Eosinophilic fasciitis leading to painless contractures

Eosinophilic fasciitis leading to painless contractures
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DOI:
10.1007/s00431-002-1038-1
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发表时间:
2002-10-01
影响因子:
3.6
通讯作者:
Hanefeld, F
Hanefeld, F
中科院分区:
医学3区
文献类型:
--
作者:
Huppke, P;Wilken, B;Hanefeld, F

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我们报告了一个11岁的女孩,她在3个月的时间里发生了多处关节痉挛。该病以手指、肘部、肩部、膝部和足部进行性受累为主要表现,不伴有其他症状。实验室检查显示嗜酸性粒细胞增多和高丙种球蛋白血症。右前臂肌肉超声和磁共振成像显示筋膜增厚,全层活检证实诊断为嗜酸性筋膜炎。在用脉冲类固醇治疗后,痉挛消失了。结论:我们的病例表明,嗜酸性筋膜炎可以在没有皮肤受累和关节炎的情况下出现,因此必须被视为儿童肌挛缩的鉴别诊断。脉冲类固醇治疗有效,无副作用。
We report on an 11-year-old girl who developed multiple joint contractures over a period of 3 months. The disease presented with progressive involvement of the fingers, elbows, shoulders, knees and feet and was not accompanied by other symptoms. Laboratory investigations showed eosinophilia and hypergammaglobulinaemia. Muscle ultrasound and magnetic resonance imaging of the right forearm revealed thickened fascia and a full thickness biopsy confirmed the diagnosis of eosinophilic fasciitis. Following treatment with pulsed steroids, the contractures resolved. Conclusion: our case shows that eosinophilic fasciitis can present without skin involvement and arthritis and therefore has to be regarded as a differential diagnosis of contractures in childhood. Pulsed steroid treatment was effective and without side-effects.