From Milan: Erdheim Chester disease-imaging and therapy

http://www.ncbi.nlm.nih.gov/pubmed/21716027

Clin Nucl Med. 2011 Aug;36(8):704-6.
Erdheim-chester disease: imaging-guided therapeutic approach.
Della Torre E, Dagna L, Mapelli P, Mellone R, Grazia Sabbadini M.
Source
From the *Università Vita-Salute San Raffaele, School of Medicine, San Raffaele Scientific Institute, Milan, Italy; Departments of †Medicine and Clinical Immunology, and ‡Nuclear Medicine, San Raffaele Scientific Institute, Milan, Italy; §Università Milano Bicocca, Milan, Italy; and ¶Department of Radiology, San Raffaele Scientific Institute, Milan, Italy.

Abstract
Erdheim-Chester disease (ECD) is a rare form of systemic non-Langerhans cell histiocytosis with characteristic bone involvement. However, extraskeletal involvement occurs in approximately half of the patients. Because of its protean findings, the diagnosis of ECD is often delayed; thus, a clinical suspicion may prompt specific imaging studies to recognize suggestive signs of organ involvement. In this study, a case of a patient with ECD with representative progressive multisystemic involvement has been reported; although the final diagnosis was confirmed by histologic analysis, imaging studies with almost pathognomonic findings guided the diagnostic process and prompted different therapeutic approaches according to the localization of the disease.

PMID: 21716027 [PubMed - in process]