title: [Imaging features of osseous and extra-osseous involvement in Erdheim-Chester disease].
abstract: Erdheim-Chester disease is a rare form of systemic non-Langerhans cell histiocytosis characterized by infiltration by lipid-laden or foamy histiocytes. Osseous involvement, major diagnostic criteria, is constant and characteristic. It presents as metaphyseal and diaphyseal osteosclerosis, mainly affecting the long bones of the lower limbs. A few cases with axial skeleton involvement have been reported. Extra-osseous lesions may affect the retroperitoneum, lungs, skin, heart, brain and orbits. Prognosis depends mainly on the extra-osseous disease, mainly heart and lung involvement. Diagnosis is based on the combination of radiographic features, nuclear medicine features and nearly pathognomonic immunohistochemical profile.
authors.affiliation: Département de radiologie, centre hospitalier universitaire d'Angers, 4, rue Larrey, 49933 Angers cedex 09, France. omar.adib@yahoo.fr
authors.forename: O
authors.initials: O
authors.lastname: Adib
authors.forename: E
authors.initials: E
authors.lastname: Baroth
authors.forename: L
authors.initials: L
authors.lastname: Perard
authors.forename: J-Y
authors.initials: JY
authors.lastname: Scoazec
authors.forename: L
authors.initials: L
authors.lastname: Vervueren
authors.forename: C
authors.initials: C
authors.lastname: Aubé
authors.forename: S
authors.initials: S
authors.lastname: Willoteaux
mesh_terms: D001706:Biopsy; D001842:Bone and Bones / Q000473:pathology*; D003952:Diagnostic Imaging; D031249:Erdheim-Chester Disease / Q000175:diagnosis*; D006644:Histiocytes / Q000473:pathology; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D010026:Osteosclerosis / Q000175:diagnosis*; D049268:Positron-Emission Tomography; D011658:Pulmonary Fibrosis / Q000175:diagnosis; D011877:Radionuclide Imaging; D013131:Spine / Q000473:pathology; D014057:Tomography, X-Ray Computed