Humans ; Sirolimus ; Cytarabine/therapeutic use* ; Erdheim-Chester Disease/drug therapy* ; Interferon-alpha/therapeutic use* ; Treatment Failure

Erdheim-Chester disease (ECD) is a disseminated xanthogranulomatous infiltrative disease of unknown origin which reveals the infiltration of different organs and bones by foamy histiocytes. Patients with ECD have specific skeletal X-ray findings, with characteristic osteosclerosis of long bones, and histiocytes stained negative for S-100 antibody and electron microscopy of their cytoplasm does not reveal Birbeck granules. ECD represents variable clinical manifestations due to systemic involvement including the liver, spleen, pancreas, peritoneum, lung, pericardium, retroperitoneum and kidney. We report a case of ECD with bilateral hydronephrosis treated with the insertion of double J catheter following to the steroid based chemotherapy with a brief review of the literature.
Catheters ; Cytoplasm ; Drug Therapy ; Erdheim-Chester Disease* ; Histiocytes ; Humans ; Hydronephrosis* ; Kidney ; Liver ; Lung ; Microscopy, Electron ; Osteosclerosis ; Pancreas ; Pericardium ; Peritoneum ; Spleen