Rare Diseases Symptoms Automatic Extraction
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A case of cutaneous mastocytosis in a child with prominent Langerhans cell infiltration.
[cutaneous mastocytosis]
We
recently
encountered
a
2
-
year
-old
boy
with
slightly
infiltrative
brown
papules
on
the
face
,
trunk
,
and
extremities
.
Stroking
of
one
of
the
papules
produced
an
urticarial
wheal
(
positive
Darier
's
sign
)
.
Histopathologic
tests
revealed
a
dense
infiltration
of
mast
cells
containing
numerous
granules
and
showing
metachromasia
under
Toluidine
blue
staining
.
Immunohistochemical
tests
revealed
that
these
cells
were
positive
for
CD
68
and
for
c-kit
.
In
addition
,
dermal
dendritic
cells
that
were
positive
for
S
100
and
CD
1
a
immunostaining
were
intermingled
with
the
mast
cells
.
We
confirmed
through
electron
microscopy
that
the
dermal
dendritic
cells
that
were
observed
adjacent
to
the
infiltration
of
mast
cells
had
Birbeck
granules
in
their
cytoplasm
,
namely
Langerhans
cells
.
However
,
because
of
the
greater
numbers
of
mast
cells
than
Langerhans
cells
,
and
because
of
the
absence
of
both
monomorphic
LC
proliferation
and
systemic
symptoms
of
Langerhans
cell
histiocytosis
,
the
present
case
favors
a
diagnosis
of
cutaneous
mastocytosis
in
a
child
with
Langerhans
cell
infiltration
.
