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Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever

OBJECTIVE: Erysipelas-like erythema is the pathognomonic skin manifestation of familial Mediterranean fever although not frequently seen in the pediatric population. This study aims to describe the differences between patients presenting with and without erysipelas-like erythema and to examine the r...

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Autores principales: Avar-Aydın, Pınar Özge, Özçakar, Zeynep Birsin, Aydın, Fatma, Karakaş, Hatice Dilara, Çakar, Nilgün, Yalçınkaya, Fatoş
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Turkish Pediatrics Association 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9682821/
https://www.ncbi.nlm.nih.gov/pubmed/35950745
http://dx.doi.org/10.5152/TurkArchPediatr.2022.22058
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author Avar-Aydın, Pınar Özge
Özçakar, Zeynep Birsin
Aydın, Fatma
Karakaş, Hatice Dilara
Çakar, Nilgün
Yalçınkaya, Fatoş
author_facet Avar-Aydın, Pınar Özge
Özçakar, Zeynep Birsin
Aydın, Fatma
Karakaş, Hatice Dilara
Çakar, Nilgün
Yalçınkaya, Fatoş
author_sort Avar-Aydın, Pınar Özge
collection PubMed
description OBJECTIVE: Erysipelas-like erythema is the pathognomonic skin manifestation of familial Mediterranean fever although not frequently seen in the pediatric population. This study aims to describe the differences between patients presenting with and without erysipelas-like erythema and to examine the relation of erysipelas-like erythema with subclinical inflammation in a large pediatric cohort of familial Mediterranean fever patients. MATERIALS AND METHODS: This retrospective study from a single pediatric rheumatology referral center included familial Mediterranean fever patients with a follow-up for at least 6 months in the last 5 years. Patients were grouped according to the presence of erysipelas-like erythema and subclinical inflammation. RESULTS: Among 515 patients with familial Mediterranean fever, 35 patients (6.8%) were found to present with erysipelas-like erythema, and the earliest age for erysipelas-like erythema was 2.9 years. All erysipelas-like erythema lesions were defined on lower extremities with concurrent arthritis in 21 patients (60.0%). Compared to other patients in the cohort, patients presented with erysipelas-like erythema had significantly higher frequencies of acute arthritis, subclinical inflammation, and biallelic exon 10 mutations, and they used significantly higher doses of colchicine at the latest visits (all P ≤ .002). Patients with subclinical inflammation more frequently presented with erysipelas-like erythema compared to others without subclinical inflammation (21.7% vs. 2.9%, P < .001). CONCLUSION: Erysipelas-like erythema is an uncommon but important finding that can be a sign of severe disease course and subclinical inflammation in the pediatric population with familial Mediterranean fever.
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spelling pubmed-96828212022-12-02 Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever Avar-Aydın, Pınar Özge Özçakar, Zeynep Birsin Aydın, Fatma Karakaş, Hatice Dilara Çakar, Nilgün Yalçınkaya, Fatoş Turk Arch Pediatr Original Article OBJECTIVE: Erysipelas-like erythema is the pathognomonic skin manifestation of familial Mediterranean fever although not frequently seen in the pediatric population. This study aims to describe the differences between patients presenting with and without erysipelas-like erythema and to examine the relation of erysipelas-like erythema with subclinical inflammation in a large pediatric cohort of familial Mediterranean fever patients. MATERIALS AND METHODS: This retrospective study from a single pediatric rheumatology referral center included familial Mediterranean fever patients with a follow-up for at least 6 months in the last 5 years. Patients were grouped according to the presence of erysipelas-like erythema and subclinical inflammation. RESULTS: Among 515 patients with familial Mediterranean fever, 35 patients (6.8%) were found to present with erysipelas-like erythema, and the earliest age for erysipelas-like erythema was 2.9 years. All erysipelas-like erythema lesions were defined on lower extremities with concurrent arthritis in 21 patients (60.0%). Compared to other patients in the cohort, patients presented with erysipelas-like erythema had significantly higher frequencies of acute arthritis, subclinical inflammation, and biallelic exon 10 mutations, and they used significantly higher doses of colchicine at the latest visits (all P ≤ .002). Patients with subclinical inflammation more frequently presented with erysipelas-like erythema compared to others without subclinical inflammation (21.7% vs. 2.9%, P < .001). CONCLUSION: Erysipelas-like erythema is an uncommon but important finding that can be a sign of severe disease course and subclinical inflammation in the pediatric population with familial Mediterranean fever. Turkish Pediatrics Association 2022-11-01 /pmc/articles/PMC9682821/ /pubmed/35950745 http://dx.doi.org/10.5152/TurkArchPediatr.2022.22058 Text en © Copyright 2022 by The Turkish Archives of Pediatrics https://creativecommons.org/licenses/by-nc/4.0/Content of this journal is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. (https://creativecommons.org/licenses/by-nc/4.0/)
spellingShingle Original Article
Avar-Aydın, Pınar Özge
Özçakar, Zeynep Birsin
Aydın, Fatma
Karakaş, Hatice Dilara
Çakar, Nilgün
Yalçınkaya, Fatoş
Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever
title Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever
title_full Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever
title_fullStr Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever
title_full_unstemmed Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever
title_short Erysipelas-Like Erythema: A Manifestation of Severe Disease Phenotype in Pediatric Patients with Familial Mediterranean Fever
title_sort erysipelas-like erythema: a manifestation of severe disease phenotype in pediatric patients with familial mediterranean fever
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9682821/
https://www.ncbi.nlm.nih.gov/pubmed/35950745
http://dx.doi.org/10.5152/TurkArchPediatr.2022.22058
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