Korean J Med.
1999 Apr;56(4):542-546.
A Case of Erdheim-Chester Disease with periodic fever and knee pain
- Affiliations
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- 1Department of Medicine, Samsung Medical Center Sungkyunkwan University, College of Medicine, Seoul, Korea.
- 2Department of Pathology, Samsung Medical Center Sungkyunkwan University, College of Medicine, Seoul, Korea.
- 3Department of Radiology, Samsung Medical Center Sungkyunkwan University, College of Medicine, Seoul, Korea.
- 4Department of Nuclear medicine, Samsung Medical Center Sungkyunkwan University, College of Medicine, Seoul, Korea.
Abstract
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ECD is a rare, non-Langerhans form of histiocytosis. The first case was reported in 1930 by
Chester and the term ECD was first described by Jaffe in 1972. Many authors have suggested that
ECD is a part of the spectrum of Langerhans cell histiocytosis(LCH), whereas others believe
ECD as a distinct lipoidosis because of the symmetric distribution of the long bone lesions on
the roentgenogram and the lack of Langerhans granules and S-100 antibody staining in the foamy
histiocytes. We experienced a case of Erdheim-Chester disease (ECD) with periodic night fever,
myalgia and both knee pain, the first case reported in Korea.