Erdheim-Chester Disease With Renal Involvement: A Case Report
Ali Kürşat GANİYUSUFOĞLU1, Gökşen GÖKŞENOĞLU1, Burçin TUNÇ2, Nurdan PAKER1
1Department of Physical Medicine and Rehabilitation, İstanbul Physical Medicine and Rehabilitation Training Research Hospaital, İstanbul, Turkey
2Department of Radiology, Ethica İncirli Hospital, İstanbul, Turkey
Keywords: Bone diseases, Erdheim-chester disease, non-Langerhans cell histiocytosis
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.
The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.
The authors received no financial support for the research and/or authorship of this article.