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Case Report

Oral Bullous Pemphigoid – A Rarity among Vesiculobullous

Lesions
P. Venkatalakshmi Aparna, S. Kailasam, Narmatha Namachivayam, Jayashree Gopinath
Department of Oral Medicine and Radiology, Ragas Dental College and Hospital, Uthandi, Chennai, Tamil Nadu, India

Abstract
Bullous pemphigoid (BP) otherwise known as parapemphigus is a chronic autoimmune subepidermal blistering disease with tense bullae that
rupture and become flaccid. It commonly affects the skin and is rare in oral mucous membrane. Considering its rarity, we are presenting a
case report of a 45‑year‑old female diagnosed with BP that had occurred predominantly in the oral cavity prior to the minimal presentation of
skin lesions, along with satisfactory management, thereby stressing the role of dentists in prior diagnosing the lesion.

Keywords: Bullous pemphigoid, direct immunofluorescence, vesiculobullous lesion

Introduction
Pemphigoid is derived from Greek word and it means a form
of blister. Bullous pemphigoid (BP) is a rare autoimmune
blistering disease (AIBD) usually affecting the elderly
people. The epidemiology of BP estimated to be 0.2 and 3 per
100,000 people per year. Among autoimmune vesiculobullous
lesion, pemphigus vulgaris is more common than BP in
India. A regional study in UK estimated an incidence of
1.4 per 100,000 people per year.[1] The mechanism behind
bullae formation are IgG autoantibodies bind to the basement
membrane, which activates complement and inflammatory
mediators. Activation of the complement system is thought
to play a critical role in attracting inflammatory cells to the
basement membrane zone, thereby releasing proteases, which
degrade hemidesmosomal proteins leading to blister formation.
Dermoepidermal cohesion is promoted by two components of
adhesion complexes, namely, 180‑ and 230–KD antigen. In
BP, sub epidermal autoantibodies are directed against these
antigens promoting loss of cell adhesion.[2] DIF is found to be
the gold standard test for differentiating among vesiculobullous
lesions particularly in case of BP.[3]
Case Report
A 45‑year‑old female patient named Thenmozhi came to Ragas
Dental College Outpatient Department with a chief complaint
of soreness in the upper and lower gum regions for past 2 years
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and history of itching in palms and feet since 2 weeks, after
which she started developing multiple bullae inside the mouth
and rupture of bullae leaving an ulcer. During masticating
hard food items and sometimes even after forceful brushing,
the incidence of bullae is provoked, which resolved within
2–3 days. She had a history of pruritus followed by appearance
of extraoral bullae and on rupture leaving discoloration in
left side of the cheek and neck regions for past 3 days. She
underwent scaling and restoration before 1 year.
On extra oral examination, pruritic macule [Figure 1] on the
left side cheek and neck regions was noted. Diffuse rash is
noted over the left‑ and right‑side zygoma, crossing over the
bridge of nose. On intraoral soft tissue examination, gingiva
appeared generalized erythematous, desquamative, ulcerated,
sloughing epithelial surface [Figure 2] with loss of stippling
and scalloping, blunt interdental papilla, soft and edematous
in consistency, generalized bleeding on probing. On inspection
of palatal mucosa, desquamation, ulceration in relation to 28
of ~1 cm in size with irregular margin [Figure 3] and a smooth,
solitary bullae, with purplish hue in relation to 17,18 [Figure 4]
Address for correspondence: Dr. Narmatha Namachivayam,
Department of Oral Medicine and Radiology, Ragas Dental College and
Hospital, Uthandi, Chennai, Tamil Nadu, India.
E‑mail: narmadabds11@gmail.com
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How to cite this article: Aparna PV, Kailasam S, Namachivayam N,
Gopinath J. Oral bullous pemphigoid – A rarity among vesiculobullous
lesions. J Indian Acad Oral Med Radiol 2018;30:432‑5.
Received: 28‑08‑2018   Accepted: 02-11-2018   Published: 17‑01‑2019

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Aparna, et al.: Oral bullous pemphigoid
Figure 1: Extra oral examination, pruritic macule was noted in cheek
and neck regions
Figure 3: Desquamation, ulceration in relation to 28
of ~1 2 cm extending anteriorly from mesial interdental margin
of 17 to distal margin of 18 posteriorly and superoinferiorly
1 cm from cervical margin of 17,18 with diffuse margin is
noted. On palpation, it was tender, soft in consistency, and no
bleeding tendency was evident. Diseases of the pemphigus
group can be easily differentiated by distinctive clinical
features. Mucous membrane pemphigoid can be differentiated
from BP by its predominant involvement of mucosal surfaces
and positive Nikolsky’s sign. Lichen planus pemphigoides is
clinically differentiated by the presence of lichen planus lesions
in addition to tense blisters.
Biopsy was performed perilesional within 1 cm over the
intact bullae in relation to palatal aspect of 17,18 [Figure 4]
under aseptic condition, and the biopsy sample was sent
simultaneously for both histopathological analysis and
direct immunofluorescence study. Histopathological report
revealed hyperparakeratinized stratified squamous epithelium
with underlying connective tissue stroma. Subepithelial
split [Figure 5a and b] is noticed. The connective tissue is
densely collagenized with moderate cellularity. The mucosa
Journal of Indian Academy of Oral Medicine & Radiology  ¦  Volume 30 ¦ Issue 4 ¦ October‑December 2018
Figure 2: Intraoral soft tissue examination, gingiva appears generalized
erythematous, desquamative, ulcerated, and sloughing epithelial surface
Figure 4: Solitary bullae, with purplish hue in relation to 17,18
is acanthotic with some neutrophilic exocytosis. Diffuse
moderate chronic inflammatory cell infiltrate is noticed.
Vascularity appears to be minimal.
Direct immunofluorescence findings revealed evidence
of linear deposition of IgG [Figure 6], C3 [Figure 7], and
fibrinogen [Figure 8] along the dermal–epidermal junction
(basement membrane zone).
Thus, the conclusive diagnosis of BP was arrived on the basis
of history, clinical presentation, and immunological findings.
She was managed with systemic steroids, prednisolone
(Omnocortil) – 10 mg initially twice daily for 3 days and
once daily for 6 days – and the dosage was reduced to half
for about 15 days. Predinosolone being an intermediate
acting steroid is given daily in the morning in a single dose of
1 mg/kg/body weight. This is to mimic the pattern of suprarenal
secretion (circadian rhythm), thereby duration of hypothalomo
hypophyseal axis was suppressed.[4] Upon review the incidence
of bullae has been reduced followed by which drug was tapered
and put under maintenance therapy.
Discussion
BP is an autoimmune vesiculobullous disease usually affecting
the elderly people; its incidence increases with age.[5] In our study
the affected individual is an elderly one. It is usually presented
with rare oral manifestations sparing the buccal mucosa,
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Aparna, et al.: Oral bullous pemphigoid

a b

c
Figure 6: Direct immunofluorescence findings revealed evidence of
Figure 5: (a and b) Hyperparakeratinized stratified squamous epithelium
linear deposition of IgG along the dermal–epidermal junction (basement
with underlying connective tissue stroma and subepithelial split. (c)
membrane zone)
Acanthotic mucosa with some neutrophilic exocytosis and diffuse
moderate chronic inflammatory cell infiltrate

Figure 8: Direct immunofluorescence findings revealed evidence of linear

Figure 7: Direct immunofluorescence findings revealed evidence of
linear deposition of C3 along the dermal–epidermal junction (basement
membrane zone)
palate, and gingivae. Oral involvement is seen prior to skin
manifestations with the commonly affected site being palate.[6]
Palate is the most commonly affected site in our case. Oral
lesions comprise of bullae/vesicle that rupture to form erosions
and ultimately leave out ulcerations.[7] Her history revealed onset
of bullae followed by rupture and leaving out painful ulcerations.
Clinical presentation of chronic desquamative gingivitis was
evident in our case. Severe itching and blister formation are the
most commonly encountered symptoms in patients.[8] Similarly,
in our case, she had a history of itching in palms and feet before
the development of bullae inside the mouth. Pruritus can be the
common clinical presentation, which may persist for several
weeks or months, although scarring is not seen, leaving out only
pigmentation in the normal skin background.[9] She had pruritic
macule on the left side and a history of extraoral bullae followed
by rupture and leaving out discoloration in the affected area.
deposition of fibrinogen along the dermal–epidermal junction (basement
membrane zone)
The Nikolsky’s sign was found to be negative.[9] Nikolsky’s
sign is present in case of pemphigus and cicatricial pemphigoid,
but not in the case of BP. In our case also, Nikolsky’s sign
was negative.
Mucous membrane pemphigoid can be differentiated from BP
by its predominant involvement of mucosal surfaces.
Apart from evaluating history, clinical presentation,
histopathological analysis is carried out followed by direct
immunofluorescence study for the differential diagnosis and
confirmation of the condition.
Direct immunofluorescence is found to be the gold standard
test. Deposition pattern of different types of immunoreactants
differentiates the various immune‑mediated diseases. Direct
immunofluorescence shows presence of IgG and C3 deposits
along the basement membrane zone[10] Histopathological report
reveal subepithelial split with some neutrophilic exocytosis.

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Aparna, et al.: Oral bullous pemphigoid
Our case also revealed same subepithelial split and direct
immunofluorescence findings.
Thus, the conclusive diagnosis of BP was arrived on the
basis of history, clinical presentation, and immunological
findings. Treatment is based on the degree of cutaneous
and oral involvement. Mostly, topical steroid (clobetasol
propionate)[11] gives satisfactory result in case of smaller area
of skin involvement, whereas larger area of skin involvement
and recurrent cases are treated satisfactorily with systemic
steroids and immunosuppressive agents. The relapse rate of
BP ranges from 27.87% to 53% after disease remission, while
the majority of relapses occur early (within 6 months) during
remission.[12]
Recommended dosage for oral prednisolone is 0.3–1.25 mg/kg
body weight/day, controls disease within 1–2 weeks, followed
by which the dose is tapered.[13] Dexamethasone (100 mg in
500 mL 5% dextrose i.v. over 2–3 h for three consecutive days)
is the preferred steroid for pulse therapy, either administered
alone or in combination with cyclophosphamide.[14]
Higher doses of systemic corticosteroids seem to be
associated with higher mortality rates, which led to the
addition of corticosteroid‑sparing agents to the treatment of
BP. The most frequently used immunosuppressive agent is
azathioprine (0.5–2.5 mg/kg body weight/day). Others being
cyclophosphamide, methotrexate, cyclosporine A, combination
tetracycline/minocycline along with nicotinamide, and more
recently, mycophenolate mofetil, a DNA synthesis inhibitor,
and methotrexate, a folate antagonist.[15]
Other drugs for treating BP include new antibody modulators,
rituximab 375mg/m2 weekly over 4 weeks[16] and omalizumab
subcutaneously 300–375 mg for every 6 weeks.[17]
IVIg – A dose of 1–2 g/kg for five consecutive day cycle of
0.4 g/kg/day, although a 3‑day cycle may be used in cases that
are nonresponsive to conventional therapy.[18]
In our case, she was managed with systemic steroids, and upon
review, the incidence of bullae has been reduced followed by
which drug was tapered and put under maintenance therapy.
Conclusion
Vesiculobullous disorders although rare in occurrence, poses
a severe life‑threatening changes as it is very difficult to
diagnose at the early stage based on the clinical pictures alone,
requiring various diagnostic modality to derive at the definitive
diagnosis. Treatment modality comprises of interdisciplinary
approach among dentists, general physician, dermatologists,
and ophthalmologists to successfully manage the patient with
AIBD.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and other
Journal of Indian Academy of Oral Medicine & Radiology  ¦  Volume 30 ¦ Issue 4 ¦ October‑December 2018
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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