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Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years.
[systemic capillary leak syndrome]
'
Idiopathic
systemic
capillary
leak
syndrome
(
SCLS
)
,
or
Clarkson
's
disease
is
an
unusual
entity
first
described
45
years
ago
.
It
presents
with
recurrent
episodes
of
shock
due
to
leakage
of
the
plasma
,
which
is
reflected
by
accompanying
hemo-concentration
,
hypo-albuminemia
and
edema
.
We
report
the
case
of
a
young
man
with
multiple
episodes
of
shock
with
generalized
edema
and
pleural
effusion
.
An
initial
diagnosis
of
sepsis
was
made
,
but
the
lack
of
fever
and
absence
of
a
demonstrable
organism
or
source
led
to
a
revision
of
diagnosis
to
SLCS
.
The
last
review
of
this
syndrome
which
analyzed
24
cases
was
published
in
1990
.
Since
then
,
75
more
cases
have
been
reported
.
This
article
reviews
these
cases
.
Diseases
Validation
Diseases presenting
"edema"
symptom
acute rheumatic fever
adrenomyeloneuropathy
aniridia
cadasil
canavan disease
classical phenylketonuria
coats disease
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
erythropoietic protoporphyria
esophageal carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
homocystinuria without methylmalonic aciduria
inclusion body myositis
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
systemic capillary leak syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
This symptom has already been validated