Scleromyxedema: clinical diagnosis and autopsy findings

Detalhes bibliográficos
Autor(a) principal: Sala,Ana Carolina Bulhões
Data de Publicação: 2016
Outros Autores: Cunha,Paulo Rowilson, Pinto,Clóvis Antônio Lopes, Alves,Célia Antônia Xavier de Moraes, Paiva,Ingrid Barreto, Araujo,Ana Paula Vieira
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Anais brasileiros de dermatologia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100048
Resumo: Abstract Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
id SBD-1_f40b2b35cf454aca7c3cd25db04785ec
oai_identifier_str oai:scielo:S0365-05962016001100048
network_acronym_str SBD-1
network_name_str Anais brasileiros de dermatologia (Online)
repository_id_str
spelling Scleromyxedema: clinical diagnosis and autopsy findingsAutopsyCyclophosphamideParaproteinemiasScleromyxedemaAbstract Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.Sociedade Brasileira de Dermatologia2016-10-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100048Anais Brasileiros de Dermatologia v.91 n.5 suppl.1 2016reponame:Anais brasileiros de dermatologia (Online)instname:Sociedade Brasileira de Dermatologia (SBD)instacron:SBD10.1590/abd1806-4841.20164527info:eu-repo/semantics/openAccessSala,Ana Carolina BulhõesCunha,Paulo RowilsonPinto,Clóvis Antônio LopesAlves,Célia Antônia Xavier de MoraesPaiva,Ingrid BarretoAraujo,Ana Paula Vieiraeng2017-03-03T00:00:00Zoai:scielo:S0365-05962016001100048Revistahttp://www.anaisdedermatologia.org.br/https://old.scielo.br/oai/scielo-oai.phpabd@sbd.org.br||revista@sbd.org.br1806-48410365-0596opendoar:2017-03-03T00:00Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)false
dc.title.none.fl_str_mv Scleromyxedema: clinical diagnosis and autopsy findings
title Scleromyxedema: clinical diagnosis and autopsy findings
spellingShingle Scleromyxedema: clinical diagnosis and autopsy findings
Sala,Ana Carolina Bulhões
Autopsy
Cyclophosphamide
Paraproteinemias
Scleromyxedema
title_short Scleromyxedema: clinical diagnosis and autopsy findings
title_full Scleromyxedema: clinical diagnosis and autopsy findings
title_fullStr Scleromyxedema: clinical diagnosis and autopsy findings
title_full_unstemmed Scleromyxedema: clinical diagnosis and autopsy findings
title_sort Scleromyxedema: clinical diagnosis and autopsy findings
author Sala,Ana Carolina Bulhões
author_facet Sala,Ana Carolina Bulhões
Cunha,Paulo Rowilson
Pinto,Clóvis Antônio Lopes
Alves,Célia Antônia Xavier de Moraes
Paiva,Ingrid Barreto
Araujo,Ana Paula Vieira
author_role author
author2 Cunha,Paulo Rowilson
Pinto,Clóvis Antônio Lopes
Alves,Célia Antônia Xavier de Moraes
Paiva,Ingrid Barreto
Araujo,Ana Paula Vieira
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Sala,Ana Carolina Bulhões
Cunha,Paulo Rowilson
Pinto,Clóvis Antônio Lopes
Alves,Célia Antônia Xavier de Moraes
Paiva,Ingrid Barreto
Araujo,Ana Paula Vieira
dc.subject.por.fl_str_mv Autopsy
Cyclophosphamide
Paraproteinemias
Scleromyxedema
topic Autopsy
Cyclophosphamide
Paraproteinemias
Scleromyxedema
description Abstract Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
publishDate 2016
dc.date.none.fl_str_mv 2016-10-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100048
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962016001100048
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/abd1806-4841.20164527
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.91 n.5 suppl.1 2016
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_ 1752126421460844544

Registros relacionados