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Peripheral edema due to increased vascular permeability: a clinical appraisal.
[systemic capillary leak syndrome]
The
release
of
vasoactive
substances
produces
reversible
changes
of
endothelial
permeability
with
consequent
edematous
syndromes
.
We
present
899
patients
referred
to
our
clinic
for
"
non-hydrostatic
non-hyponcotic
"
recurrent
edema
problems
.
Personal
and
family
histories
were
recorded
and
a
complete
physical
examination
was
carried
out
for
each
patient
.
In
chronic
situations
laboratory
tests
[
blood
cell
count
,
cryoglobulins
,
thyroid
hormones
,
complement
components
(
C
3
,
C
4
,
C
1
inhibitor
)
,
total
IgE
,
skin
testing
]
were
performed
.
Four
subgroups
of
angioedema
are
identified
for
relevant
clinical
and
etiopathogenetic
differences
.
Seventy
-
three
percent
of
patients
had
an
urticaria
-
angioedema
syndrome
responding
to
antihistamine
and
/
or
corticosteroid
treatment
(
histamine-dependent
angioedema
)
.
Twenty
-
three
percent
had
an
angioedema
related
to
a
deficiency
in
C
1
esterase
inhibitor
(
complement-dependent
angioedema
)
.
In
a
minority
of
patients
,
angioedema
was
due
to
the
pharmacological
effect
of
a
drug
(
pharmacological
angioedema
)
or
was
of
a
totally
unknown
origin
(
idiopathic
angioedema
)
.
A
generalized
increase
in
vascular
permeability
was
reported
in
3
patients
(
systemic
capillary
leak
syndrome
)
.
A
brief
survey
of
the
literature
is
given
with
the
review
of
our
patients
.
Diseases
Validation
Diseases presenting
"blood cell count"
symptom
alpha-thalassemia
aromatase deficiency
cutaneous mastocytosis
esophageal squamous cell carcinoma
familial mediterranean fever
pyomyositis
systemic capillary leak syndrome
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