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Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia

Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron’s papules on e...

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Autores principales: Ofori, Emmanuel, Ramai, Daryl, Ona, Mel, Reddy, Madhavi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5593446/
https://www.ncbi.nlm.nih.gov/pubmed/28912913
http://dx.doi.org/10.14740/gr841w
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author Ofori, Emmanuel
Ramai, Daryl
Ona, Mel
Reddy, Madhavi
author_facet Ofori, Emmanuel
Ramai, Daryl
Ona, Mel
Reddy, Madhavi
author_sort Ofori, Emmanuel
collection PubMed
description Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron’s papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer. We report a 72-year-old female with renal clear cell carcinoma, breast cancer, and papillary serous carcinoma of the uterus, who presented with progressive weakness, 30 pound weight loss, and dysphagia over the past 3 months. She also reported difficulty in ambulating, facial rash, and inability to extend her fingers. Left bicep muscle biopsy and staining with nicotinamide adenine dinucleotide tetrazolium reductase (NADH-TR) identified frequent myofibers with targetoid-like changes consistent with DM. Despite immunosuppression with intravenous corticosteroids, she did not respond to the treatment and a percutaneous gastrostomy tube was placed for enteral feeding.
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spelling pubmed-55934462017-09-14 Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia Ofori, Emmanuel Ramai, Daryl Ona, Mel Reddy, Madhavi Gastroenterology Res Case Report Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron’s papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer. We report a 72-year-old female with renal clear cell carcinoma, breast cancer, and papillary serous carcinoma of the uterus, who presented with progressive weakness, 30 pound weight loss, and dysphagia over the past 3 months. She also reported difficulty in ambulating, facial rash, and inability to extend her fingers. Left bicep muscle biopsy and staining with nicotinamide adenine dinucleotide tetrazolium reductase (NADH-TR) identified frequent myofibers with targetoid-like changes consistent with DM. Despite immunosuppression with intravenous corticosteroids, she did not respond to the treatment and a percutaneous gastrostomy tube was placed for enteral feeding. Elmer Press 2017-08 2017-08-31 /pmc/articles/PMC5593446/ /pubmed/28912913 http://dx.doi.org/10.14740/gr841w Text en Copyright 2017, Ofori et al. http://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution Non-Commercial 4.0 International License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ofori, Emmanuel
Ramai, Daryl
Ona, Mel
Reddy, Madhavi
Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia
title Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia
title_full Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia
title_fullStr Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia
title_full_unstemmed Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia
title_short Paraneoplastic Dermatomyositis Syndrome Presenting as Dysphagia
title_sort paraneoplastic dermatomyositis syndrome presenting as dysphagia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5593446/
https://www.ncbi.nlm.nih.gov/pubmed/28912913
http://dx.doi.org/10.14740/gr841w
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