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Lamellar ichthyosis presenting as bilateral spontaneous corneal perforation.
[lamellar ichthyosis]
Lamellar
Ichthyosis
is
an
autosomal
recessive
,
inherited
skin
disorder
characterized
by
thickening
,
fissuring
and
scaling
of
the
skin
.
To
report
a
case
of
lamellar
icthyosis
and
highlight
the
importance
of
monitoring
corneal
health
in
these
patients
.
We
report
a
rare
case
of
bilateral
spontaneous
corneal
perforation
in
a
patient
with
lamellar
ichthyosis
.
The
patient
presented
with
complaints
of
diminution
of
vision
,
foreign
body
sensation
,
watering
and
discharge
in
both
of
his
eyes
for
the
last
three
months
followed
by
pain
and
redness
for
one
week
.
Visual
acuities
were
light
perception
in
both
the
eyes
.
Cicatricial
ectropion
was
seen
in
both
the
lower
eyelids
.
Corneal
perforation
was
seen
in
both
the
eyes
.
Lamellar
ichthyosis
was
diagnosed
on
the
basis
of
scaling
and
excessive
dryness
of
the
entire
body
skin
and
was
confirmed
by
skin
biopsy
.
Peneterating
keratoplasty
of
both
eyes
was
done
with
cataract
surgery
of
the
right
.
The
systemic
anti-
ichthyosis
therapy
was
started
.
Ectropion
of
the
right
eye
was
corrected
,
and
on
follow-up
at
three
months
,
the
patient
had
a
visual
acuity
of
4
/
60
and
CFCF
in
the
right
and
the
left
eye
respectively
.
In
patients
with
cicatricial
ectropion
and
dry
eye
secondary
to
ichthyosis
,
corneal
health
should
be
closely
monitored
because
of
the
perforation
risk
.
