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Papillon-Lefevre syndrome: clinical presentation and a brief review.
[papillon-lefèvre syndrome]
The
Papillon-
Lefevre
syndrome
(
PLS
)
was
first
described
by
Papillon
and
Lefevre
in
1924
.
It
is
an
autosomal
recessive
disorder
characterized
by
a
diffuse
palmoplanter
hyperkeratosis
and
rapidly
progressive
and
devastating
periodontitis
,
affecting
the
primary
as
well
as
the
permanent
dentition
,
attributed
to
a
point
mutation
of
the
cathepsin
C
gene
.
This
paper
presents
a
clinical
presentation
and
a
brief
review
of
its
etiology
and
treatment
modalities
.