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Cutaneous mastocytosis: Two pediatric cases treated with topical pimecrolimus.
[cutaneous mastocytosis]
Cutaneous
mastocytosis
is
characterized
by
increased
numbers
of
skin
mast
cells
that
release
mediators
causing
pruritus
,
urticaria
,
and
flushing
.
Most
pediatric
mastocytosis
patients
exhibit
the
pattern
of
urticaria
pigmentosa
,
which
typically
appears
during
the
first
two
years
of
life
and
resolves
spontaneously
in
late
adolescence
.
However
,
while
the
disease
is
active
,
patients
are
frequently
symptomatic
and
uncomfortable
,
which
justifies
symptomatic
treatment
.
We
report
2
patients
,
a
14
-
month
-old
girl
and
a
26
-
month
-old
boy
,
with
localized
cutaneous
erythematous
lesions
with
a
positive
Darier
sign
.
In
each
,
a
punch
biopsy
confirmed
the
diagnosis
of
mastocytosis
.
Treatment
was
instituted
with
pimecrolimus
cream
twice
a
day
and
oral
antihistamine
.
An
almost
complete
response
was
achieved
after
4
months
of
therapy
in
both
patients
,
with
no
clinical
evidence
of
recurrence
after
4
years
and
2
years
of
follow-up
,
respectively
.
In
children
,
the
treatment
of
mastocytosis
is
directed
primarily
to
avoiding
potential
mast
cell
degranulating
agents
and
alleviating
symptoms
.
Topical
calcineurin
inhibitors
act
by
inhibiting
T
-
cell
activation
and
cytokine
release
;
they
may
suppress
mast
cell-
mediated
reactions
by
reducing
their
degranulation
.
These
two
cases
suggest
that
in
localized
cutaneous
mastocytosis
they
are
a
safe
and
efficacious
alternative
to
topical
steroid
therapy
.
Diseases
Validation
Diseases presenting
"almost complete response"
symptom
cutaneous mastocytosis
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