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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...

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Autores principales: Llamas-Molina, José María, Velasco-Amador, Juan Pablo, De la Torre-Gomar, Francisco Javier, Carrero-Castaño, Alejandro, Ruiz-Villaverde, Ricardo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
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.
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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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