Huppke Peter, Wilken Bernd, Brockmann Knut, Sattler Bernd, Hanefeld Folker
Abteilung Kinderheilkunde, Schwerpunkt Neuropädiatrie, Georg-August-Universität Göttingen, Robert Koch Strasse 40, 37075 Göttingen, Germany.
Eur J Pediatr. 2002 Oct;161(10):528-30. doi: 10.1007/s00431-002-1038-1. Epub 2002 Aug 21.
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.
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.
我们报告了一名11岁女孩,她在3个月内出现了多处关节挛缩。该病表现为手指、肘部、肩部、膝盖和足部的进行性受累,且无其他伴随症状。实验室检查显示嗜酸性粒细胞增多和高球蛋白血症。右前臂的肌肉超声和磁共振成像显示筋膜增厚,全层活检确诊为嗜酸性筋膜炎。经脉冲类固醇治疗后,挛缩症状得以缓解。
我们的病例表明,嗜酸性筋膜炎可在无皮肤受累和关节炎的情况下出现,因此必须被视为儿童挛缩的鉴别诊断之一。脉冲类固醇治疗有效且无副作用。