Pemphigus vulgaris

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1. Pemphigus Vulgaris

2. What is it?
•This patient
complains of this
painful outbreak.
You tell them they
have
•A) Poor hygiene
•B) Bullous
Pemphigoid
•C) Pemphigus
Vulgaris
•D) Bullous Impetigo

3. Pemphigus Vulgaris
•Pemphigus
vulgaris is the
most common
form of pemphigus
•Oral lesions
usually precede
the skin blisters by
weeks to months
(80% present with
oral lesions first)

4. Pemphigus Vulgaris
•The primary
lesion is a
flaccid
blister/bullae
that easily
ruptures,
leaving erosions
and crusting,
and eventual
marked post-
inflammatory
changes

5. Pemphigus Vulgaris
•In severe cases
the oral epithelium
is completely
denuded and is
associated with
intraoral pain that
is particularly
worsened by
eating
•Infrequently other
mucous
membranes
maybe involved
(ocular and
genitourinary)

6. Pemphigus Vulgaris
•Nonpruritic skin
blisters varying
in size from 1 to
several cm’s
gradually
appear and
maybe localized
for a
considerable
time

7. Pemphigus Vulgaris
•The lesions
become
invariably more
generalized if
left untreated.
•It is usually
most
accentuated in
the
intertriginous
areas

8. Pemphigus Vulgaris
•The etiology is
from autoimmunity
to the pemphigus
vulgaris antigen
(desmoglein 3) a
member of the
cadherin family
and a normal
component of
human
keratinocyte cell
membranes

9. Pemphigus Vulgaris •DIF reveals IgG in
the intercellular
regions of the
epidermis in and
around the
affected parts of
the skin or mucous
membranes
•C3, IgM and IgA
are found much
less frequently
•Indirect IF studies
are positive and
can be used as an
indicator of
disease activity
(monkey
esophagus)

10. Pemphigus Vulgaris
•The basal cells
lose their
intercellular
bridges but they
remain attached to
the dermis, giving
a ‘tombstone
appearance’
•The blister cavity
usually contains a
few acantholytic
cells which often
show degenerative
changes

11. Diagnosis
•Clinical picture
•Skin biopsy for
light microscopy
•Skin biopsy for
direct IF studies
•Indirect IF
studies (levels
correlate with
disease activity)

12. Pemphigus Vegetans
•This is a variant of
P. Vulgaris in
which markedly
hyperplastic
erosive plaques
develop, primarily
in intertriginous
regions (groin and
axillary vaults)
•At times, such
lesions occur in
some patients with
longstanding
pemphigus
vulgaris

13. Pemphigus Vegetans
•P. Vegetans is
characterized by
flaccid bullae that
become erosions and
form fungoid
vegetations or
papillomatous
proliferations
especially in the body
folds
•The bullae rupture and
become exuberant
with verrucous
vegetations, capped
by crusts and
surrounded by a zone
of inflammation

14. Pemphigus
Other variants:
•P. erythematosus
•P. Foliaceous
•P. Vulgaris
•P. Vegetans

15. P. Vulgaris Treatment
Oral steroids
Azathioprine
Cyclosporine
Plasmapheresis
Rituximab