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E-IJD CORRESPONDENCE
Year : 2015  |  Volume : 60  |  Issue : 2  |  Page : 218
Concurrent occurrence of pemphigus vulgaris and oral submucous fibrosis: An unusual presentation


1 Department of Oral and Maxillofacial Pathology and Microbiology, Rishiraj College of Dental Sciences and Research Centre, Bhopal, Madhya Pradesh, India
2 Department of Oral and Maxillofacial Pathology and Microbiology, People's College of Dental Sciences and Research Centre, Bhopal, Madhya Pradesh, India
3 Department of Oral and Maxillofacial Pathology and Microbiology, People's Dental Academy, Bhopal, Madhya Pradesh, India
4 Department of Oral and Maxillofacial Pathology and Microbiology, Ram Krishna Dharmarth Foundation Dental College, Bhopal, Madhya Pradesh, India

Date of Web Publication3-Mar-2015

Correspondence Address:
Samar Khan
Department of Oral and Maxillofacial Pathology and Microbiology, Rishiraj College of Dental Sciences and Research Centre, Bhopal, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.152597

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How to cite this article:
Khan S, Tijare MS, Jain M, Nagpal N, Desai AP. Concurrent occurrence of pemphigus vulgaris and oral submucous fibrosis: An unusual presentation. Indian J Dermatol 2015;60:218

How to cite this URL:
Khan S, Tijare MS, Jain M, Nagpal N, Desai AP. Concurrent occurrence of pemphigus vulgaris and oral submucous fibrosis: An unusual presentation. Indian J Dermatol [serial online] 2015 [cited 2020 Apr 1];60:218. Available from: http://www.e-ijd.org/text.asp?2015/60/2/218/152597


Sir,

A 19-year-old male patient reported with a history of restricted mouth opening of nearly 20 mm since 1 year, painful ulcerations for duration of 6 months and superadded burning sensation from the last 2 months. The patient also complains of multiple vesicles approximately 20-25 in number which were present in the back and front of the body, associated with itching from last 1.5 months. These vesicles subside spontaneously in 2-3 days without intervention. History revealed that the lesion had an insidious onset about 5 months ago on the lower lip and slowly progressed to multiple ulcers in the following 2-3 months.

On general examination the patient was moderately built, well nourished and well oriented to time, place and person. The patient gave a history of continuous fever for 2-3 days since 1 month. Multiple vesicles and scabs present on the chest and back and with no significant medical history.

Intra-oral examination revealed poor oral hygiene with erythematous gingiva. There were multiple small irregular erosions, area of ulceration and intense erythema. Multiple ulcers with severe erosion were present on the right and left buccal mucosa [Figure 1]. On palpation the lesion was tender with tendency to bleed. Nikolsky's sign was positive on buccal mucosa.
Figure 1: Buccal mucosa showing ulceration and reduced mouth opening

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On extra-oral examination the skin over the face was associated with multiple papules on the left side of the face and two ruptured vesicles with crust formation and there were multiple vesicles and with scab formation was found to be present on the skin of chest and back [Figure 2]. Provisional diagnosis of Oral submucous fibrosis and suspicion of pemphigus vulgaris was given. Differential diagnosis of bullous pemphigoid, linear IgA bullous dermatitis, erythema multiforma and bullous lichen planus was given.
Figure 2: Multiple vesicles with scab on skin of back

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Investigation

Routine investigations including complete hemogram, peripheral smear, exfoliative cytology followed by special investigation of biopsy and immunofluorescence was carried out.

Cytology report showed acantholytic- rounded Tzanck cells which confirmed the diagnosis pemphigus vulgaris [Figure 3].
Figure 3: Smear showing acantholytic Tzanck cells. (H and E, ×1000)

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An incisional biopsy was performed from the lower labial mucosa and the H and E- stained soft tissue specimen showed parakeratinized epithelium showing intraepithelial cleft formation and rounded epithelial cells with relatively large hyperchromatic nucleus (Tzanck cells) within the cleft [Figure 4] and [Figure 5]. Underlying connective tissue shows prominent blood capillaries reaching high up into the papillary layer along with chronic inflammatory cell infiltrate and hemorrhage.
Figure 4: Intraepithelial cleft with prominent blood capillaries and hyalinization of stroma showing interlacing bundles of collagen fibers. (H and E stain, ×200)

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Figure 5: Intraepithelial cleft showing acantholytic cells. (H and E stain, ×400)

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The specimen was then submitted for confirmatory diagnosis. (Direct immunofluorescence) showed positive intracellular deposits of IgG which confirmed the diagnosis of pemphigus vulgaris [Figure 6].
Figure 6: Direct immunofluorescence showing intracellular deposition of IgG

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Treatment

The patient responded to therapy on systemic steroids with a initial dose of 60 mg/kg/day, calcium supplements, cimetidine, mouth wash, Anabel gel was also given-; the prednisolone dose was tapered down to 10 mg/kg/day and the patient is still under the course of low dose of steroids.


   Discussion Top


The case report is the rare association of Oral submucous fibrosis with pemphigus with only two documented studies in the literature showing this association where the two lesions had lead to malignant transformation. [1],[2] Association of OSMF and pemphigus is a mere chance or do they share common pathogenicity is yet to be elucidate but there are certain common factors such as: (1) similarity of both these conditions which is presumed to have an autoimmune pathogenesis that has evoked a possibility of autoimmune basis for the occurrence of both the diseases in a single spectrum. (2) An increased frequency of HLA haplotypic pairs in OSMF and HLA-DR presentation of Dsg-3 derived peptides may suggest an MHC- mediated immunological derangement operating in both the disease. [3],[4] (3) Cell- mediated and humoral response may also suggest to play a role in present association as studies have shown increase in serum level of IgA, IgD, IgE and circulating immune complexes in OSMF which is yet again common finding in pemphigus vulgaris.

So it can thus be postulated that it is the presence of these immunological factors which might be the reason for their concurrent occurrence. But future research should be done to substantiate the role of such factors in order to determine the occurrence. Therefore, to expound that such an association is a mere chance or is significant lies down on further molecular and immunological studies in order to substantiate the above- suggested pathogenesis.

 
   References Top

1.
Hay RJ, Calnan CD. Oral submucous fibrosis in a patient with pemphigus vulgaris. Clin Exp Dermatol 1979;4:381-3.  Back to cited text no. 1
    
2.
Dayal PK, Joshi HN, Dayal JP. Concomitant occurrence of oral submucous fibrosis, pemphigus and squamous cell carcinoma. Indian J Pathol Microbiol 1988;31:334-7.  Back to cited text no. 2
    
3.
Rajendran R. Oral submucous fibrosis: Etiology, pathogenesis, and future research. Bull World Health Organ 1994;72:985-96.  Back to cited text no. 3
    
4.
Scully C, Challacombe SJ. Pemphigus vulagris: Update on eitiopathogenesis, oral manifestations, and management. Crit Rev Oral Biol Med 2002;13:397-408.  Back to cited text no. 4
    


    Figures

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



 

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