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
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.