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Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman

Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM – anti-melanoma differentiation-associated gene...

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Autores principales: Agulló, Alfredo, Hinds, Brian, Larrea, Mónica, Yanguas, Ignacio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5803943/
https://www.ncbi.nlm.nih.gov/pubmed/29441299
http://dx.doi.org/10.4103/idoj.IDOJ_72_17
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author Agulló, Alfredo
Hinds, Brian
Larrea, Mónica
Yanguas, Ignacio
author_facet Agulló, Alfredo
Hinds, Brian
Larrea, Mónica
Yanguas, Ignacio
author_sort Agulló, Alfredo
collection PubMed
description Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM – anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash). The patients did not develop interstitial lung disease or internal malignancies and experienced a rapid response to prednisolone and intravenous immunoglobulins. After 2 years, she has no relapse of her cutaneous disease and continues 5 mg prednisolone and 2 g/kg kilogram of intravenous immunoglobulin every 3 months for maintenance. Our case highlights the clinical heterogeneity of CADM and underscores the importance of a comprehensive approach to DM patients. It was previously postulated that anti-MDA5 antibody could target vascular cells and compromise vascular function, the presence of livedo racemosa lesions, and MDA5 antibodies in a patient with negative thrombophilia workup, reinforce this idea. This is the first case, to our knowledge, of CADM with acral panniculitis and livedo racemosa.
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spelling pubmed-58039432018-02-13 Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman Agulló, Alfredo Hinds, Brian Larrea, Mónica Yanguas, Ignacio Indian Dermatol Online J Case Report Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM – anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash). The patients did not develop interstitial lung disease or internal malignancies and experienced a rapid response to prednisolone and intravenous immunoglobulins. After 2 years, she has no relapse of her cutaneous disease and continues 5 mg prednisolone and 2 g/kg kilogram of intravenous immunoglobulin every 3 months for maintenance. Our case highlights the clinical heterogeneity of CADM and underscores the importance of a comprehensive approach to DM patients. It was previously postulated that anti-MDA5 antibody could target vascular cells and compromise vascular function, the presence of livedo racemosa lesions, and MDA5 antibodies in a patient with negative thrombophilia workup, reinforce this idea. This is the first case, to our knowledge, of CADM with acral panniculitis and livedo racemosa. Medknow Publications & Media Pvt Ltd 2018 /pmc/articles/PMC5803943/ /pubmed/29441299 http://dx.doi.org/10.4103/idoj.IDOJ_72_17 Text en Copyright: © 2018 Indian Dermatology Online Journal http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Agulló, Alfredo
Hinds, Brian
Larrea, Mónica
Yanguas, Ignacio
Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman
title Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman
title_full Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman
title_fullStr Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman
title_full_unstemmed Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman
title_short Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman
title_sort livedo racemosa, reticulated ulcerations, panniculitis and violaceous plaques in a 46-year-old woman
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5803943/
https://www.ncbi.nlm.nih.gov/pubmed/29441299
http://dx.doi.org/10.4103/idoj.IDOJ_72_17
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