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[Associated Langerhans cell histiocytosis and Erdheim-Chester disease].
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
non-
Langerhans
cell
histiocytosis
with
multiple
organ
involvement
affecting
middle
-aged
adults
.
A
case
of
ECD
associated
with
Langerhans
cell
histiocytosis
(
LCH
)
is
reported
herein
.
A
75
-
year
-old
woman
presented
maculopapular
skin
lesions
on
her
trunk
,
associated
with
constrictive
pericarditis
and
pleurisy
present
for
1
year
.
The
skin
biopsy
militated
in
favour
of
LCH
since
it
revealed
a
histiocytic
infiltrate
with
a
positive
CD
1
a
marker
at
immunohistochemistry
(
IHC
)
.
The
association
with
ECD
was
diagnosed
on
the
basis
of
pericarditis
,
periaortitis
,
pleurisy
,
pulmonary
involvement
and
retroperitoneal
fibrosis
.
The
patient
was
treated
with
interferon-α
2
a
with
good
initial
results
,
but
died
from
septic
shock
a
year
and
a
half
later
,
a
few
months
after
discontinuing
interferon
due
to
poor
tolerability
.
The
clinical
,
radiographic
and
histological
arguments
in
favour
of
ECD
clearly
differ
from
those
for
LCH
.
However
,
as
already
reported
,
the
two
illnesses
may
be
associated
,
thus
underlining
the
possible
existence
of
a
link
between
these
two
histiocytic
proliferations
emanating
from
the
same
medullary
precursor
.
Two
hypotheses
have
been
advanced
in
an
attempt
to
explain
this
association
:
the
first
involves
a
stimulus
that
might
lead
to
independent
proliferation
of
the
two
cell
lines
while
the
second
suggests
the
existence
of
a
transformation
pathway
from
one
form
of
proliferation
to
the
other
.
Screening
for
associated
ECD
should
be
routinely
performed
in
patients
presenting
LCH
with
signs
evocative
of
ECD
.
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erdheim-chester disease
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