|Year : 2018 | Volume
| Issue : 4 | Page : 432-435
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
|Date of Submission||28-Aug-2018|
|Date of Acceptance||02-Nov-2018|
|Date of Web Publication||17-Jan-2019|
Dr. Narmatha Namachivayam
Department of Oral Medicine and Radiology, Ragas Dental College and Hospital, Uthandi, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
| 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
|How to cite this article:|
Aparna P V, Kailasam S, Namachivayam N, Gopinath J. Oral bullous pemphigoid – a rarity among vesiculobullous lesions. J Indian Acad Oral Med Radiol 2018;30:432-5
|How to cite this URL:|
Aparna P V, Kailasam S, Namachivayam N, Gopinath J. Oral bullous pemphigoid – a rarity among vesiculobullous lesions. J Indian Acad Oral Med Radiol [serial online] 2018 [cited 2019 Apr 21];30:432-5. Available from: http://www.jiaomr.in/text.asp?2018/30/4/432/250252
| 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. 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. DIF is found to be the gold standard test for differentiating among vesiculobullous lesions particularly in case of BP.
| 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 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] 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.
|Figure 1: Extra oral examination, pruritic macule was noted in cheek and neck regions|
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|Figure 2: Intraoral soft tissue examination, gingiva appears generalized erythematous, desquamative, ulcerated, and sloughing epithelial surface|
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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 5]a and [Figure 5]b is noticed. The connective tissue is densely collagenized with moderate cellularity. The mucosa is acanthotic with some neutrophilic exocytosis. Diffuse moderate chronic inflammatory cell infiltrate is noticed. Vascularity appears to be minimal.
|Figure 5: (a and b) Hyperparakeratinized stratified squamous epithelium with underlying connective tissue stroma and subepithelial split. (c) Acanthotic mucosa with some neutrophilic exocytosis and diffuse moderate chronic inflammatory cell infiltrate|
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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).
|Figure 6: Direct immunofluorescence findings revealed evidence of linear deposition of IgG along the dermal–epidermal junction (basement membrane zone)|
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|Figure 7: Direct immunofluorescence findings revealed evidence of linear deposition of C3 along the dermal–epidermal junction (basement membrane zone)|
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|Figure 8: Direct immunofluorescence findings revealed evidence of linear deposition of fibrinogen along the dermal–epidermal junction (basement membrane zone)|
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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. 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. In our study the affected individual is an elderly one. It is usually presented with rare oral manifestations sparing the buccal mucosa, palate, and gingivae. Oral involvement is seen prior to skin manifestations with the commonly affected site being palate. 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. 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. 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. 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.
The Nikolsky's sign was found to be negative. 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 Histopathological report reveal subepithelial split with some neutrophilic exocytosis. 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) 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.
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. 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.
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.
Other drugs for treating BP include new antibody modulators, rituximab 375mg/m2 weekly over 4 weeks and omalizumab subcutaneously 300–375 mg for every 6 weeks.
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.
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 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
Conflicts of interest
There are no conflicts of interest.
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