Erdheim-Chester Disease (ECD) is a non-inherited multifocal lipid storing histiocytosis. It is a rare disease characterised by lipid-laden monocyte infiltration of long bones causing cortical sclerosis and characteristic X-ray appearances. It also involves extraskeletal tissue in up to 50% of cases including retroperitoneal and renal infiltration. We report a patient with long standing ECD with widespread extraskeletal involvement, including significant renal infiltration, presenting with left hydronephrosis secondery obstruction from a proximal ureteric calculas.
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Dundee, P., Bouchier-Hayes, D., Iles, L. et al. Renal Calculi in a Patient with Erdheim–Chester Disease. Int Urol Nephrol 37, 453–456 (2005). https://doi.org/10.1007/s11255-004-0020-4
- Erdheim-Chester Disease