Erdheim-Chester disease: a distinct lipoidosis or part of the spectrum of histiocytosis?

Abstract
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.

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