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Cutaneous mastocytosis with atypical mast cells and giant cytoplasmic granules.
[cutaneous mastocytosis]
A
12
-
year
-old
male
presented
with
an
8
-
year
history
of
five
firm
cream
colored
papules
on
the
right
vertex
of
the
scalp
.
A
biopsy
showed
a
dense
infiltrate
of
monomorphous
mast
cells
involving
the
dermis
and
extending
into
the
subcutis
.
A
relatively
well-circumscribed
cluster
of
larger
cells
showed
pleomorphic
nuclei
with
bilobed
and
multilobed
morphology
.
Both
mast
cell
populations
had
an
eosinophilic
cytoplasm
filled
with
granules
ranging
in
size
from
small
to
giant
forms
.
By
immunohistochemistry
,
the
cells
expressed
CD
117
,
tryptase
and
CD
68
,
and
were
negative
for
AE
1
/
AE
3
,
CD
1
a
,
CD
2
and
CD
2
5
.
S-
100
staining
revealed
only
faint
cytoplasmic
positivity
and
myeloperoxidase
had
an
inhomogeneous
patchy
pattern
,
with
an
overall
staining
of
less
than
5
%
of
the
cells
.
A
diagnosis
of
cutaneous
mastocytosis
was
made
and
after
6
months
follow-up
,
no
progression
observed
.
Clinical
correlation
and
awareness
of
these
unusual
morphologic
features
as
being
part
of
the
spectrum
of
cutaneous
mastocytosis
are
important
to
avoid
an
erroneous
diagnosis
of
malignancy
.
Although
pleomorphic
,
multilobed
nuclear
morphology
and
giant
cytoplasmic
granules
have
not
been
associated
with
an
aggressive
behavior
or
systemic
mastocytosis
,
close
clinical
observation
is
warranted
in
this
context
.
Diseases
Validation
Diseases presenting
"colored papules on the right vertex of the scalp"
symptom
cutaneous mastocytosis
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