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Papillon-lefèvre syndrome: a series of six cases in the same family.
[papillon-lefèvre syndrome]
Papillon-
Lefèvre
syndrome
(
PLS
)
is
a
rare
,
autosomal
recessive
heterogeneous
disorder
,
which
is
characterized
by
palmoplantar
hyperkeratosis
,
early
loss
of
primary
and
permanent
teeth
,
and
associated
calcification
of
the
dura
mater
.
Herein
we
described
six
cases
of
PLS
in
the
same
family
.
In
this
series
,
six
cases
(
two
females
and
four
males
)
with
the
mean
age
of
15
.
6
±
10
.
4
years
were
recruited
.
Palmoplantar
hyperkeratosis
was
detected
in
all
of
the
cases
,
leading
to
a
difficult
and
painful
walking
in
two
cases
due
to
lesions
on
the
soles
.
Skin
lesions
were
sharply
distinct
from
adjacent
normal
skin
in
all
cases
.
Other
skin
lesions
were
located
in
the
external
malleolus
(
5
/
6
)
,
knee
(
4
/
6
)
,
elbow
(
4
/
6
)
,
toe
and
dorsal
fingers
(
3
/
6
)
,
and
the
thighs
(
2
/
6
)
.
In
three
cases
,
all
permanent
teeth
were
exfoliated
.
In
three
others
,
no
primary
teeth
remained
.
Severe
gingivitis
was
observed
in
three
patients
.
Radiologic
study
confirmed
alveolar
bone
destruction
in
five
cases
.
Delayed
diagnosis
and
insufficient
treatment
of
PLS
patients
can
affect
patient
's
life
of
by
causing
edentulism
at
a
young
age
and
may
impose
PLS
patients
to
increased
risk
of
social
,
psychological
,
and
economical
burdens
.
Diseases
Validation
Diseases presenting
"early loss"
symptom
dentin dysplasia
papillon-lefèvre syndrome
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