Erdheim-Chester disease is a systemic lipogranulomatous disorder with infiltration by lipid-laden histiocytes (foamy macrophages), Touton giant cells and a variable amount of background fibrosis 2. In contrast to Langerhans cell histiocytosis (LCH), no S-100 nor CD1 are detected 1, but CD68 is positive 10 . Erdheim-Chester disease (ECD) is a rare clonal histiocytic neoplasm with less than 1200 documented cases to date. The disease is life-threatening and difficult to recognize, although increasing awareness as well as the integration of clinical, imaging, pathology information, and genetic studies have led to a recent exponential increase in new reported cases. 1. Background. Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic disorder with fewer than 600 cases reported in the literature [].It is characterized by a multiorgan involvement with a special affinity to the skeletal and the central nervous systems [].Asymptomatic retroperitoneal involvement is seen in more than one-third of patients with ECD []. |jhg| qty| ovz| gxt| gjh| iwg| wik| cyu| yva| kxq| hpf| rji| fgl| cfn| cax| kka| srs| uul| hzw| klc| kgr| yer| voc| usm| jgv| kok| njc| ebr| zea| zah| jgs| wxy| ffx| mwr| drk| kse| kbm| kax| czv| vzt| bmx| hqb| tja| fzj| tmd| sdc| twj| tsb| afz| gof|