Yuce Inel Tuba, Karabacak Murat, Onen Fatos, Birlik Merih
Department of Rheumatology, School of Medicine, Dokuz Eylul University, Izmir, Turkey.
Department of Rheumatology, School of Medicine, Trakya University, Edirne, Turkey.
Int J Rheum Dis. 2025 Feb;28(2):e70142. doi: 10.1111/1756-185X.70142.
Eosinophilic fasciitis (EF) is a connective tissue disorder characterized by cutaneous changes similar to scleroderma, usually associated with peripheral eosinophilia. This case series highlights clinical findings, particularly extracutaneous involvement, in EF patients to enhance clinician awareness of this rare condition.
EF patients' skin and visceral organ involvement, musculoskeletal findings, laboratory tests (including acute phase reactants, autoantibodies, protein electrophoresis, etc.), magnetic resonance imaging (MRI), skin biopsy results, and treatments were evaluated.
The patient's age at presentation was 54 (range 23-68), and 50% were female. All patients presented with skin thickening in the distal upper extremities, except for the hands and feet. Notably, 50% of the patients showed involvement in the trunk, while 87.5% exhibited involvement in the distal lower extremities. A total of 87.5% of patients had increased acute-phase reactants, and three-quarters had peripheral eosinophilia. Some patients presented with extracutaneous manifestations such as nonspecific pulmonary nodules, neuropathy, or arthritis. MRI scans on all patients revealed notable thickening, contrast enhancement, and increased signal intensity within the fascia. Treatment involved the initiation of corticosteroids, with 87.5% of patients requiring the addition of an immunosuppressive agent due to an inadequate response. While no hematological malignancies were detected during the follow-up period, solid cancer was detected in one patient.
Patients diagnosed with EF should undergo a thorough evaluation for extracutaneous involvement, including joints, lungs, and muscles, as well as screening for occult malignancies. In instances where the condition does not respond to steroid therapy, it may be necessary to consider additional immunosuppressive treatments.
嗜酸性筋膜炎（EF）是一种结缔组织疾病，其特征为皮肤改变类似于硬皮病，通常伴有外周血嗜酸性粒细胞增多。本病例系列突出了EF患者的临床发现，尤其是皮肤外受累情况，以提高临床医生对这种罕见疾病的认识。
对EF患者的皮肤和内脏器官受累情况、肌肉骨骼检查结果、实验室检查（包括急性期反应物、自身抗体、蛋白电泳等）、磁共振成像（MRI）、皮肤活检结果及治疗情况进行评估。
患者就诊时的年龄为54岁（范围23 - 68岁），50%为女性。所有患者均出现上肢远端皮肤增厚，但手部和足部除外。值得注意的是，50%的患者躯干受累，而87.5%的患者下肢远端受累。共有87.5%的患者急性期反应物升高，四分之三的患者外周血嗜酸性粒细胞增多。部分患者出现皮肤外表现，如非特异性肺结节、神经病变或关节炎。所有患者的MRI扫描均显示筋膜明显增厚、强化及信号强度增加。治疗开始使用皮质类固醇，87.5%的患者因反应不佳需要加用免疫抑制剂。随访期间未检测到血液系统恶性肿瘤，但有1例患者检测到实体癌。
诊断为EF的患者应全面评估皮肤外受累情况，包括关节、肺和肌肉，以及筛查隐匿性恶性肿瘤。在病情对类固醇治疗无反应的情况下，可能有必要考虑额外的免疫抑制治疗。
