Case report: Erdheim-Chester disease: polyostotic sclerosing histiocytosis.
Erdheim-Chester disease is characterised by diffuse metaphyseal and diaphyseal sclerosis of long tubular bones of the appendicular skeleton. In addition, there is an inconstant tendency to visceral involvement by lipo-granulomatous infiltration. Histologically, this condition has marked similarities to Hand-Schuller-Christian disease. A further case is presented which supports the view that Erdheim-Chester disease is part of the spectrum of histiocytoses and not a distinct pathological entity. Polyostotic sclerosing histiocytosis would be an appropriate alternative label for this condition.
D000328:Adult; D005260:Female; D006801:Humans; D007719:Knee Joint / Q000000981:diagnostic imaging; D008064:Lipidoses / Q000000981:diagnostic imaging* / Q000473:pathology; D008206:Lymphatic Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D010026:Osteosclerosis / Q000000981:diagnostic imaging* / Q000473:pathology; D010384:Pelvic Bones / Q000000981:diagnostic imaging; D011859:Radiography; D011877:Radionuclide Imaging; D013577:Syndrome; D013977:Tibia / Q000473:pathology