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T-lymphocytes are directly involved in the clinical expression of migratory circinate erythema in epidermolysis bullosa simplex patients.
[epidermolysis bullosa simplex]
Epidermolysis
bullosa
simplex
with
migratory
circinate
erythema
(
EBS
-MCE
)
is
a
rare
EBS
subtype
characterised
by
migratory
blistering
lesions
that
resolve
with
brownish
pigmentation
.
It
is
caused
by
a
recurrent
readthrough
mutation
,
c
.
1649
delG
,
in
the
tail
of
keratin
5
.
Here
,
we
report
a
child
with
EBS
-MCE
and
investigated
the
immunologic
mechanisms
underlying
the
migratory
lesions
in
this
patient
.
A
skin
biopsy
from
the
patient
from
an
active
border
of
an
erythematous
lesion
was
used
for
the
immunohistochemical
characterisation
of
the
inflammatory
infiltrate
and
for
TUNEL
assay
to
detect
apoptotic
cells
.
We
found
abundant
CD
4
+
and
CD
8
+
T
lymphocytes
infiltrating
the
papillary
dermis
and
lining
the
dermal-epidermal
junction
.
A
number
of
these
cells
expressed
the
activation
marker
CD
69
.
CD
83
+
dendritic
cells
were
present
both
in
the
epidermis
and
papillary
dermis
.
Finally
,
TUNEL
staining
showed
apoptosis
of
basal
and
suprabasal
keratinocytes
.
These
findings
suggest
a
critical
role
of
the
cellular
immunity
in
determining
the
EBS
-MCE
phenotype
.
Diseases
Validation
Diseases presenting
"papillary dermis"
symptom
child syndrome
epidermolysis bullosa simplex
junctional epidermolysis bullosa
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