ERDHEIM-CHESTER-DISEASE - RADIOGRAPHIC FINDINGS IN 5 PATIENTS

We present the case histories of five patients with Erdheim-Chester di sease, a rare lipoidosis that has several typical radiographic feature s. In all the patients, the diaphyses and metaphyses of the extremitie s demonstrated a symmetric pattern of diffuse or patchy increased dens ity, a coarsened trabecular pattern, medullary sclerosis, and cortical thickening. The epiphyses were spared in four patients and partially involved in one. The axial skeleton was involved in one patient. Radio tracer Te-99m accumulated in areas of radiographic abnormalities in al l patients. In one patient, MRT demonstrated an abnormal signal, corre sponding to radiographic abnormalities. The signal was hypointense to muscle on T1-weighted sequences and heterogeneously hyperintense and h ypointense to normal bone marrow on T2-weighted sequences. Xanthogranu lomatous lesions infiltrated the retroperitoneum in one patient, the t estes in one patient, the eyelids in one patient, and the orbits in tw o patients.