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Clinical aspects of paediatric mastocytosis: a review of 101 cases.
[cutaneous mastocytosis]
Cutaneous
mastocytosis
(
CM
)
is
a
typical
presentation
of
mastocytosis
in
children
.
However
,
systemic
mastocytosis
may
also
occur
in
children
.
We
tried
to
characterize
the
clinical
features
of
childhood-onset
mastocytosis
and
estimate
the
value
of
the
SCORMA
(
SCORing
Mastocytosis
)
Index
and
serum
tryptase
levels
as
disease
severity
parameters
.
In
a
survey
of
101
children
mastocytosis
was
diagnosed
and
classified
according
to
World
Health
Organization
criteria
.
In
all
the
cases
serum
tryptase
levels
and
the
SCORMA
Index
were
done
to
assess
the
extent
and
intensity
of
the
disease
.
Cutaneous
mastocytosis
was
diagnosed
in
100
children
;
84
%
of
them
presented
maculopapular
CM
,
10
%
mastocytoma
and
6
%
diffuse
cutaneous
mastocytosis
.
Moreover
,
systemic
mastocytosis
with
bone
marrow
infiltration
and
associated
with
maculopapular
CM
was
found
in
one
case
.
There
was
a
positive
correlation
of
serum
tryptase
level
to
the
SCORMA
Index
.
Both
the
mean
tryptase
level
and
the
mean
SCORMA
Index
were
elevated
in
diffuse
cutaneous
mastocytosis
children
when
compared
with
other
forms
CM
.
A
significantly
higher
mean
tryptase
level
was
found
in
children
with
flushing
,
hypotension
,
diarrhoea
,
extensive
bullous
lesions
and
osteoporosis
or
osteopenia
.
Mastocytosis
in
children
usually
has
a
benign
course
.
Nevertheless
,
severe
mediator-related
symptoms
and
systemic
involvement
may
appear
.
Therefore
,
a
multidisciplinary
approach
involving
careful
monitoring
of
the
serum
tryptase
level
,
SCORMA
Index
and
the
organ
function
is
recommended
.
Both
tryptase
levels
and
the
SCORMA
Index
are
of
a
great
value
as
disease
severity
parameters
and
they
should
be
assessed
simultaneously
in
all
mastocytosis
patients
.
Diseases
Validation
Diseases presenting
"mastocytosis in children"
symptom
cutaneous mastocytosis
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