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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
.
Diseases
Validation
Diseases presenting
"ichthyosis"
symptom
child syndrome
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
harlequin ichthyosis
hirschsprung disease
junctional epidermolysis bullosa
kallmann syndrome
lamellar ichthyosis
This symptom has already been validated