Korean J Urol.  2001 Apr;42(4):453-456.

A Case of Erdheim-Chester Disease with Bilateral Hydronephrosis

• Lee W
• Park NC

Abstract

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.

Keyword

Erdheim-Chester disease; Bilateral hydronephrosis

MeSH Terms

Catheters
Cytoplasm
Drug Therapy
Erdheim-Chester Disease*
Histiocytes
Humans
Hydronephrosis*
Kidney
Liver
Lung
Microscopy, Electron
Osteosclerosis
Pancreas
Pericardium
Peritoneum
Spleen