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Harlequin ichthyosis: a case report of prolonged survival.
[harlequin ichthyosis]
Harlequin
ichthyosis
(
HI
)
is
a
rare
type
of
congenital
ichthyosis
associated
with
poor
survival
.
We
report
,
with
photographic
record
,
a
male
baby
born
with
HI
.
To
the
best
of
our
knowledge
,
this
is
the
first
reported
case
of
HI
in
Saudi
Arabia
,
where
the
child
has
survived
beyond
7
years
.
The
baby
was
born
at
37
weeks
of
gestation
from
consanguineous
parents
with
no
inherited
skin
disorder
in
the
family
.
The
mother
was
28
years
old
with
three
normal
previous
pregnancies
and
healthy
babies
.
At
birth
,
the
baby
's
skin
had
thick
scales
separated
by
deep
fissuring
.
He
was
managed
in
intensive
care
with
supportive
treatment
and
frequent
application
of
lubricants
,
emollients
and
urea
cream
.
The
skin
gradually
became
softened
,
and
began
to
shed
after
6
weeks
.
After
8
months
of
inpatient
management
,
he
was
discharged
.
He
is
currently
7
years
old
and
is
still
being
treated
for
non-
bullous
congenital
ichthyosiform
erythroderma
.
Diseases
Validation
Diseases presenting
"type of congenital ichthyosis"
symptom
harlequin ichthyosis
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