Paraneoplastic
pemphigus
is
an
autoimmune
disorder
initiated
by
an
____________________
neoplasm
.
Tumor
antigens
are
hypothesized
to
evoke
both
a
humoral
____________________
a
cellular
immune
response
that
leads
to
blistering
in
____________________
and
other
epithelia
.
Affected
organ
systems
include
the
____________________
,
respiratory
tract
,
and
gastrointestinal
tract
.
A
patient
with
renal
and
neurologic
involvement
has
been
reported
.
Most
patients
have
had
an
associated
malignancy
,
often
a
lymphoproliferative
disorder
,
but
it
has
also
been
reported
to
____________________
in
association
with
benign
tumors
.
____________________
neoplasms
include
non
-
Hodgkin
lymphoma
,
chronic
lymphocytic
____________________
,
Castleman
disease
,
thymoma
,
Waldenström
macroglobulinemia
,
Hodgkin
lymphoma
,
various
carcinomas
,
sarcomas
,
and
malignant
melanoma
.
Passive
transfer
of
paraneoplastic
pemphigus
sera
____________________
blistering
in
neonatal
mice
,
proving
that
the
antibodies
present
are
pathogenic
.
Paraneoplastic
pemphigus
patients
exhibit
many
different
autoantibodies
to
proteins
of
the
plakin
family
,
____________________
intracellular
component
of
desmosomes
and
hemidesmosomes
,
including
envoplakin
(
210
-
kd
)
,
periplakin
(
190
-
kd
)
,
bullous
pemphigoid
antigen
I
(
230
-
kd
)
,
desmoplakin
I
(
250
-
kd
)
,
desmoplakin
II
(
210
-
kd
)
,
plectin
(
500
-
kd
)
,
and
alpha2
-
macroglobulin
-
like
?
1
(
170
-
kd
)
.
They
can
also
____________________
antibodies
to
antigens
associated
with
pemphigus
vulgaris
(
desmoglein
3
,
130
-
kd
)
and
pemphigus
foliaceus
(
desmoglein
1
,
160
-
kd
)
,
as
well
as
several
others
.
In
a
2011
review
,
Czernik
et
al
summarize
the
increasing
role
of
cellular
immunity
in
paraneoplastic
pemphigus
,
evidenced
by
lesional
mononuclear
cells
and
elevated
IL
-
6
levels
in
sera
from
paraneoplastic
____________________
patients
.
