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嗜酸性筋膜炎

Eosinophilic fasciitis.

作者信息

Pinal-Fernández Iago, Milisenda José César, Selva-O'Callaghan Albert

机构信息

Muscle Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Montgomery, EE. UU.; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, EE. UU..

Muscle Research Unit, Internal Medicine Service, Hospital Clinic. Barcelona University; CIBERER and IDIBAPS, Barcelona, España.

出版信息

Med Clin (Barc). 2025 Jun 20;165(4):107066. doi: 10.1016/j.medcli.2025.107066.

Abstract

Eosinophilic fasciitis is a rare scleroderma-like syndrome of unknown cause. It is characterized by painful induration and progressive thickening of the muscular fascia and subcutaneous tissue of the limbs and trunk. The most common laboratory findings include peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate. The diagnosis is confirmed through a full-thickness wedge biopsy of the affected skin, revealing inflammation and thickening of the deep fascia. The differential diagnosis includes scleroderma, morphea, myofasciitis in graft-versus-host disease, and epidemic fasciitis syndromes caused by toxins, such as eosinophilia-myalgia syndrome and toxic oil syndrome. Although the diagnosis is based on clinical, laboratory, and histological findings, no universal diagnostic criteria exist. Glucocorticoids are the standard treatment, although some patients may improve spontaneously.

摘要

嗜酸性筋膜炎是一种病因不明的罕见的硬皮病样综合征。其特征为四肢和躯干的肌筋膜及皮下组织出现疼痛性硬结和进行性增厚。最常见的实验室检查结果包括外周血嗜酸性粒细胞增多、高球蛋白血症和红细胞沉降率升高。通过对受累皮肤进行全层楔形活检确诊,显示深筋膜炎症和增厚。鉴别诊断包括硬皮病、局限性硬皮病、移植物抗宿主病中的肌筋膜炎以及由毒素引起的流行性筋膜炎综合征,如嗜酸性粒细胞增多性肌痛综合征和中毒性油综合征。尽管诊断基于临床、实验室和组织学检查结果,但尚无通用的诊断标准。糖皮质激素是标准治疗方法,不过一些患者可能会自发改善。

本文引用的文献

1
Treatment and Monitoring of Eosinophilic Fasciitis.嗜酸性筋膜炎的治疗与监测
Curr Treatm Opt Rheumatol. 2025;11. doi: 10.1007/s40674-024-00222-6. Epub 2025 Jan 23.
2
Pathological autoantibody internalisation in myositis.肌炎中的病理性自身抗体内化。
Ann Rheum Dis. 2024 Oct 21;83(11):1549-1560. doi: 10.1136/ard-2024-225773.
3
Is there a Role for Anti-IL-5 Therapies in Eosinophilic Fasciitis?抗白细胞介素-5疗法在嗜酸性筋膜炎中是否有作用?
Mediterr J Rheumatol. 2023 Dec 30;34(4):414-417. doi: 10.31138/mjr.301223.itr. eCollection 2023 Dec.
9
Intravenous immunoglobulin for refractory eosinophilic fasciitis: A retrospective analysis from 3 tertiary care centers.
J Am Acad Dermatol. 2023 Jun;88(6):1354-1356. doi: 10.1016/j.jaad.2019.12.022. Epub 2019 Dec 14.

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