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http://dx.doi.org/10.14476/jomp.2021.46.2.63

A Case of Mucous Membrane Pemphigoid: From Diagnosis to Therapeutic Strategy  

Kang, Kun-Hwa (Department of Oral Medicine, School of Dentistry, Kyungpook National University)
Byun, Jin-Seok (Department of Oral Medicine, School of Dentistry, Kyungpook National University)
Jung, Jae-Kwang (Department of Oral Medicine, School of Dentistry, Kyungpook National University)
Choi, Jae-Kap (Department of Oral Medicine, School of Dentistry, Kyungpook National University)
Publication Information
Journal of Oral Medicine and Pain / v.46, no.2, 2021 , pp. 63-68 More about this Journal
Abstract
Mucous membrane pemphigoid (MMP) is a heterogeneous group of chronic, autoimmune subepithelial blistering diseases, predominantly involving oral mucosal membrane. Because of its pathophysiology of autoimmune reaction, MMP-related gingivitis would not respond to conventional periodontal treatments. We present a case of a 65-year-old female with a chief complaint of chronic generalized buccal gingivitis, unimproved after periodontal treatment for four months. Based on the clinical findings, histological examination, and direct immunofluorescence microscopy, it was diagnosed as MMP. The symptoms were relieved with immunomodulatory therapy using corticosteroids and the supportive management of professional plaque control. MMP can cause pathological involvement throughout the oral and other mucosae of the body, thus leading to functional impairment through repeated inflammatory cascades. Therefore, accurate diagnosis is essential to properly manage local and possible systemic complications of MMP.
Keywords
Gingivitis; Mucous membrane pemphigoid; Subepithelial autoimmune disease; Topical corticosteroids;
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1 Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002;138:370-379.
2 Xu HH, Werth VP, Parisi E, Sollecito TP. Mucous membrane pemphigoid. Dent Clin North Am 2013;57:611-630.   DOI
3 Chan LS. Mucous membrane pemphigoid. Clin Dermatol 2001;19:703-711.   DOI
4 Bertram F, Brocker EB, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges 2009;7:434-440.
5 Ahmed AR, Hombal SM. Cicatricial pemphigoid. Int J Dermatol 1986;25:90-96.   DOI
6 Balding SD, Prost C, Diaz LA, et al. Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. J Invest Dermatol 1996;106:141-146.   DOI
7 Kirtschig G, Marinkovich MP, Burgeson RE, Yancey KB. Antibasement membrane autoantibodies in patients with anti-epiligrin cicatricial pemphigoid bind the alpha subunit of laminin 5. J Invest Dermatol 1995;105:543-548.   DOI
8 Luke MC, Darling TN, Hsu R, et al. Mucosal morbidity in patients with epidermolysis bullosa acquisita. Arch Dermatol 1999;135:954-959.
9 Neff AG, Turner M, Mutasim DF. Treatment strategies in mucous membrane pemphigoid. Ther Clin Risk Manag 2008;4:617-626.   DOI
10 Chan LS, Majmudar AA, Tran HH, et al. Laminin-6 and laminin-5 are recognized by autoantibodies in a subset of cicatricial pemphigoid. J Invest Dermatol 1997;108:848-853.   DOI
11 Tyagi S, Bhol K, Natarajan K, Livir-Rallatos C, Foster CS, Ahmed AR. Ocular cicatricial pemphigoid antigen: partial sequence and biochemical characterization. Proc Natl Acad Sci U S A 1996;93:14714-14719.   DOI
12 Georgoudis P, Sabatino F, Szentmary N, et al. Ocular mucous membrane pemphigoid: current state of pathophysiology, diagnostics and treatment. Ophthalmol Ther 2019;8:5-17.   DOI
13 Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013;381:320-332.   DOI
14 Scully C, Lo Muzio L. Oral mucosal diseases: mucous membrane pemphigoid. Br J Oral Maxillofac Surg 2008;46:358-366.   DOI
15 Bagan J, Jimenez Y, Murillo J, Bagan L. Oral mucous membrane pemphigoid: a clinical study of 100 low-risk cases. Oral Dis 2018;24:132-134.   DOI
16 Al-Abeedi F, Aldahish Y, Almotawa Z, Kujan O. The differential diagnosis of desquamative gingivitis: review of the literature and clinical guide for dental undergraduates. J Int Oral Health 2015;7(Suppl 1):88-92.
17 Buonavoglia A, Leone P, Dammacco R, et al. Pemphigus and mucous membrane pemphigoid: an update from diagnosis to therapy. Autoimmun Rev 2019;18:349-358.   DOI
18 Uzun S, Durdu M. The specificity and sensitivity of Nikolskiy sign in the diagnosis of pemphigus. J Am Acad Dermatol 2006;54:411-415.   DOI
19 Meijer JM, Diercks GFH, de Lang EWG, Pas HH, Jonkman MF. Assessment of diagnostic strategy for early recognition of bullous and nonbullous variants of pemphigoid. JAMA Dermatol 2019;155:158-165.   DOI
20 Goletz S, Hashimoto T, Zillikens D, Schmidt E. Anti-p200 pemphigoid. J Am Acad Dermatol 2014;71:185-191.   DOI
21 Miserocchi E, Baltatzis S, Roque MR, Ahmed AR, Foster CS. The effect of treatment and its related side effects in patients with severe ocular cicatricial pemphigoid. Ophthalmology 2002;109:111-118.   DOI
22 Chan LS. Ocular and oral mucous membrane pemphigoid (cicatricial pemphigoid). Clin Dermatol 2012;30:34-37.   DOI
23 Bernard P, Prost C, Lecerf V, et al. Studies of cicatricial pemphigoid autoantibodies using direct immunoelectron microscopy and immunoblot analysis. J Invest Dermatol 1990;94:630-635.   DOI
24 Ahmed AR, Kurgis BS, Rogers RS 3rd. Cicatricial pemphigoid. J Am Acad Dermatol 1991;24(6 Pt 1):987-1001.   DOI
25 Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol 2009;60:595-603.   DOI
26 Foster CS, Chang PY, Ahmed AR. Combination of rituximab and intravenous immunoglobulin for recalcitrant ocular cicatricial pemphigoid: a preliminary report. Ophthalmology 2010;117:861-869.   DOI
27 Rashid H, Lamberts A, Diercks GFH, et al. Oral lesions in autoimmune bullous diseases: an overview of clinical characteristics and diagnostic algorithm. Am J Clin Dermatol 2019;20:847-861.   DOI
28 Ahmed AR, Shetty S. A comprehensive analysis of treatment outcomes in patients with pemphigus vulgaris treated with rituximab. Autoimmun Rev 2015;14:323-331.   DOI
29 Schellinck AE, Rees TD, Plemons JM, Kessler HP, Rivera-Hidalgo F, Solomon ES. A comparison of the periodontal status in patients with mucous membrane pemphigoid: a 5-year follow-up. J Periodontol 2009;80:1765-1773.   DOI
30 Jascholt I, Lai O, Zillikens D, Kasperkiewicz M. Periodontitis in oral pemphigus and pemphigoid: a systematic review of published studies. J Am Acad Dermatol 2017;76:975-978.e3.   DOI