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[Monoclonal gammopathies].
[systemic capillary leak syndrome]
Monoclonal
gammopathies
can
induce
clinical
or
biological
symptoms
,
independent
of
any
associated
hemopathy
.
Cryoglobulins
types
I
and
II
are
responsible
for
cutaneous
lesions
,
peripheral
neuropathy
,
and
membranoproliferative
glomerulopathy
.
Peripheral
neuropathies
associated
with
monoclonal
gammopathy
are
chronic
,
distal
,
symmetrical
and
progressive
,
in
two
thirds
of
the
cases
the
monoclonal
component
reacts
with
a
neuronal
antigen
principally
myelin
associated
glycoprotein
.
POEMS
syndrome
is
characterized
by
:
polyneuropathy
,
organomegaly
,
endocrine
disorder
,
monoclonal
component
,
and
skin
diseases
.
Deposits
of
light
chains
of
immunoglobulins
are
responsible
for
a
glomerulopathy
,
and
sometimes
affect
other
organs
(
skin
,
heart
)
.
Other
manifestations
are
described
:
angioneurotic
oedema
,
acquired
Willebrand
disease
,
systemic
capillary
leak
syndrome
,
Fanconi
syndrome
.
Treatment
of
the
gammopathy
can
control
associated
affections
.
Diseases
Validation
Diseases presenting
"cutaneous lesions"
symptom
child syndrome
cutaneous mastocytosis
cystinuria
dystrophic epidermolysis bullosa
gm1 gangliosidosis
harlequin ichthyosis
inclusion body myositis
lamellar ichthyosis
malignant atrophic papulosis
papillon-lefèvre syndrome
systemic capillary leak syndrome
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