Juvenile Eosinophilic Fasciitis: A Single Center Case Series

Author:

Stubbs Leigh Anna1ORCID,Ogunbona Oluwaseun2,Beil Emily2,Szafron Vibha2,Adesina Adekunle2,Anvari Sara2,Lai Jamie2,Ramirez Andrea2,Ditzler Matthew2,De Guzman Marietta2ORCID

Affiliation:

1. Cook Children\'s Health Care System: Cook Children's Medical Center

2. Baylor College of Medicine

Abstract

AbstractBackground:Eosinophilic fasciitis (EF) is a rare disease characterized by skin induration and musculoskeletal abnormalities. Diagnostic criteria for EF are based on adult populations. There is a need to expand the literature on EF in children due to limited reported cases and potential differences compared to adults.Methods:We conducted a retrospective review of medical records for six pediatric patients diagnosed with EF at our institution between November 2011 and April 2023. Inclusion criteria required patients to be under 18 years of age at the time of diagnosis and to have confirmed diagnosis through clinical history, imaging, and histology.Results:Most of our cohort were female (83%) and non-Hispanic white (50%). Age at diagnosis ranged from 4 to 16 years. Duration of symptoms before diagnosis varied from 1 to 12 months. Follow-up periods ranged from 14 to 123 months. Concurrent medical conditions included localized scleroderma, acquired thrombophilia, and juvenile idiopathic arthritis. Patients presented with progressive painful swelling, severe joint limitations, and positive prayer sign. Initial regimens involved corticosteroids and methotrexate. Hydroxychloroquine, immunoglobulin, mycophenolate mofetil, rituximab, and tocilizumab were also used depending on the patient’s disease severity and course.Conclusions:Juvenile EF may manifest as swelling and progressive induration without apparent skin abnormalities. Unlike adult populations, no underlying malignancies or associations with trauma were observed in our cohort. Our cases did not exhibit systemic involvement observed in previous studies on juvenile EF. While non-specific, the prayer sign may aid in early recognition of juvenile EF and help prevent long-term disability.

Publisher

Research Square Platform LLC

Reference27 articles.

1. Eosinophilic fasciitis: Current concepts;Fett N;Clin Dermatol,2018

2. Li SC, Pope E. Textbook of Pediatric Rheumatology. In: Petty RE, Wedderburn LR, Laxer RM, Mellins ED, Lindsley CB, Fuhlbrigge RC, editors. Textbook of Pediatric Rheumatology. Eighth ed. Philadelpha, Pennsylvania: Elseiver; 2021.

3. In situ immunophenotype of the inflammatory infiltrate in eosinophilic fasciitis;Toquet C;J Rheumatol,2003

4. Serum levels of tissue inhibitor of metalloproteinase-1 and 2 in patients with eosinophilic fasciitis;Jinnin M;Br J Dermatol,2004

5. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review;Ferreli C;Clin Rev Allergy Immunol,2017

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3