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Initial expression of the Papillon-Lefevre syndrome in consanguine family.
[papillon-lefèvre syndrome]
A
rare
case
of
Papillon-
Lefevre
syndrome
is
discussed
with
clinicoradiological
presentation
.
The
purpose
of
the
case
report
is
to
make
the
medical
community
aware
of
this
rare
syndrome
and
its
association
with
consanguinity
.
Papillon-
Lefevre
syndrome
is
an
extremely
rare
genodermatosis
of
autosomal-recessive
inheritance
which
usually
manifests
itself
between
the
ages
of
6
months
to
4
years
characterised
by
diffuse
palmoplanter
hyperkeratosis
(
keratoderma
)
,
and
rapidly
progressive
and
devastating
periodontitis
,
affecting
the
primary
as
well
as
permanent
dentition
.
Papillon-
Lefevre
syndrome
results
from
a
combination
of
host
and
bacterial
factors
,
including
recessive
gene
,
consanguinity
,
specific
periodontal
pathogens
and
lack
of
thorough
oral
hygiene
.
The
present
case
report
describes
Papillon-
Lefevre
syndrome
and
its
association
with
consanguinity
in
a
3
-
year
-old
girl
.
