Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease),

Detalhes bibliográficos
Autor(a) principal: Almeida Jr,Hiram Larangeira de
Data de Publicação: 2021
Outros Autores: Rodeghiero,Raphael Goveia, Suzuki,Patrícia Naomi Ando, Ogawa,Marília Marufuji
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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spelling 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
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