| 解燕春，曾艳平，柳胤，梁静静，卢祖能.嗜酸性筋膜炎2例报道并文献复习[J].神经损伤功能重建,2018,13(10):515-519 |
| 嗜酸性筋膜炎2例报道并文献复习 |
| Clinical and Pathological Analysis of Eosinophilic Fasciitis: Case Report and Literature Review |
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| DOI： |
| 中文关键词: 嗜酸性筋膜炎 临床特点 病理特点 文献复习 |
| 英文关键词: eosinophilic fasciitis clinical features pathological features literature review |
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| 中文摘要: |
| 目的：探讨嗜酸性筋膜炎（EF）的临床和病理特点。方法：回顾性分析我院2例确诊为EF的患者的临
床表现、实验室检查、影像学资料及病理检查，并结合文献进行回顾性分析。结果：2例患者中女性和男性各
1例，2例均表现为对称性肢体肿胀僵硬；2例外周血嗜酸性粒细胞增多及血沉增快；1例高丙种球蛋白血症
及C反应蛋白增高；肌肉MRI提示筋膜增厚，T2WI呈高信号；受累区域全层皮肤肌肉活检可见筋膜增厚伴
炎性细胞浸润。检索既往报道的EF患者496例，最常见的表现是对称性肢体肿胀僵硬。实验室检查常显示
外周血嗜酸性粒细胞增多、高丙种球蛋白血症及血沉增快。典型的肌肉MRI表现是T1加权成像显示受累筋
膜增厚，T2加权成像呈高信号，增强扫描增厚的筋膜明显强化。典型的病理改变是筋膜增厚、纤维化并伴炎
性细胞浸润。结论：EF是一种以对称性肢体肿胀硬化、外周血嗜酸性粒细胞增多、高丙种球蛋白血症、血沉
增快等为主要特征的少见病，系统受累较少，激素治疗有效。 |
| 英文摘要: |
| To investigate the clinical and pathological features of eosinophilic fasciitis (EF).
Methods: The clinical presentation, laboratory examination, imaging characteristics, and pathological features
of two patients diagnosed with EF were retrospectively analyzed, and the literature was reviewed. Results: The
2 cases (1 female and 1 male) presented with symmetrical edema and stiffening of the limbs. Peripheral
eosinophilic granulocyte increase and increased erythrocyte sedimentation rate were present in both cases, and
hypergammaglobulinemia and increased C-reactive protein were found in 1 case. Muscle MRI revealed
thickening and high signal intensity on T2WI in deep fasciae. Full-thickness wedge biopsy of the clinically
affected skin and muscle showed thickening of the muscular fascia with accumulation of inflammatory cells. We
reviewed the reported data of 496 cases of EF. Most of the patients presented with symmetrical edema and
stiffening of the limbs. The characteristic laboratory findings in EF were peripheral eosinophilia, increased
erythrocyte sedimentation rate, and hypergammaglobulinemia. Muscle MRI characteristics of EF were
thickening on T1-weighted images, increased signal intensity on T2-weighted images, and contrast enhancement
in thickened muscle fascia following gadolinium injection. Pathological features of EF were fascial thickening
and fibrosis and inflammatory cell infiltration. Conclusion: EF is a rare disorder characterized by symmetrical
edema and stiffening of the limbs, peripheral eosinophilia, hypergammaglobulinemia, and increased erythrocyte
sedimentation rate. Systemic involvement is rare, and corticosteroid treatment is effective． |
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