Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview
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 neopla...
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Dermatologia
2019-10-01
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| Series: | Anais Brasileiros de Dermatologia |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962019000400388&tlng=en |
| _version_ | 1818719422206967808 |
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| author | Celina Wakisaka Maruta Denise Miyamoto Valeria Aoki Ricardo Gomes Ribeiro de Carvalho Breno Medeiros Cunha Claudia Giuli Santi |
| author_facet | Celina Wakisaka Maruta Denise Miyamoto Valeria Aoki Ricardo Gomes Ribeiro de Carvalho Breno Medeiros Cunha Claudia Giuli Santi |
| author_sort | Celina Wakisaka Maruta |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-17T20:06:41Z |
| format | Article |
| id | doaj.art-a1c854ca49324310b6a06936fb30d8f6 |
| institution | Directory Open Access Journal |
| issn | 0365-0596 |
| language | English |
| last_indexed | 2024-12-17T20:06:41Z |
| publishDate | 2019-10-01 |
| publisher | Sociedade Brasileira de Dermatologia |
| record_format | Article |
| series | Anais Brasileiros de Dermatologia |
| spelling | doaj.art-a1c854ca49324310b6a06936fb30d8f62022-12-21T21:34:19ZengSociedade Brasileira de DermatologiaAnais Brasileiros de Dermatologia0365-05962019-10-0194438839810.1590/abd1806-4841.20199165Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overviewCelina Wakisaka Marutahttps://orcid.org/0000-0002-0541-5526Denise Miyamotohttps://orcid.org/0000-0002-4133-4475Valeria Aokihttps://orcid.org/0000-0003-4256-4413Ricardo Gomes Ribeiro de Carvalhohttps://orcid.org/0000-0002-2491-4558Breno Medeiros Cunhahttps://orcid.org/0000-0003-2177-0496Claudia Giuli Santihttps://orcid.org/0000-0003-3650-4254Abstract: 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962019000400388&tlng=enAutoantibodiesAutoimmunityParaneoplastic syndromesPemphigusSkin diseases, vesiculobullous |
| spellingShingle | Celina Wakisaka Maruta Denise Miyamoto Valeria Aoki Ricardo Gomes Ribeiro de Carvalho Breno Medeiros Cunha Claudia Giuli Santi Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview Anais Brasileiros de Dermatologia Autoantibodies Autoimmunity Paraneoplastic syndromes Pemphigus Skin diseases, vesiculobullous |
| title | 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_short | Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview |
| title_sort | paraneoplastic pemphigus a clinical laboratorial and therapeutic overview |
| topic | Autoantibodies Autoimmunity Paraneoplastic syndromes Pemphigus Skin diseases, vesiculobullous |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962019000400388&tlng=en |
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