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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
"skin diseases"
symptom
cutaneous mastocytosis
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erythropoietic protoporphyria
fabry disease
harlequin ichthyosis
inclusion body myositis
junctional epidermolysis bullosa
kindler syndrome
lamellar ichthyosis
oculocutaneous albinism
papillon-lefèvre syndrome
sneddon syndrome
systemic capillary leak syndrome
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