Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
[An ophthalmologic diagnostic error leading to a rare systemic diagnosis: Erdheim-Chester disease].
[erdheim-chester disease]
Erdheim-
Chester
disease
is
a
rare
systemic
disease
.
The
diagnosis
is
difficult
due
to
significant
clinical
and
morphological
polymorphism
.
Orbital
involvement
is
rare
,
but
constitutes
a
classic
means
of
detection
.
We
report
the
case
of
a
60
-
year
-old
man
,
who
consulted
for
evaluation
of
bilateral
retro-orbital
tumors
.
These
tumors
had
been
discovered
on
head
CT
two
years
previously
during
work-up
of
proptosis
.
Two
biopsies
were
performed
.
The
first
one
revealed
polymorphous
inflammatory
tissue
.
The
second
one
revealed
intense
granulomatous
reaction
,
rich
in
non-
specific
foamy
histiocytes
.
Thoracic
-
abdominal
-
pelvic
CT
scan
detected
peri-
aortic
and
retroperitoneal
infiltration
.
The
association
of
these
signs
pointed
to
a
diagnosis
of
Erdheim-
Chester
disease
,
confirmed
by
the
re
-examination
of
the
histological
samples
.
Erdheim-
Chester
disease
is
a
rare
non-
Langerhans
histiocytosis
with
a
specific
tropism
for
perivascular
and
fatty
connective
tissue
.
The
cause
is
not
known
.
The
diagnosis
of
this
systemic
disease
is
histological
.
In
the
case
of
bilateral
intra-orbital
tumors
,
the
diagnosis
of
Erdheim-
Chester
disease
must
be
considered
.
Diseases
Validation
Diseases presenting
"polymorphous inflammatory tissue"
symptom
erdheim-chester disease
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom