Erdheim-Chester Disease

Case contributed by Dr Michael P Hartung

Presentation

Abdominal discomfort.

Patient Data

Age: 55 years
Gender: Male
CT

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. 

X-ray

Medullary sclerotic lesions throughout the left femur and imaged proximal tibia. 

Nuclear medicine

Whole body bone scan with increased radiotracer uptake in the humeral heads, patchy medullar 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

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.