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Orthodontic treatment considerations for a patient with erythropoietic protoporphyria.
[erythropoietic protoporphyria]
Erythropoietic
protoporphyria
(
EPP
)
is
an
inherited
blood
disorder
in
which
formation
of
the
heme
group
of
hemoglobin
is
defective
.
Specifically
,
a
deficiency
of
the
enzyme
ferrochelatase
leads
to
the
accumulation
of
protoporphyrin
,
resulting
in
often
painful
photosensitivity
of
the
skin
and
tissues
.
The
prevalence
of
EPP
is
estimated
at
1
:
75
,
000
to
1
:
200
,
000
.
Photosensitivity
is
exhibited
upon
exposure
to
light
with
specific
wavelengths
through
the
creation
of
reactive
oxygen
products
(
oxidants
)
,
activation
of
the
complement
system
,
and
mast
cell
degranulation
.
The
aim
of
this
article
is
to
report
the
orthodontic
treatment
of
an
11
-
year
-old
boy
with
EPP
,
a
Class
III
skeletal
relationship
,
and
an
anterior
crossbite
.
Orthodontic
treatment
established
normal
overbite
and
overjet
.
Short
-term
periodontal
and
dental
tissue
responses
to
treatment
were
noted
.
Extra
care
was
needed
when
collecting
photographic
and
radiographic
records
for
this
patient
and
during
some
treatment
procedures
to
avoid
causing
a
photosensitive
reaction
of
the
skin
or
oral
mucosa
.
Diseases
Validation
Diseases presenting
"resulting in often painful photosensitivity of the skin and tissues"
symptom
erythropoietic protoporphyria
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