ERDHEIM-CHESTER DISEASE - REPORT OF A CAS E, REVIEW OF THE LITERATURE, AND DISCUSSION OF RELATIONSHIPS WITH LANGHERANS CELL HISTIOCYTOSIS

Citation
E. Pertuiset et al., ERDHEIM-CHESTER DISEASE - REPORT OF A CAS E, REVIEW OF THE LITERATURE, AND DISCUSSION OF RELATIONSHIPS WITH LANGHERANS CELL HISTIOCYTOSIS, Revue du rhumatisme, 60(9), 1993, pp. 601-609
Citations number
29
Categorie Soggetti
Rheumatology
Journal title
ISSN journal
11698446
Volume
60
Issue
9
Year of publication
1993
Pages
601 - 609
Database
ISI
SICI code
1169-8446(1993)60:9<601:ED-ROA>2.0.ZU;2-B
Abstract
Erdheim-Chester disease is an endogenous, non-genetically-determined l ipidosis characterized by infiltrates of foamy, lipid-laden histiocyte s and by bilateral symmetric foci of sclerosis in appendicular long bo nes. The clinical spectrum ranges from focal bone lesions to systemic disease with life-threatening visceral involvement. In one third of pa tients, roentgenograms show focal osteolysis within areas of sclerosis . Authors report a new case of Erdheim-Chester disease documented by t wo bone biopsies in different sites. Features in their patient include d : 1) osteolysis and sclerosis of the long bones of the limbs and max illas, with CT scan evidence of cortical rupture; 2) on magnetic reson ance imaging studies, heterogeneous foci of decreased signal intensity on T1 images and heterogeneous areas of moderately increased signal i ntensity on T2-weighted images; 3) increased serum osteocalcin levels; 4) laboratory evidence of chronic inflammation with no extraosseous m anifestations. The clinical, radiological, and pathological features o f Erdheim-Chester disease are different from those of Langherans cell histiocytosis. However, three cases of patients with both conditions h ave been reported in the literature, suggesting that there may be link s between the two diseases.