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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
"sepsis"
symptom
acute rheumatic fever
carcinoma of the gallbladder
congenital diaphragmatic hernia
cushing syndrome
cystinuria
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
focal myositis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
homocystinuria without methylmalonic aciduria
inclusion body myositis
junctional epidermolysis bullosa
kindler syndrome
lamellar ichthyosis
locked-in syndrome
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
scrub typhus
systemic capillary leak syndrome
typhoid
This symptom has already been validated