Ganiyusufoğlu Ali Kürşat, Gökşenoğlu Gökşen, Tunç Burçin, Paker Nurdan
Department of Physical Medicine and Rehabilitation, İstanbul Physical Medicine and Rehabilitation Training Research Hospaital, İstanbul, Turkey.
Department of Radiology, Ethica İncirli Hospital, İstanbul, Turkey.
Arch Rheumatol. 2017 Feb 1;32(2):167-170. doi: 10.5606/ArchRheumatol.2017.5953. eCollection 2017 Jun.
Erdheim-Chester disease is a type of non-Langerhans cell histiocytosis. It is a rare, multisystem disorder with unknown etiology. Heterogeneity of the clinical symptoms makes the diagnosis challenging. On the other hand, knowing the signs and radiological findings of the disease helps to establish a correct diagnosis. In this article, we present a 51-year-old male patient with skeletal and urinary system manifestations who finally underwent right nephrectomy due to renal insufficiency. The diagnosis was suspected by the retroperitoneal infiltrative process and radiological findings of the tubular bones. Erdheim-Chester disease diagnosis was confirmed with CD68(+) CD1a(-) histiocytes detected by immunohistochemical analysis of the nephrectomy specimen.
厄尔德海姆-切斯特病是一种非朗格汉斯细胞组织细胞增多症。它是一种病因不明的罕见多系统疾病。临床症状的异质性使得诊断具有挑战性。另一方面，了解该疾病的体征和影像学表现有助于做出正确诊断。在本文中，我们介绍了一名51岁男性患者，其有骨骼和泌尿系统表现，最终因肾功能不全接受了右肾切除术。通过腹膜后浸润性病变和管状骨的影像学表现怀疑了诊断。通过对肾切除标本进行免疫组织化学分析检测到CD68(+) CD1a(-)组织细胞，从而确诊为厄尔德海姆-切斯特病。
