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Papillon-Lefévre syndrome: a highly-suspected case.
[papillon-lefèvre syndrome]
Papillon-
Lefévre
syndrome
(
PLS
)
is
characterized
by
severe
periodontal
disease
extending
to
destruction
of
the
alveolar
bone
surrounding
deciduous
teeth
and
palmoplantar
hyperkeratosis
of
the
skin
.
Increased
susceptibility
to
infection
has
been
reported
among
individuals
with
the
cathepsin
C
(
CTSC
)
gene
mutation
.
This
article
reports
a
7
-
year
-old
Japanese
girl
who
presented
with
deciduous
tooth
mobility
and
was
diagnosed
as
having
PLS
.
Radiographic
examination
revealed
loosening
of
deciduous
incisors
and
the
right
second
molar
of
the
maxilla
,
and
destruction
of
the
alveolar
bone
around
the
residual
deciduous
dentition
.
However
,
there
was
no
destruction
of
the
alveolar
bone
around
the
permanent
molars
.
The
patient
did
not
show
the
typical
signs
of
CTSC
polymorphism
,
which
almost
always
negatively
impacts
certain
activating
enzymes
.
With
respect
to
immune
function
,
analysis
of
the
patient
's
leukocytes
indicated
that
H
(
2
)
O
(
2
)
,
chemotactic
and
phagocytotic
functions
were
within
the
normal
range
.
However
,
the
special
precautions
normally
applied
to
prevent
infections
in
PLS
patients
undergoing
dental
treatment
were
taken
.
