Erdheim-Chester disease: a case report and literature overview

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