Erdheim-Chester disease: case report with unique postmortem magnetic resonance imaging, high-resolution radiography, and pathologic correlation.
Erdheim-Chester disease is an infiltrative form of histiocytosis characterized by replacement of normal tissues by lipid-laden histiocytes. The disease typically infiltrates the medullary portion of the diaphysis and metaphysis of long bones, producing a characteristic radiological pattern dominated by bone sclerosis. It usually affects adults of 40 years of age with a clinical spectrum ranging from an asymptomatic focal bone lesion to multisystemic disease. This case report documents unique imaging and pathologic findings of Erdheim-Chester disease using close postmortem pathologic-imaging correlation.
de Abreu
Marcelo Rodrigues
MR
Department of Radiology, Hospital Mae de Deus, Pedro Chaves Barcelos 1127/401, Porto Alegre RS 90450-010, Brazil. marcelorad@hotmail.com
Castro
Miguel Oliveira
MO
Chung
Christine
C
Trudell
Debra
D
Biswal
Sandip
S
Wesselly
Michelle
M
Resnick
Donald
D
D001842:Bone and Bones / Q000000981:diagnostic imaging* / Q000473:pathology*; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011859:Radiography