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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...

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Autores principales: Derbyshire, Mark Lewis, Brady, Amy Leigh, Farah, Ramsay Sami
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2023
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.
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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
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