Advertisement

Mucous Membrane Pemphigoid

  • Lawrence S. ChanEmail author
  • Young Kwak
  • Naveed Sami
Chapter

Abstract

Mucous membrane pemphigoid (MMP) is a term categorizing a group of autoimmune sub-epithelial blistering diseases affecting primarily mucous membrane areas. These diseases mainly affect one or more mucous membranes, and can also affect the skin in a minor fashion. The common features that define this group of diseases are: (1). Mucous membranes are the predominantly affected areas; (2). Pathology of the lesional epithelium demonstrates a sub-epithelial blister; (3). Immunopathology of the peri-lesional epithelium shows linear deposition of immunoglobulin and/or complement component (C3) at the epithelial basement membrane. The therapeutic strategy should be based on both the progressiveness of the disease and the specific mucous membranes affected. Systemic treatments currently available for physicians to employ include dapsone, corticosteroids, immunosuppressives, intravenous immunoglobulin, and, most recently, biologics such as rituximab, an anti-CD20 monoclonal antibody to mature B cells.

Keywords

Mucous membrane Antibody Antigen Basement membrane Immunosuppressives Corticosteroid Dapsone Azathioprine Mycophenolate mofetil Rituximab 

References

  1. 1.
    Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. The first international consensus on mucous membrane pemphigoid. Arch Dermatol. 2002;138(3):370–9.CrossRefPubMedGoogle Scholar
  2. 2.
    Arafat SN, Suelves AM, Spurr-Michaud S, Chodosh J, Foster CS, Dohlman CH, et al. Neutrophil collagenase, gelatinase, and myeloperoxidase in tears of patients with Stevens-Johnson syndrome and ocular cicatricial pemphigoid. Ophthalmology. 2014;121(1):79–87.PubMedCentralCrossRefPubMedGoogle Scholar
  3. 3.
    Lavallee A, Bourret-Massicotte D, Laughrea PA. Childhood mucous membrane pemphigoid: a case with exclusively ocular involvement. Cornea. 2013;32(10):1399–401.Google Scholar
  4. 4.
    Shamim T, Varghese VI, Shameena PM, Sudha S. Pemphigus vulgaris in oral cavity: clinical analysis of 71 cases. Med Oral Patol Oral Cir Bucal. 2008;13(10):E622–4.PubMedGoogle Scholar
  5. 5.
    Mohan P, Srinivas CR, Leelakrishnan V. A rare initial presentation of esophageal involvement in pemphigus. Dis Esophagus. 2013;26(3):351.CrossRefPubMedGoogle Scholar
  6. 6.
    Akhyani M, Chams-Davatchi C, Naraghi Z, Daneshpazhooh M, Toosi S, Asgari M, et al. Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases. Br J Dermatol. 2008;158(3):478–82.CrossRefPubMedGoogle Scholar
  7. 7.
    Anhalt GJ, Kim SC, Stanley JR, Korman NJ, Jabs DA, Kory M, et al. Paraneoplastic pemphigus: an autoimmune mucocutaneous disease associated with neoplasis. N Engl J Med. 1990;323(25):1729–35.CrossRefPubMedGoogle Scholar
  8. 8.
    Egan CA, Zone JJ. Linear IgA bullous dermatosis. Int J Dermatol. 1999;38(11):818–27.CrossRefPubMedGoogle Scholar
  9. 9.
    Woodley DT, Briggaman RA, O’Keefe EJ, Inman AO, Queen LL, Gammoan WR. Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita. N Engl J Med. 1984;310(16):1007–13.CrossRefPubMedGoogle Scholar
  10. 10.
    Diaz LA, Ratrie 3rd H, Saunders WS, Futamara S, Squiquera HL, Anhalt GJ, et al. Isolation of a human epidermal cDNA corresponding to the 180-kD autoantigen recognized by bullous pemphigoid and herpes gestationis sera. Immunolocalization of this protein to the hemidesmosome. J Clin Invest. 1990;86(4):1088–94.PubMedCentralCrossRefPubMedGoogle Scholar
  11. 11.
    Chan LS, Fine JD, Briggaman RA, Woodley DT, Hammerberg C, Drugge RJ, et al. Identification and partial characterization of a novel 105-kDalton lower lamina lucida autoantigen associated with a novel immune-mediated subepidermal blistering disease. J Invest Dermatol. 1993;101(3):262–7.CrossRefPubMedGoogle Scholar
  12. 12.
    Shimanovich I, Hirako Y, Sitaru C, Hashimoto T, Brocker EB, Butt E, et al. The autoantigen of anti-p200 pemphigoid is an acidic noncollagenous N-linked glycoprotein of the cutaneous basement membrane. J Invest Dermatol. 2003;121(6):1402–8.CrossRefPubMedGoogle Scholar
  13. 13.
    Zillikens D, Mascaro JM, Rose PA, Liu Z, Ewing SM, Caux F, et al. A highly sensitive enzyme-linked immunosorbent assay for the detection of circulating anti-BP180 autoantibodies in patients with bullous pemphigoid. J Invest Dermatol. 1997;109(5):679–83.CrossRefPubMedGoogle Scholar
  14. 14.
    Chen M, Chan LS, Cai X, O’Toole EA, Sample JC, Woodley DT. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol. 1997;108(1):68–72.CrossRefPubMedGoogle Scholar
  15. 15.
    Zone JJ, Taylor TB, Kadunce DP, Meyer LJ. Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis. J Clin Invest. 1990;85(3):812–20.PubMedCentralCrossRefPubMedGoogle Scholar
  16. 16.
    Chan LS, Majmurdar AA, Tran HH, Meier F, Schumburg-Lever G, Chen M, et al. Laminin-6 and laminin-5 are recognized by autoantibodies in a subset of cicatricial pemphigoid. J Invest Dermatol. 1997;108(6):843–53.CrossRefGoogle Scholar
  17. 17.
    Bernard P, Antonicelli F, Bedane C, Joly P, Le Roux-Villet C, Duvert-Lehembre S, et al. Prevalence and clinical significance of anti-laminin 332 autoantibodies detected by a novel enzyme-linked immunosorbent assay in mucous membrane pemphigoid. JAMA Dermatol. 2013;149(5):533–40.CrossRefPubMedGoogle Scholar
  18. 18.
    Rashid KA, Foster CS, Ahmed AR. Identification of epitopes within integrin b4 for binding of auto-antibodies in ocular cicatricial and mucous membrane pemphigoid: preliminary report. Invest Ophthalmol Vis Sci. 2013;54(12):7707–16.CrossRefPubMedGoogle Scholar
  19. 19.
    Hayakawa T, Furumura M, Fukano H, Li X, Ishii N, Hamada T, et al. Diagnosis of oral mucous membrane pemphigoid by means of combined serologic testing. Oral Surg Oral Med Oral Pathol Oral Radiol. 2014;117(4):483–96.CrossRefPubMedGoogle Scholar
  20. 20.
    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;120(2):161–71.CrossRefPubMedGoogle Scholar
  21. 21.
    Günther C, Wozel G, Meurer M, Pfeiffer C. Topical tacrolimus treatment for cicatricial pemphigoid. J Am Acad Dermatol. 2004;50(2):325–6.CrossRefPubMedGoogle Scholar
  22. 22.
    Assmann T, Becker J, Ruzicka T, Megahed M. Topical tacrolimus for oral cicatricial pemphigoid. Clin Exp Dermatol. 2004;29(6):674–6.CrossRefPubMedGoogle Scholar
  23. 23.
    Suresh L, Martinez Calixto LE, Radfar L. Successful treatment of mucous membrane pemphigoid with tacrolimus. Spec Care Dentist. 2006;26(2):66–70.CrossRefPubMedGoogle Scholar
  24. 24.
    Lee HY, Blazek C, Beltraminelli H, Borradori L. Oral mucous membrane pemphigoid: complete response to topical tacrolimus. Acta Derm Venereol. 2011;91(5):604–5.CrossRefPubMedGoogle Scholar
  25. 25.
    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(10):1071–6.CrossRefPubMedGoogle Scholar
  26. 26.
    Marzano AV, Dassoni F, Caputo R. Treatment of refractory blistering autoimmune diseases with mycophenolic acid. J Dermatolog Treat. 2006;17(6):370–6.CrossRefPubMedGoogle Scholar
  27. 27.
    Munyangango EM, Le Roux-Villet C, Doan S, et al. Oral cyclophosphamide without corticosteroids to treat mucous membrane pemphigoid. Br J Dermatol. 2013;168(2):381–90.CrossRefPubMedGoogle Scholar
  28. 28.
    Chaidemenos G, Sidiropoulos T, Katsioula P, Koussidou-Eremondi T. Colchicine in the management of mucous membrane pemphigoid. Dermatol Ther. 2011;24(4):443–5.CrossRefPubMedGoogle Scholar
  29. 29.
    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(1):e49–56.CrossRefPubMedGoogle Scholar
  30. 30.
    Cafaro A, Broccoletti R, Arduino PG. Low-level laser therapy for oral mucous membrane pemphigoid. Lasers Med Sci. 2012;27(6):124.CrossRefGoogle Scholar
  31. 31.
    Canizares MJ, Smith DI, Conners MS, Maverick KJ, Heffernan MP. Successful treatment of mucous membrane pemphigoid with etanercept in 3 patients. Arch Dermatol. 2006;142(11):1457–61.CrossRefPubMedGoogle Scholar
  32. 32.
    Kennedy JS, Devillez RL, Henning JS. Recalcitrant cicatricial pemphigoid treated with the anti-TNF-alpha agent etanercept. J Drugs Dermatol. 2010;9(1):68–70.PubMedGoogle Scholar
  33. 33.
    Taverna JA, Lerner A, Bhawan J, Demierre M-F. Successful adjuvant treatment of recalcitrant mucous membrane pemphigoid with anti-CD20 antibody rituximab. J Drugs Dermatol. 2007;6(7):731–2.PubMedGoogle Scholar
  34. 34.
    Schumann T, Schmidt E, Booken N, Goerdt S, Goebeler M. Successful treatment of mucous membrane pemphigoid with the anti-CD-20 antibody rituximab. Acta Derm Venereol. 2009;89(1):101–2.CrossRefPubMedGoogle Scholar
  35. 35.
    Le Roux-Villet C, Prost-Squarcioni C, Alexandre M, et al. Rituximab for patients with refractory mucous membrane pemphigoid. Arch Dermatol. 2011;147(7):843–9.CrossRefPubMedGoogle Scholar
  36. 36.
    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(10):1238–40.CrossRefPubMedGoogle Scholar
  37. 37.
    Wozniak K, Waszczykowska E, Hashimoto T, et al. Anti-epiligrin cicatricial pemphigoid initially limited to the upper respiratory tract. Br J Dermatol. 2006;154(4):779–81.CrossRefPubMedGoogle Scholar
  38. 38.
    Segura S, Iranzo P, Martínez-de Pablo I, et al. High-dose intravenous immunoglobulins for the treatment of autoimmune mucocutaneous blistering diseases: evaluation of its use in 19 cases. J Am Acad Dermatol. 2007;56(6):960–7.CrossRefPubMedGoogle Scholar
  39. 39.
    Gürcan HM, Ahmed AR. Intravenous immunoglobulin treatment in laryngeal pemphigoid. Clin Exp Dermatol. 2009;34(8):884–6.CrossRefPubMedGoogle Scholar
  40. 40.
    Yu JT, Chong LY, Lee KC. A recalcitrant case of cicatricial pemphigoid. Hong Kong Med J. 2007;13(2):157–60.PubMedGoogle Scholar
  41. 41.
    Nishmura R, Fujimoto N, Kito K, Uchiyama K, Koga H, Hodohara K, et al. Refractory mucous membrane pemphigoid which developed after allogeneic stem cell transplantation and was successfully treated with rituximab. Eur J Dermatol. 2013;23(4):562–4.Google Scholar
  42. 42.
    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(3):499–506.CrossRefPubMedGoogle Scholar
  43. 43.
    Czemik A, Toosi S, Bystryn JC, Grando SA. Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: an update. Autoimmunity. 2012;45(1):111–8.CrossRefGoogle Scholar
  44. 44.
    Suelves AM, Arcinue CA, Gonzalez-Martin JM, Kruh JN, Foster CS. Analysis of a novel protocol of pulsed intravenous cyclophosphamide for recalcitrant or severe ocular inflammatory disease. Ophthalmology. 2013;120(6):1201–9.CrossRefPubMedGoogle Scholar
  45. 45.
    Nottage JM, Hammersmith KM, Murchison AP, Felipe AF, Penne R, Raber I. Treatment of mucous membrane pemphigoid with mycophenolate mofetil. Cornea. 2013;32(6):810–5.Google Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyUniversity of Illinois College of MedicineChicagoUSA
  2. 2.Department of DermatologyUniversity of Alabama-BirminghamBirminghamUSA

Personalised recommendations