Cargando…

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...

Descripción completa

Detalles Bibliográficos
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
Descripción
Sumario: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.