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Erdheim-Chester disease: a case report and literature overview

  • Musculoskeletal radiology
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Abstract.

Erdheim-Chester (EC) disease belongs to the group of lipoid granulomatosis. Symmetric sclerosis of the meta- and diaphysis of long tubular bones are pathognomonic radiologic changes. Additionally, other skeletal segments can be affected. Extraskeletal manifestations can occur in almost all organs; lungs, pericardium, retroperitoneum, skin, and orbita play particularly important roles. The last case of 38 cases of Erdheim-Chester disease with an extraordinary mediastinal and perirenal involvement is described. For the second time following the initial description by Chester, an axial skeletal pattern of eburnated vertebra is shown.

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Received: 18 June 1997; Revision received: 16 February 1998; Accepted: 20 March 1998

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Kenn, W., Stäbler, A., Zachoval, R. et al. Erdheim-Chester disease: a case report and literature overview. Eur Radiol 9, 153–158 (1999). https://doi.org/10.1007/s003300050647

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  • DOI: https://doi.org/10.1007/s003300050647

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