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Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome
Dermatomyofibromas are a rare, benign, acquired neoplasm with less than 150 reported cases worldwide. The etiologic factors that contribute to the development of these lesions are currently unknown. To our knowledge, there have been only six previously reported cases of patients presenting with mult...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10294281/ https://www.ncbi.nlm.nih.gov/pubmed/37383324 http://dx.doi.org/10.1159/000530423 |
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author | Derbyshire, Mark Lewis Brady, Amy Leigh Farah, Ramsay Sami |
author_facet | Derbyshire, Mark Lewis Brady, Amy Leigh Farah, Ramsay Sami |
author_sort | Derbyshire, Mark Lewis |
collection | PubMed |
description | Dermatomyofibromas are a rare, benign, acquired neoplasm with less than 150 reported cases worldwide. The etiologic factors that contribute to the development of these lesions are currently unknown. To our knowledge, there have been only six previously reported cases of patients presenting with multiple dermatomyofibromas, and in each of these cases, there were less than ten lesions present. Herein, we describe a patient who developed more than 100 dermatomyofibromas over a period of years, and we argue that the patient’s concurrent Ehlers-Danlos syndrome could have contributed to this unique presentation by inducing an increased fibroblast-to-myofibroblast transition. |
format | Online Article Text |
id | pubmed-10294281 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-102942812023-06-28 Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome Derbyshire, Mark Lewis Brady, Amy Leigh Farah, Ramsay Sami Case Rep Dermatol Single Case Dermatomyofibromas are a rare, benign, acquired neoplasm with less than 150 reported cases worldwide. The etiologic factors that contribute to the development of these lesions are currently unknown. To our knowledge, there have been only six previously reported cases of patients presenting with multiple dermatomyofibromas, and in each of these cases, there were less than ten lesions present. Herein, we describe a patient who developed more than 100 dermatomyofibromas over a period of years, and we argue that the patient’s concurrent Ehlers-Danlos syndrome could have contributed to this unique presentation by inducing an increased fibroblast-to-myofibroblast transition. S. Karger AG 2023-06-26 /pmc/articles/PMC10294281/ /pubmed/37383324 http://dx.doi.org/10.1159/000530423 Text en © 2023 The Author(s). Published by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Single Case Derbyshire, Mark Lewis Brady, Amy Leigh Farah, Ramsay Sami Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome |
title | Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome |
title_full | Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome |
title_fullStr | Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome |
title_full_unstemmed | Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome |
title_short | Multiple Dermatomyofibromas in a Patient with Ehlers-Danlos Syndrome |
title_sort | multiple dermatomyofibromas in a patient with ehlers-danlos syndrome |
topic | Single Case |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10294281/ https://www.ncbi.nlm.nih.gov/pubmed/37383324 http://dx.doi.org/10.1159/000530423 |
work_keys_str_mv | AT derbyshiremarklewis multipledermatomyofibromasinapatientwithehlersdanlossyndrome AT bradyamyleigh multipledermatomyofibromasinapatientwithehlersdanlossyndrome AT farahramsaysami multipledermatomyofibromasinapatientwithehlersdanlossyndrome |