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Hand-Schüller-Christian disease and Erdheim-Chester disease: coexistence and discrepancy.
[erdheim-chester disease]
Langerhans
cell
histiocytosis
(
LCH
)
and
Erdheim-
Chester
disease
(
ECD
)
share
similar
clinical
features
and
mechanisms
.
In
very
rare
circumstances
,
the
two
diseases
coexist
in
the
same
patient
.
Here
we
report
such
a
patient
,
who
was
first
diagnosed
with
Hand-
Schüller-
Christian
disease
(
HSC
)
,
a
type
of
LCH
.
Several
years
later
,
the
patient
presented
with
severe
exophthalmos
and
osteosclerosis
on
radiograph
.
New
biopsy
revealed
ECD
.
We
also
analyze
54
cases
of
LCH
and
6
cases
of
ECD
diagnosed
in
our
hospital
,
as
well
as
their
progression
during
a
follow-up
period
of
8
years
.
In
five
cases
of
HSC
(
9
.
3
%
of
LCH
)
,
a
triad
of
central
diabetes
insipidus
,
hyperprolactinemia
,
and
pituitary
stalk
thickening
on
magnetic
resonance
imaging
(
MRI
)
preceded
the
typical
bone
lesions
by
4
-
9
years
.
In
addition
,
LCH
was
featured
as
elevated
plasma
alkaline
phosphatase
(
ALP
)
,
which
was
normal
in
ECD
.
Combined
with
a
literature
review
,
several
features
are
summarized
to
differentiate
ECD
from
HSC
.
In
patients
with
diabetes
insipidus
,
concomitant
hyperprolactinemia
and
pituitary
stalk
thickening
on
MRI
indicate
a
possible
HSC
.
Additionally
,
if
osteosclerosis
is
observed
in
a
patient
with
LCH
,
the
coexistence
of
ECD
should
be
considered
.
Diseases
Validation
Diseases presenting
"pituitary stalk thickening on magnetic resonance"
symptom
erdheim-chester disease
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