Display Settings:

Format

Send to:

Choose Destination
Hong Kong Med J. 2013 Oct;19(5):451-4. doi: 10.12809/hkmj133738.

Erdheim-Chester disease: an uncommon cause of upper urinary tract obstruction.

Author information

  • 1Department of Surgery, Caritas Medical Centre, 111 Wing Hong Street, Shamshuipo, Kowloon, Hong Kong.

Abstract

Erdheim-Chester disease is a rare non-Langerhans form of systemic histiocytosis of unknown origin. We describe a 45-year-old man presenting with bilateral hydronephrosis suggestive of extrinsic urinary tract obstruction. Computed tomography revealed extensive hypodense soft tissue infiltration in the retroperitoneum surrounding the kidneys. Needle biopsy of the retroperitoneal soft tissue revealed aggregates of lipid-laden histiocytes expressing CD68 but negative for CD1a and S100 protein. The diagnosis of Erdheim-Chester disease was supported by typical radionuclide bone scinitigraphic findings. Treatment with prednisolone, sirolimus, and regular ureteric stent revision was initiated to achieve adequate urinary tract drainage. To our knowledge, this is the second patient with Erdheim-Chester disease reported in Hong Kong. A high index of suspicion is required to avoid delay in the diagnosis of this rare disease.

KEYWORDS:

Erdheim-Chester disease; Histiocytosis; Sirolimus

PMID:
24088590
[PubMed - indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Icon for Hong Kong Academy of Medicine Press
    Loading ...
    Write to the Help Desk