Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement.
Erdheim-Chester disease (ECD) is a rare form of histiocytosis of unknown origin characterized by tissue infiltration by lipid-laden histiocytes. Typically, the diaphyseal and metaphyseal portions of the tubular bones are affected, leading to a characteristic radiographic pattern of bone sclerosis. Orbital involvement is not infrequent and is manifested by exophthalmos and periorbital xanthomatous lesions, with associated visual problems. This case report documents imaging and pathologic findings in a patient with ECD with extensive orbital involvement.
De Abreu
Marcelo R
MR
Department of Radiology, Hospital Mae de Deus e Mae de Deus Center, Porto Alegre, Brazil.
Chung
Christine B
CB
Biswal
Sandip
S
Haghighi
Parvis
P
Hesselink
John
J
Resnick
Donald
D
D001842:Bone and Bones / Q000473:pathology; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000473:pathology; D005094:Exophthalmos / Q000209:etiology / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007676:Kidney Failure, Chronic / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009408:Nerve Compression Syndromes / Q000175:diagnosis / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000473:pathology; D009915:Orbit / Q000473:pathology; D009916:Orbital Diseases / Q000175:diagnosis* / Q000473:pathology; D010026:Osteosclerosis / Q000175:diagnosis / Q000473:pathology; D012185:Retroperitoneal Fibrosis / Q000473:pathology; D015354:Vision, Low / Q000209:etiology / Q000473:pathology; D014973:Xanthomatosis / Q000209:etiology / Q000473:pathology