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Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature

Morphea profunda or subcutaneous (deep) morphea is a variant of localized morphea, characterized by one or more ill-defined, deep sclerotic plaque. Preferential sites are the abdomen, trunk, sacral area, or extremities. The presence of hyperplastic lymphoid follicles in the context of the sclerotic...

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Autores principales: Cassisa, Angelo, Vannucchi, Margherita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8788542/
https://www.ncbi.nlm.nih.gov/pubmed/35076471
http://dx.doi.org/10.3390/dermatopathology9010003
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author Cassisa, Angelo
Vannucchi, Margherita
author_facet Cassisa, Angelo
Vannucchi, Margherita
author_sort Cassisa, Angelo
collection PubMed
description Morphea profunda or subcutaneous (deep) morphea is a variant of localized morphea, characterized by one or more ill-defined, deep sclerotic plaque. Preferential sites are the abdomen, trunk, sacral area, or extremities. The presence of hyperplastic lymphoid follicles in the context of the sclerotic bands of morphea is rarely described. Localized scleroderma is sustained by a profibrotic inflammatory profile. Transforming growth factor-β (TGF-β), an imbalance between functional subclasses of T-lymphocytes (innate immune cells) has a role in activate collagen deposition. In this case report, we present two cases of morphea profunda with lymphoid follicular hyperplasia. A systematic review of the literature on the pathophysiology of localized scleroderma is also presented, with particular reference to the presence of lymphoid structures.
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spelling pubmed-87885422022-01-26 Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature Cassisa, Angelo Vannucchi, Margherita Dermatopathology (Basel) Case Report Morphea profunda or subcutaneous (deep) morphea is a variant of localized morphea, characterized by one or more ill-defined, deep sclerotic plaque. Preferential sites are the abdomen, trunk, sacral area, or extremities. The presence of hyperplastic lymphoid follicles in the context of the sclerotic bands of morphea is rarely described. Localized scleroderma is sustained by a profibrotic inflammatory profile. Transforming growth factor-β (TGF-β), an imbalance between functional subclasses of T-lymphocytes (innate immune cells) has a role in activate collagen deposition. In this case report, we present two cases of morphea profunda with lymphoid follicular hyperplasia. A systematic review of the literature on the pathophysiology of localized scleroderma is also presented, with particular reference to the presence of lymphoid structures. MDPI 2022-01-10 /pmc/articles/PMC8788542/ /pubmed/35076471 http://dx.doi.org/10.3390/dermatopathology9010003 Text en © 2022 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 Case Report
Cassisa, Angelo
Vannucchi, Margherita
Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
title Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
title_full Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
title_fullStr Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
title_full_unstemmed Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
title_short Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
title_sort morphea profunda with tertiary lymphoid follicles: description of two cases and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8788542/
https://www.ncbi.nlm.nih.gov/pubmed/35076471
http://dx.doi.org/10.3390/dermatopathology9010003
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