Erdheim-Chester disease (bones)
Erdheim-Chester disease is a very rare xanthogranulomatous, non-Langerhans cell systemic histiocytosis with an unknown etiology and pathogenesis.
Localization
symmetric osteosclerosis of the long bones
vertebral and axial skeleton
osteolytic lesions
Synopsis
diffuse macrophagic infiltration
large, foamy histiocytes
rare Touton-like giant cells
lymphocytic aggregates
fibrosis
Immunochemistry
Ultrastructure
lack of Birbeck granules
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