SlideShare utilise les cookies pour améliorer les fonctionnalités et les performances, et également pour vous montrer des publicités pertinentes. Si vous continuez à naviguer sur ce site, vous acceptez l’utilisation de cookies. Consultez nos Conditions d’utilisation et notre Politique de confidentialité.
SlideShare utilise les cookies pour améliorer les fonctionnalités et les performances, et également pour vous montrer des publicités pertinentes. Si vous continuez à naviguer sur ce site, vous acceptez l’utilisation de cookies. Consultez notre Politique de confidentialité et nos Conditions d’utilisation pour en savoir plus.
“A CASE REPORT”
INDIAN DENTAL ACADEMY
Leader in continuing Dental Education
Pemphigus is derived from the Greek word
‘Pemphix’ meaning bubble or a blister .
Pemphigus describes a group of chronic
bullous diseases, Originally named by
‘Wichman’ in 1791.
Pemphigus refers to a group of
autoimmune blistering diseases of
the skin and mucous membrane
characterized histologically by
intradermal blisters and
immunopathologically by the
finding of in vivo bound and
circulating immunoglobulin G
(IgG) antibody directed against the
cell surface of keratinocytes.
Indian Dental academy
• Leader continuing dental education
• Offer both online and offline dental courses
• Pemphigus Vulgaris has been reported to occur
world wide, incidence varies from 0.5 to 5 cases
(Langan SM et al).
Male to female ratio is approximately equal .
Mean age of onset is 40-70 years of age .
(Turki Hamida et al)
As the Oral manifestations in Pemphigus
occur much before the skin lesions , Dental
Professionals must be sufficiently familiar
with Clinical manifestations for early
Diagnosis and Management.
A 48 year old male patient
reported to the our OPD for the
treatment of recurring
ulcerations in his mouth at
different sites since 1 year.
Burning sensation on eating spicy
He also complained of
appearance of blisters that came
into existence after the oral
ulcerations, involving the legs,
scalp and back.
The Patients past Medical and Drug history
were non contributory and there was no
significant Medical Problem.
Patient had no adverse habits .
The Vital signs were within normal limits.
Healed Scars of the blisters were seen on the scalp and nose
Nikolskys sign was POSITIVE
Patient wasPatient was moderately built and nourishedmoderately built and nourished
and weighed 85 kgs.and weighed 85 kgs.
Patient wasPatient was well oriented , conscious andwell oriented , conscious and
Denuded blisters of various sizes ranging from the size
0.25x0.25 cm to 0.5cmx0.5cms were seen on the lower lip,
legs and back.
Central erythmatous zone with epidermal crusting at the
periphery was seen on the blisters.
LESION ON THE LEG LESION ON THE LIP
LESIONS ON THE SCALP LESIONS ON THE BACK
No eye involvement was seen.
INTRA ORAL EXAMINATION
Shallow ulcers of the size
ranging from 5cmsx3cms to
4cmsx2cms were present on
the buccal mucosa of the left
side, extending from
premolar region till the
The ulcers had irregular
borders with mild erythema
around the periphery.The
surface was covered with
One hemorrhagic bulla
of the size 3cms x 2cms
was present on the buccal
mucosa opposite the 1st
Other parts of the oral
cavity were not involved.
Generalized attrition was
present with moderate
stains and calculus
The ulcers were tender
Tendency to bleeding
on slightest provocation
The floor of the ulcer
was covered with yellow
PROVISIONAL DIAGNOSIS: Pemphigus
Age (48 years) and Chronicity.
Presence of multiple ulcers as well as Bullous lesions
involving mucocutaneous surfaces (Oral Cavity and
Oral ulcers preceded cutaneous lesions.
No response to earlier treatment by other physicians.
All blood investigations were within the
normal limits except ESR which was found
to be on a higher side(24mm/hr).
•The Serum IgG antibody titre of the patient
presence of eosinophilic
rounded cells with
either present in
clumps or as individual
The acantholytic cells
were interspread with
representing cells of the
Incisional BiopsyParakeratinized stratified
squamous epithelium with
intra epithelial split also
showing basal cells abutting
on the underlying
Tzank cells were seen
within the split arranged in
the groups or single cells.
Compatible with clinical
diagnosis of Pemphigus
The patient was put on
0.5mg of Tab Betnesol TDS
X 8days Swish and Swallow
Dose was tapered on week
basis for a period of 3 weeks.
Maintenance therapy was
2-Week Follow Up
Betadine mouth rinses were advised to
combat secondary infection.
Supportive therapy with high dosage of
Antioxidants were also prescribed along with
B-complex & Zinc supplements.
Considerable improvement in the skin and
oral lesions was seen after 2 weeks of therapy.
Subsequent referral were made to
dermatologist & physician for follow-up and
Pemphigus Vulgaris is the most common form and
frequently involves the mouth.
(Weinberg et al.)
Patients with active disease have circulating
and tissue bound autoantibodies of both the
immunoglobulin G1(IgG1) and
immunoglobulin G4 (IgG4) subclasses.
(Scully et al)
SUPRA BASILAR BULLA
Abnormal IgG Production
Binding of specific IgG
antibodies to an antigen on
the epithelial membrane
Epithelial cell seperation
The cause of Pemphigus Vulgaris remains
unknown. However, several relevant factors
have been identified.
Vulgaris may develop
vegetative lesions in the
skin folds forming
This form can be more
resistant to therapy and
can remain in one place
for a long period of time.
Vegetative Pemphigus Vulgaris:
Mucous membranes typically are affected first
and preceded by cutaneous lesions.
Oral lesions are the “The first to Show and Last to
Oral lesions may begin
as the classic bulla
Shallow ulcers are
seen as the bullae
Erosions can be seen in
any part of the oral
Involve the larynx with
surfaces may be
DIF SHOWING DEPOSITION OF
IMMUNOREACTANTS IN THE INTERCELLULAR
AREAS BETWEENTHE SURFACE EPITHELIAL CELLS
The aim of treatment in Pemphigus
vulgaris is the same as in other
autoimmune bullous diseases , which is to
decrease the blister formation and
promote healing of blisters and erosions
and determine the minimal dose of
medication necessary to control the
(Ahmed et al.,1980; Becker and Gaspari 1993)
High doses of systemic corticosteroids along
with immunosuppressive drugs such as
Azithioprine, Dapsone,Cyclosporine initially to
clear the lesion
Maintain the patient on the low dose to control the
(Mourellou et al.,1995; Bystryn and Steinman,1996; Korman
In this case report, we describe the management of a
patient who had previously undergone treatment ,
whose complaints were not relieved for a long time.
We also distinguish the diagnosis of oral pemphigus
vulgaris and other similar oral lesions and the
importance of role of oral physician in early diagnosis
1. Mignogna et al: Oral pemphigus: long term behaviour and clinical
response to treatment with deflazacort in sixteen cases. J Oral Pathol
2. Tsuruta D, Kobayashi H: Recent Patents in Pemphigus Research,
Prophylaxis,Diagnosis and Treatment in USA (1988-2006). Recent
Patents on Inflammation & Allergy Drug Discovery 2007;1:77-81.
3. Azimi H, Golforoushan F: Comparing the Results of Classic
Treatment of Pemphigus Vulgaris with that of Cyclophosphamide
and Corticosteroid Pulse Therapy.
4. Azizi A, Lawaf S: Oral pemphigus: The management of oral mucous
membrane pemphigoid with dapsone and topical corticosteroid. J
Oral Pathol Med 2008;37:341-344.
5. Shafer, Hine, Levy. Textbook of Oral pathology. Fifth edition. 2005
6. Malcolm A, Vernon J B, Martin S G. Burket’s Oral Medicine-Diagnosis
& Treatment. Ninth edition. 1994 J. B. Lippincott Company.
7. Neville W B et al.Oral & Maxillofacial Pathology. Second edition. 2002