关键词: Shulman’s disease eosinophilic fasciitis tocilizumab

来  源:   DOI:10.31138/mjr.34.1.78   PDF(Pubmed)

Abstract:
Eosinophilic fasciitis (EF) is a rare connective-tissue disorder that is characterised by subacute onset of erythema, oedema, and induration of the skin and soft tissues of the limbs and trunk. Although several triggers have been hypothesised to be associated with EF, the aetiology of eosinophilic fasciitis (EF) is still unclear, and several treatment regimens have been proposed to treat this disease. In this article, we report a case of a 72-year-old gentleman with multiple comorbidities who presented to the clinic for diffuse skin thickening present on his forearms, thighs, legs bilaterally, and over the pelvis. The patient was diagnosed with EF and failed multiple treatment regimens including prednisone, methotrexate, rituximab, but finally responded and was maintained on tocilizumab. In this article, we will review the current understanding of EF, diagnostic approach, popular treatments and review other cases of EF in which tocilizumab was used.
摘要:
嗜酸性筋膜炎(EF)是一种罕见的结缔组织疾病,其特征是亚急性发作的红斑,水肿,四肢和躯干的皮肤和软组织硬化。尽管假设有几个触发器与EF相关,嗜酸性筋膜炎(EF)的病因尚不清楚,已经提出了几种治疗方案来治疗这种疾病。在这篇文章中,我们报告了一个72岁的绅士有多种合并症,他出现在他的前臂弥漫性皮肤增厚的诊所,大腿,双腿两侧,在骨盆上。患者被诊断为EF,包括泼尼松在内的多种治疗方案均失败。甲氨蝶呤,利妥昔单抗,但最终有反应,并继续服用托珠单抗。在这篇文章中,我们将回顾当前对EF的理解,诊断方法,流行的治疗方法,并回顾使用托珠单抗的其他EF病例。
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