CTICasesErdheim-Chester disease
Imaging findings
Radiographs of the lower extremities show symmetric, patchy osteosclerosis of the distal femurs and proximal tibias. Contrast-enhanced CT chest and abdomen show soft tissue infiltration surrounding the descending aorta and kidneys, which was cold on PET CT, indicating a chronic, fibrotic stage.
Key takeaways
Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis classified as a histiocytic neoplasm. Classic imaging findings include bilateral, symmetric osteosclerosis of the long bones, along with perinephric soft tissue infiltration ('hairy kidney') and periaortic sheath infiltration ('coated aorta').
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