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Harlequin ichthyosis: a case report.
[harlequin ichthyosis]
Harlequin
Ichthyosis
(
HI
)
is
a
very
rare
subtype
of
a
group
of
hereditary
disorders
that
have
in
common
the
accumulation
and
shedding
of
significant
amounts
of
hyperkeratotic
epidermis
.
HI
has
a
very
specific
clinical
presentation
that
includes
eclabium
,
fish
mouth
appearance
,
mouth
breathing
and
xerostomia
.
The
addition
of
a
very
high
caloric
demand
to
the
clinical
features
place
these
affected
individuals
at
extremely
high
risk
for
developing
rampant
dental
decay
.
This
case
report
discusses
a
two
-
year
-old
boy
with
HI
that
presents
with
severe
early
childhood
caries
.
The
specific
accommodations
that
were
made
to
accomplish
required
dental
work
under
general
anesthesia
are
presented
.
A
case
is
made
for
the
inclusion
of
dentists
in
the
management
of
these
cases
once
a
diagnosis
is
made
.
