Erdheim-Chester disease is a form of Histiocytosis which involves the
adults and is distinct from Histiocytosis X. It is characterized by a
constant diaphyseal and metaphyseal bone involvement predominating in
the lower links. The diagnosis can readily be envisaged when the typic
al radiological findings are present. Bone involvement may be isolated
and well tolerated, or can be associated with systemic involvement an
d a severe prognosis. We describe three cases of women aged 46, 50 and
73 years. One patient presented with isolated bone lesions, while the
two others had a multiorgan localization. From the three cases and fr
om an extensive review of the literature, we describe the spectrum of
bone and visceral lesions that can be seen by imaging. The emphasis is
put on lesions of the skeletal system, the retroperitoneum, the nervo
us system, and the pericardium. Furthermore, the relationships between
Erdheim-Chester disease and Histiocytosis X are discussed.