Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview
Autor(a) principal: | |
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Data de Publicação: | 2019 |
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=S0365-05962019000400388 |
Resumo: | Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively. |
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Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overviewAutoantibodiesAutoimmunityParaneoplastic syndromesPemphigusSkin diseases, vesiculobullousAbstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.Sociedade Brasileira de Dermatologia2019-08-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962019000400388Anais Brasileiros de Dermatologia v.94 n.4 2019reponame:Anais brasileiros de dermatologia (Online)instname:Sociedade Brasileira de Dermatologia (SBD)instacron:SBD10.1590/abd1806-4841.20199165info:eu-repo/semantics/openAccessMaruta,Celina WakisakaMiyamoto,DeniseAoki,ValeriaCarvalho,Ricardo Gomes Ribeiro deCunha,Breno MedeirosSanti,Claudia Giulieng2019-10-11T00:00:00Zoai:scielo:S0365-05962019000400388Revistahttp://www.anaisdedermatologia.org.br/https://old.scielo.br/oai/scielo-oai.phpabd@sbd.org.br||revista@sbd.org.br1806-48410365-0596opendoar:2019-10-11T00:00Anais brasileiros de dermatologia (Online) - Sociedade Brasileira de Dermatologia (SBD)false |
dc.title.none.fl_str_mv |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
title |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
spellingShingle |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview Maruta,Celina Wakisaka Autoantibodies Autoimmunity Paraneoplastic syndromes Pemphigus Skin diseases, vesiculobullous |
title_short |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
title_full |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
title_fullStr |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
title_full_unstemmed |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
title_sort |
Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
author |
Maruta,Celina Wakisaka |
author_facet |
Maruta,Celina Wakisaka Miyamoto,Denise Aoki,Valeria Carvalho,Ricardo Gomes Ribeiro de Cunha,Breno Medeiros Santi,Claudia Giuli |
author_role |
author |
author2 |
Miyamoto,Denise Aoki,Valeria Carvalho,Ricardo Gomes Ribeiro de Cunha,Breno Medeiros Santi,Claudia Giuli |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Maruta,Celina Wakisaka Miyamoto,Denise Aoki,Valeria Carvalho,Ricardo Gomes Ribeiro de Cunha,Breno Medeiros Santi,Claudia Giuli |
dc.subject.por.fl_str_mv |
Autoantibodies Autoimmunity Paraneoplastic syndromes Pemphigus Skin diseases, vesiculobullous |
topic |
Autoantibodies Autoimmunity Paraneoplastic syndromes Pemphigus Skin diseases, vesiculobullous |
description |
Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-08-01 |
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=S0365-05962019000400388 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962019000400388 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/abd1806-4841.20199165 |
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.94 n.4 2019 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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1752126423420633088 |