[Erdheim-Chester disease]

Abstract

Erdheim-Chester disease (ECD) is characterized by lipid granuloma in the long tubular bones, which leads to pathognomonic symmetrical sclerosis of their metaphyses and diaphyses. Lipid granuloma may also be present in numerous other mesenchymal tissues, especially lung, orbit and retroperitoneal space. The clinical course and prognosis of the disease depend on these lesions. Reviewing 30 cases published since 1931 and a personal case with S100 positive cells, we present the typical radiological and clinical findings. There is striking resemblance to chronic disseminated histiocytosis X.