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Do mutations in the basement membrane zone affect the human periodontium? Review with special reference to epidermolysis bullosa.
[kindler syndrome]
Epidermolysis
bullosa
(
EB
)
is
a
group
of
diseases
characterized
by
the
development
of
blisters
or
erosions
following
minor
trauma
to
the
skin
.
Oral
findings
that
have
been
associated
with
EB
include
keratin-filled
cysts
;
blistering
of
the
mucosa
,
tongue
,
and
lips
;
perioral
carcinomas
;
ankyloglossia
;
lingual
papilla
atrophy
;
caries
;
enamel
hypoplasia
;
rapid
attrition
of
the
teeth
;
and
obliteration
of
the
oral
vestibule
.
Defects
at
the
basal
cell
/
basement
membrane
/
connective
tissue
levels
correspond
to
the
mutations
in
basal
cell
keratins
,
hemidesmosome
components
,
and
type
VII
collagen
,
respectively
.
In
a
number
of
types
of
EB
,
structural
defects
in
the
skin
have
been
shown
and
genetic
mutations
determined
.
Although
there
are
no
publications
documenting
the
prevalence
of
periodontal
diseases
in
patients
with
epidermolysis
bullosa
,
it
is
likely
that
some
molecular
defects
in
the
basement
membrane
zone
could
increase
the
susceptibility
of
a
patient
to
periodontal
disease
,
as
this
has
been
noted
in
the
related
disorder
of
Weary-
Kindler
syndrome
.
Early
-onset
periodontal
disease
can
be
expected
to
develop
in
some
types
of
EB
patients
,
even
in
the
absence
of
common
periodontal
pathogens
,
because
of
a
reduced
resistance
at
the
junctional
epithelial
complex
.
Diseases
Validation
Diseases presenting
"susceptibility of a patient"
symptom
kindler syndrome
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