Rare Diseases Symptoms Automatic Extraction
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Adhesive stripping to remove epidermis in junctional epidermolysis bullosa for revertant cell therapy.
[junctional epidermolysis bullosa]
Replacing
mutant
skin
in
epidermolysis
bullosa
(
EB
)
by
epithelial
sheets
of
transduced
autologous
keratinocytes
is
the
essential
surgical
step
of
ex
vivo
gene
therapy
.
The
same
applies
for
revertant
cell
therapy
in
which
epithelial
sheets
of
revertant
autologous
keratinocytes
are
used
.
Revertant
cells
can
be
found
in
patches
of
normal
skin
in
patients
with
junctional
EB
(
JEB
)
due
to
revertant
mosaicism
caused
by
in
vivo
reversions
.
To
develop
a
technique
of
adhesive
tape
stripping
as
a
method
for
epidermis
removal
to
prepare
the
acceptor
site
for
revertant
cell
therapy
in
a
patient
with
revertant
mosaic
JEB
.
We
performed
revertant
cell
therapy
on
a
patient
with
mosaic
type
XVII
collagen-
deficient
non-
Herlitz
JEB
.
Skin
biopsies
were
taken
from
revertant
skin
on
the
wrist
.
Graft
production
took
place
on
a
3
T
3
-
J
2
feeder
layer
resulting
in
two
6
x
7
cm
grafts
.
An
innovative
method
that
uses
the
pathological
plane
of
least
resistance
of
JEB
skin
was
developed
to
prepare
the
acceptor
site
.
A
polyacrylate
adhesive
plaster
was
placed
on
the
skin
and
then
pulled
off
with
the
epidermis
.
The
epidermis
was
easily
removed
with
the
plaster
.
The
skin
separated
at
the
level
of
the
lamina
lucida
,
leaving
a
bloodless
wound
bed
of
naked
lamina
densa
.
Transplantation
was
successful
;
the
acceptor
site
healed
without
scarring
.
However
,
blistering
could
be
provoked
.
The
functional
repair
was
not
achieved
due
to
the
low
percentage
of
revertant
cells
in
the
graft
.
We
conclude
that
adhesive
stripping
is
a
simple
,
effective
and
almost
painless
procedure
for
removing
epidermis
for
ex
vivo
cell
therapy
in
EB
.
Diseases
Validation
Diseases presenting
"the low percentage of revertant cells in the graft"
symptom
junctional epidermolysis bullosa
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