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Papillon-Lefèvre syndrome: Case report and review of the literature.
[papillon-lefèvre syndrome]
Papillon-
Lefèvre
syndrome
is
a
very
rare
syndrome
of
autosomal
recessive
inheritance
characterized
by
palmar
-
plantar
hyperkeratosis
and
early
onset
of
a
severe
destructive
periodontitis
leading
to
premature
loss
of
both
primary
and
permanent
dentitions
.
Various
etiopathogenic
factors
are
associated
with
the
syndrome
;
but
a
recent
report
has
suggested
that
the
condition
is
linked
to
mutations
of
the
cathepsin
C
gene
.
Two
cases
of
Papillon-
Lefèvre
syndrome
in
the
same
family
,
having
all
of
the
characteristic
features
are
presented
.
An
11
-
year
-old
girl
,
and
her
elder
sister
,
a
13
-
year
-old
girl
complained
of
loose
teeth
and
discomfort
in
chewing
along
with
recurrently
swollen
and
friable
gums
.
Both
patients
also
had
premature
shedding
of
their
deciduous
teeth
.
The
family
history
revealed
consanguineous
marriage
of
the
parents
.
Both
patients
presented
with
persistent
thickening
,
flaking
and
scaling
of
the
skin
of
palms
and
soles
.
Severe
generalized
periodontal
destruction
with
mobility
of
teeth
was
evident
on
intraoral
examination
;
orthopantomograph
examination
showed
severe
generalized
loss
of
alveolar
bone
in
both
the
patients
.
