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嗜酸性筋膜炎(舒尔曼病)的临床、影像与病理相关性。

Eosinophilic fasciitis (Shulman disease) with clinical, imaging and pathological correlation.

机构信息

Radiology, Hospital Beatriz Ângelo, Loures, Portugal

Reumatology, Instituto Português de Reumatologia, Lisboa, Lisboa, Portugal.

出版信息

BMJ Case Rep. 2021 Dec 30;14(12):e246151. doi: 10.1136/bcr-2021-246151.

Abstract

Eosinophilic fasciitis (EF) is a rare subacute fibrosing disorder of unknown aetiology, characterised by thickening of the muscular fascia and subcutaneous tissue, leading to swelling of limbs and trunk and sparing fingers and toes. Eosinophilic infiltration and degranulation may prompt tissue damage and consequent fibrosis due to the accumulation of collagen and extracellular matrix proteins. MRI is the best imaging modality for diagnosis, depicting fascial thickening and enhancement. MRI may also have a significant role in excluding alternative diagnosis and guiding the skin-muscle biopsy.We report a case of EF with clinical and pathological correlation, highlighting the diagnostic value of MRI for early diagnosis and further treatment.

摘要

嗜酸性筋膜炎(EF)是一种罕见的亚急性纤维性疾病,病因不明,其特征为肌肉筋膜和皮下组织增厚,导致四肢和躯干肿胀,手指和脚趾不受累。嗜酸性粒细胞浸润和脱颗粒可能会导致组织损伤和随后的纤维化,这是由于胶原蛋白和细胞外基质蛋白的积累。磁共振成像(MRI)是诊断该病的最佳影像学方式,可显示筋膜增厚和增强。MRI 还可能在排除其他诊断和指导皮肤-肌肉活检方面具有重要作用。我们报告了一例 EF 的病例,其临床表现和病理改变具有相关性,突出了 MRI 在早期诊断和进一步治疗中的诊断价值。

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