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Case Reports in Dentistry

Volume 2015, Article ID 593940, 3 pages
http://dx.doi.org/10.1155/2015/593940

Case Report
Oral Lesions: The Clue to Diagnosis of Pemphigus Vulgaris

Diana Kuriachan, Rakesh Suresh, Mahija Janardhanan, and Vindhya Savithri

Department of Oral Pathology and Microbiology, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham,
Kochi 682041, India
Correspondence should be addressed to Rakesh Suresh; dr.rakesh.s.rao@gmail.com

Received 1 October 2015; Accepted 10 November 2015

Academic Editor: Pia Lopez Jornet

Copyright © 2015 Diana Kuriachan et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.

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. It is therefore important that the oral manifestations of the disease are recognized on time, to make a
proper diagnosis and initiate timely treatment. Here we present a case of Pemphigus Vulgaris (PV) that presented with oral lesions at
multiple sites including tongue, to highlight the importance of timely recognition of the oral lesions during routine dental practice
for the diagnosis and management of this disease.

1. Introduction 2. Case Report of Pemphigus

Vulgaris at Multiple Intraoral Sites,
Pemphigus Vulgaris (PV) is the most common variant of the
with No Involvement of Skin
Pemphigus group of potentially fatal autoimmune diseases
characterized by cutaneous or mucosal blistering and shows A 55-year-old gentleman presented with painful nonhealing
oral lesions as early manifestations of the disease in nearly ulcers on the left buccal mucosa and left posterolateral border
50% of the cases [1, 2]. Its peak incidence is between the of tongue four months ago. History revealed that he had
fourth and fifth decade of life [3]. Clinically oral lesions burning sensation at both sites for the past six months. He
precede skin lesions in many cases and appear as blisters was aware of one blister which appeared and burst rapidly
which rupture rapidly resulting in painful erosions. Buccal on the buccal mucosa, after which ulcerations appeared on
mucosa, lips, and soft palate are most commonly involved both the sites. There was no history of skin lesions. Intraoral
[4]. Diagnosis is based on the identification of clinical man- examination revealed a 2 cm × 2 cm ovoid shallow ulcer
ifestations and confirmation through biopsy. Demonstration with sloping margins along the line of occlusion of 35 to
of immunoglobulins, in the spinous cell junctions by distinct 37 on the left buccal mucosa (Figure 1) and a 1 cm × 1 cm
ovoid ulcer with yellow crusted surface on the left postero-
immunofluorescence (IF), is often used for the final confir-
lateral border of the tongue (Figure 2). After ascertaining the
mation of PV [5, 6]. As the oral presentation of the disease
absence of traumatic agents like sharp tooth/cusp, dentures,
is often the first indicator that can lead to the final diagnosis, and so forth, a provisional diagnosis of vesiculobullous
it is very critical for the dental practitioner to recognize the lesions, namely, Pemphigus, Pemphigoid, or Bullous Lichen
oral lesions of PV at a sufficiently early stage to initiate further Planus, was considered. Incisional biopsy was performed
investigations and treatment. We present a case of PV where and adequate tissue bits were taken from both sites for
the patient presented with ulcerations at multiple oral sites histopathologic examination. Bits from the perilesional area
including tongue and the final diagnosis was made by the were also sent for direct IF studies separately. Histopathologic
timely interpretation of these manifestations. features of the sections from both the sites were similar and
2 Case Reports in Dentistry

Figure 1: Ulcer on the left buccal mucosa, ovoid in shape.

Figure 3: Epithelium exhibiting suprabasal split (H&E stain, ×100).

Figure 2: Ulcer with yellow crusted surface on the left posterolateral

border of tongue.
Figure 4: Acantholytic Tzanck cells within the suprabasal split
(H&E stain, ×400).
showed ulcerated stratified squamous epithelium exhibit-
ing suprabasal split (Figure 3). Many round acantholytic
(Tzanck) cells with hyperchromatic nuclei were observed Our patient presented with oral lesions in the form of
within the split (Figure 4). Basal cells were seen attached to ulcerations four months ago and was aware of one blister
the underlying connective tissue, below the split. A dense forming prior to this. As the oral cavity is subject to trauma
inflammatory cell infiltrate consisting mainly of plasma cells during mastication, the thin roof of the blister ruptures easily
was seen in the connective tissue. These microscopic features and forms an erosion or ulcer in the area. This patient
were suggestive of PV. The direct IF showed deposits of IgG did not develop any lesion on the skin [10]. As reported
and C3 (complement) in a fish-net pattern along the spinous in literature our patient too presented with the two most
intercellular zone, which confirmed the diagnosis of PV. common symptoms related to PV, that is, pain and burning
sensation. Many cases of PV have been reported to begin
3. Discussion as generalized lesions involving multiple intraoral sites as in
our case where the patient developed lesions on the buccal
Derived from the Greek word meaning “blister,” Pemphigus mucosa and the tongue. Though buccal mucosa has been
is a group of potentially life-threatening autoimmune muco- reported to be one of the most commonly affected sites,
cutaneous disorders characterized by intraepithelial blister tongue, which was also affected in our case, is a rare site for
formation [1]. The blisters occur in the epithelium where the PV [9]. Since the clinical features of PV are similar to those
patients IgG autoantibodies produced in response to trig- seen in Cicatricial Pemphigoid and Bullous Lichen Planus, its
gering factors target two structured proteins of desmosomes diagnosis needs to be confirmed with routine histopathology
identified as Desmogleins 1 and 3. Recently, a new Pemphigus and IF studies. Tissue bits from both sites were taken in
antigen Desmoglein 4 and other non-Desmoglein antigens our case and the histologic features were suggestive of PV.
like human 𝛼-9-acetylcholine receptor that regulates ker- The diagnostic features were the presence of a suprabasilar
atinocyte adhesion and keratinocyte annexin like molecules split and acantholytic Tzanck cells in the split, both produced
binding acetylcholine termed pemphaxin and catenin are also due to the intraepithelial blister formation. Direct IF study
thought to play a role in its etiopathogenesis [7, 8]. Thin showed the typical “fish-net” pattern of IgG and complement
separation at the desmosomal region triggers the acantholysis C3 deposits in the spinous layer, the site for the autoimmune
and suprabasal spilt. reaction in PV. Both histopathology and IF studies confirmed
Pemphigus usually affects patients in the fourth and fifth the diagnosis of PV.
decades of life, with females reportedly being affected more PV is generally treated with oral, intralesional, and topical
frequently than males [3]. More than 50% of the affected corticosteroids [11]. The present treatment regime in PV is
patients have been reported with initial manifestations on based on systemic immunosuppressant like corticosteroids
the oral mucosa followed by skin involvement. The average along with adjuvants like methotrexate, cyclophosphamide,
duration of the oral lesions is found to be between 3 months and so forth [6]. Drugs like cholinergic agonists are thought
and one year [9]. to reverse the acantholysis in PV [5]. Our patient, in
Case Reports in Dentistry 3

consultation with the department of dermatology, was put [8] V. T. Nguyen, “Pemphigus vulgaris antibody identifies pem-
on 100 mg dexamethasone for 3 days along with 500 mg phaxin: a novel keratinocyte annexin-like molecule binding
of cyclophosphamide. Two more cycles of this regime at Acetycholine,” The Journal of Biological Chemistry, vol. 275, no.
intervals of 4 weeks each are planned. The patient was put on 38, pp. 29466–29476, 2000.
30 mg Wysolone tablets during the interim 4-week period. A [9] T. Shamim, V. I. Varghese, P. M. Shameena, and S. Sudha,
review after 2 weeks of the initial steroid therapy showed that “Pemphigus vulgaris in oral cavity: clinical analysis of 71 cases,”
the cheek and the tongue ulcerations were resolving which Medicina Oral, Patologia Oral y Cirugia Bucal, vol. 13, no. 10,
indicated a positive response to the treatment. Article ID 1111111613, pp. 622–626, 2008.
PV, a potentially fatal disease with most cases showing [10] T. Shamim, V. Ipe Varghese, P. M. Shameena, and S. Sudha,
initial oral manifestations, requires early diagnosis as well “Oral pemphigus vulgaris: clinicopathologic study of 20 cases,”
Indian Journal of Pathology and Microbiology, vol. 50, no. 3, pp.
as early treatment to prevent future complications. The
498–501, 2007.
diagnosis of PV is based on 3 main factors, clinical features,
[11] J.-C. Bystryn and J. L. Rudolph, “Pemphigus,” The Lancet, vol.
histopathology, and immunofluorescence studies. Many a
366, no. 9479, pp. 61–73, 2005.
time, keen observation of the oral symptoms leading to a
histopathologic examination will suffice for a final diagnosis.
This in turn can facilitate early treatment which will be
highly beneficial to the patient’s recovery. Nevertheless, long
term regular follow-up is essential to identify the possible
remissions of this disease.

Conflict of Interests
The authors declare that there is no conflict of interests
regarding the publication of this paper.

Authors’ Contribution
Diana Kuriachan and Rakesh Suresh were responsible for
concept, design and content, literature search, and preparing,
editing, and reviewing the paper. Mahija Janardhanan and
Vindhya Savithri were responsible for design and content and
editing and reviewing the paper. The paper has been read and
approved by all the authors.

References
[1] M. Black, M. D. Mignogna, and C. Scully, “Number II. Pemphi-
gus vulgaris,” Oral Diseases, vol. 11, no. 3, pp. 119–130, 2005.
[2] T. Hashimoto, “Recent advances in the study of the pathophys-
iology of pemphigus,” Archives of Dermatological Research, vol.
295, no. 1, pp. S2–S11, 2003.
[3] A. Iamaroon, P. Boonyawong, P. Klanrit, S. Prasongtunskul, and
K. Thongprasom, “Characterization of oral pemphigus vulgaris
in Thai patients,” Journal of Oral Science, vol. 48, no. 1, pp. 43–46,
2006.
[4] M. D. Mignogna, L. Lo Muzio, and E. Bucci, “Clinical features of
gingival pemphigus vulgaris,” Journal of Clinical Periodontology,
vol. 28, no. 5, pp. 489–493, 2001.
[5] F. Femiano, “Pemphigus vulgaris; recent advances in our under-
standing of its pathogenesis,” Minerva Stomatologica, vol. 56, no.
4, pp. 215–223, 2007.
[6] C. Scully and S. J. Challacombe, “Pemphigus vulgaris: update
on etiopathogenesis, oral manifestations, and management,”
Critical Reviews in Oral Biology and Medicine, vol. 13, no. 5, pp.
397–408, 2002.
[7] A. Kljuic, H. Bazzi, J. P. Sundberg et al., “Desmoglein 4 in hair
follicle differentiation and epidermal adhesion: evidence from
inherited hypotrichosis and acquired pemphigus vulgaris,” Cell,
vol. 113, no. 2, pp. 249–260, 2003.
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