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Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome
Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition attributed to plasma cell proliferation and the deposition of immunoglobulin light chains in the skin without association with systemic amyloidosis or hematological dyscrasias. It is not uncommon for patients diagnosed with...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10139233/ https://www.ncbi.nlm.nih.gov/pubmed/37108553 http://dx.doi.org/10.3390/ijms24087378 |
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author | Llamas-Molina, José María Velasco-Amador, Juan Pablo De la Torre-Gomar, Francisco Javier Carrero-Castaño, Alejandro Ruiz-Villaverde, Ricardo |
author_facet | Llamas-Molina, José María Velasco-Amador, Juan Pablo De la Torre-Gomar, Francisco Javier Carrero-Castaño, Alejandro Ruiz-Villaverde, Ricardo |
author_sort | Llamas-Molina, José María |
collection | PubMed |
description | Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition attributed to plasma cell proliferation and the deposition of immunoglobulin light chains in the skin without association with systemic amyloidosis or hematological dyscrasias. It is not uncommon for patients diagnosed with PLCNA to also suffer from other auto-immune connective tissue diseases, with Sjögren’s syndrome (SjS) showing the strongest association. This article provides a literature review and descriptive analysis to better understand the unique relationship between these two entities. To date, 34 patients with PLCNA and SjS have been reported in a total of 26 articles. The co-existence of PLCNA and SjS has been reported, especially in female patients in their seventh decade of life with nodular lesions on the trunk and/or lower extremities. Acral and facial localization, which is a typical localization of PLCNA in the absence of SjS, seems to be much more unusual in patients with associated SjS. |
format | Online Article Text |
id | pubmed-10139233 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-101392332023-04-28 Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome Llamas-Molina, José María Velasco-Amador, Juan Pablo De la Torre-Gomar, Francisco Javier Carrero-Castaño, Alejandro Ruiz-Villaverde, Ricardo Int J Mol Sci Review Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition attributed to plasma cell proliferation and the deposition of immunoglobulin light chains in the skin without association with systemic amyloidosis or hematological dyscrasias. It is not uncommon for patients diagnosed with PLCNA to also suffer from other auto-immune connective tissue diseases, with Sjögren’s syndrome (SjS) showing the strongest association. This article provides a literature review and descriptive analysis to better understand the unique relationship between these two entities. To date, 34 patients with PLCNA and SjS have been reported in a total of 26 articles. The co-existence of PLCNA and SjS has been reported, especially in female patients in their seventh decade of life with nodular lesions on the trunk and/or lower extremities. Acral and facial localization, which is a typical localization of PLCNA in the absence of SjS, seems to be much more unusual in patients with associated SjS. MDPI 2023-04-17 /pmc/articles/PMC10139233/ /pubmed/37108553 http://dx.doi.org/10.3390/ijms24087378 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Llamas-Molina, José María Velasco-Amador, Juan Pablo De la Torre-Gomar, Francisco Javier Carrero-Castaño, Alejandro Ruiz-Villaverde, Ricardo Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome |
title | Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome |
title_full | Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome |
title_fullStr | Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome |
title_full_unstemmed | Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome |
title_short | Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome |
title_sort | localized cutaneous nodular amyloidosis: a specific cutaneous manifestation of sjögren’s syndrome |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10139233/ https://www.ncbi.nlm.nih.gov/pubmed/37108553 http://dx.doi.org/10.3390/ijms24087378 |
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