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2016 ; 2016
(ä): 2670495
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Multisystem Radiologic Manifestations of Erdheim-Chester Disease
#MMPMID27340583
Lodhi U
; Sarmast U
; Khan S
; Yaddanapudi K
Case Rep Radiol
2016[]; 2016
(ä): 2670495
PMID27340583
show ga
Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans' cell
histiocytosis that affects individuals between the ages of 50 and 70 with an
equal distribution among males and females. It is associated with significant
morbidity and mortality that is mostly due to infiltration of critical organs.
Some of the sites that Erdheim-Chester Disease affects include the skeletal
system, central nervous system, cardiovascular system, lungs, kidneys
(retroperitoneum), and skin. The most common presenting symptom of
Erdheim-Chester Disease is bone pain although a large majority of patients are
diagnosed incidentally during a workup for a different disease process.
Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to
other infiltrative processes. Therefore, a multimodality diagnostic approach is
employed with imaging being at the forefront. As of date, a comprehensive
radiologic review of the manifestations of Erdheim-Chester Disease has rarely
been reported. Here we present radiologic findings of an individual suffering
from Erdheim-Chester Disease.