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[Anaesthesia for children with epidermolysis bullosa].
[kindler syndrome]
Epidermolysis
bullosa
(
EB
)
is
a
heterogeneous
group
of
inherited
rare
diseases
,
which
are
characterized
by
trauma-induced
blister
formation
of
the
skin
and
mucosa
.
The
underlying
cause
is
a
functional
deficiency
of
structural
proteins
of
the
epidermis
or
the
dermis
.
Depending
on
the
level
of
the
blister
formation
,
EB
is
divided
into
EB
simplex
(
intra-epidermal
)
,
junctional
EB
(
within
the
lamina
lucida
)
,
dystrophic
EB
(
below
the
lamina
lucida
)
and
Kindler
syndrome
(
variable
level
of
split
formation
)
.
Besides
different
distinct
blister
formation
and
pain
symptoms
secondary
problems
like
anaemia
,
oesophageal
stenosis
,
cardiomyopathy
or
squamous
cell
carcinoma
may
occur
.
Since
causal
therapies
are
not
available
strict
prevention
of
friction
and
trauma
is
essential
to
avoid
blister
formation
.
Anaesthesia
challenges
exist
in
the
field
of
bedding
procedures
,
care
of
the
skin
,
monitoring
,
airway
management
und
analgesia
.
This
article
gives
a
review
over
the
EB
and
highlights
in
detail
the
corresponding
anaesthesia
characteristics
.
