| 何贤松,俞欣,周迪,高祥福.嗜酸性筋膜炎[J].浙江中西医结合杂志,2016,26(12): |
| 嗜酸性筋膜炎 |
| Eosinophilic fasciitis |
| 投稿时间：2016-04-15 修订日期：2016-04-20 |
| DOI： |
| 中文关键词: 嗜酸性筋膜炎 嗜酸性粒细胞增多症 激素 |
| 英文关键词:Eosinophilic fasciitis Eosinophilia Corticosteroids |
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| 中文摘要: |
| 嗜酸性筋膜炎是一种不明病因和发病机制的、具有硬皮病样症状的、罕见的结缔组织病,实验检查可见外周血嗜酸性粒细胞增多、高丙种球蛋白血症、血沉加快等。全层皮肤的活检显示深筋膜增厚和筋膜炎,是诊断嗜酸性筋膜炎的基础,核磁共振成像显示受累筋膜的高信号,有助于诊断和评估疗效。需要与嗜酸性粒细胞-肌痛综合征、嗜酸性细胞增多综合征、硬皮病、多发性肌炎等疾病鉴别。口服糖皮质激素仍是主要的治疗手段,必要时可结合免疫抑制剂,特异性单抗等治疗方法。 |
| 英文摘要: |
| Eosinophilic fasciitis (EF) is a rare scleroderma-like syndrome with an unknown etiology and pathogenesis.Peripheral blood eosinophilia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate are the main laboratory findings. Full-thickness wedge biopsy of the clinically affected skin showing in?ammation and thickening of deep fascia is essential to establish the diagnosis. Magnetic resonance imaging may evidence an increased signal intensity within the fascia.Differential diagnosis should be ruled out, including eosinophilia myalgia syndrome (EMS) after L-tryprophane ingestion, hypereosinophilic syndromes (HES), systemic sclerosis and Polymyositis. Oral corticosteroids remain the main-stay treatment and may be associated to an immunosuppressive drug and monoclonal antibody. |
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