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Unusual orbital involvement in Erdheim Chester disease: a radiological diagnosis.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
an
exceedingly
rare
,
disseminated
non-
Langerhan
cell
histiocytosis
with
multisystem
involvement
,
having
characteristic
sclerotic
skeletal
lesions
.
We
present
an
unusual
case
primarily
manifesting
as
an
extensive
orbital
disease
,
with
low
-grade
systemic
involvement
.
Owing
to
its
rarity
and
therefore
lack
of
general
awareness
it
remains
a
difficult
clinical
and
pathologic
diagnosis
.
Immuno-histochemistry
of
the
biopsy
specimen
is
diagnostic
.