Presentation
Abdominal discomfort.
Patient Data
Symmetric stranding surrounding both kidneys and extending into the renal hila and along the course of the proximal ureters with increased thickening and enhancement. No obstruction.
Left humerus radiographs with vague areas of medullary sclerosis in the humeral neck and diaphysis.
Medullary sclerotic lesions throughout the left femur and imaged proximal tibia.
Whole body bone scan with increased radiotracer uptake in the humeral heads, patchy medullary uptake in the proximal humeral diaphysis and radius/ulna. Pronounced bilateral hip and lower extremity uptake.
Case Discussion
This case begins with an unusual (but highly characteristic) appearance of symmetric perinephric and perihilar stranding - the "hairy kidney" sign. It is appropriate to confirm the suspicion of Erdheim-Chester disease with long bone radiographic which looks for the characteristic medullary sclerotic lesions, and finally with a bone scan confirming increased uptake in this same pattern. ECD is a non-Langerhans histiocytosis with multisystem involvement, but most commonly presents with findings surrounding the kidneys and involvement of the long bones.
Case courtesy of Dr. Barry Rush
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