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Symptomatic response to imatinib mesylate in cutaneous mastocytosis associated with chronic myelomonocytic leukemia.
[cutaneous mastocytosis]
Mastocytosis
is
an
uncommon
disorder
defined
by
increased
and
abnormal
mast
cells
in
one
or
more
tissues
.
Cutaneous
mastocytosis
(
cm
)
is
limited
to
the
skin
,
with
varying
degrees
of
rash
,
pruritus
,
and
disfigurement
.
Systemic
mastocytosis
(
sm
)
typically
involves
the
bone
marrow
,
sometimes
in
association
with
other
bone
marrow
disorders
,
including
chronic
myelomonocytic
leukemia
(
cmml
)
.
Mastocytosis
has
been
associated
with
somatic
mutations
in
the
gene
encoding
the
tyrosine
kinase
Kit
,
leading
to
identification
of
Kit
as
a
therapeutic
target
.
The
Kit
inhibitor
imatinib
mesylate
is
approved
for
aggressive
sm
.
We
present
an
unusual
patient
with
disabling
pruritus
from
telangiectasia
macularis
eruptiva
perstans
,
a
subtype
of
cm
,
and
cmml
,
but
with
no
evidence
of
systemic
mast
cell
disease
.
She
was
treated
with
imatinib
and
experienced
marked
improvement
in
her
pruritus
.
Concomitant
cm
and
cmml
have
not
previously
been
reported
,
and
the
present
report
is
the
first
of
successful
imatinib
therapy
in
an
adult
patient
with
cm
.
Diseases
Validation
Diseases presenting
"somatic mutations in the gene encoding the tyrosine kinase kit"
symptom
cutaneous mastocytosis
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