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Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature.
[cutaneous mastocytosis]
Diffuse
cutaneous
mastocytosis
(
DCM
)
is
an
extremely
rare
disease
characterized
by
mast
cell
(
MCs
)
infiltration
of
the
entire
skin
.
Little
is
known
about
the
natural
course
of
DCM
.
We
decided
to
characterize
clinical
manifestations
,
the
frequency
of
MCs
mediator-related
symptoms
and
anaphylaxis
,
risk
of
systemic
mastocytosis
(
SM
)
and
prognosis
,
based
on
10
cases
of
DCM
,
the
largest
series
published
to
date
.
Diffuse
cutaneous
mastocytosis
,
DCM
was
confirmed
by
histopathological
examination
of
skin
samples
in
all
cases
.
SCORing
Mastocytosis
(
SCORMA
)
Index
was
used
to
assess
the
intensity
of
DCM
.
The
analysis
of
clinical
symptoms
and
laboratory
tests
,
including
serum
tryptase
levels
was
performed
.
Bone
marrow
biopsy
was
done
only
in
selected
cases
.
Large
haemorrhagic
bullous
variant
of
DCM
(
five
cases
)
and
infiltrative
small
vesicular
variant
(
five
cases
)
were
identified
.
The
skin
symptoms
appeared
in
age-dependent
manner
;
blistering
predominated
in
infancy
,
whereas
grain-leather
appearance
of
the
skin
and
pseudoxanthomatous
presentation
developed
with
time
.
SM
was
not
recognized
in
any
of
the
patients
.
Mast
cell
mediator-related
symptoms
were
present
in
all
cases
.
Anaphylactic
shock
occurred
in
three
patients
.
Follow-up
performed
in
seven
cases
revealed
slight
improvement
of
skin
symptoms
,
reflected
by
decrease
of
SCORMA
Index
in
all
of
them
.
Serum
tryptase
levels
declined
with
time
in
six
cases
.
Diffuse
cutaneous
mastocytosis
,
DCM
is
a
heterogeneous
,
severe
,
cutaneous
disease
,
associated
with
mediator-related
symptoms
and
risk
of
anaphylactic
shock
.
Although
our
results
suggest
generally
favourable
prognosis
,
the
review
of
the
literature
indicate
that
SM
may
occur
.
Therefore
,
more
guarded
prognosis
should
be
given
in
DCM
patients
.
Diseases
Validation
Diseases presenting
"cutaneous mastocytosis"
symptom
cutaneous mastocytosis
This symptom has already been validated