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Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations.
[cutaneous mastocytosis]
Cutaneous
mastocytosis
in
children
is
a
generally
benign
disease
that
can
present
at
birth
and
is
often
associated
with
mast
cell
mediator-related
symptoms
including
pruritus
,
flushing
,
and
abdominal
pain
with
diarrhea
.
The
most
common
form
of
presentation
is
urticaria
pigmentosa
,
also
referred
to
as
maculopapular
mastocytosis
.
Flares
of
lesions
are
induced
by
triggers
such
as
physical
stimuli
,
changes
in
temperature
,
anxiety
,
medications
,
and
exercise
.
The
skin
lesions
are
typically
present
on
the
extremities
.
Symptoms
respond
to
topical
and
systemic
anti-mediator
therapy
including
antihistamines
and
cromolyn
sodium
.
Remission
at
puberty
is
seen
in
a
majority
of
cases
.
Progression
to
systemic
mastocytosis
with
involvement
of
extracutaneous
organs
is
not
common
.
The
cause
of
cutaneous
mastocytosis
is
unknown
and
familial
cases
are
rare
.
Mutations
of
c-kit
have
been
observed
in
the
skin
of
those
affected
.
The
diagnosis
is
established
on
clinical
grounds
and
the
findings
on
skin
biopsy
.
Bone
marrow
studies
are
recommended
if
there
is
suspicion
of
progression
of
disease
to
an
adult
form
,
if
cytoreductive
therapy
is
contemplated
,
or
if
skin
lesions
remain
present
and
/
or
tryptase
levels
remain
elevated
after
puberty
.
The
use
of
chemotherapy
,
including
kinase
inhibitors
,
is
strongly
discouraged
unless
severe
hematologic
disease
is
present
,
since
malignant
evolution
is
extremely
rare
.
Diseases
Validation
Diseases presenting
"bone marrow studies"
symptom
cutaneous mastocytosis
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