Department of Paediatric Cardiology and Rheumatology, Central Clinical Hospital of the Medical University of Lodz, 36/50 Sporna St., 91-738, Lodz, Poland.
Rheumatol Int. 2023 Sep;43(9):1755-1764. doi: 10.1007/s00296-023-05362-x. Epub 2023 Jun 14.
Diffuse fasciitis with eosinophilia (EF) is a rare condition classified as a part of the connective tissue disorders. The clinical presentation of this condition can be diverse, however the main symptoms include symmetrical swelling and hardening of distal parts of limbs accompanied by peripheral eosinophilia. The diagnostic criteria are not specified. In inconclusions cases Magnetic Resonance Imaging (MRI) and skin to muscle biopsy may be useful. The pathogenesis and ethiology remain unknown, but extensive physical exertion, certain infectious factors, such as Borrelia burgdorferi, or medications may serve as a trigger. EF affects equally women and men, mainly in their middle age, however the disease can occur at any age. The standard therapy contents gluccocorticosteroids. As a second-line treatment, methotrexate is usually chosen. In this article we compare world reports of EF in paediatric patients with the cases of two adolescent male patients recently hospitalized in the Department of Paediatric Rheumatology.
弥漫性筋膜炎伴嗜酸性粒细胞增多症(EF)是一种罕见的疾病,被归类为结缔组织疾病的一部分。这种疾病的临床表现多种多样,但主要症状包括四肢远端对称性肿胀和硬化,伴有外周嗜酸性粒细胞增多。诊断标准尚未明确。在不典型病例中,磁共振成像(MRI)和皮肤-肌肉活检可能有用。发病机制和病因尚不清楚,但剧烈的体力活动、某些感染因素,如伯氏疏螺旋体,或药物可能是触发因素。EF 影响男女比例相等,主要发生在中年,但任何年龄都可能发病。标准治疗包括糖皮质激素。作为二线治疗,通常选择甲氨蝶呤。本文比较了世界范围内儿科 EF 患者的报告,以及最近在儿科风湿病科住院的两名青少年男性患者的病例。