Rare Diseases Symptoms Automatic Extraction
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Eponym: Papillon-Lefevre syndrome.
[papillon-lefèvre syndrome]
Papillon-
Lefevre
Syndrome
(
PLS
)
is
a
very
rare
autosomal
recessive
disorder
characterized
by
palmoplantar
hyperkeratosis
and
severe
early
onset
periodontitis
,
affecting
the
primary
and
permanent
dentition
.
The
syndrome
was
first
described
by
Papillon
and
Lefevre
in
1924
.
Genetic
,
immunologic
,
and
microbiologic
factors
are
suggested
as
responsible
for
the
initiation
and
progression
of
the
disease
.
A
point
mutation
of
cathepsin
C
gene
has
recently
been
detected
in
PLS
.
A
multidisciplinary
approach
is
important
for
management
.
The
prognosis
has
improved
with
the
early
recognition
of
the
syndrome
,
effective
professional
supervision
,
and
home
care
.