Case Report

Oral Mucous Membrane Pemphigoid With Positive Nikolsky’s Sign: A Case Report


Mucous membrane pemphigoid (MMP) is a rare inflammatory, autoimmune, and subepithelial vesiculobullous disease in which tissue-bound autoantibodies are produced against one or more components of the basement membrane. Oral lesions of the pemphigoid begin in the form of vesicles or bullae that often involve throughout the mouth but may be confined to specific areas, especially the gingiva, in a pattern known as desquamative gingivitis. The positive Nikolsky's sign is characteristic of pemphigus vulgaris, in which a blister can appear on the normal-appearing skin if exerting lateral pressure, and is very rare in the mucosa and other vesiculobullous diseases. Here we report a case of mucous membrane pemphigoid that developed as desquamated gingivitis in a 46-year-old woman with positive Nikolsky's sign in the gingival mucosa. In the histopathologic view, a subepithelial cleft was observed. The results of direct and indirect immunofluorescence tests and related therapeutic interventions are also presented. Positive Nikolsky's sign can be observed in the mucosa as well as in the mucous membrane pemphigoid in addition to pemphigus vulgaris, and vesiculobullous lesions should be diagnosed based on the sum of clinical, histopathological, and immunofluorescence findings.

1. Rao S, Hegde S, Ajila V, Babu S, Buch SA. Mucous membrane pemphigoid presenting as bleeding gums and burning sensation of mouth: a case report. Arch Orofac Sci. 2018; 13(1): 45-9.
2. Shenoy S, Kedlaya MN, HR S, Ramesh A. Oral mucous membrane pemphigoid with contact dermatitis rare clinical entity. a case report of meilodosis. Nitte Univ J Health Sci. 2018; 8(3): 41-4.
3. Neville BW, Daam DD, Allen CM, Chi AC. Oral and maxillofacial pathology.4th ed. Missouri: Saunders co; 2016. p. 718
4. Soni AG. Nikolsky's sign - A clinical method to evaluate damage at epidermal-dermal junction. J Indian Acad Oral Med Radiol. 2018; 30 (1):68-72.
5. Chen S, Mattei P, Fischer M, Gay JD, Milner SM, Price LA. Linear IgA bullous dermatosis. Eplasty. 2013; 13:ic49.
6. Gomes IO, DE Morais HO, Chagas WP, Petillo CR, Antero SA, Israel MS, et al. Treatment of mucous membrane pemphigoid with low-level laser therapy. Oral Surg Oral Med Oral Pathol Oral Radiol. 2020; 129(1):e26.
IssueVol 58, No 12 (2020) QRcode
SectionCase Report(s)
Benign mucous membrane pemphigoid Mouth Mucous membrane

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How to Cite
Akbari N, Mozafari G, Abbaszadeh H. Oral Mucous Membrane Pemphigoid With Positive Nikolsky’s Sign: A Case Report. Acta Med Iran. 2021;58(12):654-657.