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Unusual protrusion of conjunctiva in two neonates with harlequin ichthyosis.
[harlequin ichthyosis]
We
present
two
patients
who
developed
severe
protrusion
of
the
conjunctiva
and
chemosis
secondary
to
Harlequin
ichthyosis
(
HI
)
.
Case
1
was
a
male
infant
diagnosed
with
HI
who
had
parchment-like
appearance
and
conjunctival
protrusion
with
severe
chemosis
.
Case
2
was
a
female
infant
on
whom
HI
had
been
suspected
before
birth
through
ultrasonography
.
She
showed
thickened
skin
over
the
entire
body
and
conjunctival
protrusion
with
severe
chemosis
.
For
both
cases
,
a
vitamin
A
derivative
was
applied
and
the
hyperkeratotic
layer
was
peeled
off
every
day
.
Great
care
was
taken
to
sterilize
and
moisten
the
ocular
surface
.
The
conjunctival
protrusion
gradually
improved
and
other
systemic
conditions
were
successfully
treated
.
HI
is
a
rare
condition
,
but
affected
infants
are
surviving
longer
than
previously
and
hence
guidelines
for
ocular
management
are
now
required
.
Gentle
and
patient
debridement
of
the
hyperkeratotic
skin
and
moisturizing
were
important
in
treating
the
unusual
conjunctival
protrusion
.
Diseases
Validation
Diseases presenting
"conjunctival protrusion with severe chemosis"
symptom
harlequin ichthyosis
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