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嗜酸性筋膜炎:病例系列

Eosinophilic Fasciitis: A Case Series.

作者信息

Macedo Brás Elisa, Sousa António-Pedro, Ferreira da Silva Nuno, Salvador Fernando

机构信息

Internal Medicine, Centro Hospitalar de Trás-os-Montes e Alto Douro, Vila Real, PRT.

出版信息

Cureus. 2025 Jan 18;17(1):e77615. doi: 10.7759/cureus.77615. eCollection 2025 Jan.

Abstract

Eosinophilic fasciitis (EF) is a rare and frequently underdiagnosed condition, with limited representation in the scientific literature. Early and accurate diagnosis is critical to preventing the progression of the disease and the development of irreversible damage. EF is characterized by thickening and fibrosis of the subcutaneous fascia and skin, typically affecting the extremities, trunk, and neck, with peripheral eosinophilia occasionally observed. While most cases are considered idiopathic, a thorough evaluation is essential to rule out differential diagnoses or associated underlying conditions. The disease significantly impacts patients' daily activities and is associated with substantial morbidity. Management of EF primarily involves the use of corticosteroids and other immunosuppressive therapies. The majority of patients demonstrate favorable responses to treatment, particularly when intervention occurs before the onset of advanced fibrosis. In this paper, we present a case series involving four patients diagnosed and treated at our institution. We provide a detailed analysis of the diagnostic process, therapeutic approaches, and follow-up strategies employed, contributing to the broader understanding of this rare disease.

摘要

嗜酸性筋膜炎(EF)是一种罕见且常被漏诊的疾病,在科学文献中的描述有限。早期准确诊断对于防止疾病进展和不可逆损伤的发生至关重要。嗜酸性筋膜炎的特征是皮下筋膜和皮肤增厚及纤维化,通常累及四肢、躯干和颈部,偶尔可见外周嗜酸性粒细胞增多。虽然大多数病例被认为是特发性的,但进行全面评估以排除鉴别诊断或相关潜在疾病至关重要。该疾病对患者的日常活动有显著影响,并伴有大量发病情况。嗜酸性筋膜炎的治疗主要包括使用皮质类固醇和其他免疫抑制疗法。大多数患者对治疗反应良好,尤其是在晚期纤维化出现之前进行干预时。在本文中,我们介绍了一组在我们机构诊断和治疗的4例患者。我们对所采用的诊断过程、治疗方法和随访策略进行了详细分析,有助于更广泛地了解这种罕见疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0eef/11831705/94ddf5a9fbc3/cureus-0017-00000077615-i01.jpg

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