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首页> 外文期刊>Italian Journal of Medicine >An unusual Erdheim-Chester disease with orbital involvement: a case report
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An unusual Erdheim-Chester disease with orbital involvement: a case report

机译：一种不寻常的Erdheim-Chester病，伴有眶受累：一例报告

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Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis with multiorgan involvement and a specific tropism for perivascular and fatty connective tissue, of unclear origin, with poor response to therapy. Its identification is difficult because of the variable clinical presentation and its lack of knowledge. We report the case of a 63-years-old woman, with a history of bilateral orbital pseudotumor, who comes to our attention because of progressively worsening asthenia, vomiting and systemic inflammation. Total body computerized tomography scan showed a volumetric increase of choroid plexus of the temporal horn of the left lateral ventricle, presence of solid retrobulbar tissue at the level of both maxillary sinuses, lung fibrosis, and retroperitoneal and peri-aortic infiltration. The association of these signs addressed to a diagnosis of Erdheim-Chester disease. Thus, although extremely rare, the diagnosis of Erdheim-Chester disease must be considered in the case of bilateral retro-orbital tumors and multisystemic involvement.
机译：Erdheim-Chester病是一种罕见的非朗格汉斯细胞组织细胞增生症，多器官受累，血管周围和脂肪结缔组织的特异性趋向性，来源不明，对治疗反应较差。由于临床表现形式多样且缺乏知识，因此很难对其进行鉴定。我们报告了一例63岁的女性，有双侧眼眶假瘤病史，由于无力，呕吐和全身性炎症逐渐加重，引起了我们的关注。全身计算机断层扫描显示，左心室颞角的脉络丛神经体积增加，上颌窦水平存在实体球后组织，肺纤维化以及腹膜后和主动脉周围浸润。这些体征与诊断Erdheim-Chester病有关。因此，尽管极为罕见，但在双侧眼眶后肿瘤和多系统受累的情况下，必须考虑Erdheim-Chester病的诊断。

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