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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 33  |  Issue : 2  |  Page : 222-225

Mucocutaneous pemphigus vegetans-A rare case report


Department of Oral Medicine and Radiology, Saveetha Dental College and Hospitals, Chennai, Tamil Nadu, India

Date of Submission29-Dec-2020
Date of Acceptance08-Apr-2021
Date of Web Publication23-Jun-2021

Correspondence Address:
Dr. T N Uma Maheswari
Department of Oral Medicine and Radiology, Saveetha Dental College and Hospitals, Saveetha Institute of Medical and Technical Sciences, Saveetha University, Chennai - 600 077, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_282_20

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   Abstract 


Pemphigus vegetans, a rare clinical variant of pemphigus vulgaris which is a group of vesiculobullous autoimmune diseases. The characteristic clinical feature of pemphigus vegetans is formation of vegetative plaques in oral mucosa. A 40 year old female patient reported with mucocutaneous ulcerative lesions with lip crustations. This patient was under 8 months follow up with periodic remissions and exacerbation. Hence, this case report highlights the significance of early diagnosis, appropriate therapeutic intervention and periodic follow up of pemphigus vegetans to achieve complete remission and prevent adverse complications of long term immunomodulatory therapy.

Keywords: Autoimmune disorders, pemphigus vulgaris, pemphigus vegetans, hirsutism


How to cite this article:
Jayapriya R, Chaudhary M, Uma Maheswari T N, Muthukrishnan A. Mucocutaneous pemphigus vegetans-A rare case report. J Indian Acad Oral Med Radiol 2021;33:222-5

How to cite this URL:
Jayapriya R, Chaudhary M, Uma Maheswari T N, Muthukrishnan A. Mucocutaneous pemphigus vegetans-A rare case report. J Indian Acad Oral Med Radiol [serial online] 2021 [cited 2021 Jul 29];33:222-5. Available from: https://www.jiaomr.in/text.asp?2021/33/2/222/319075




   Introduction Top


Pemphigus belongs to a group of autoimmune potentially life threatening diseases that cause blisters and erosions of skin and mucous membrane.[1],[2] Pemphigus vegetans is a rarest variant of pemphigus vulgaris. The epidemiology of pemphigus vegetans represents incidence of pemphigus vulgaris ranges from 0.42 to 1.62 cases per 100,000.[3] Pemphigus vegetans comprise less than 1-2% of all pemphigus cases[4] and worldwide the incidence of pemphigus vulgaris is approximately 0.7 per 100000. Oral mucosal manifestations of pemphigus vegetans account 60-80% which represent a granular or cobblestone appearance on an erythematous base.


   Case Report Top


Clinical description

A 40 year old female housewife patient reported to our department with a complaint of pain and ulcers in the lips and mouth for 2 months. History reveals 8 months back patient developed ulcers in the tongue for which she consulted a general physician and haematological investigations were done and was diagnosed with anaemia with her haemoglobin 7gm% and one sitting of blood transfusion was done. Two months later she developed fluid filled blisters involving neck, thorax and thigh sparing the lower extremities then she was referred to a dermatologist and she was under empirical systemic corticosteroids 20 mg prednisolone and was not subjected to any investigation and she discontinued medications without any medical advice. Dermal lesions healed with pigmentation and scarring. Two months back she developed ulcers in the lips, tongue, and mouth which were painful. Pain was severe, continuous, burning in nature and aggravated on eating spicy food, ulcers developed following erythema of lips, interfered with mastication.

On extraoral examination, multiple scars in the forehead, neck, arms , hands , upper body till the thigh sparing the lower extremities were seen. Redness of the left eye and lower lip was bilaterally swollen and appeared everted with multiple ulceration, erosions with fissuring and crustations. On intraoral examination, there was a generalised red and erythematous desquamation of the marginal gingiva, multiple ulcers seen in left and right buccal mucosa, upper and lower labial mucosa, tongue and soft palate as seen in [Figure 1] and [Figure 2].
Figure 1: (a) Reveals conjunctivitis in left eye. (b) Multiple blisters with scarring dermal lesions

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Figure 2: Reveals severe bleeding ulcerations and crustations of tongue, buccal mucosa and lips

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Diagnostic assessment

On correlation of history and clinical examination such as patient initially experienced fluid filled cutaneous lesions which healed with systemic steroids and currently having ocular erythema with generalised irregular oral ulceration, lip crustations and dysphagia it was provisionally diagnosed as an immune bullous disorder like pemphigus vulgaris. Differential diagnosis included pemphigus vulgaris, paraneoplastic pemphigus, linear IgA, pemphigoid and erythema multiforme. An incisional biopsy was done from the left buccal mucosa and a histopathological report revealed epithelial thickening with prolonged rete pegs, intraepithelial clefts in suprabasal cells. Eosinophilic microabscesses were seen within the epithelium. Other microscopic changes were intraepithelial edema and eosinophilic infiltration, chronic inflammatory cells infiltration in the lamina propria [Figure 3].
Figure 3: Histopathological features (arrows) revealing (a) intra epithelial cleft, (b) eosinophilic microabscess

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Therapeutic intervention

Patient was started on systemic corticosteroid oral prednisolone 5 mg thrice a day for a week with super potent topical corticosteroid clobetasol propionate 0.05% to be applied on the dermal lesions twice daily for a week, betamethasone 0.5mg tablet to be crushed and mixed with water and use as a mouthwash once daily for a week and a multivitamin tablet once daily for a month and the patient was reviewed after a week. For ocular involvement, the patient had visited an ophthalmologist who had advised eye lubricants drops.

Follow-up and outcome

First follow up was after a week, intraoral and extra oral reduction in the lesion was observed as seen in [Figure 4]. The second follow up was done after 1 month. Remission of the oral and dermal lesions was observed as seen in [Figure 5] and she was put on a maintenance dose of 10mg of systemic prednisolone. After a week of tapering systemic prednisolone she had recurrence of oral and dermal lesions and failed to respond to the previous treatment so intramuscular dexamethasone 8 mg per day for 5 days, systemic oral prednisolone 40 mg prednisolone for 2 weeks and 10 mg cetirizine was given. Third follow up was after 2 weeks, the dermal and oral lesions were reduced. Hence, a maintenance dose of 20mg of systemic oral prednisolone was prescribed. Fourth follow up was done after 1 month with complete resolution of dermal and oral lesions were observed as seen in [Figure 6]. The patient failed to report for 5 months and was still on 20mg prednisolone so She developed side effects of systemic corticosteroids like increase in body weight and hirsutism as shown in [Figure 7] and [Table 1].
Table 1: Timeline of events that took place in this case

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Figure 4: Remission of (a) intraoral (b) extra oral lesions after one week follow-up

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Figure 5: Recurrence of oral and dermal lesions after one month maintenance phase

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Figure 6: Post treatment follow-up shows complete resolution of dermal and oral lesions after 4 months of treatment

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Figure 7: The adverse effects of long term corticosteroid therapy

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   Discussion Top


Pemphigus vulgaris is a rare intraepithelial blistering disorder which was life threatening but with recent therapeutic advancement mortality is reduced. A localised variant of pemphigus vulgaris is pemphigus vegetans. Pemphigus Vegetans Neumann type has a similar course to pemphigus vulgaris and it requires higher doses of corticosteroids and has relapses and remissions.[5] Hallopeau patients have fewer relapses and respond to lower doses of corticosteroids.[6] The first line of treatment is systemic corticosteroid. Ocular findings are usually seen in cicatricial pemphigoid but this was seen as a unique clinical feature in the present case. Prednisone (1-2 mg / kg/ day) can be administered and the dose is increased by 25% to 50% every week and slowly tapered by reducing dose by 2.5- to 5.0 mg decrements every 3–7 days until physiologic dose (5 to 7.5 mg of prednisone per day) is reached[7] Inappropriate use of systemic corticosteroids will lead to complications like moon facies/buffalo Hump/ truncal obesity, increased blood sugar, decreased bone density/ avascular necrosis.


   Conclusion Top


Pemphigus vegetans, a rare variant of pemphigus vulgaris which poses a diagnostic challenge. Hence a dentist should know the clinical features of both dermal and oral lesions for early diagnosis and timely management. Multiple drug treatment approaches are required in management. Constant follow up is required to assess disease progression and should be closely monitored to prevent any adverse effects.

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.
Stanley JR. Pemphigus. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York: McGrawHill; 2008. p. 459-68.  Back to cited text no. 1
    
2.
James WD, Berger TG, Elston DM. Andrews Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia: WB Saunders Co; 2006. p. 459-62.  Back to cited text no. 2
    
3.
Mahoney MG, Wang Z, Rothenberger K, Koch PJ, Amagai M, Stanley JR. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest 1999;103:461-8.  Back to cited text no. 3
    
4.
Rivollier C, Vaillant L, Machet MC, Martin L, Jan V, Huttenberger B, et al. [Paraneoplastic pemphigus: A pustular form during chronic lymphoid leukemia]. Ann Dermatol Venereol 2001;128:644-8.  Back to cited text no. 4
    
5.
Vorobyev A, Ludwig RJ, Schmidt E. Clinical features and diagnosis of epidermolysis bullosa acquisita. Expert Rev Clin Immunol 2017;13:157-69.  Back to cited text no. 5
    
6.
de Almeida HL Jr, Neugebauer MG, Guarenti IM, Aoki V. Pemphigus vegetans associated with verrucous lesions: Expanding a phenotype. Clinics (Sao Paulo) 2006;61:279-82.  Back to cited text no. 6
    
7.
Goes PEM, Luna AHB, De Figueiredo LS, Montenegro EDAS, Da Silveira KG, Maia FPA, et al. Pemphigus vulgaris with oral mucosa and skin involvement: A case report. Oral Surg Oral Med Oral Pathol Oral Radiol 2015;120:e57.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
 
 
    Tables

  [Table 1]



 

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