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Combined therapy in a patient with Papillon-Lefèvre syndrome: a 13-year follow-up.
[papillon-lefèvre syndrome]
Papillon-
Lefèvre
syndrome
(
PLS
)
is
an
autosomal
recessive
disease
characterized
by
hyperkeratosis
of
the
palms
and
soles
combined
with
premature
loss
of
the
primary
and
permanent
dentition
.
Several
treatment
regimens
have
been
recommended
in
the
literature
;
however
,
a
definitive
treatment
protocol
has
not
been
established
.
This
case
report
evaluates
the
success
of
combined
therapy
in
managing
a
patient
with
PLS
.
A
6
-
year
-old
girl
diagnosed
with
PLS
presented
with
aggressive
periodontal
destruction
of
her
primary
and
permanent
dentitions
.
After
extraction
of
periodontally
affected
teeth
,
the
edentate
region
was
rehabilitated
with
different
temporary
dentures
until
her
skeletal
growth
was
complete
.
At
the
same
time
,
her
orthodontic
treatment
was
performed
.
The
early
loss
of
her
incisors
resulted
in
inadequate
alveolar
bone
height
and
width
for
esthetic-
advanced
prosthetic
rehabilitation
.
Alveolar
bone
augmentation
was
performed
,
and
6
months
later
,
two
intraosseous
dental
implants
were
placed
into
the
central
incisor
zone
.
After
13
years
of
treatment
and
follow-up
,
the
patient
had
periodontally
healthy
permanent
dentition
.
She
had
practiced
meticulous
oral
hygiene
,
and
the
orthodontic
treatment
was
successful
and
without
incident
.
Alveolar
ridge
augmentation
and
placement
of
an
intraosseous
implant
with
guided
bone
regeneration
were
performed
successfully
.
This
case
report
demonstrates
that
individually
developed
treatment
protocols
can
provide
long
-term
dental
/
periodontal
success
in
patients
with
PLS
.
A
multidisciplinary
approach
with
advanced
periodontal
surgery
,
orthodontic
and
prosthetic
treatment
,
and
implant
therapy
may
be
an
appropriate
treatment
modality
for
dental
rehabilitation
in
patients
with
PLS
.
Diseases
Validation
Diseases presenting
"permanent dentitions"
symptom
papillon-lefèvre syndrome
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