Nishiyama K, Shimuzu J, Takeda K, Sakuta M
Department of Neurology, Japanese Red Cross Medical Center.
Rinsho Shinkeigaku. 1991 May;31(5):543-5.
Eosinophilic fasciitis is an uncommon disorder characterized by peripheral blood eosinophilia, hypergamma-globulinemia, and inflammation of fascia and skin. Its typical symptom in the acute stage includes muscle pain and swelling, followed by slowly progressive joint contractures. We report an 11-year-old girl with pathological diagnosis of chronic fasciitis, probable eosinophillic fasciitis. Her first symptom was chronic symmetrical joint contractures, which developed when she was 4 years old. She had atypical course as compared with patients described in literature, lack of acute symptom and very early onset of the disease. Her muscle CT scan revealed very fibrotic fascia, which might be responsible for joint contractures. Muscle and fascia biopsies were performed to confirm the diagnosis. The fascia was very fibrotic containing mononuclear cell infiltration, compatible with a chronic stage of eosinophilic fasciitis. In conclusion muscle CT scan is useful for differential diagnosis of this disorder in patients presenting with chronic symmetrical joint contractures, but without acute inflammatory symptom.
嗜酸性筋膜炎是一种罕见的疾病,其特征为外周血嗜酸性粒细胞增多、高球蛋白血症以及筋膜和皮肤炎症。急性期的典型症状包括肌肉疼痛和肿胀,随后逐渐出现进行性关节挛缩。我们报告一名11岁女孩,病理诊断为慢性筋膜炎,可能为嗜酸性筋膜炎。她的首发症状是慢性对称性关节挛缩,4岁时就已出现。与文献中描述的患者相比,她的病程不典型,缺乏急性症状且疾病起病非常早。她的肌肉CT扫描显示筋膜纤维化非常严重,这可能是导致关节挛缩的原因。进行了肌肉和筋膜活检以确诊。筋膜纤维化非常严重,伴有单核细胞浸润,符合嗜酸性筋膜炎的慢性期表现。总之,对于出现慢性对称性关节挛缩但无急性炎症症状的患者,肌肉CT扫描有助于该疾病的鉴别诊断。