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Inherited junctional epidermolysis bullosa (Herlitz type) in German black-headed mutton sheep.
[junctional epidermolysis bullosa]
This
report
describes
the
microscopical
,
immunohistochemical
and
ultrastructural
findings
in
the
first
ovine
cases
of
the
Herlitz
type
of
inherited
junctional
epidermolysis
bullosa
.
Sixteen
German
black-headed
mutton
lambs
and
one
crossbred
lamb
had
blisters
and
ulceration
of
the
skin
and
mucous
membranes
in
addition
to
alterations
of
the
horn
of
the
hooves
.
Microscopically
,
there
was
separation
of
the
dermoepidermal
junction
,
which
was
confirmed
to
be
located
in
the
lamina
lucida
of
the
basement
membrane
by
electron
microscopy
.
In
areas
of
subepidermal
splitting
the
hemidesmosomes
were
missing
and
in
adjacent
areas
they
appeared
to
be
rudimentary
and
reduced
in
number
.
Immunohistochemistry
for
laminin
5
revealed
a
markedly
reduced
expression
of
this
molecule
on
the
dermal
side
of
the
blisters
,
while
expression
of
collagen
VII
was
normal
.
Diseases
Validation
Diseases presenting
"markedly reduced expression of this molecule on the dermal side"
symptom
junctional epidermolysis bullosa
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