Pemphigus is a potentially life threatening autoimmune disease that causes blisters and erosions of the skin and the mucous membrane.

1. Pemphigus
Dr Shrikant Phatak
Consultant & Head of ENT Deptt
Choithram Hospital Indore

2. Pemphigus
1. A wide number of dermatological conditions
can affect the oral cavity. Pemphigus is one of
them.
2. Pemphigus vulgaris (PV) is an autoimmune
disease causing blisters and erosions of the
skin and mucous membranes associated with
intraepithelial acantholysis.
3. When only mucosal involvement is present it
may be difficult to diagnose.

3. Pemphigus
1. The oral cavity may be the only site of
involvement for a year or so, and this may lead
to delayed diagnosis and inappropriate
treatment of a potentially fatal disorder
2. Such patients may first present to ENT Surgeon
with the symptoms of burning sensation,
painful oral ulceration dysphagia, and
hoarseness of voice.
3. Hence ENT surgeon must be aware of the
clinical manifestations of pemphigus vulgaris.

4. Pemphigus
• . Oral blisters have a thin roof, which readily
rupture in the face of trauma, leading to
multiple chronic painful ulcers, bleeding
ulcers, and erosions that heal with difficulty.
• Antibodies against specific proteins in the skin
and mucous membranes called desmogleins
are responsible for Acantholysis.

5. Pemphigus
The lesions most commonly start in areas of
frictional trauma such as the buccal mucosa
along the occlusal plane, followed by palate,
gingiva, pharynx, larynx, oesophagus,

6. Pemphigus
Painful ulcers with irregular,
ragged margins seen on the
lower lip and labial mucosa,
and bilateral buccal mucosa
extending posteriorly up to the
retro molar trigon
Lower lip showing ruptured
blister with erosion,
the dorsum of the tongue has
a thick, diffuse, white coating,
while the buccal mucosae
showed erosions with
pseudomembrane formation [

7. Pemphigus
The classic lesion of
pemphigus is a thin-
walled bulla arising on
otherwise normal skin or
mucosa, which rapidly
breaks and continues to
extend peripherally,
eventually leaving large
denuded areas.

8. Pemphigus
• Nikolsky's sign is
pathognomic for
pemphigus and is
elicited by applying
lateral or tangential
pressure to the
mucosa or skin in the
peri-lesional area
resulting in shearing
away of the epidermis
in the normal areas.

9. Pemphigus
Gingiva is the least
commonly affected site
Desquamative gingivitis is
the manifestation of the
disease when gingiva is
affected.

10. Pemphigus
Histopathological features
include intraepithelial
acantholysis and presence
of Tzanck cells.

11. Pemphigus
Corticosteroid on a long term
basis in high doses play the
main role in treatment.
Azathioprim ,Methotrexate
Rituximab also play a role
depending on individual
case.

12. Disclaimer
• The information contained in the presentation is based on the personal
experience and cases collected at Choithram Hospital Indore over the last
20 years.
• It is intended for the use of Medical students. ENT postgraduates
Dermatology students.
• The views expressed are purely on personal opinion. viewers can make
their own opinion. For any confusion please contact sole author.
• Everybody is allowed to copy or download the material best suited to him.
I am not responsible for any controversies arising out of the presentation.
• For any suggestions or corrections you may please contact
phatak_shrikant@yahoo.in

13. • Thank you