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[Childhood-onset mastocytosis].
[cutaneous mastocytosis]
Mastocytoses
are
a
group
of
rare
diseases
characterized
,
in
most
cases
,
by
a
benign
proliferation
and
accumulation
of
mast
cells
in
different
tissues
.
In
children
,
mastocytosis
affects
usually
exclusively
the
skin
and
differs
in
many
aspects
from
adult-onset
mastocytosis
.
Except
for
diffuse
cutaneous
mastocytosis
,
which
is
an
uncommon
subtype
of
childhood-onset
mastocytosis
,
involvement
of
the
bone
marrow
or
additional
extracutaneous
organs
is
rarely
seen
in
children
.
The
prognosis
of
childhood-onset
mastocytosis
is
usually
very
good
.
Compared
to
adult
patients
who
most
commonly
experience
chronic
-stable
or
slowly
progressive
disease
,
mastocytosis
in
children
is
mostly
transient
and
self-limiting
.
In
this
review
,
we
present
and
discuss
the
subtypes
of
childhood-onset
mastocytosis
,
recent
advances
in
the
understanding
of
their
pathogenesis
as
well
as
similarities
and
differences
between
adult-
and
childhood-onset
mastocytosis
.
