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[Urticaria pigmentosa: two different clinical presentations in pediatric patients].
[cutaneous mastocytosis]
Urticaria
pigmentosa
(
UP
)
is
the
most
frequent
clinical
feature
of
cutaneous
mastocytosis
.
It
usually
begins
in
a
bimodal
way
:
a
peak
of
incidence
from
birth
to
the
age
of
3
and
the
other
one
between
2
(
nd
)
and
6
(
th
)
decades
of
life
.
Darier
's
sign
is
constant
over
the
affected
skin
without
affecting
the
surrounding
skin
.
When
UP
starts
early
,
it
has
a
good
prognosis
disappearing
into
adolescence
,
while
late
onset
is
often
associated
with
persistent
or
systemic
involvement
.
This
article
reports
two
cases
of
UP
,
one
with
the
classic
description
of
the
disease
and
the
other
with
an
unusual
clinical
presentation
,
prompting
the
pediatrician
to
incorporate
both
forms
as
different
manifestations
of
the
same
entity
.
Diseases
Validation
Diseases presenting
"late onset"
symptom
adrenomyeloneuropathy
cadasil
canavan disease
congenital adrenal hyperplasia
cowden syndrome
cutaneous mastocytosis
homocystinuria without methylmalonic aciduria
junctional epidermolysis bullosa
kabuki syndrome
krabbe disease
neonatal adrenoleukodystrophy
omenn syndrome
phenylketonuria
primary hyperoxaluria type 1
proteus syndrome
pyruvate dehydrogenase deficiency
thoracic outlet syndrome
triple a syndrome
zellweger syndrome
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