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Techniques for toddlers: linear band incision for harlequin ichthyosis with associated compartment syndrome.
[harlequin ichthyosis]
Harlequin
ichthyosis
(
HI
)
is
a
rare
autosomal
recessive
disorder
of
cornification
in
which
children
are
born
with
an
extremely
thick
stratum
corneum
that
becomes
a
restrictive
circumferential
encasement
around
the
orifices
,
limbs
,
chest
,
and
abdomen
,
resulting
in
limb
contractures
.
We
present
a
neonate
diagnosed
in
utero
with
HI
.
The
infant
was
born
with
encasing
bands
of
thickened
skin
creating
strictures
that
were
causing
digital
and
limb
cyanosis
(
compartment
syndrome
)
.
We
treated
the
child
using
a
new
technique
of
lysis
of
the
encasing
bands
that
we
call
linear
band
incision
,
using
a
new
escharotomy-like
procedure
while
the
infant
was
undergoing
a
3
-
week
course
oral
acitretin
therapy
.
The
technique
involved
linear
incision
and
lysis
of
encasements
that
resulted
in
reperfusion
of
the
injured
limbs
and
prevention
of
further
digital
necrosis
.
The
child
is
currently
a
healthy
8
-
year
-old
boy
with
skin
manifestations
resembling
congenital
ichthyosiform
erythroderma
.
He
has
use
of
all
of
the
limbs
that
were
released
in
the
procedures
and
is
maintained
on
frequent
application
of
bland
emollients
.
Linear
band
incision
is
a
potentially
life-
and
limb
-saving
technique
in
children
with
HI
.
Diseases
Validation
Diseases presenting
"skin manifestations"
symptom
child syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
familial mediterranean fever
harlequin ichthyosis
inclusion body myositis
kindler syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
sneddon syndrome
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