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[Kindler syndrome. A new bullous dermatosis].
[kindler syndrome]
The
Kindler
syndrome
is
a
new
form
of
inherited
epidermolysis
bullosa
and
the
first
genodermatosis
caused
by
a
defect
of
the
focal
adhesions
.
Kindlin-
1
,
the
deficient
protein
,
plays
an
essential
role
in
integrin
activation
and
in
the
adhesion
of
keratinocytes
to
the
extracellular
matrix
.
The
adhesion
defect
leads
to
skin
blistering
which
begins
at
birth
and
ameliorates
with
age
,
and
to
mucosal
fragility
which
leads
to
scarring
and
stricture
formation
.
Skin
atrophy
and
poikiloderma
develop
progressively
.
Photosensitivity
is
rather
mild
,
but
squamous
cell
carcinomas
develop
on
sun-exposed
areas
mainly
after
the
age
of
40
years
.
The
most
important
differential
diagnoses
are
epidermolysis
bullosa
with
mottled
pigmentation
and
dystrophic
epidermolysis
bullosa
.
Management
aims
to
treat
the
symptoms
and
prevent
complications
.
