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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
.
