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Pyogenic liver abscess and peritonitis due to Rhizopus oryzae in a child with Papillon-Lefevre syndrome.
[papillon-lefèvre syndrome]
Papillon-
Lefevre
syndrome
(
PLS
)
is
an
autosomal
recessive
disease
that
is
characterized
by
symmetric
palmoplantar
keratodermatitis
and
severe
periodontal
destruction
.
Mutations
in
the
cathepsin
C
gene
(
CTSC
)
have
recently
been
detected
in
PLS
.
Immune
dysregulation
,
due
to
a
mutation
in
CTSC
,
increases
the
risk
of
pyogenic
infections
in
PLS
patients
.
A
child
with
PLS
is
presented
here
with
liver
abscesses
and
peritonitis
caused
by
Rhizopus
oryzae
.
His
liver
abscess
and
peritonitis
were
cured
with
amphotericin
B
without
surgical
care
.
This
is
the
first
case
in
the
literature
liver
abscess
due
to
Rhizopus
oryzae
in
a
child
with
PLS
.
Diseases
Validation
Diseases presenting
"symmetric palmoplantar keratodermatitis"
symptom
papillon-lefèvre syndrome
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