, scanning Microscopy, electron, transmission Skin diseases, genetic |
topic |
Lipoid proteinosis of Urbach and Wiethe Microscopy electron, scanning Microscopy, electron, transmission Skin diseases, genetic |
description |
Abstract Lipoid proteinosis is a rare autosomal recessive disease, characterized by hyaline deposits of PAS-positive material in tissues due to mutations in the ECM1 gene. This study evaluated the ultrastructure of the skin of a 6-year-old child affected by this condition. The light microscopy identified PAS-positive hyaline deposits, which were more intense in the papillary dermis. Scanning electron microscopy of the dermis showed a compact papillary dermis and fibrillar deposits in the middle dermis. Transmission electron microscopy clearly showed the deposition of fibrillar material in the dermis, forming clusters adherent to elastic fibers, between the collagen bundles and the collagen fibers, and also filling up the cytoplasm of dermal fibroblasts. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021-12-01 |
dc.type.driver.fl_str_mv |
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 |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962021000600730 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962021000600730 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1016/j.abd.2021.04.010 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Dermatologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Dermatologia |
dc.source.none.fl_str_mv |
Anais Brasileiros de Dermatologia v.96 n.6 2021 reponame:Anais brasileiros de dermatologia (Online) instname:Sociedade Brasileira de Dermatologia (SBD) instacron:SBD |
instname_str |
Sociedade Brasileira de Dermatologia (SBD) |
instacron_str |
SBD |
institution |
SBD |
reponame_str |
Anais brasileiros de dermatologia (Online) |
collection |
Anais brasileiros de dermatologia (Online) |
repository.name.fl_str_mv |
Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD) |
repository.mail.fl_str_mv |
abd@sbd.org.br||revista@sbd.org.br |
_version_ |
1752126424619155456 |