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Kindler syndrome.
[kindler syndrome]
Kindler
syndrome
is
a
rare
genodermatosis
characterized
by
acral
bullae
and
photosensitivity
.
The
photosensitivity
improves
with
advancing
age
and
results
in
progressive
poikiloderma
and
cutaneous
atrophy
,
and
many
additional
features
have
also
been
described
.
This
report
describes
two
male
Kindler
syndrome
patients
with
classical
features
of
acral
blistering
and
photosensitivity
in
childhood
,
and
subsequent
development
of
poikiloderma
,
leukokeratosis
of
oro-ano-
genital
mucosae
,
phimosis
and
meatal
stenosis
.
The
first
patient
had
additional
ophthalmic
features
of
chronic
simple
conjunctivitis
caused
by
persistent
irritation
,
multiple
stromal
nebular
corneal
opacities
and
thickened
corneal
nerves
.
The
second
patient
showed
skeletal
changes
,
namely
a
dome-shaped
skull
(
turri-cephaly
)
,
bifid
fourth
rib
,
missing
fifth
rib
,
short
fourth
and
fifth
metacarpals
and
mandibular
abnormalities
.
This
is
the
first
report
of
such
ophthalmic
and
skeletal
features
of
Kindler
syndrome
.