Gerritzen Nicolas, Ziob Jana, Brossart Peter, Schäfer Valentin S
Medizinische Klinik III, Onkologie, Hämatologie, Rheumatologie und klinische Immunologie, Universitätsklinikum Bonn, Venusberg Campus 1, 53127, Bonn, Deutschland.
Klinik und Poliklinik für Dermatologie und Allergologie, Universitätsklinikum Bonn, Bonn, Deutschland.
Z Rheumatol. 2024 Mar;83(2):134-138. doi: 10.1007/s00393-022-01207-3. Epub 2022 May 16.
Eosinophilic fasciitis (EF, also known as Shulman syndrome) is an uncommon connective tissue disease characterized by inflammatory thickening of the fasciae as well as swelling and hardening of the skin. It mostly affects the lower extremities. Swollen and indurated skin, together with the groove sign, are typical clinical signs. So far, biopsy evidence of inflammation and thickening of the fascia has been the gold standard for diagnosis. Magnetic resonance imaging (MRI) is mentioned in the literature as an alternative method for confirming the diagnosis. We present a case of asymmetric EF in a 54-year-old German male. He came with painful induration of the right forearm, with a characteristic groove sign and limitation of motion of the right hand. The blood count revealed eosinophilia with 0.57 G/l or 9.6% (normal: 0.05-0.5 G/l and 0.5-5.5%), ANA and ENA were negative. The diagnosis was confirmed histologically and we were able to detect a thickened fascia in MRI and ultrasound imaging. The EF also appeared in the left lateral malleolus during the course of the illness. Treatment was carried out with prednisolone and methotrexate.
嗜酸性筋膜炎(EF,也称为舒尔曼综合征)是一种罕见的结缔组织疾病,其特征为筋膜的炎症性增厚以及皮肤的肿胀和硬化。它主要累及下肢。皮肤肿胀和硬结以及沟槽征是典型的临床体征。到目前为止,筋膜炎症和增厚的活检证据一直是诊断的金标准。文献中提到磁共振成像(MRI)是确诊的一种替代方法。我们报告一例54岁德国男性的不对称性嗜酸性筋膜炎病例。他因右前臂疼痛性硬结前来就诊,伴有特征性的沟槽征和右手活动受限。血常规显示嗜酸性粒细胞增多,为0.57 G/l或9.6%(正常:0.05 - 0.5 G/l和0.5 - 5.5%),抗核抗体(ANA)和可提取性核抗原(ENA)均为阴性。组织学检查确诊,并且我们在MRI和超声成像中均检测到筋膜增厚。病程中左侧外踝也出现了嗜酸性筋膜炎。采用泼尼松龙和甲氨蝶呤进行治疗。