Rare Diseases Symptoms Automatic Extraction
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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
.