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Case Report

Gingival pemphigus vulgaris preceding

cutaneous lesion: A rare case report
Saroj K. Rath, M. Reenesh

Department of Abstract:
Periodontics, Armed Pemphigus is a group of autoimmune diseases characterized by formation of intraepithelial bullae in skin and
Forces Medical College, the mucous membrane. Pemphigus vulgaris affects the oral mucosa in nearly all cases. Pemphigus vulgaris is
Pune, India characterized by auto antibodies directed against desmosome‑associated protein antigens (desmoglein‑3) found
in epithelial and epidermal intercellular substance. We report here a case of pemphigus vulgaris of gingiva in
an adult female patient at an early stage followed by dermatologic involvement. Perilesional incision was taken
and histopathological and direct immunofluorescence was done for identification of specific antibodies. The
oral lesions were treated with 0.1% Triamcinolone acetonide ointment and Prednisolone 20 mg twice daily with
multivitamins was administered systemically for skin lesion.
Key words:
Desquamative gingivitis, pemphigus, perilesional biopsy, vulgaris

INTRODUCTION female patient at an early stage followed by

dermatologic involvement.

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A number of dermatological diseases may
present gingival manifestations sometimes
in the form of desquamative lesions of the gingiva
www.jisponline.com
or gingival ulcerations. Desquamative gingivitis
DOI: (DG) denotes a particular clinical picture and is
10.4103/0972-124X.106922
not a diagnosis in itself.[1] The common disease
Quick Response Code: with relevance of these diseases include Lichen
planus, Pemphigoid, Pemphigus, Erythema
multiforme, and Lupus erythematosus.
Pemphigus is a group of autoimmune diseases
characterized by formation of intraepithelial
bullae in skin and the mucous membrane.
Pemphigus vulgaris is the most common of
pemphigus which also includes pemphigus
foliaceus, pemphigus vegetans, and pemphigus
erythematosus.[2] Pemphigus is a rare case of
desquamative gingivitis, one study showed
that only 2.3% of DG is due to pemphigus.[3]
Studies have shown world‑wide incidence of
0.1 to 0.5 cases of pemphigus vulgaris per year
per 1lakh individuals.[2,4] Pemphigus vulgaris
affects the oral mucosa in nearly all cases
and more importantly, the oral mucosa is
the site of the first lesion in the majority of
Address for
correspondence: cases. Pemphigus vulgaris is characterized by
Dr. Saroj K. Rath, auto antibodies directed against desmosome
Department of associated protein antigens (desmoglein‑3)
Periodontics, Armed found in epithelial and epidermal intercellular
Forces Medical College, substance. Since the desmosome is the primary
Pune – 411 040, India. attachment mechanism between keratinocytes,
E‑mail: drrathfamily@
yahoo.com
the inflammatory destruction of that attachment
leads to the characteristic fluid filled bullae and
Submission: 01‑07‑2011 subsequent ulceration. We report here a case
Accepted: 12‑09‑2012 of pemphigus vulgaris of gingiva in an adult
CASE REPORT
A 36‑year‑old married female [Figure 1], resident
of Pune, housewife by occupation referred
by Department of Dermatology, Command
Hospital Pune for the gingival lesion to division
of Periodontics, Department of dental surgery,
AFMC, Pune, with chief complaint of burning
sensation of the oral cavity for the last 3 months.
The patient was apparently asymptomatic
before 3 months. She noticed ulcers in the mouth
[Figure 2] and felt severe burning sensation,
specially aggravated on taking hot and spicy food.
The vesicles on the gum ruptured to produce pain
and severe itching in the mouth. She also noticed
small eruptions over skin on face, back [Figures 3
and 4] extremities just 3 weeks before, for which
she reported to the dermatologist.
The medical history and family history was clear
and patient was not taking any other drugs and
had no ocular or genital lesions. The patient
had a proper oral hygiene habit without any
deleterious habits.
On general physical examination, the patient was
moderately built and nourished with vital signs
within normal limits. She had numerous flaccid
vesicles and ruptured bullae of varying diameter
seen over the back and nose, which are mainly
seen as ulcerated eroded surfaces. On extra oral
examination no relevant findings were observed.
On intra‑oral examination, denuded and
spontaneously bleeding gingival zones were

588 Journal of Indian Society of Periodontology - Vol 16, Issue 4, Oct-Dec 2012
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Rath and Reenesh: Gingival pemphigus vulgaris preceding cutaneous lesion

revealed. The clinical picture of persistent superficial ulcers with
a positive Nikolsky’s sign strongly suggested a vesicullobulous
disorder. Based on the history, clinical signs, and symptoms
of both intaoral and extraoral lesions, a provisional diagnosis
of desquamative gingivitis was given.
After routine hemogram, urine and blood sugar examination
of patient was taken up for incisional perilesional biopsy with
intact epithelium. The sample was sent for histopathological
examination in normal saline to Department of Pathology.
The patient was taken back to Department of Dermatology
for opinion and biopsy of skin was taken from back
for histopathological examination and for the direct
immunofluorescence assay in Michel’s solution. On
histopathological examination, acantholysis was revealed
along areas of ulceration without dysplastic changes with
polymorphonuclear leucocytes infiltration. Basal cells of
epithelium remained attached to the basement membrane.
Higher resolution showed perivesicular edema with loss of
cohesiveness and hyperchromatic epithelial cells called “Tzank
cells” [Figures 5 and 6], suggesting diagnosis of pemphigus
vulgaris. The direct immunofluorescence showed intercellular
deposition of C3, IgG, and IgM in stratum spinosum
[Figures 7 and 8] confirming the diagnosis of pemphigus
vulgaris.
The oral lesions were treated with 0.1% Triamcinolone
acetonide ointment and skin lesion with topical application of
0.05% Clobetasol with Soframycin twice daily. Prednisolone
20_mg twice daily with multivitamins was administered
systemically. The patient is under control and still under
review [Figure 9].
DISCUSSION
It is reported that most cases of DG are cases of severe
mucocutaneous diseases.[3,5,6] In the present case report of
pemphigus vulgaris, the oral lesions were the first sign of the
disease with subsequent dermatologic involvement as observed
by several other studies.[7,8] Mucous membrane pemphigoid
and erosive lichen planus are the most common causes of
DG accounting for 48.9% and 23.6% respectively, pemphigus
vulgaris being the least common cause.[3]
Histopathological examination and direct immunofluorescence
testing are necessary to make a confirmed diagnosis of
pemphigus vulgaris responsible for DG.[5,6,9]

Figure 1: Patients photograph Figure 2: Intra oral lesion

Figure 3: Lesion on face Figure 4: Skin lesion

Journal of Indian Society of Periodontology - Vol 16, Issue 4, Oct-Dec 2012 589
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Rath and Reenesh: Gingival pemphigus vulgaris preceding cutaneous lesion

Figure 5: Histopathology under ( H and E ×10) Figure 6: Histopathology under (H and E ×40)

Figure 7: Direct immunoflorescence 1 Figure 8: Direct immunoflorescence 2

not rendered at any level. The reasons for delayed diagnosis

may be explained by the patient symptoms being confined to
gingiva and clinically mild in nature and the sign and symptoms
occurring in repeated cycles of remission and exacerbation.
In this report, we recognized the acantholytic cells in the
histopathologic study which was not sufficient for a confirmed
diagnosis of pemphigus vulgaris. The confirmed diagnosis
was made after the direct immunofluorescence assay method
was followed from the skin lesion. This is because acantholytic
cells may also appear in Impetigo, Darier’s disease, transient
acantholytic dermatosis, viral infections, and carcinoma.[10]

To summarize, in the present case, a definitive diagnosis of

pemphigus vulgaris was made based on a general assessment
of positive Nikosky’s sign, presence of acantholysis in
histopathology and findings of direct immunofluorescence.

Figure 9: Post treatment after 15 days
Pemphigus vulgaris is potentially fatal considering the nature of
the disease. It is essential to establish a early definitive diagnosis,
timely therapy and follow up. The present case under discussion
has visited various dental clinics for her oral lesions, when she
had not developed the skin lesion. But definitive diagnosis was
High dose topical corticosteroid ointment may control limited
oral disease. In most cases, however, disease control or
remission is achieved using systemic corticosteroids alone or in
combination with immuno‑modulating medications. The most
important aspect of pemphigus vulgaris is its early recognition,
diagnosis, and treatment. The dentist has a unique opportunity
to make the diagnosis and institute treatment.

590 Journal of Indian Society of Periodontology - Vol 16, Issue 4, Oct-Dec 2012
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Rath and Reenesh: Gingival pemphigus vulgaris preceding cutaneous lesion

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Dermatol Clin 1993;11:453‑60.
5. Endo H, Rees TD, Matsue M. Disease progression from mucosal
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pemphigus vulgaris 2001;28:489‑501.
8. Siegel MA, Basciunas BA. Oral presentation and management of
vesiculobullous disorder. Semin Dermatol 1994;13:78‑81.
9. Nisengard RJ, Levine RA. Diagnosis and management of
desquamative gingivitis. Periodontal Insights 1995;2:4‑10.
10. Scully C, Challacombe SJ. Pemphigus vulgaris, update on
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How to cite this article: Rath SK, Reenesh M. Gingival pemphigus
vulgaris preceding cutaneous lesion: A rare case report. J Indian
Soc Periodontol 2012;16:588-91.
Source of Support: Nil, Conflict of Interest: None declared.

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