Erdheim-Chester disease: a case report.
10.3346/jkms.1999.14.3.323
- Author:
Yong Koo PARK
1
;
Kyung Nam RYU
;
Bang HUH
;
Jae Do KIM
Author Information
1. Department of Pathology, College of Medicine, Kyung Hee University, Seoul, Korea. damia@chollian.net
- Publication Type:Case Report
- Keywords:
Erdheim-Chester disease;
Histiocytosis;
Immunohistochemistry
- MeSH:
Adult;
Bone Marrow/pathology;
Case Report;
Histiocytosis/pathology*;
Human;
Knee/radiography;
Knee/pathology;
Magnetic Resonance Imaging;
Male;
Osteosclerosis/radiography;
Osteosclerosis/pathology
- From:Journal of Korean Medical Science
1999;14(3):323-326
- CountryRepublic of Korea
- Language:English
-
Abstract:
A 42-year-old man with Erdheim-Chester disease (EC) is presented. This is the first case of this disease reported in Korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. Magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.
