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[A man in his forties with swelling in both orbits].
[erdheim-chester disease]
Erdheim-
Chester
disease
.
A
multi-disiplinary
challenge
.
The
histiocytoses
are
a
diverse
,
but
rare
group
of
disorders
with
symptoms
affecting
many
organs
,
varying
from
self-limiting
,
localised
lesions
to
disseminated
multi-organ
disease
.
The
diagnostic
challenges
are
illustrated
and
discussed
in
the
following
case
.
A
man
in
his
forties
was
admitted
to
hospital
due
to
pain
in
his
right
eye
and
visual
disturbances
.
MRI
imaging
detected
a
mass
in
his
right
orbit
and
a
minor
mass
in
his
left
orbit
.
The
histological
results
of
the
mass
in
his
right
orbit
revealed
an
inflammatory
process
with
lymphocytes
and
macrophages
and
no
sign
of
vasculitis
,
infection
or
malignancy
.
The
diagnosis
pseudotumor
orbita
was
made
and
treatment
with
corticosteroids
was
initiated
.
He
did
not
respond
to
corticosteroids
or
radiotherapy
and
increasing
symptoms
necessitated
rehospitalisation
.
Further
tests
disclosed
a
multisystem
disease
which
affected
the
aorta
,
skeleton
,
lung
,
heart
and
kidney
.
The
biopsy
was
reconsidered
and
the
disease
was
classified
as
a
histiocytosis
with
CD
68
positive
and
CD
1
a
negative
cells
.
The
diagnosis
Erdheim-
Chester
was
given
,
about
14
months
after
the
initial
hospitalisation
.
Treatment
with
interferon
α
was
started
.
Diseases
Validation
Diseases presenting
"pain in his right eye"
symptom
erdheim-chester disease
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