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Table of Contents
CASE REPORT
Year : 2018  |  Volume : 5  |  Issue : 3  |  Page : 34-38

Pemphigus vulgaris: Blistering mucocutaneous disease


1 Final Year Student, Department of Oral Medicine and Radiology, VS Dental College and Hospital, Bengaluru, Karnataka, India
2 Reader, Department of Oral Medicine and Radiology, VS Dental College and Hospital, Bengaluru, Karnataka, India
3 Professor and HOD, Department of Oral Medicine and Radiology, VS Dental College and Hospital, Bengaluru, Karnataka, India
4 Professor, Department of Oral Medicine and Radiology, VS Dental College and Hospital, Bengaluru, Karnataka, India

Date of Web Publication30-Jan-2019

Correspondence Address:
Dr. Harshita Thakur
Department of Oral Medicine and Radiology, VS Dental College and Hospital, Bengaluru - 560 004, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/INPC.INPC_2_18

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  Abstract 


Pemphigus is a group of potentially fatal dermatoses with both cutaneous and oral manifestations characterized by the appearance of vesicle or bullae. Their manifestations in the oral cavity often precede those on the skin by many months or may remain as the only symptoms of the disease. Most patients could be initially misdiagnosed. Timely recognition and therapy of oral lesions are critical if treatment is instituted during this time, the disease is easier to control and the chance for early remission of the disorder is enhanced. Topical, systemic or intralesional corticosteroids are the treatment of choice for pemphigus vulgaris. Although the mortality has decreased to <10% since corticosteroids appeared, high doses, and prolonged treatment can cause side effects. They can be prescribed with different immunosuppressive therapy to reduce the cumulative dose of corticosteroids. Among the different immunosuppressive drugs, we find azathioprine, methotrexate, and cyclophosphamide and more recently mycophenolate mofetil.

Keywords: Corticosteroids, immunosuppressive drugs, pemphigus vulgaris


How to cite this article:
Thakur H, Satish S, Choudary S, Manjunath M, Deepak T A. Pemphigus vulgaris: Blistering mucocutaneous disease. Int J Prev Clin Dent Res 2018;5:34-8

How to cite this URL:
Thakur H, Satish S, Choudary S, Manjunath M, Deepak T A. Pemphigus vulgaris: Blistering mucocutaneous disease. Int J Prev Clin Dent Res [serial online] 2018 [cited 2019 Apr 24];5:34-8. Available from: http://www.ijpcdr.org/text.asp?2018/5/3/34/251049




  Introduction Top


Pemphigus vulgaris (PV) is characterized by intraepithelial blister formation that results from breakdown of the cellular adhesion between epithelial cells.[1] In 1964, autoantibodies against keratinocyte surfaces were described in patients with pemphigus.[2]

Clinical and experimental observation indicates that the circulating autoantibodies are pathogenic. An immunogenetic predisposition is well established. Diagnosis is based on the identification of clinical manifestations and confirmation through biopsy. Demonstration of immunoglobulins, in the spinous cell junctions by distinct immunofluorescence (IF), is often used for the final confirmation of PV.[3],[4] As the oral presentation of the disease is often the first indicator that can lead to the final diagnosis, it is very critical for the dental practitioner to recognize the oral lesions of PV at a sufficiently early stage to initiate further investigations and treatment. We present a case of PV where the patient presented with ulcerations at multiple oral sites including tongue, and the final diagnosis was made by the timely interpretation of these manifestations.


  Case Report Top


A 50-year-old woman reported to the Department of Oral Medicine and Radiology, VSDCH with a complaint of burning sensation and bleeding gums and lips for 7 months. The patient reported that burning sensation occurred on intake of hot and spicy food with continuous bleeding with a history of ulcers in the skin. She had received fluconazole Once daily for a week and then discontinued the treatment. Personal and family history was uneventful. On the extraoral examination, well-defined ulcerative lesion (ruptured vesicles) was present on the symphysis, back of the neck, and upper abdomen region [Figure 1] and [Figure 2]. On intraoral examination, multiple ulcers with ill-defined margins, erythematous, and sloughy floor were noted on the vermillion border of the upper and lower lip, labial mucosa, buccal mucosa, hard and soft palate, dorsum surface of the tongue and the floor of the mouth [Figure 3], [Figure 4], [Figure 5], [Figure 6]. On palpation, the base of ulcer was soft tender and easily bled on touch. A diagnosis of PV was made after a skin biopsy, which yielded a positive Tzancktest.
Figure 1: Extra oral view shows irregular crustations on lips

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Figure 2: Eroded area on the ventral surface of arm

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Figure 3: Eroded vesicle on the left torso

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Figure 4: Multiple erosion on the tongue

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Figure 5: Multiple erosion on left buccal mucosa

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Figure 6: Multiple erosion extending to soft palate

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The patient was referred to the Clinical Dermatology, KIMS Hospital for treatment after the informed consent was obtained and was initially under injection Betnesol OD for 6 days, injection Taxim 1 g BD for 6 days, Tess buccal paste application OD for 6 days, Betadine mouthwash BD for 6 days, tablet fluconazole 150 OD on day 1 and day 5 and 6, tablet Omnacortil 20 mg OD on day 5 and day 6, tablet Endoxan 50 mg OD on day 5 and 6.

The patient recovered and was directed for regular follow-up. The patient was reviewed after a month, and the lesions showed healing without scarring [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12].
Figure 7: Healed lesion on the lips

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Figure 8: Scab formation on the upper arm

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Figure 9: Healed lesion on the left torso

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Figure 10: Completely healed lesion on the tongue

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Figure 11: Remission of lesions on left buccal mucosa

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Figure 12: Remission of lesions on right buccal mucosa

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  Discussion Top


Derived from the Greek word meaning “blister,” Pemphigus is a group of potentially life-threatening autoimmune mucocutaneous disorders characterized by intraepithelial blister formation.[5] The blisters occur in the epithelium where the patient's IgG autoantibodies produced in response to triggering factors target two structured proteins of desmosomes identified as Desmogleins 1 and 3. Recently, a new Pemphigus antigen Desmoglein 4 and other non-Desmoglein antigens such as human α-9-acetylcholine receptor that regulates keratinocyte adhesion and keratinocyte annexin such as molecules binding acetylcholine termed pemphaxin and catenin are also thought to play a role in its etiopathogenesis.[6],[7] Thin separation at the desmosomal region triggers the acantholysis and suprabasal spilt. Since the clinical features of PV are similar to those seen in Cicatricial Pemphigoid and Bullous Lichen Planus, its diagnosis needs to be confirmed with routine histopathology and IF studies. Tissue bits from both sites were taken in our case, and the histologic features were suggestive of PV. The diagnostic features were the presence of a suprabasilar split and acantholytic Tzanck cells in the split, both produced due to the intraepithelial blister formation. Direct IF study showed the typical “fish-net” pattern of IgG and complement C3 deposits in the spinous layer, the site for the autoimmune reaction in PV. Both histopathology and IF studies confirmed the diagnosis of PV.

An important aspect of patient management is early diagnosis when lower doses of medication can be used for shorter periods of time to control the disease. Dental professionals must be sufficiently familiar with clinical manifestation of PV to ensure early diagnosis and treatment since this in turn determines the prognosis and course of the disease. The institution of early treatment could prevent serious involvement of other mucosa and cutaneous sites and fatal complications. PV is generally managed with local and systemic corticosteroid therapy. Treatment is administered in two phases: a loading phase, to control the disease, and a maintenance phase, which is further divided into consolidation and treatment tapering. Local treatment consists of a paste, an ointment or a mouthwash administered alone or in conjunction with systemic treatment. Intralesional injections of corticosteroids have been used for the management of persistent lesions.[8] In cases of extensive oral lesions or involvement of other mucosa and skin, systemic corticosteroid therapy is initiated immediately. The initial of prednisone 0.5–2 mg/kg is recommended.[9] Depending on the response, the dose is gradually decreased to the minimum therapeutic dose, taken once a day in the morning to minimize side effects. When steroids are used for longer periods of time, adjuvants such as azathioprine or cyclophosphamide are added to the regimen to reduce the complications of long-term corticosteroid therapy. Before the advent of corticosteroid therapy, pemphigus was fatal, with a mortality rate of up to 75% in the first year. It is still a serious disorder; however, the 5%–10% mortality rate is now primarily due to the side effects of therapy.[10] The different treatment options are summarized in [Table 1].[11]
Table 1: Treatment options

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  Conclusion Top


Dental professionals must be sufficiently familiar with the clinical manifestations of PV to ensure early diagnosis and treatment which in turn determines the prognosis and course of the disease.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ljubojević S, Lipozencić J, Brenner S, Budimcić D. Pemphigus vulgaris: A review of treatment over a 19-year period. J Eur Acad Dermatol Venereol 2002;16:599-603.  Back to cited text no. 1
    
2.
Beutner EH, Jordon RE. Demonstration of skin antibodies in sera of pemphigus vulgaris patients by indirect immunofluorescent staining. Proc Soc Exp Biol Med 1964;117:505-10.  Back to cited text no. 2
    
3.
Femiano F. Pemphigus vulgaris: Recent advances in our understanding of its pathogenesis. Minerva Stomatol 2007;56:215-23.  Back to cited text no. 3
    
4.
Scully C, Challacombe SJ. Pemphigus vulgaris: Update on etiopathogenesis, oral manifestations, and management. Crit Rev Oral Biol Med 2002;13:397-408.  Back to cited text no. 4
    
5.
Black M, Mignogna MD, Scully C. Number II. Pemphigus vulgaris. Oral Dis 2005;11:119-30.  Back to cited text no. 5
    
6.
Kljuic A, Bazzi H, Sundberg JP, Martinez-Mir A, O'Shaughnessy R, Mahoney MG, et al. Desmoglein 4 in hair follicle differentiation and epidermal adhesion: Evidence from inherited hypotrichosis and acquired pemphigus vulgaris. Cell 2003;113:249-60.  Back to cited text no. 6
    
7.
Nguyen VT, Ndoye A, Grando SA. Pemphigus vulgaris antibody identifies pemphaxin. A novel keratinocyte annexin-like molecule binding acetylcholine. J Biol Chem 2000;275:29466-76.  Back to cited text no. 7
    
8.
Fellner MJ, Sapadin AN. Current therapy of pemphigus vulgaris. Mt Sinai J Med 2001;68:268-78.  Back to cited text no. 8
    
9.
Tóth GG, Jonkman MF. Therapy of pemphigus. Clin Dermatol 2001;19:761-7.  Back to cited text no. 9
    
10.
Lever WF, Schaumburg-Lever G. Treatment of pemphigus vulgaris. Results obtained in 84 patients between 1961 and 1982. Arch Dermatol 1984;120:44-7.  Back to cited text no. 10
    
11.
Harman KE, Albert S, Black MM, British Association of Dermatologists. Guidelines for the management of pemphigus vulgaris. Br J Dermatol 2003;149:926-37.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]
 
 
    Tables

  [Table 1]



 

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