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Epidermal nevus syndromes.
[child syndrome]
The
term
"
epidermal
nevus
syndrome
"
(
ENS
)
has
been
used
to
describe
the
association
of
epidermal
hamartomas
and
extra
-
cutaneous
abnormalities
.
Epidermal
nevi
follow
the
lines
of
Blaschko
.
The
majority
of
the
extra
-
cutaneous
manifestations
involve
the
brain
,
eye
,
and
skeletal
systems
.
Several
subsets
with
characteristic
features
have
been
delineated
including
the
nevus
sebaceous
syndrome
,
Proteus
syndrome
,
CHILD
syndrome
,
Becker
nevus
syndrome
,
nevus
comedonicus
syndrome
,
and
phakomatosis
pigmentokeratotica
.
Epidermal
nevi
have
been
associated
with
benign
and
malignant
neoplasms
.
Advances
in
molecular
biology
have
revealed
that
the
manifestations
of
ENS
are
due
to
genomic
mosaicism
.
It
is
likely
that
the
varied
clinical
manifestations
of
ENS
are
due
in
great
part
to
the
functional
effects
of
specific
genetic
defects
.
Optimal
management
of
the
patient
with
ENS
involves
an
interdisciplinary
approach
.
Amelioration
of
the
cutaneous
features
of
ENS
has
been
difficult
but
there
have
been
advances
,
especially
in
the
use
of
lasers
.
Diseases
Validation
Diseases presenting
"nevus syndrome"
symptom
child syndrome
cowden syndrome
malignant atrophic papulosis
oculocutaneous albinism
proteus syndrome
sneddon syndrome
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