Erdheim-Chester disease: a distinct lipoidosis or part of the spectrum of histiocytosis?
Erdheim-Chester disease has always been considered a distinct lipoidosis based on clinical and radiographic criteria. Pathologically, it has been indistinguishable from Hand-Schüller-Christian disease. Analysis of the 15 reported cases lends some doubt as to the diagnostic criteria. A new case is presented that strongly suggests that Erdheim-Chester disease is actually part of the spectrum of the histiocytoses.
D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D004803:Eosinophilic Granuloma / Q000000981:diagnostic imaging; D005260:Female; D005360:Fibula / Q000000981:diagnostic imaging*; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D008064:Lipidoses / Q000175:diagnosis / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D010026:Osteosclerosis / Q000000981:diagnostic imaging; D011859:Radiography; D013977:Tibia / Q000000981:diagnostic imaging*