Urologic Manifestations and Hydronephrosis as Initial Presentation of Erdheim-Chester Disease: A Rare Form of Non-Langerhans Histiocytosis

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis that is classified as a malignancy of myeloid progenitor cells, with only 1,000 confirmed cases in the literature so far. It often manifests as a multi-system disorder with an initial presentation predominantly in the long bones, central nervous system (CNS), and retroperitoneal space, sometimes causing urologic symptoms as a result. ECD often presents indolently and in a spectrum of different ways, making it challenging to identify and treat. We report a case of a 63-year-old female with ECD that first presented with abdominal pain and acute renal injury due to ECD-related retroperitoneal fibrosis. We also explore the literature at large around ECD, its diagnosis, pathophysiology, and advances in treatments.