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An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis

Juvenile dermatomyositis (JDM) is a rare condition worldwide, affecting children younger than 16 years. It is characterized by weakness in the proximal skeletal muscles and a pathognomonic skin rash. Patients with JDM develop complications that are usually a consequence of vasculopathy affecting mul...

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Autores principales: Asante, Adelaide Ankomaa, Nsaful, Josephine, Dey, Dzifa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9381251/
https://www.ncbi.nlm.nih.gov/pubmed/35982709
http://dx.doi.org/10.1155/2022/8758263
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author Asante, Adelaide Ankomaa
Nsaful, Josephine
Dey, Dzifa
author_facet Asante, Adelaide Ankomaa
Nsaful, Josephine
Dey, Dzifa
author_sort Asante, Adelaide Ankomaa
collection PubMed
description Juvenile dermatomyositis (JDM) is a rare condition worldwide, affecting children younger than 16 years. It is characterized by weakness in the proximal skeletal muscles and a pathognomonic skin rash. Patients with JDM develop complications that are usually a consequence of vasculopathy affecting multiple organ systems. Occult gastrointestinal (GI) perforation is an uncommon complication and is associated with an increased risk of mortality due to a delay in diagnosis. We report on a 14-year-old male with JDM with an aggressive course over two years and severe clinical manifestations. The patient developed necrotizing fasciitis, an unusual rapidly progressing lethal infection of the fascia resulting from bowel contents seeping from multiple intestinal perforations. This case, less commonly seen in males, highlights the occurrence of multiple phenomena—JDM complicated by skin and gastrointestinal vasculopathy with resultant development of multiple GI perforations and consequently life-threatening necrotizing fasciitis of the leg. Physicians need a high index of suspecting GI perforation in JDM patients as the delayed recognition of this complication can result in significant morbidity and/or mortality since the typical symptoms of perforation may be absent.
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spelling pubmed-93812512022-08-17 An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis Asante, Adelaide Ankomaa Nsaful, Josephine Dey, Dzifa Case Rep Rheumatol Case Report Juvenile dermatomyositis (JDM) is a rare condition worldwide, affecting children younger than 16 years. It is characterized by weakness in the proximal skeletal muscles and a pathognomonic skin rash. Patients with JDM develop complications that are usually a consequence of vasculopathy affecting multiple organ systems. Occult gastrointestinal (GI) perforation is an uncommon complication and is associated with an increased risk of mortality due to a delay in diagnosis. We report on a 14-year-old male with JDM with an aggressive course over two years and severe clinical manifestations. The patient developed necrotizing fasciitis, an unusual rapidly progressing lethal infection of the fascia resulting from bowel contents seeping from multiple intestinal perforations. This case, less commonly seen in males, highlights the occurrence of multiple phenomena—JDM complicated by skin and gastrointestinal vasculopathy with resultant development of multiple GI perforations and consequently life-threatening necrotizing fasciitis of the leg. Physicians need a high index of suspecting GI perforation in JDM patients as the delayed recognition of this complication can result in significant morbidity and/or mortality since the typical symptoms of perforation may be absent. Hindawi 2022-08-09 /pmc/articles/PMC9381251/ /pubmed/35982709 http://dx.doi.org/10.1155/2022/8758263 Text en Copyright © 2022 Adelaide Ankomaa Asante et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Asante, Adelaide Ankomaa
Nsaful, Josephine
Dey, Dzifa
An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
title An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
title_full An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
title_fullStr An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
title_full_unstemmed An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
title_short An Unusual Cause of Necrotising Fasciitis in a Young Male with Juvenile Dermatomyositis
title_sort unusual cause of necrotising fasciitis in a young male with juvenile dermatomyositis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9381251/
https://www.ncbi.nlm.nih.gov/pubmed/35982709
http://dx.doi.org/10.1155/2022/8758263
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