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Imaging in Erdheim-Chester disease: classic features and new insights.
[erdheim-chester disease]
Erdheim-
Chester
disease
is
a
rare
non-
Langerhans
cell
histiocytosis
.
Osseous
involvement
is
the
most
frequent
feature
with
bilateral
and
symmetric
osteosclerotic
changes
in
long
bone
diaphyseal
and
metaphyseal
regions
,
classically
sparing
epiphyses
.
99
mTc
scintigraphy
shows
bilateral
and
symmetrical
metaphysal
and
diaphyseal
increased
uptake
in
almost
all
the
patients
,
even
asymptomatic
.
Other
classical
features
on
CT
-scan
,
very
evocative
of
Erdheim-
Chester
disease
,
must
be
recognised
:
e
.
g
.
'
coated
'
aorta
,
'
hairy
kidney
'
patterns
.
New
imaging
techniques
such
as
MRI
have
led
to
a
better
description
of
cardiac
and
central
nervous
system
involvements
.
Pachymeningitis
and
right
atrium
wall
infiltration
are
new
evocative
images
of
this
disease
.
Fluorodeoxyglucose
Positron
Emission
Tomography
in
the
diagnosis
or
prognosis
assessment
is
still
discussed
.
The
objective
of
this
review
is
to
discuss
the
place
of
each
imaging
technique
in
Erdheim-
Chester
disease
in
2013
.
Diseases
Validation
Diseases presenting
"symmetric osteosclerotic changes in long bone"
symptom
erdheim-chester disease
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