QJM: An International Journal of Medicine, 2017, 1–2

doi: 10.1093/qjmed/hcx164
Advance Access Publication Date: 16 August 2017
Clinical picture

CLINICAL PICTURE

Pemphigus vulgaris
A 36-year-old female presented with burning sensation of the which ultimately results in the formation of suprabasilar bulla.3
gingiva which increased on consumption of spicy foodstuff. The differential diagnosis of pemphigus vulgaris includes aph-
Intraoral examination revealed severe generalized erythema thous stomatits, lichenplanus, oral candidiasis and pemphigoid
with detachment of superficial layer of gingiva (Figure 1A, black which can identified by clinical findings, immunofluorescence
arrow). Extraoral examination of the skin of hand revealed mul- tests, histopathological examination and periodic acid Schiff
tiple bullae (Figure 1B, black arrows) with positive Nikolsky sign test using smear samples for candidial hyphae. Treatment con-
1 and Asboe—Hansen sign or Nikolsky sign 2. Perilesional sists of systemic and topical steroids with constant follow-up
biopsy samples from gingiva and skin showed suprabasal acan- visits to prevent the recurrence of the disease.
tholytic blisters and intercellular edema in the lower epithe-
lium. Direct immunofluorescence demonstrated IgG in the
Photographs and text from: T. Chaubal, Department of
intercellular regions of the epithelium. Desmoglein 3 ELISA test
Periodontics and Oral Implantology, D.Y.Patil University School
was positive for anti desmoglein 3 antibodies in the serum.
of Dentistry, Nerul, Navi Mumbai, Maharashtra 400706, India; R.
Based on the above investigations, the diagnosis arrived was
Bapat, Division of Clinical Dentistry, School of Dentistry,
Pemphigus vulgaris. Treatment instituted was topical applica-
International Medical University, Kuala Lumpur 57000,
tion of 0.1% triamcinolone acetonide on the oral lesions for
Malaysia; P. Wadkar, Department of Periodontics and Oral
4 weeks and systemic prednisolone 80 mg for 2 weeks. Systemic
Implantology, D.Y.Patil University School of Dentistry, Nerul,
prednisolone was reduced in a staggered manner for further
Navi Mumbai, Maharashtra 400706, India. Email:
4 weeks; oral and skin lesions regressed after 6 weeks. Gingival
tanayvc@gmail.com
and skin lesions healed completely without scarring at 1-year
follow-up visit. Pemphigus vulgaris is an autoimmune, intraepi-
thelial, blistering disease affecting the skin and mucous mem-
branes.1 The incidence rate of pemphigus is 1–16 cases per year Conflict of interest: None declared.
per million people.2 Pemphigus vulgaris is associated with the
binding of IgG autoantibodies to desmoglein 3, a transmem- References
brane glycoprotein adhesion molecule present on desmosomes
1. Grando SA. Pemphigus autoimmunity: hypotheses and real-
resulting in loss of intercellular adhesion and separation of cells
ities. Autoimmunity 2012; 45:7–35.
called as acantholysis in lower layers of stratum spinosum

Figure 1. (A): Oral cavity showing detachment of the epithelium of gingiva (black arrow). (B): Skin of hand showing multiple bullae (black arrows).

C The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved.
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2. Uzun S, Durdu M, Akman A, Gunasti S, Uslular C, Memisoglu 3. Ben Lagha N, Poulesquen V, Roujeau JC, Alantar A, Maman L.
HR, et al. Pemphigus in the Mediterranean region of Turkey: a Pemphigus vulgaris: a case-based update. J Can Dent Assoc
study of 148 cases. Int J Dermatol 2006; 45:523–8. 2005; 71:667–72.