J Korean Med Sci.  1999 Jun;14(3):323-326. 10.3346/jkms.1999.14.3.323.

Erdheim-Chester disease: a case report

Affiliations
  • 1Department of Pathology, College of Medicine, Kyung Hee University, Seoul, Korea. damia@chollian.net

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.

Keyword

Erdheim-Chester disease; Histiocytosis; Immunohistochemistry

MeSH Terms

Adult
Bone Marrow/pathology
Case Report
Histiocytosis/pathology*
Human
Knee/radiography
Knee/pathology
Magnetic Resonance Imaging
Male
Osteosclerosis/radiography
Osteosclerosis/pathology

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