Erdheim-Chester disease with skeletal and lung involvement

Case contributed by Dr Andrew Dixon

Presentation

2 month history of SOB and right knee pain.

Patient Data

Age: 65
Gender: Male

CXR shows reticular interstitial pattern with preserved lung volume. 

Appearance of symmetrical metaphyseal sclerosis and corresponding increased uptake on Tc-MDP bone scan (shown below).

HRCT reveals predominantly cystic mid and upper zone disease with interstitial thickening, a few nodules and preservation of lung volumes (the same appearance as Langerhan's Cell Histiocytosis).

Symmetrical metaphyseal sclerosis and corresponding increased uptake on Tc-MDP bone scan.

Case Discussion

Initial CXR showed reticular interstitial pattern with preserved lung volume. Right knee x-ray revealed femoral and tibial metaphyseal sclerosis. Lung HRCT showed predominantly cystic mid and upper zone disease with interstitial thickening, a few nodules and preservation of lung volumes. Erdheim-Chester Disease (a non-langerhan's cell histiocytosis) was suspected, and the classic symmetrical metaphyseal sclerosis with increased uptake on Tc-MDP bone scan was confirmed when both knees were imaged. The patient died 8 months following symptom onset.

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Case information

rID: 9351
Case created: 7th Apr 2010
Last edited: 27th Sep 2015
Inclusion in quiz mode: Included

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