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Dystrophic epidermolysis bullosa in a child.
[dystrophic epidermolysis bullosa]
Epidermolysis
Bullosa
(
EB
)
is
a
form
of
severe
skin
adhesion
defect
due
to
the
disruption
of
the
dermal-
epidermal
junction
.
It
is
classified
into
simplex
and
dystrophic
forms
depending
on
the
level
at
which
the
junction
is
compromised
.
Repeated
ulcerations
and
bullae
formation
in
the
mouth
lead
to
scarring
that
brings
about
various
changes
in
the
oral
cavity
.
These
include
loss
of
sulcular
depth
,
ankyloglossia
,
limited
mouth
opening
and
other
dentoalveolar
changes
.
At
present
while
there
is
no
cure
for
EB
,
the
therapeutic
approaches
are
essentially
aimed
at
controlling
the
infections
and
maintaining
an
acceptable
quality
of
life
.
Dental
management
should
aim
at
maintaining
a
functional
dentition
that
would
help
in
mastication
and
favour
nutrition
.
Oral
manifestations
and
dental
management
in
a
child
diagnosed
with
dystrophic
EB
since
birth
are
presented
here
.
Diseases
Validation
Diseases presenting
"oral cavity"
symptom
cowden syndrome
dedifferentiated liposarcoma
dentinogenesis imperfecta
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
oral submucous fibrosis
papillon-lefèvre syndrome
pleomorphic liposarcoma
proteus syndrome
sneddon syndrome
well-differentiated liposarcoma
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