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Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey

Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and nondermatologic involvements like neurological and renal disorders. We report a case series of the data regarding the characteristi...

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Autores principales: Ansari, Abtin, Erfani, Zohre, Daneshpazhooh, Maryam, Mahmoudi, Leila, Saffarian, Zahra, Kamyab, Kambiz, Mahmoudi, Hamidreza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9294944/
https://www.ncbi.nlm.nih.gov/pubmed/35950147
http://dx.doi.org/10.1159/000525211
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author Ansari, Abtin
Erfani, Zohre
Daneshpazhooh, Maryam
Mahmoudi, Leila
Saffarian, Zahra
Kamyab, Kambiz
Mahmoudi, Hamidreza
author_facet Ansari, Abtin
Erfani, Zohre
Daneshpazhooh, Maryam
Mahmoudi, Leila
Saffarian, Zahra
Kamyab, Kambiz
Mahmoudi, Hamidreza
author_sort Ansari, Abtin
collection PubMed
description Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and nondermatologic involvements like neurological and renal disorders. We report a case series of the data regarding the characteristics and treatment of 14 patients diagnosed with scleromyxedema and their follow-up. Patients entered the study based on scleromyxedema diagnosis criteria. Comorbidities were also recorded to evaluate their effect on the treatment process. Clinicopathological and laboratory findings and responses to their treatment were evaluated separately. There was a significant improvement after administering intravenous immunoglobulin (IVIG). Despite the lack of a definite treatment for this condition, the present study shows that the application of IVIG can improve both cutaneous and systemic symptoms. Younger patients, in particular, responded significantly to the use of IVIG. More studies are required to investigate the potential efficacy of IVIG in the treatment of scleromyxedema.
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spelling pubmed-92949442022-08-09 Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey Ansari, Abtin Erfani, Zohre Daneshpazhooh, Maryam Mahmoudi, Leila Saffarian, Zahra Kamyab, Kambiz Mahmoudi, Hamidreza Case Rep Dermatol Case Series Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and nondermatologic involvements like neurological and renal disorders. We report a case series of the data regarding the characteristics and treatment of 14 patients diagnosed with scleromyxedema and their follow-up. Patients entered the study based on scleromyxedema diagnosis criteria. Comorbidities were also recorded to evaluate their effect on the treatment process. Clinicopathological and laboratory findings and responses to their treatment were evaluated separately. There was a significant improvement after administering intravenous immunoglobulin (IVIG). Despite the lack of a definite treatment for this condition, the present study shows that the application of IVIG can improve both cutaneous and systemic symptoms. Younger patients, in particular, responded significantly to the use of IVIG. More studies are required to investigate the potential efficacy of IVIG in the treatment of scleromyxedema. S. Karger AG 2022-06-27 /pmc/articles/PMC9294944/ /pubmed/35950147 http://dx.doi.org/10.1159/000525211 Text en Copyright © 2022 by The Author(s). Published 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 Case Series
Ansari, Abtin
Erfani, Zohre
Daneshpazhooh, Maryam
Mahmoudi, Leila
Saffarian, Zahra
Kamyab, Kambiz
Mahmoudi, Hamidreza
Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey
title Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey
title_full Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey
title_fullStr Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey
title_full_unstemmed Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey
title_short Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey
title_sort clinical characteristics and treatment outcomes of scleromyxedema: a 10-year retrospective survey
topic Case Series
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9294944/
https://www.ncbi.nlm.nih.gov/pubmed/35950147
http://dx.doi.org/10.1159/000525211
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