Case Author(s): Jeffrey Yu, M.D. and Tom R. Miller, M.D., Ph.D. , 12/14/99 . Rating: #D5, #Q3

Diagnosis: Erdheim-Chester Disease

Brief history:

46 year-old man with bilateral pretibial pain, right greater than left

Images:

Anterior tibial/ankle flow images

View main image(bs) in a separate image viewer

View second image(bs). Anterior tibial/ankle immediate static images

View third image(bs). Anterior knee/tibial/ankle immediate static images

View fourth image(bs). Delayed whole-body images

Full history/Diagnosis is available below

Diagnosis: Erdheim-Chester Disease

Full history:

46 year-old man with bilateral pretibial pain, right greater than left and a macular rash over the same distribution. Recently, the patient has been having difficulty with night sweats and has been taking aspirin for pain relief.

Radiopharmaceutical:

Tc-99m MDP, i.v.

Findings:

Flow images over the tibia and ankles, immediate static images over the lower extremities, as well as delayed whole-body scintigrams were obtained. There is markedly increased uptake of radiotracer involving the distal and proximal tibia on all three phases of the bone scan. Flow and immediate static images over the femurs were not obtained but there is increased uptake in the distal femurs bilaterally on the delayed images.

There is also increased uptake in the majority of the ulnae radii bilaterally, in the posterior left rib, in the anterior aspect of the first right rib, and in the proximal aspect of the left humerus.

Plain radiographs of these areas demonstrate corresponding abnormalities consisting of scalloping of the cortex and a cystic and sclerotic mixed medullary pattern with thickened trabeculae.

Discussion:

Erdheim-Chester Disease is a rare lipidosis with the following features:

Age: 50-70y

Clinical: Ranges from joint pain --> systemic involvement (heart, liver, spleen, pancreas, pericardium, lungs, adrenals, aorta, lymph nodes, bowel, orbit, and bone)

Histology: Replacement of bone marrow by foamy lipid, histiocytes, and giant cells causing medullary fibrosis and osteosclerosis

X-ray: Symmetric, appendicular skeleton >> axial skeleton Long bones invariably affected in the diaphysis and metaphysis with:

- Patchy or diffuse increase in trabecular pattern

- Medullary sclerosis

- Cortical thickening

Nucs: Increased uptake with Tc-99m MDP and Gallium-67. Photopenia with In-111 and Tc-99m SC

Prognosis: 1/3 Fatal

Treatment: Radiation, chemotherapy, steroids

Followup:

This was a known, biopsy-proven diagnosis prior to the bone scan. The bone scan was performed to evaluate extent of disease.

View followup image(xr). There are multiple small mixed, lytic, and sclerotic lesions throughout the tibia, primarily located in the ends of the diaphysis and the metaphysis of the tibia. There is also a small lytic lesion in the proximal fibular diaphysis, as well as in the distal femur. When compared to the previous plain xrays examinations, there has been no significant interval change. These areas of abnormality correlate with the areas of increased radiopharmaceutical uptake on the bone scan.

Major teaching point(s):

A Zebra and an Aunt Minnie.

Differential Diagnosis List

1) Hemangioma 2) Lymphangioma 3) Multiple myeloma 4) Hand-Schuller-Christian disease

ACR Codes and Keywords:

References and General Discussion of Bone Scintigraphy (Anatomic field:Skeletal System, Category:Other generalized systemic disorder)

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Case number: bs115

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