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[Chronic edema, monoclonal dysglobulinemia and profuse telangiectasia: a distinct entity?].
[systemic capillary leak syndrome]
Monoclonal
gammopathy
and
edema
are
features
encountered
during
several
diseases
,
especially
systemic
capillary
leak
syndrome
.
The
diagnoses
of
POEMS
syndrome
,
edematous
systemic
scleroderma
and
a
fortuitous
association
may
be
also
discussed
.
We
report
the
cases
of
two
patients
which
did
not
fulfill
the
criteria
for
such
diagnoses
.
Although
the
2
cases
share
some
discrepancies
,
they
have
also
similar
and
particular
features
:
association
of
chronic
edema
,
monoclonal
gammopathy
and
profuse
and
acquired
telangiectasias
.
The
meaning
of
these
cases
remains
to
be
clarified
.
It
might
be
an
entity
close
to
the
systemic
capillary
leak
syndrome
but
characterized
by
the
chronicity
of
edema
and
by
a
distinct
cutaneous
sign
,
the
occurrence
of
numerous
telangiectasias
.
These
cases
emphasize
that
the
acquired
and
profuse
telangiectasias
belong
to
the
wide
range
of
cutaneous
abnormalities
which
may
be
associated
with
monoclonal
paraproteinemia
.
Diseases
Validation
Diseases presenting
"wide range"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
adrenomyeloneuropathy
alexander disease
allergic bronchopulmonary aspergillosis
alpha-thalassemia
aromatase deficiency
benign recurrent intrahepatic cholestasis
cadasil
carcinoma of the gallbladder
congenital toxoplasmosis
cowden syndrome
cystinuria
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
fabry disease
gm1 gangliosidosis
harlequin ichthyosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
legionellosis
neonatal adrenoleukodystrophy
oral submucous fibrosis
pendred syndrome
phenylketonuria
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
proteus syndrome
pyruvate dehydrogenase deficiency
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
trochlear dysplasia
well-differentiated liposarcoma
werner syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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