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Cutaneous mastocytosis, problems of clinical diagnosis of four cases.
[cutaneous mastocytosis]
Mastocytosis
is
a
rare
disease
characterized
by
a
pathological
increased
of
mast
cells
in
one
or
more
tissues
,
particularly
in
the
skin
,
bone
marrow
,
liver
,
spleen
,
lymph
nodes
and
gastrointestinal
tract
.
Cutaneous
mastocytosis
represents
over
90
%
of
cases
found
with
predilection
in
children
.
The
aim
of
the
paper
was
to
summarize
the
authors
'
clinical
,
histopathological
and
immunohistochemical
observations
on
patients
with
cutaneous
mastocytosis
.
We
present
four
cases
of
cutaneous
mastocytosis
,
sporadic
form
,
customized
by
clinical
presentation
and
age
of
onset
:
two
installed
in
the
neonatal
period
,
a
case
with
onset
in
infancy
and
another
in
adulthood
.
For
the
assessment
of
the
severity
and
the
effectiveness
of
the
treatment
,
we
used
SCORMA
Index
.
We
performed
in
each
patient
histopathological
examination
of
the
skin
(
Hematoxylin-
Eosin
and
Giemsa
stains
)
,
the
dosage
of
mediators
(
serum
tryptase
level
,
serum
histamine
levels
,
urinary
histamine
metabolites
)
and
the
balance
of
expansion
(
complete
blood
cell
count
,
liver
biological
investigations
,
abdominal
ultrasound
,
skeletal
radiography
,
chest
radiography
)
.
For
the
adult
with
mastocytosis
,
we
performed
abdominal
scanner
and
cytological
study
of
the
bone
marrow
.
Following
investigations
carried
out
in
each
case
,
we
mentioned
the
diagnosis
of
cutaneous
mastocytosis
,
and
also
excluded
several
diseases
confounded
by
clinically
and
histologically
aspect
.
Considering
the
fact
that
the
balance
expansion
was
negative
,
we
excluded
the
diagnosis
of
systemic
mastocytosis
.
The
presence
of
anemia
and
protein
energetic
malnutrition
in
children
with
mastocytosis
involves
carrying
out
balance
extension
for
the
exclusion
of
a
systemic
form
of
the
disease
.
Histopathological
examination
of
the
skin
using
special
stains
,
the
dosage
of
mediators
(
serum
tryptase
level
,
serum
histamine
levels
,
urinary
histamine
metabolites
)
and
balance
expansion
establish
the
diagnosis
of
cutaneous
mastocytosis
and
also
exclude
many
confusions
because
of
the
clinical
presentation
.
Diseases
Validation
Diseases presenting
"mastocytosis"
symptom
allergic bronchopulmonary aspergillosis
cutaneous mastocytosis
epidermolysis bullosa simplex
This symptom has already been validated