Mucous Membrane Pemphigoid: A Review of Literature

Main Article Content

Surawut Pongsiriwet
Pansakorn Sangkaew

Abstract

Mucous membrane pemphigoid (MMP) is a chronic, autoimmune subepithelial vesiculobullous disease that predominantly involves the mucous membrane and occasionally the skin. Most patients with MMP affecting the oral mucosa manifest desquamative gingivitis. The diagnosis of MMP must be based on clinical, histopathologic, and immunopathologic features. Corticosteroids and immunosuppressive drugs are the main therapeutic agents used in the treatment of MMP. This article reviews the etiology, pathogenesis, clinical presentation, diagnosis and treatment of MMP.

Article Details

How to Cite
Pongsiriwet, S. ., & Sangkaew, P. . (2018). Mucous Membrane Pemphigoid: A Review of Literature. Chiang Mai Dental Journal, 39(1), 25–42. Retrieved from https://he01.tci-thaijo.org/index.php/cmdj/article/view/197241
Section
Review article

References

Xu HH, Werth VP, Parisi E, Sollecito TP. Mucousmembrane pemphigoid. Dent Clin North Am 2013;57: 611-630.

Di Zenzo G, Carrozzo M, Chan LS. Urban legendseries: mucous membrane pemphigoid. Oral Dis 2014;20: 35-54.

Scully C, Carrozzo M, Gandolfo S, Puiatti P, MonteilR. Update on mucous membrane pemphigoid: a heterogeneousimmune-mediated subepithelialblistering entity. Oral Surg Oral Med Oral PatholOral Radiol Endod 1999; 88: 56-68.

Scully C, Lo Muzio L. Oral mucosal diseases: mucousmembrane pemphigoid. Br J Oral Maxillofac Surg2008; 46: 358-366.

Sollecito TP, Parisi E. Mucous membrane pemphigoid.Dent Clin North Am 2005; 49: 91-106.

Bagan J, Lo Muzio L, Scully C. Mucosal diseaseseries. Number III. Mucous membrane pemphigoid.Oral Dis 2005; 11(4): 197-218.

ฐิติกานต์ เหล่าธรรมทัศน์. มิวคัส เมมเบรน เพมฟิกอยด์.ใน กอบกาญจน์ ทองประสม (บรรณาธิการ). เวชศาสตร์ช่องปากขั้นสูง. พิมพ์ครั้งที่ 2. กรุงเทพฯ: สำนักพิมพ์แห่งจุฬาลงกรณ์มหาวิทยาลัย; 2552: 205-222.

Chan LS, Ahmed AR, Anhalt GJ, et al. The firstinternational consensus on mucous membranepemphigoid: definition, diagnostic criteria, pathogenicfactors, medical treatment, and prognostic indicators.Arch Dermatol 2002; 138(3): 370-379.

Rashid KA, Gurcan HM, Ahmed AR. Antigenspecificity in subsets of mucous membranepemphigoid.J Invest Dermatol 2006; 126: 2631–2636.

Bhol KC, Goss L, Kumari S, Colon JE, Ahmed AR.Autoantibodies to human alpha6 integrin in patientswith oral pemphigoid. J Den Res 2001; 80: 1711–1715

Domloge-Hultsch N, Gammon W, Briggaman R, GilS, Carter W, Yancey K. Epiligrin, the major humankeratinocyte integrin ligand, is a target in both anacquired autoimmune and an inherited subepidermal blistering skin disease. J Clin Invest 1992; 90: 1628–1633.

Egan CA, Lazarova Z, Darling TN, Yee C, YanceyKB. Anti epiligrin cicatricial pemphigoid: clinicalfindings, immunopathogenesis, and significantassociations. Medicine (Baltimore) 2003; 82: 177–186.

Chan RY, Bhol K, Tesavibul M, et al. The role of antibody to human beta4 integrin in conjunctivalbasement membrane separation: possible in vitromodel for ocular cicatricial pemphigoid. Invest Ophthalmol Vis Sci 1999; 40: 2283–2290.

Egan CA, Yee C, Zillikens D, Yancey KB. Anti-p200 pemphigoid: diagnosis and treatment of a case presenting as an inflammatory subepidermal blistering disease. J Am Acad Dermatol 2002; 46: 786-789.

Vassileva S. Drug-induced pemphigoid: bullous and cicatricial. Clin Dermatol 1998; 16: 379–387.

Bruch-Gerharz D, Hertl M, Ruzicka T. Mucous membrane pemphigoid: clinical aspects, immunopathological features and therapy. Eur J Dermatol 2007; 17: 191-200.

Delgado JC, Turbay D, Yunis EJ, et al. A common major histocompatibility complex class II allele HLADQB1* 0301 is present in clinical variants of pemphigoid. Proc Natl Acad Sci USA 1996; 93: 8569-8571.

Ahmed AR, Foster S, Zaltas M, et al. Association of DQw7 (DQB1*0301) with ocular cicatricial pemphigoid. Proc Natl Acad Sci U S A 1991; 88: 11579-11582.

Carrozzo M, Fasano ME, Broccoletti R, et al. HLADQB1 alleles in Italian patients with mucous membrane pemphigoid predominantly affecting the oral cavity. Br J Dermatol 2001; 145: 805-808.

Eversole LR. Immunopathology of oral mucosal ulcerative, desquamative, and bullous diseases. Selective review of the literature. Oral Surg Oral Med Oral Pathol 1994; 77: 555–571.

Eversole LR. Adhesion molecules and oral mucosal diseases. Oral Dis 1996; 2: 185–187.

Yeh SW, Usman AQ, Ahmed AR. Profile of autoantibody to basement membrane zone proteins in patients with mucous membrane pemphigoid: longterm follow up and influence of therapy. Clin Immunol 2004; 112: 268–272.

Carrozzo M, Cozzani E, Broccoletti R, et al. Analysis of antigens targeted by circulating IgG and IgA antibodies in patients with mucous membrane pemphigoid predominantly affecting the oral cavity. J Periodontol 2004; 75: 1302–1308.

Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013; 381: 320-332.

Rashid KA, Gurcan HM, Ahmed AR. Antigen specificity in subsets of mucous membrane pemphigoid. J Invest Dermatol 2006; 126: 2631-2636.

Balding SD, Prost C, Diaz LA, Bernard P, Bedane C, Aberdam D, Giudice GJ. Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. J Invest Dermatol 1996; 106(1):141-146.

Leverkus M, Bhol K, Hirako Y, et al. Cicatricial pemphigoid with circulating autoantibodies to beta4 integrin, bullous pemphigoid 180 and bullous pemphigoid 230. Br J Dermatol 2001; 145(6): 998- 1004.

Bhol KC, Goss L, Kumari S, et al. Autoantibodies to human alpha6 integrin in patients with oral pemphigoid. J Dent Res 2001; 80: 1711–1715.

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.

Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 1995; 131: 48–52.

Bertram F, Brocker EB, Zillikens D, et al. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges 2009; 7: 434–440.

Radford CF, Rauz S, Williams GP, et al. Incidence, presenting features, and diagnosis of cicatrizing conjunctivitis in the United Kingdom. Eye (Lond) 2012; 26: 1199–1208.

Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin

diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 1995; 131: 48–52.

Daniels TE, Quadra-White C. Direct immunofluorescence in oral mucosal disease: a diagnostic analysis of 130 cases. Oral Surg Oral Med Oral Pathol 1981; 51: 38–47.

Chan LS. Mucous membrane pemphigoid. Clin Dermatol 2001; 19: 703–711.

Thorne JE, Anhalt GJ, Jabs DA. Mucous membrane pemphigoid and pseudopemphigoid. Ophthalmology; 111: 45–52.

Siegel MA, Balciunas BA. Oral presentation and management of vesiculobullous disorders. Semin Dermatol 1994; 13(2): 78-86

Regezi JA, Sciubba JJ, Jordan RCK. Oral pathology. Clinical pathologic correlations. 6th ed. St. Louis: MoElsevier/Saunders; 2012: 15-17.

Taylor J, McMillan R, Shephard M, et al. World Workshop on Oral Medicine VI: a systematic review of the treatment of mucous membrane pemphigoid. Oral Surg Oral Med Oral Pathol Oral Radiol 2015;(2): 161-171.

Arduino PG, Lopetuso E, Carcieri P, et al. Professional oral hygiene treatment and detailed oral hygiene instructions in patients affected by mucous membrane pemphigoid with specific gingival localization: a pilot study in 12 patients. Int J Dent Hyg 2012; 10: 138-141.

Lozada F, Silverman S Jr. Topically applied fluocinonide in an adhesive base in the treatment of oral vesiculoerosive diseases. Arch Dermatol 1980; 116(8): 898-901.

Gonzalez-Moles MA, Ruiz-Avila I, RodriguezArchilla A, et al. Treatment of severe erosive gingival lesions by topical application of clobetasol propionate in custom trays. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 95(6): 688-692.

Fistarol SK, Itin PH. Anti-inflammatory treatment. Curr Probl Dermatol 2011; 40: 58-70.

Al Johani KA, Hegarty AM, Porter SR, Fedele S. Calcineurin inhibitors in oral medicine. J Am Acad Dermatol 2009; 61(5): 829-840.

Lee HY, Blazek C, Beltraminelli H, et al. Oral mucous membrane pemphigoid: complete response to topical tacrolimus. Acta Derm Venereol 2011; 91: 604–605.

Suresh L, Martinez Calixto LE, Radfar L. Successful treatment of mucous membrane pemphigoid with tacrolimus. Spec Care Dentist 2006; 26: 66–70.

Assmann T, Becker J, Ruzicka T, et al. Topical tacrolimus for oral cicatricial pemphigoid. Clin Exp Dermatol 2004; 29: 674–676.

Moghadam-Kia S, Werth VP. Prevention and treatment of systemic glucocorticoid side effects. Int J Dermatol 2010; 49: 239–248.

Arash A, Shirin L. The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid. J Oral Pathol Med 2008; 37:341–344.

Rogers RS 3rd, Mehregan DA. Dapsone therapy of cicatricial pemphigoid. Semin Dermatol 1988; 7:201–205.

Ciarrocca KN, Greenberg MS. A retrospective study of the management of oral mucous membrane pemphigoid ith dapsone. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999; 88: 159–163.

Kasperkiewicz M, Schmidt E. Current treatment of autoimmune blistering diseases. Curr Drug Discov Technol 2009; 6(4): 270-280.

Thorne JE, Woreta FA, Jabs DA, Anhalt GJ. Treatment of ocular mucous membrane pemphigoid with immunosuppressive drug therapy. Ophthalmology 2008; 115: 2146-2152.

Hayashi H, Sakai J, Matsuura H, et al. Cyclophosphamide and amniotic membrane transplantation in the management of ocular disease in a case of antiepiligrin cicatricial pemphigoid. Clin Exp Dermatol 2009; 34: e477–479.

Munyangango EM, Le Roux-Villet C, Doan S, et al. Oral cyclophosphamide without corticosteroids to treat mucous membrane pemphigoid. Br J Dermatol 2013; 168: 381-390.

Staines K, Hampton PJ. Treatment of mucous membrane pemphigoid with the combination of mycophenolate mofetil, dapsone, and prednisolone: a case series. Oral Surg Oral Med Oral Pathol Oral Radiol 2012; 114: e49–56.

Doycheva D, Deuter C, Blumenstock G, et al. Longterm results of therapy with mycophenolate mofetil in ocular mucous membrane pemphigoid. Ocul Immunol Inflamm 2011; 19: 431–438.

Rallis E, Anyfantakis V. Coexistent psoriasis and bullous pemphigoid responding to mycophenolate mofetil monotherapy. Skinmed 2008; 7: 101–102.

McCluskey P, Chang JH, Singh R, et al. Methotrexate therapy for ocular cicatricial pemphigoid. Ophthalmology; 111: 796–801.

Gangaputra S, Newcomb CW, Liesegang TL, et al. Methotrexate for ocular inflammatory diseases. Ophthalmology 2009; 116: 2188–2198.

Alonso A, Bignone ML, Brunzini M, et al. Ocular autoimmune pemphigoid and cyclosporin A. Allergol Immunopathol (Madr) 2006; 34: 113–115.

Kacmaz RO, Kempen JH, Newcomb C, et al. Cyclosporine for ocular inflammatory diseases. Ophthalmology 2010; 117: 576–584.

Williams DY, Oziemski M, Varigos G. Cyclosporine therapy of life-threatening cicatricial pemphigoid affecting the respiratory tract. Int J Dermatol 1995; 34: 639–641.

Foster CS, Neumann R, Tauber J. Long-term results of systemic chemotherapy for ocular cicatricial pemphigoid. Doc Ophthalmol 1992; 82: 223–229.

Poskitt L, Wojnarowska F. Minimizing cicatricial pemphigoid orodynia with minocycline. Br J Dermatol ; 132: 784–789.

Carrozzo M, Arduino P, Bertolusso G, Cozzani E, Parodi A. Systemic minocycline as a therapeutic option in predominantly oral mucous membrane pemphigoid: a cautionary report. Int J Oral Maxillofac

Surg 2009; 38: 1071-1076.

Reiche L, Wojnarowska F, Mallon E. Combination therapy with nicotinamide and tetracyclines for cicatricial pemphigoid: further support for its efficacy. Clin Exp Dermatol 1998; 23: 254–257.

Sakamoto K, Mori K, Hashimoto T, et al. Antiepiligrin cicatricial pemphigoid of the larynx successfully treated with a combination of tetracycline and niacinamide. Arch Otolaryngol Head Neck Surg 2002; 1420–1423.

O’Neill ID, Scully C. Biologics in oral medicine: principles of use and practical considerations. Oral Dis 2012; 18: 525–536.

O’Neill ID. Off-label use of biologicals in the management of inflammatory oral mucosal disease.J Oral Pathol Med 2008; 37: 575–581.

Canizares MJ, Smith DI, Conners MS, et al. Successful treatment of mucous membrane pemphigoid with etanercept in 3 patients. Arch Dermatol 2006; 142: 1457–1461.

Cusano F, Iannazzone SS, Riccio G, et al. Coexisting bullous pemphigoid and psoriasis successfully treated with etanercept. Eur J Dermatol 2010; 20: 520.

Kennedy JS, Devillez RL, Henning JS. Recalcitrant cicatricial pemphigoid treated with the anti-TNF-alpha agent etanercept. J Drugs Dermatol 2010; 9: 68–70.

Prey S, Robert PY, Drouet M, et al. Treatment of ocular cicatricial pemphigoid with the tumour necrosis factor alpha antagonist etanercept. Acta Derm Venereol 2007; 87: 74–75.

Sacher C, Rubbert A, Konig C, et al. Treatment of recalcitrant cicatricial pemphigoid with the tumor necrosis factor alpha antagonist etanercept. J Am Acad Dermatol 2002; 46: 113–115.

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.

Shetty S, Ahmed AR. Critical analysis of the use of rituximab in mucous membrane pemphigoid: a review of the literature. J Am Acad Dermatol 2013; 68: 499–506.

Le Roux-Villet C, Prost-Squarcioni C, Alexandre M, et al. Rituximab for patients with refractory mucous membrane pemphigoid. Arch Dermatol 2011; 147: 843–849.

Ross AH, Jaycock P, Cook SD, et al. The use of rituximab in refractory mucous membrane pemphigoid with severe ocular involvement. Br J Ophthalmol 2009; 93: 421–422.

Kasperkiewicz M, Shimanovich I, Ludwig RJ, et al. Rituximab for treatment-refractory pemphigus and pemphigoid: a case series of 17 patients. J Am Acad Dermatol 2011; 65: 552–558.

Mignogna MD, Leuci S, Piscopo R, et al. Intravenous immunoglobulins and mucous membrane pemphigoid. Ophthalmology 2008; 115: 752–752.e1.

Iaccheri B, Roque M, Fiore T, et al. Ocular cicatricial pemphigoid, keratomycosis, and intravenous immunoglobulin therapy. Cornea 2004; 23: 819–822.

Kharfi M, Khaled A, Anane R, Fazaa B, Kamoun MR. Early onset childhood cicatricial pemphigoid: a case report and review of the literature. Pediatr Dermatol 2010; 27: 119-124.

Chaidemenos G, Sidiropoulos T, Katsioula P, Koussidou-Eremondi T. Colchicine in the management of mucous membrane pemphigoid. Dermatol Ther 2011; 24: 443-445.

Suelves AM, Arcinue CA, Gonzalez-Martin JM, Kruh JN, Foster C. Analysis of a novel protocol of pulsed intravenous cyclophosphamide for recalcitrant or severe ocular inflammatory disease. Ophthalmology; 120: 1201-1209.

Sami N, Letko E, Androudi S, Daoud Y, Foster CS, Ahmed AR. Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: a long-term follow-up. Ophthalmology 2004; 111: 1380-1382.

Segura S, Iranzo P, Martinez-de Pablo I, et al. Highdose intravenous immunoglobulins for the treatment of

autoimmune mucocutaneous blistering diseases: evaluation of its use in 19 cases. J Am Acad Dermatol

; 56: 960-967.

Lourari S, Herve C, Doffoel-Hantz V, et al. Bullous and mucous membrane pemphigoid show a mixed response to rituximab: experience in seven patients. J Eur Acad Dermatol Venereol 2011; 25: 1238-1240.

Trimarchi M, Bellini C, Fabiano B, et al. Multiple mucosal involvement in cicatricial pemphigoid. Acta Otorhinolaryngol Ital 2009; 29: 222–225.

Higgins TS, Cohen JC, Sinacori JT. Laryngeal mucous membrane pemphigoid: a systematic review and pooled-data analysis. Laryngoscope 2010; 120: 529–536.

Cafaro A, Broccoletti R, Arduino PG. Low-level laser therapy for oral mucous membrane pemphigoid. Lasers Med Sci 2012; 27: 1247–1250.

Yilmaz HG, Kusakci-Seker B, Bayindir H, et al. Low-level laser therapy in the treatment of mucous membrane pemphigoid: a promising procedure. J Periodontol 2010; 81: 1226–1230.