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Hand surgery for dystrophic epidermolysis bullosa.
[dystrophic epidermolysis bullosa]
Epidermolysis
bullosa
(
EB
)
is
a
group
of
inherited
,
mechanobullous
disorders
caused
by
mutations
in
various
structural
proteins
in
the
skin
.
The
manifestation
of
these
disorders
in
the
hand
is
of
digital
contractures
and
pseudosyndactyly
or
"
cocoon
hands
,
"
causing
significant
functional
impairment
.
Our
preferred
surgical
treatment
of
these
patients
involves
separation
of
the
digits
from
the
palm
by
releasing
the
finger
flexion
contractures
and
separating
them
,
primarily
the
adducted
thumb
.
However
,
recurrence
is
common
.
Our
hypothesis
was
that
functional
improvement
is
gained
irrespective
of
recurrence
of
contractures
.
We
retrospectively
evaluated
4
patients
,
2
male
and
2
female
,
whose
average
age
was
11
years
,
treated
surgically
by
the
separation
of
all
their
digits
and
by
coverage
with
skin
grafts
.
The
follow-up
period
was
between
1
and
3
½
years
.
Partial
recurrence
of
the
deformity
was
observed
in
all
patients
.
Recurrence
was
more
pronounced
in
the
nondominant
hand
,
especially
between
the
digits
and
of
flexion
contractures
,
but
did
not
preclude
the
use
of
precision
or
oppositional
pinch
at
final
follow-up
.
The
patient
with
the
longest
follow-up
has
been
referred
for
revision
surgery
to
gain
further
release
of
contractures
.
Significant
rehabilitation
goals
were
achieved
in
all
4
patients
after
surgery
.
After
6
months
,
both
of
the
younger
patients
were
measured
for
finger
dexterity
,
which
showed
lower
scores
than
the
norm
,
although
this
was
felt
to
be
dependent
on
which
daily
manual
activities
they
were
more
familiar
with
.
These
tests
could
not
have
been
performed
before
surgery
.
All
patients
and
families
felt
the
effort
was
worthy
.
Separating
the
thumb
and
straightening
the
digits
was
found
to
be
significant
,
yet
the
indication
for
separating
all
the
digits
is
debatable
.
The
need
for
revision
surgery
,
to
maintain
the
digit
function
,
is
clear
.
Level
4
,
case
series
.
Diseases
Validation
Diseases presenting
"significant functional impairment"
symptom
dystrophic epidermolysis bullosa
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