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Periodontal disease associated to systemic genetic disorders.
[papillon-lefèvre syndrome]
A
number
of
systemic
disorders
increase
patient
susceptibility
to
periodontal
disease
,
which
moreover
evolves
more
rapidly
and
more
aggressively
.
The
underlying
factors
are
mainly
related
to
alterations
in
immune
,
endocrine
and
connective
tissue
status
.
These
alterations
are
associated
with
different
pathologies
and
syndromes
that
generate
periodontal
disease
either
as
a
primary
manifestation
or
by
aggravating
a
pre-existing
condition
attributable
to
local
factors
.
This
is
where
the
role
of
bacterial
plaque
is
subject
to
debate
.
In
the
presence
of
qualitative
or
quantitative
cellular
immune
alterations
,
periodontal
disease
may
manifest
early
on
a
severe
localized
or
generalized
basis--
in
some
cases
related
to
the
presence
of
plaque
and
/
or
specific
bacteria
(
severe
congenital
neutropenia
or
infantile
genetic
agranulocytosis
,
Chediak-
Higiashi
syndrome
,
Down
syndrome
and
Papillon-
Lefévre
syndrome
)
.
In
the
presence
of
humoral
immune
alterations
,
periodontal
damage
may
result
indirectly
as
a
consequence
of
alterations
in
other
systems
.
In
connective
tissue
disorders
,
bacterial
plaque
and
alterations
of
the
periodontal
tissues
increase
patient
susceptibility
to
gingival
inflammation
and
alveolar
resorption
(
Marfan
syndrome
and
Ehler-
Danlos
syndrome
)
.
The
management
of
periodontal
disease
focuses
on
the
control
of
infection
and
bacterial
plaque
by
means
of
mechanical
and
chemical
methods
.
Periodontal
surgery
and
even
extraction
of
the
most
seriously
affected
teeth
have
also
been
suggested
.
There
are
variable
degrees
of
consensus
regarding
the
background
systemic
disorder
,
as
in
the
case
of
Chediak-
Higiashi
syndrome
,
where
antibiotic
treatment
proves
ineffective
;
in
severe
congenital
neutropenia
or
infantile
genetic
agranulocytosis
,
where
antibiotic
prophylaxis
is
suggested
;
and
in
Papillon-
Lefévre
syndrome
,
where
an
established
treatment
protocol
is
available
.
Diseases
Validation
Diseases presenting
"seriously affected teeth"
symptom
papillon-lefèvre syndrome
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