J Korean Bone Joint Tumor Soc.  2013 Jun;19(1):28-32. 10.5292/jkbjts.2013.19.1.28.

Erdheim Chester Disease (ECD): A Case Report

• Lee JH
• Jung ST
• Choi YD

• KMID: 1431657
• DOI: http://doi.org/10.5292/jkbjts.2013.19.1.28

Abstract

Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.

Keyword

Langerhans cell histiocytosis

MeSH Terms

Biopsy
Erdheim-Chester Disease
Histiocytosis
Histiocytosis, Langerhans-Cell
Humans
Knee
Korea
Lower Extremity
Neoplasm Metastasis
Orthopedics

Figure

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  Figure 1. On computed tomography, there are soft tissue invasions around both kidney.

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  Figure 2. On histologic finding, pathologic features revealed collagenous fibroadipose tissue with lymphoplasma and histiocytes. The result immunohistochemical staining were a positive for CD68, and negative for S-100, CD1a.

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  Figure 3. On PET CT, it showed high metabolic lesions of SUV 4.7 in heart (A), kidney (B), bilateral distal femoral, and proximal tibia (C, D).

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  Figure 4. X-ray also showed osteosclerotic and osteolytic lesions on both distal femur and proximal tibia.

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  Figure 5. The bone biopsy revealed an intraosseous fibrosis. H & E (Hematoxylin & Eosin) staining.

Reference

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