CTICasesErdheim-Chester disease
Imaging findings
Chest and abdominal CT show diffuse coarse interstitial thickening and septal lines in the lungs, a soft tissue cuff around the abdominal aorta, retroperitoneal fat infiltration, and a bilateral symmetric soft tissue rind surrounding both kidneys. PET/CT shows intense FDG uptake in the peri-nephric rinds, orbital cavities, and aortic cuffs.
Key takeaways
Erdheim-Chester disease is a rare, clonal histiocytic neoplasm characterized by systemic infiltration of lipid-laden histiocytes. Classic imaging features include the hairy kidney appearance, coated aorta, and skeletal/orbital involvement. Pulmonary involvement manifests as septal and bronchovascular thickening.
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