title: Erdheim-Chester disease with extensive marrow necrosis: a case report and literature review.
abstract: Erdheim-Chester disease is a rare systemic disorder characterized by a fibrosing xanthogranulomatous infiltration of multiple organs. We report a case of Erdheim-Chester disease with diffuse necrosis leading to difficulty in making a prompt diagnosis. Radiologically, osteosclerotic lesions with osteolytic element involved metadiaphyses of both proximal tibia, and retroperitoneal infiltrations encasing both kidneys, both adrenals, and aorta were found. A biopsy of the tibia showed diffuse infiltration of foamy histiocytes, Touton-type giant cells, and fibroblastic cells associated with extensive coagulative necrosis. Immunohistochemically, foamy histiocytes were positive for CD68 and peanut agglutinin and negative for S-100 protein. A few Langerhans' cells, which were difficult to identify in hematoxylin-eosin stain, were highlighted by immunostain for S-100 protein. The patient received supportive therapy and was alive 1 1/2 years after diagnosis, with newly developed bilateral retrobulbar lesions and worsened heart failure.
authors.affiliation: Department of Diagnostic Pathology, Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, Korea.
authors.forename: N R
authors.initials: NR
authors.lastname: Kim
authors.forename: Y H
authors.initials: YH
authors.lastname: Ko
authors.forename: Y H
authors.initials: YH
authors.lastname: Choe
authors.forename: H G
authors.initials: HG
authors.lastname: Lee
authors.forename: B
authors.initials: B
authors.lastname: Huh
authors.forename: G H
authors.initials: GH
authors.lastname: Ahn
mesh_terms: D001847:Bone Diseases / Q000473:pathology* / Q000628:therapy; D001853:Bone Marrow / Q000473:pathology*; D005487:Foam Cells / Q000378:metabolism / Q000473:pathology; D015726:Giant Cells / Q000378:metabolism / Q000473:pathology; D006099:Granuloma / Q000473:pathology* / Q000628:therapy; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D007801:Langerhans Cells / Q000378:metabolism / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D010026:Osteosclerosis / Q000000981:diagnostic imaging / Q000473:pathology; D009418:S100 Proteins / Q000378:metabolism; D013977:Tibia / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D009418:S100 Proteins