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Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient

Clinically amyopathic dermatomyositis (CADM) is a rare form of DM characterized by unique cutaneous and pulmonary features with no muscle involvement. A subset of patients with CADM has a specific antibody known as anti-melanoma differentiation-associated protein 5 (MDA5). The systemic associations...

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Autores principales: Alqahtani, Nouf, Aleissa, Majed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8543314/
https://www.ncbi.nlm.nih.gov/pubmed/34720922
http://dx.doi.org/10.1159/000519104
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author Alqahtani, Nouf
Aleissa, Majed
author_facet Alqahtani, Nouf
Aleissa, Majed
author_sort Alqahtani, Nouf
collection PubMed
description Clinically amyopathic dermatomyositis (CADM) is a rare form of DM characterized by unique cutaneous and pulmonary features with no muscle involvement. A subset of patients with CADM has a specific antibody known as anti-melanoma differentiation-associated protein 5 (MDA5). The systemic associations of anti-MDA-5 CADM warrant an early recognition and management to prevent fetal sequelae. It is seen more commonly in white and Asian female individuals. The clinical features of anti-MDA5 antibody-positive CADM in other ethnic groups are not well reported. Here, we describe a case of CADM with identified autoantibodies against MDA5 in a Sudanese female patient presenting with characteristic cutaneous features in association with MDA5 autoantibodies: ulcerated Gottron's papules, painful palmar papules, shawl sign, and heliotrope sign. No evidence of pulmonary or systemic involvement was identified. Treatment with prednisolone and mycophenolate mofetil was initiated. This case emphasizes the importance of keeping a high level of suspicion and to recognize the unique clinical feature of this type of DM aiding in early treatment and preventing fatal outcomes.
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spelling pubmed-85433142021-10-29 Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient Alqahtani, Nouf Aleissa, Majed Case Rep Dermatol Single Case Clinically amyopathic dermatomyositis (CADM) is a rare form of DM characterized by unique cutaneous and pulmonary features with no muscle involvement. A subset of patients with CADM has a specific antibody known as anti-melanoma differentiation-associated protein 5 (MDA5). The systemic associations of anti-MDA-5 CADM warrant an early recognition and management to prevent fetal sequelae. It is seen more commonly in white and Asian female individuals. The clinical features of anti-MDA5 antibody-positive CADM in other ethnic groups are not well reported. Here, we describe a case of CADM with identified autoantibodies against MDA5 in a Sudanese female patient presenting with characteristic cutaneous features in association with MDA5 autoantibodies: ulcerated Gottron's papules, painful palmar papules, shawl sign, and heliotrope sign. No evidence of pulmonary or systemic involvement was identified. Treatment with prednisolone and mycophenolate mofetil was initiated. This case emphasizes the importance of keeping a high level of suspicion and to recognize the unique clinical feature of this type of DM aiding in early treatment and preventing fatal outcomes. S. Karger AG 2021-09-28 /pmc/articles/PMC8543314/ /pubmed/34720922 http://dx.doi.org/10.1159/000519104 Text en Copyright © 2021 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
Alqahtani, Nouf
Aleissa, Majed
Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient
title Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient
title_full Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient
title_fullStr Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient
title_full_unstemmed Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient
title_short Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient
title_sort cutaneous features of anti-mda-5 antibody-positive amyopathic dermatomyositis in a sudanese patient
topic Single Case
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8543314/
https://www.ncbi.nlm.nih.gov/pubmed/34720922
http://dx.doi.org/10.1159/000519104
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