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Erdheim-Chester disease

Erdheim-Chester disease
#00029402
Author: Eli L. Diamond, Lorenzo Dagna, David M. Hyman, Giulio Cavalli, Filip Janku, Juvianee Estrada-Veras, Marina Ferrarini, Omar Abdel-Wahab, Mark L. Heaney, Paul J. Scheel, Nancy K. Feeley, Elisabetta Ferrero, Kenneth L. McClain1, Augusto Vaglio, Thomas Colby,
Category: Macrophage/Histiocytic and dendritic cell Neoplasms and disorders (2015) > L group > Erdheim-Chester disease
Published Date: 07/24/2014

Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis described by Jakob Erdheim and William Chester. Characteristic histopathologic and radiographic findings of ECD. (A) PET and (B) 99mTc imaging demonstrating symmetric diametaphyseal radiotracer uptake in the long bones of the legs (arrows) commonly seen in ECD patients. R indicates the patient's right side. (C) CT and (D) MRI scans revealing sclerotic lesions of the metaphyses of femur and tibia (arrows). (E) Hematoxylin-eosin–stained biopsy section of ECD lesion revealing lipid-laden histiocytes characteristic of ECD. (F) IHC stain for CD68 revealing positivity of histiocytes.