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Pulmonary lymphangioleiomyomatosis: a rare case.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
is
an
uncommon
lung
disease
primarily
affecting
women
of
childbearing
age
.
It
is
characterized
by
the
progressive
proliferation
and
infiltration
of
smooth
muscle
-like
cells
,
which
lead
to
cystic
destruction
of
the
lung
parenchyma
;
obstruction
of
airways
,
blood
vessels
,
and
lymphatics
;
and
loss
of
pulmonary
function
.
We
present
the
case
of
a
46
-
year
-old
female
patient
with
chest
pain
,
cough
,
sputum
,
and
dyspnea
on
exertion
for
three
weeks
.
Minimal
pneumothorax
was
noted
,
and
the
patient
was
referred
to
our
center
for
further
investigation
and
treatment
.
High
-resolution
computed
tomography
revealed
numerous
bilateral
thin
-walled
air
cysts
and
interstitial
thickening
affecting
the
central
and
peripheral
part
of
the
upper
zone
of
the
lung
.
We
performed
an
open
-
lung
biopsy
to
confirm
lymphangioleiomyomatosis
.
Our
aim
is
to
discuss
the
pathogenesis
and
other
lesions
noted
in
the
differential
diagnosis
of
this
rare
disease
.
Diseases
Validation
Diseases presenting
"blood vessels"
symptom
cadasil
coats disease
cushing syndrome
esophageal adenocarcinoma
hereditary cerebral hemorrhage with amyloidosis
hydrocephalus with stenosis of the aqueduct of sylvius
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
oral submucous fibrosis
pyomyositis
sneddon syndrome
von hippel-lindau disease
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