Erdheim-Chester疾病;一个31岁的妇女提出骨痛和眼科受累:一个病例报告

Soheil Abdollahi Yeganeh, A. Dehghan, J. Jahanshahi, Sepehr Shirouei, Mahdi Arjipour, S. Borzouei
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摘要

埃尔德海姆-切斯特病(ECD)是一种罕见的非朗格汉斯组织细胞疾病,没有实际已知的发病率。它主要通过促炎细胞因子的表达增加影响多个器官,最终导致组织细胞在多个部位活化和浸润。31岁女性处女,表现为进行性全身骨痛和视力模糊。她接受了全面的临床和临床外评估。根据最终结果,建立ECD诊断。我们开始主要使用干扰素-α、皮质类固醇、雌激素、左甲状腺素和去氨加压素进行治疗。对于患有影响多器官的神秘慢性疾病的成人,我们应该始终考虑ECD作为鉴别诊断。尽管ECD仍然罕见,但近几十年来该病的检出率显著提高。全面的临床和临床评价是必要的,以作出明确的诊断和确定疾病的程度。
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Erdheim-Chester disease; a 31-year-old woman presented with bone pain and ophthalmologic involvement: a case report
Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder with no actual known incidence rate. It primarily affects multiple organs via increased expression of proinflammatory cytokines, eventually leading to histiocyte activation and infiltration at multiple sites. A 31-year-old virgin female presented with progressive generalized bone pain and blurred vision. She underwent a comprehensive clinical and paraclinical evaluation. Based on the final results, the diagnosis of ECD was established. We started treatment mainly using Interferon-α and corticosteroids, estrogen, levothyroxine, and desmopressin acetate. In adults with a mysterious chronic disease affecting multiple organs, we should always consider ECD as a differential diagnosis. Although ECD is still rare, the detection rate of this disease has increased significantly in recent decades. A comprehensive clinical and paraclinical evaluation is necessary to make a definitive diagnosis and determine the extent of the disease.
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