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Oral manifestion of Langerhans cell histiocytosis mimicking inflammation.
[papillon-lefèvre syndrome]
Langerhans
cell
histiocytosis
(
LCH
)
is
a
rare
idiopathic
disease
characterized
by
the
clonal
proliferation
of
Langerhans
cells
.
LCH
affects
five
children
per
million
population
.
The
peak
incidence
is
from
1
to
4
years
of
age
.
LCH
involves
the
head
and
neck
region
quite
commonly
.
Oral
soft
tissue
lesions
are
also
common
.
The
differential
diagnosis
of
oral
LCH
includes
leukemia
,
neutropenia
,
prepubertal
periodontitis
,
hypophosphatasia
,
fibrous
dysplasia
,
and
Papillon-
Lefevre
syndrome
.
The
prognosis
of
LCH
depends
on
early
detection
and
appropriate
management
.
Surgical
management
alone
is
used
in
50
%
of
cases
with
an
additional
23
%
of
the
lesions
being
treated
with
both
surgery
and
radiation
therapy
.
A
case
of
LCH
in
a
6
-
year
-old
girl
involving
the
mid
root
level
of
developing
first
permanent
molar
with
a
floating
developing
tooth
bud
of
permanent
second
molar
mimicking
an
inflammation
is
reported
.
Diseases
Validation
Diseases presenting
"early detection"
symptom
22q11.2 deletion syndrome
acute rheumatic fever
adrenomyeloneuropathy
allergic bronchopulmonary aspergillosis
carcinoma of the gallbladder
child syndrome
cholangiocarcinoma
classical phenylketonuria
congenital diaphragmatic hernia
cowden syndrome
cystinuria
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal squamous cell carcinoma
fabry disease
homocystinuria without methylmalonic aciduria
inclusion body myositis
kallmann syndrome
krabbe disease
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pyomyositis
von hippel-lindau disease
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