Case Report Pemphigus Vulgaris As Oral Mucosal Ulcer Progressive To Skin Lesion

Updat Dent. Coll .
j 2013; 3(2):43-47
Case Report
Pemphigus Vulgaris as Oral Mucosal Ulcer Progressive to Skin Lesion:
*S. M. Anwar Sadata, Akhter Imamb
a
Resident Surgeon, Oral& Maxillofacial Surgery, Dhaka Dental College Hospital

Dental Surgeon, Sadar Hospital, Kurigram
ARTICLE INFO
ABSTRACT
Article History:
Among all the life threatening diseases, autoimmune diseases are

among most notorious and complicated diseases. As these diseases
have no specific cause or responsible organism, it is therefore very
difficult to distinguish and diagnose and thus stands as a major
threat for public health. It is assumed that the reason for these
diseases may be from genetic to environmental factors, from stress
to idiopathic origin. Pemphigus Vulgaris(PV) is such an
autoimmune blistering disease that frequently affects the mucous
membrane and skin. Very often it starts from oral cavity and
Dentists therefore the first to recognize. This paper describes the
case of a patient presenting with a one-year history of painful
ulcerated gingiva, tongue, floor of the mouth and even on GIT who
is finally diagnosed as having PV spreading to generalized skin.
Received : 1 October2012
Accepted : 17 March 2013
Key Words:
Autoimmune disease,
Autoantibody,
Mucocuteneous disease,
Pemphigus vulgaris
however, often regarded as difficult to diagnose,

since the initial oral lesions may be relatively
nonspecific, manifesting as superficial erosions
or ulcerations and rarely presenting with the
formation of intact bullae2,4,5,6. Diagnostic delays
of greater than 6 months are common in patients
with oral PV4. The average interval from the
onset to confirmation of the diagnosis of PV has
been reported to be 6.8 months6 or 27.2 weeks2.
Historically, studies of autoimmune responses
had been conducted by analyzing the presence
and/or concentration of single antibodies in
biological
fluids
using
conventional
immunoassays,
such
as
ELISA,
radioimmunoassay, immunoblot, and others.
More recently, antigen microarrays have been
constructedand validated for over a dozen
autoimmune diseases7.This paper describes the
case of a patient presenting with a one-year
history of painful ulcerated gingiva, tongue,
floor of the mouth and even on GIT who is
finally diagnosed as having PV spreading to
generalized skin.
Introduction:
Pemphigus Vulgaris (PV) is an autoimmune
intraepithelial blistering disease involving the
skin and mucous membrane1. PV is
characterized by acantholysis in the epithelium1.
It affects both sexes almost equally and is more
common in middle-aged and elderly patients2,3.
Systemic corticosteroid therapy is associated
with a dramatic improvement of the condition;
however, complications of medical therapy still
remain a concern. In many PV patients, the oral
lesions are followed by the development of skin
lesions3,5. Consequently, if oral
PV can be
recognized in its early stages, treatment may be
initiated to prevent progression of the disease to
skin involvement. Early oral lesions of PV are,
*Address of Correspondence:
Dr. S. M. Anwar Sadat
BDS, BCS, MCPS, FCPS, MS (OMS)
Resident Surgeon, Oral & Maxillofacial Surgery
Dhaka Dental College Hospital
Contact no: 01711156023
e-mail: [email protected]
43
Update Dental College Journal
Vol 3 Issue 2, October-2013
Casereport:
In 2011, a 63-year-old woman was referred with
a one-year history of painful ulcerated gingiva,
tongue, soft and hard palate, angle and floor of
the mouth. The patient noticed peeling out of the
gingival epithelium while she brushed her teeth.
She had initially received periodontal treatment,
including scaling, periodontal treatment and oral
hygiene instructions, from a general dentist;
however, she had noted no improvement of the
burning sensation and peeling away of her
mucosa.She also reported an increased salivation
with sticky discharge. Her condition was getting
worsen and consequently she developed skin
lesion all over her body within weeks. She had
no history of hypertension, diabetes or any other
systemic
diseases.On
examination,bullous
lesions were found in oral mucosa, gingiva,
tongue , angle and floor of mouth (fig 1, 2 & 3).
The bullae were seen to rupture with discharge
of sticky fluids.Nikolskys sign showed a
positive reaction, and the epithelium could be
peeled away easily by slightly scratching the
surface of the mucosa and skin. Endoscopy of
GIT confirmed the involvement of ulcer on
GIT.However patient was admitted to the
hospital and systemic corticosteroids were given
and she responded quickly with disappearance
of lesions in oral cavity, angle of mouth and skin
(fig 4 & 5).
The cytological smear was collectred before
obtaining biopsy specimens. Smears were
prepared by exfoliating from the labial gingiva
using a cytobrush (Medscand Medical AB,
Malmo, Sweden). In the cytological smear,
collective acantholytic cells (Tzank cells) were
recognized. These cells enabled a presumptive
diagnosis of PV to be made. A gingival biopsy
was obtained from the perilesional site and
submitted for routine histopathology and the
direct immunofluorescence (DIF) test. DIF was
performed using conjugates for IgG, IgA, IgM,
C3, and fibrinogen, and it revealed deposition of
IgG and C3 between the epithelial cells. A
definitive diagnosis of PV was made based on
these clinical and histopathological and
immunofluorescent findings.
Fig-1 shows multiple blistering

involving gingiva and buccal mucosa
lesions
Fig-2.shows blistering lesions involving angle of

the mouth
Fig-3 shows the lesions on hand skin
Fig-4 shows disappearance of lesions in angle of

mouth after treatment
44
Pemphigus vulgaris
Sadat SMA & Imam A
protein constituent of the desmosomes11. Most

patients with PV have circulating IgG
autoantibodies
against
Dsg311,12.
These
antibodies bind to the Dsg3 on the epithelial cell
membrane and may evoke acantholysis11,12.
Acantholytic cells are often found in
intraepithelial blisters. These cells show
degenerative changes, including round, swollen
hyper chromatic nuclei with a clear perinuclear
halo in cytoplasm. Acantholytic cells can be
confirmed in the cytological smear obtained by
exfoliating from the oral mucosa13.In general,
autoimmune diseases are characterized by the
presence of multiple types of autoantibodies
mediating a coordinated immunological attack
against a fraction of the tissue proteome.
Multiplex analysis of autoantibody responses
against a spectrum of candidate antigens
represents a powerful screening tool to delineate
biomarker signatures in autoimmunity, allowing
elucidation of the overall autoimmune process
rather than individual components14. The
availability of multiplex technologies has made
possible the simultaneous detection of several
different autoantibodies overcoming some of the
limitations of conventional methods15. For
instance, antigen arrays proved to be 4- to 8-fold
more sensitive than conventional ELISA
analyses for detection of autoantibodies specific
for some autoantigens16.In this report, we
recognized acantholytic cells in the cytological
smear,also a DIF which enabled a presumptive
diagnosis of PV to be made. However, it is
necessary to perform a biopsy since the
appearance of acantholytic cells alone does not
allow a definitive diagnosis, but only permits a
presumptive diagnosis of PV. This is because
acantholytic cells may also appear in other
diseases such as impetigo, Dariers disease,
transient
acantholyticdermatosis,
viral
infections, and carcinoma17. PV often be
bewildered
by
pemphigoid
or
DG(DesqamatieGingiitis), but DG is a clinical
manifestation of the gingiva that is characterized
by desquamation of the gingival epithelium,
chronic redness, ulceration, and/or blister
formation6,18,19,20. Nisengard and Levine10 cited
the following as the standard in making a
clinical diagnosis of DG: (1) gingival erythema
not resulting from plaque, (2) gingival
desquamation, (3) other intraoral and sometimes
Fig-5 shows disappearance of lesions from hand

skin
In this patient, although it took only two weeks
from her first visit to our hospital until a
definitive diagnosis of PV was made, one year
had elapsed from the onset of the oral lesions to
the definitive diagnosis. However, after the
remission of her skin lesions, she was given
prednisolone 1.1mg/kg/body wt., 100 mg
azathioprine/day, calcium supplement and
omeprazole. The prednisolone was in tapered
mode at the rate of 2.5 mg decreasing in every
two weeks. Finally, it stopped at 5 mg in every
alternative day for six months. After two years
she was instructed to lower the dose of
azathioprine from 100 mg /day to 50 mg/day and
prednisolone was completely stopped. However,
she had not reported the recurrence of the
lesions till now.
Discussion:
The term pemphigus was originally named by
Wichman in 19718. Pemphigus is a group of
potentially life
threatening autoimmune
mucocuteneous disease characterized by
epithelial blistering affecting cutaneous and/or
mucosal surfaces, the term being derived from
the Greek word pempix(bubble or blister).
Although vulgaris means common in Latin, the
worldwide incidence of PV is very low and has
been reported to be 0.1-0.5 per 100,000 persons
per year. PVappears to occur in males and
females in an equal ratio and is most frequently
reported in patients between fourth and sixth
decades of life9,10.
PV is an autoimmune disease that is
characterized by acantholysis in the epithelium1.
The main antigen in PV is desmoglein (Dsg) 3, a
45
Update Dental College Journal
Vol 3 Issue 2, October-2013
6. Endo H, Rees TD, Matsue M,Kuyama K, Nakadai

M, Yamamoto H. Early detection and successful
management of oral pemphigus vulgaris: a case
report. Journal of Periodontology 2005; 76(1): 154
160.
extra oral lesions, and (4) complaint of sore

mouth, particularly with spicy foods. It is
reported that most cases of DG are caused by
several mucocutaneous diseases6,18,19. Mucous
membranepemphigoid and erosive lichen planus
are the most frequent causes of DG, accounting
for 48.9% and 23.6%, respectively, of all cases
of DG21 whilePemhigoid lesions are widespread
tense blisters, bullae can occur on normalappearing, as well as erythematous, skin
surfaces. The bullae usually heal without
scarring or milia formation. There are mild
clinical difference in between Pemphigus and
pemphigoid.
7. Fathman CG, Soares L, Chan SM, Utz PJ (2005)

an array of possibilities for the study of
autoimmunity. Nature 435: 605611.
8. Available from:
/DERM/ topic319.htm.
http//www.emedicine.com
9. Rabinowitz LG, Easterly NB, Inflammatory

bullous disease in children, DermatolClin 1993;
11:565-81
10. Migningna MD, Lomuzio L, Zeppa P, Ruocco ,
Bucci E. Immunocytochemical detection of
autoantiody deposit in T zank smears from patients
with oral Pemphigus . J Oral Pathol Med 1997;26254-7
Conclusion:
PV is a potentially fatal disease if not treated
timely and perfectly. As it is very difficult to
diagnose except the confirmatory test, it can be
convoluted with the many other oral lesions like
Erosive Lichen Planus, Systemic Lupus
Erythematosus, Pemphigoid, Desquamative
Gingivitis etc. Many often physiciansfail to
diagnose it at its early stage and patients often
may not receive the appropriate treatment.
Therefore physicians should be more aware
regarding the ulcers especially oral ulcers. And
since dentists foreplay the role in treating with
oral diseases, therefore they must be familiar
with all these potentially fatal diseases.
11.
Amagai
M.
Autoimmunity
against
desmosomalcadherins in pemphigus. Journal of
Dermatological Science 1999; 20(2): 92102.
12. Harman KE, Gratian MJ,
Bhogal BS,
Challacombe SJ, Mlack MM. A study of desmoglein
1 autoantibodies in pemphigus vulgaris: racial
differences in frequency and the association with a
more severe phenotype. British Journal of
Dermatology 2000; 143(2): 343348.
13. Bucci E. Immunocytochemical detection of
autoantibody deposits in Tzanck smears frompatients
with oral pemphigus. Journal of Oral Pathology
andMedicine 1997;26(6): 254-257.
Referrences:
1. Scully C,Mignogna M. Oral mucosal disease:
pemphigus. British Journal of Oral and Maxillofacial
Surgery 2008; 46(4): 272277.
14. Hueber W, Robinson WH (2006) Proteomic

biomarkers for autoimmune disease. Proteomics 6:
41004105.
2. Scully C, Almeida OP, Porter SR, Gilkes JJH.

Pemphigus vulgaris: the manifestations and longter
management of 55 patients with oral lesions. British
Journal of Dermatology1999; 140 (1): 8489.
15. Tozzoli R.The diagnostic role of autoantibodies

in the prediction of organ-specific autoimmune
diseases.ClinChem Lab Med 2008; 46: 577587.
16. Robinson WH, DiGennaro C, Hueber W, Haab
BB, Kamachi M, et al. (2002) Autoantigen
microarrays for multiplex characterization of
autoantibody responses. Nat Med 8: 295301.
3.Kavusi S, Daneshpazhooh M, Farahani F, Abedini

R, Lajevardi V, Chams-davatchi C, Outcome of
pemphigus vulgaris.
Journal of the European
Academy of Dermatology and Venereology 2008;
22(5): 580584.
17. Scully C,Challacombe SJ. Pemphigus vulgaris:

update on etiopathogenesis, oral manifestations, and
management. Critical Reviews in Oral Biology and
Medicine 2002;13(5):397408.
4.Sirois DA, Fatahzadeh M, Roth R, Ettlin D.

Diagnostic patterns and delays in pemphigus
vulgaris: experience with 99 patients. Archives of
Dermatology2000;136(12): 15691570.
18.Endo H, Rees TD, Hallmon WW et al., Disease

progression from mucosal to mucocutaneous
involvement in a patient with desquamative gingivitis
associated with pemphigus vulgaris. Journal of
Periodontology 2008; 79(2): 369375.
5. Zegarelli DJ, Zegarelli EV. Intraoral pemphigus

vulgaris. Oral Surgery Oral Medicine and Oral
Pathology1977; 44(3): 384393.
46
Pemphigus vulgaris
Sadat SMA & Imam A
19. Russo LL, Fedele S,Guiglia R et al., Diagnostic

pathwaysand clinical significance of desquamative
gingivitis.
Journal of Periodontology 2008;
79(1):1424.
20. Endo H, Rees TD, Sisilia F et al., Atypical

gingival manifestations that mimic mucocutaneous
diseases in a patient with contact stomatitis caused by
toothpaste. The Journal of Implant and Advanced
Clinical Dentistry2010;2(2):101 106.
21. Nisengard RJ, Rogers RS, The treatment of
desquamative gingival lesions.
Journal of
Periodontology 1987; 58(3): 167172.
47

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Updat Dent. Coll .

Resident Surgeon, Oral& Maxillofacial Surgery, Dhaka Dental College Hospital

Among all the life threatening diseases, autoimmune diseases are

however, often regarded as difficult to diagnose,

Update Dental College Journal

Vol 3 Issue 2, October-2013

Fig-1 shows multiple blistering

Fig-2.shows blistering lesions involving angle of

Fig-3 shows the lesions on hand skin

Fig-4 shows disappearance of lesions in angle of

Sadat SMA & Imam A

protein constituent of the desmosomes11. Most

Fig-5 shows disappearance of lesions from hand

Update Dental College Journal

Vol 3 Issue 2, October-2013

6. Endo H, Rees TD, Matsue M,Kuyama K, Nakadai

extra oral lesions, and (4) complaint of sore

7. Fathman CG, Soares L, Chan SM, Utz PJ (2005)

9. Rabinowitz LG, Easterly NB, Inflammatory

14. Hueber W, Robinson WH (2006) Proteomic

2. Scully C, Almeida OP, Porter SR, Gilkes JJH.

15. Tozzoli R.The diagnostic role of autoantibodies

3.Kavusi S, Daneshpazhooh M, Farahani F, Abedini

17. Scully C,Challacombe SJ. Pemphigus vulgaris:

4.Sirois DA, Fatahzadeh M, Roth R, Ettlin D.

18.Endo H, Rees TD, Hallmon WW et al., Disease

5. Zegarelli DJ, Zegarelli EV. Intraoral pemphigus

Sadat SMA & Imam A

19. Russo LL, Fedele S,Guiglia R et al., Diagnostic

20. Endo H, Rees TD, Sisilia F et al., Atypical

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