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Papillon-Lefevre syndrome: Reporting consanguinity as a risk factor.
[papillon-lefèvre syndrome]
Papillon-
Lefevre
syndrome
(
PLS
)
is
an
autosomal
recessive
genetic
disorder
characterized
by
palmoplantar
hyperkeratosis
associated
with
severe
early
-onset
periodontitis
and
premature
loss
of
primary
and
permanent
teeth
.
This
report
describes
two
cases
of
PLS
in
28
-
year
-old
female
and
16
-
year
-old
male
siblings
with
consanguineously
married
parents
.
The
patients
presented
to
the
Department
of
Public
Health
Dentistry
of
a
dental
education
and
research
institute
in
India
with
thickening
,
flaking
,
and
scaling
of
the
skin
on
the
palms
and
soles
of
the
feet
.
On
oral
examination
,
the
female
patient
presented
completely
resorbed
maxillary
and
mandibular
alveolar
ridges
with
retention
of
only
the
third
molars
.
The
male
patient
retained
only
teeth
18
,
13
,
28
,
38
,
and
45
.
Based
on
complete
histories
and
clinical
examination
findings
,
a
final
diagnosis
of
PLS
was
made
and
treatment
was
initiated
using
an
interdisciplinary
dental
approach
in
both
cases
.
Diseases
Validation
Diseases presenting
"only teeth"
symptom
papillon-lefèvre syndrome
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