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[CLOVES syndrome: a malformational syndrome closely resembling Proteus syndrome].
[proteus syndrome]
CLOVES
syndrome
(
Congenital
Lipomatous
asymmetric
Overgrowth
of
the
trunk
with
lymphatic
,
capillary
,
venous
,
and
combined-
type
Vascular
malformations
,
Epidermal
nevi
,
Skeletal
anomalies
)
is
a
sporadic
malformational
syndrome
that
has
recently
been
described
(
mutation
of
PIK
3
CA
)
,
with
asymmetric
body
hypertrophy
,
lipomatous
hamartoma
of
the
trunk
and
numerous
malformations
.
We
report
a
case
of
CLOVES
syndrome
initially
diagnosed
as
Proteus
syndrome
,
revealed
by
infection
of
a
dorsal
lipomatous
hamartoma
.
The
patient
presented
with
both
right
lower
limb
hypertrophy
and
capillary
and
venous
-lymphatic
malformations
,
associated
with
dorsal
capillary
malformations
,
left
cervico-
facial
hypertrophy
,
and
cervical
epidermal
hamartoma
,
all
of
which
are
consistent
with
Proteus
syndrome
.
Imaging
of
the
dorsal
lipomatous
hamartoma
associated
with
capillary
as
well
as
underlying
venous
-lymphatic
malformations
and
syringomyelia
resulted
in
subsequent
correction
of
the
earlier
diagnosis
of
Proteus
syndrome
to
that
of
CLOVES
syndrome
.
Several
malformational
syndromes
are
associated
with
tissue
hypertrophy
,
hamartoma
and
vascular
malformations
.
Diagnosis
of
CLOVES
syndrome
may
be
delayed
due
to
its
fairly
close
phenotypic
similarity
to
Proteus
syndrome
.
Nevertheless
,
the
prognosis
and
complications
differ
.
Our
case
underlines
the
importance
of
considering
a
diagnosis
of
CLOVES
syndrome
in
the
presence
of
lipomatous
hamartoma
with
hemi-
hypertrophy
and
epidermal
hamartoma
,
in
order
to
enable
adequate
follow-up
with
specific
monitoring
for
the
possible
complications
associated
with
this
disease
.
Diseases
Validation
Diseases presenting
"capillary and venous-lymphatic malformations"
symptom
proteus syndrome
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