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Proteus syndrome: a case report.
[proteus syndrome]
Proteus
syndrome
is
a
rare
,
sporadically
occurring
hamartomatous
disorder
with
complex
multisystem
involvement
and
wide
clinical
variability
.
Clinical
characteristics
include
craniofacial
abnormalities
;
asymmetrical
overgrowth
of
the
trunk
,
limbs
,
and
digits
;
lipomas
;
and
vascular
malformations
.
Cystic
lung
disease
is
noted
in
approximately
10
per
cent
of
patients
.
These
cystic
malformations
may
lead
to
cystic
pulmonary
emphysema
,
which
may
cause
significant
morbidity
for
the
patient
.
We
describe
the
case
of
a
27
-
year
-old
woman
with
known
Proteus
syndrome
who
developed
acute
on
chronic
respiratory
failure
.
Subsequent
CT
scan
of
the
chest
was
significant
for
extensive
bullous
disease
with
nearly
complete
replacement
of
the
right
lung
tissue
.
Our
operative
management
and
the
patient
's
clinical
course
are
described
.
Diseases
Validation
Diseases presenting
"cystic lung disease"
symptom
lymphangioleiomyomatosis
proteus syndrome
This symptom has already been validated