Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
First Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuokashimoaizuki, Eiheiji, Fukui, 910-1193, Japan.
Clin Rheumatol. 2021 Jun;40(6):2477-2483. doi: 10.1007/s10067-020-05416-6. Epub 2020 Sep 24.
Eosinophilic fasciitis (EF) is a rare connective tissue disease that causes inflammation and fibrosis of the fascia, inducing pain and motor dysfunction. Characteristic skin manifestations, such as edema, erythema, induration, peau d'orange appearance, and the groove sign, are of diagnostic significance and observed in the majority of patients with EF. We herein report a case of EF without these characteristic skin manifestations. A 66-year-old Japanese woman developed progressive limb pain and motor dysfunction. No skin changes were observed. We diagnosed the patient with EF based on the clinical course, magnetic resonance imaging, and en bloc biopsy containing fascia and muscle. Oral prednisolone therapy markedly attenuated limb pain and motor dysfunctions. Through a systemic search of the medical literature, we retrieved 4 juvenile cases and 8 adult cases of EF without characteristic skin manifestations during the clinical course. We herein present a systemic review on EF without skin manifestations and discuss differences between the two proposed sets of diagnostic criteria of EF.
嗜酸性筋膜炎(EF)是一种罕见的结缔组织疾病,可引起筋膜炎症和纤维化,导致疼痛和运动功能障碍。特征性皮肤表现,如水肿、红斑、硬结、橘皮样外观和沟状征,具有诊断意义,且在大多数 EF 患者中观察到。本文报告一例无这些特征性皮肤表现的 EF 病例。一名 66 岁日本女性出现进行性肢体疼痛和运动功能障碍,无皮肤改变。我们根据临床病程、磁共振成像和包含筋膜和肌肉的整块活检诊断为 EF。口服泼尼松龙治疗显著减轻了肢体疼痛和运动功能障碍。通过对医学文献的系统检索,我们共检索到 4 例青少年 EF 病例和 8 例成人 EF 病例,在疾病过程中均无特征性皮肤表现。本文对无皮肤表现的 EF 进行了系统综述,并讨论了两套 EF 诊断标准之间的差异。