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[Mal de Meleda. 16 cases].
[papillon-lefèvre syndrome]
Mal
de
Meleda
is
a
recessive
transgression
palmoplantar
keratoderma
.
We
report
the
epidemiological
,
clinical
and
progressiveness
aspects
of
the
disease
in
16
patients
of
a
large
consanguineous
tunisian
family
.
The
age
ranged
from
3
to
90
years
.
Nine
were
male
and
7
female
patients
.
Onset
in
early
infancy
was
characterized
by
erythema
of
the
palms
and
soles
,
followed
by
a
diffuse
yellowish
hyperkeratosis
.
Thickness
of
the
dorsal
aspect
of
the
fingers
started
in
childhood
.
Extension
of
hyperkeratosis
to
the
dorsal
of
the
hand
were
reported
only
in
adults
and
usually
precede
the
involvement
of
the
feet
Palmoplantar
hyperhidrosis
with
fetid
odor
between
the
toes
was
frequently
seen
as
well
as
conical
distal
phalanges
.
In
some
adults
keratoderma
gave
contracture
of
the
fingers
leading
to
limitation
of
movements
Mal
de
Meleda
must
be
differentiated
from
other
recessive
palmoplantar
keratoderma
such
as
Papillon-
Lefévre
syndrome
and
Mal
de
Naxos
.
the
remaining
inherited
palmoplantar
keratoderma
being
autosomal
dominant
.
Mal
de
Meleda
is
a
rare
disease
but
is
still
relatively
frequent
in
some
tunisian
regions
,
due
to
the
high
rate
of
consanguinity
Mal
de
Meleda
remains
an
afflicting
and
disabling
disease
.
Diseases
Validation
Diseases presenting
"inherited palmoplantar keratoderma being autosomal"
symptom
papillon-lefèvre syndrome
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