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嗜酸性筋膜炎:现状与遗留挑战。

Eosinophilic Fasciitis: Current and Remaining Challenges.

机构信息

"Sfanta Maria" Clinical Hospital, Ion Mihalache blvd, 37-39, 011172 Bucharest, Romania.

"Carol Davila" University of Medicine, Dionisie Lupu Street, nr 37, 020021 Bucharest, Romania.

出版信息

Int J Mol Sci. 2023 Jan 19;24(3):1982. doi: 10.3390/ijms24031982.

Abstract

Eosinophilic fasciitis (EF), defined as diffuse fasciitis with eosinophilia by Shulman in 1974, is a disease with unknown etiology and whose pathogenesis is still being researched. The diagnosis is based on the clinical aspects (skin induration with an "orange peel" appearance), the lab results (eosinophilia, increased inflammatory markers), the skin biopsy with the pathognomonic histopathological result, as well as the typical MRI changes. The treatment includes glucocorticoids and immunosuppressive drugs. Due to severe refractory cases, the treatment remains a challenge. EF is still a disease with potential for further research.

摘要

嗜酸性筋膜炎(EF),由 Shulman 于 1974 年定义为弥漫性筋膜炎伴嗜酸性粒细胞增多症,是一种病因不明、发病机制仍在研究中的疾病。其诊断基于临床方面(皮肤硬化伴“橘皮样”外观)、实验室结果(嗜酸性粒细胞增多、炎症标志物增加)、具有特征性组织病理学结果的皮肤活检以及典型的 MRI 改变。治疗包括糖皮质激素和免疫抑制剂。由于严重的难治性病例,治疗仍然是一个挑战。EF 仍然是一种具有进一步研究潜力的疾病。

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