pemphigus vulgaris/ dental courses

PEMPHIGUS VULGARIS
“A CASE REPORT”
INDIAN DENTAL ACADEMY
Leader in continuing Dental Education
www.indiandentalacademy.com

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.
Shafers.
Definition

Indian Dental academy
• www.indiandentalacademy.com
• 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
per 1,00,000.
(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)
INCIDENCE

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.

CASE REPORT

CHIEF COMPLAINT
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
food .
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
EXAMINATION
 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
cooperativecooperative..

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
INSPECTION
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
retromolar area.
The ulcers had irregular
borders with mild erythema
around the periphery.The
surface was covered with
yellowish slough.

One hemorrhagic bulla
of the size 3cms x 2cms
was present on the buccal
mucosa opposite the 1st
molar.
DENTAL
Other parts of the oral
cavity were not involved.
Generalized attrition was
present with moderate
stains and calculus

PALPATION
The ulcers were tender
on palpation
Tendency to bleeding
on slightest provocation
was present
The floor of the ulcer
was covered with yellow
colored slough

PROVISIONAL DIAGNOSIS: Pemphigus
Age (48 years) and Chronicity.
Presence of multiple ulcers as well as Bullous lesions
involving mucocutaneous surfaces (Oral Cavity and
Skin).
Oral ulcers preceded cutaneous lesions.
No response to earlier treatment by other physicians.

DIFFERENTIAL DIAGNOSIS
Bullous Pemphigoid
Cicatricial Pemphigoid
Erythema Multiforme
Erosive Lichen Planus
Allergic Stomatitis
Dermatitis Herpetiformis

INVESTIGATIONS
ROUTINE BLOOD
SERUM IgG ANTIBODY TITRE
EXFOLIATIVE CYTOLOGY
INCISIONAL BIOPSY

BLOOD INVESTIGATIONS
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
was 1333mg/dl.

Exfoliative Cytology
Cytosmear showed
presence of eosinophilic
rounded cells with
darkly stained
hyperchromatic nucleus
either present in
clumps or as individual
cells.
The acantholytic cells
were interspread with
numerous exfoliated
buccal cells
representing cells of the
keratinizing layers.
IMPRESSION:
Acantholytic-Tzank cells
probably representing
Pemphigus Vulgaris.

Incisional BiopsyParakeratinized stratified
squamous epithelium with
intra epithelial split also
showing basal cells abutting
on the underlying
connective tissue.
Tzank cells were seen
within the split arranged in
the groups or single cells.
IMPRESSION:
Compatible with clinical
diagnosis of Pemphigus
Vulgaris.
Suprabasilar Split

FINAL DIAGNOSIS
PEMPHIGUS VULGARIS

Treatment
The patient was put on
0.5mg of Tab Betnesol TDS
X 8days Swish and Swallow
therapy
Dose was tapered on week
basis for a period of 3 weeks.
Maintenance therapy was
followed.
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
further care.

PRE- TREATMENT 2- Week- Follow upwww.indiandentalacademy.com

PRE- TREATMENT 2 Week-Follow Upwww.indiandentalacademy.com

PRE- TREATMENT
2 Week-Follow Upwww.indiandentalacademy.com

DISCUSSION
Variants of Pemphigus are:
Pemphigus vulgaris
Pemphigus foliaceus
Pemphigus paraneoplastic
Drug Induced Pemphigus

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)

PATHOPHYSIOLOGY
SUPRA BASILAR BULLA
Abnormal IgG Production
Binding of specific IgG
antibodies to an antigen on
the epithelial membrane
(Desmoglin-3)
Epithelial cell seperation
Acantholysis

CAUSES
The cause of Pemphigus Vulgaris remains
unknown. However, several relevant factors
have been identified.
Genetic Factor
Age
Disease Association
Drugs

CLINICAL FEATURES
SKIN LESIONS

Ordinary Pemphigus
Vulgaris may develop
vegetative lesions in the
skin folds forming
vegetating granulations.
This form can be more
resistant to therapy and
can remain in one place
for a long period of time.
Vegetative Pemphigus Vulgaris:

MUCOSAL MANIFESTATIONS
Mucous membranes typically are affected first
and preceded by cutaneous lesions.
 Oral lesions are the “The first to Show and Last to
go” (Navaille)
 Buccal mucosa
 Palate
 Gingiva

Oral lesions may begin
as the classic bulla
Shallow ulcers are
seen as the bullae
break rapidly.
Erosions can be seen in
any part of the oral
cavity.
Involve the larynx with
subsequent
hoarseness.

Other mucosal
surfaces may be
involved,
including the
conjunctiva,
esophagus,
vagina, cervix,
penis, urethra
and anus.

DIF SHOWING DEPOSITION OF
IMMUNOREACTANTS IN THE INTERCELLULAR
AREAS BETWEENTHE SURFACE EPITHELIAL CELLS
PEMPHIGOIDPEMPHIGUS

MANAGEMENT
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
disease process.
(Ahmed et al.,1980; Becker and Gaspari 1993)

SYSTEMIC STEROIDS
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
condition
(Mourellou et al.,1995; Bystryn and Steinman,1996; Korman
2000)

ALTERNATIVE TREATMENT TO
CORTICOSTEROIDS
DRUGS:
1) Mycophenolate Mophetil
2) Rituximab
3) Etanercept
4) Immunoglobulins
5) Dapsone
6) Cyclosporine
THERAPIES:
1) Plasmapheresis
2) Extracorpeal Photochematherapy
3) Immunoadsorption.

MANAGEMENT

CONCLUSION
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
and treatment.

BIBLIOGRAPHY
1. Mignogna et al: Oral pemphigus: long term behaviour and clinical
response to treatment with deflazacort in sixteen cases. J Oral Pathol
Med 1999;29:145-52.
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
Elsevier.
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
Saunders.

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