Skip to main content
SpringerLink
Log in

Mucous Membrane Pemphigoid

  • Chapter
  • First Online:
Autoimmune Bullous Diseases

  • 817 Accesses

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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 119.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 159.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

  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. 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.

    PubMed  Google Scholar 

  5. Mohan P, Srinivas CR, Leelakrishnan V. A rare initial presentation of esophageal involvement in pemphigus. Dis Esophagus. 2013;26(3):351.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  8. Egan CA, Zone JJ. Linear IgA bullous dermatosis. Int J Dermatol. 1999;38(11):818–27.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  21. Günther C, Wozel G, Meurer M, Pfeiffer C. Topical tacrolimus treatment for cicatricial pemphigoid. J Am Acad Dermatol. 2004;50(2):325–6.

    Article  PubMed  Google Scholar 

  22. Assmann T, Becker J, Ruzicka T, Megahed M. Topical tacrolimus for oral cicatricial pemphigoid. Clin Exp Dermatol. 2004;29(6):674–6.

    Article  PubMed  CAS  Google Scholar 

  23. Suresh L, Martinez Calixto LE, Radfar L. Successful treatment of mucous membrane pemphigoid with tacrolimus. Spec Care Dentist. 2006;26(2):66–70.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  26. Marzano AV, Dassoni F, Caputo R. Treatment of refractory blistering autoimmune diseases with mycophenolic acid. J Dermatolog Treat. 2006;17(6):370–6.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  30. Cafaro A, Broccoletti R, Arduino PG. Low-level laser therapy for oral mucous membrane pemphigoid. Lasers Med Sci. 2012;27(6):124.

    Article  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  39. Gürcan HM, Ahmed AR. Intravenous immunoglobulin treatment in laryngeal pemphigoid. Clin Exp Dermatol. 2009;34(8):884–6.

    Article  PubMed  Google Scholar 

  40. Yu JT, Chong LY, Lee KC. A recalcitrant case of cicatricial pemphigoid. Hong Kong Med J. 2007;13(2):157–60.

    PubMed  Google Scholar 

  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. 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.

    Article  PubMed  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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 

Download references

Author information

Authors and Affiliations

  1. Department of Dermatology, University of Illinois College of Medicine, 808 S. Wood St., R380, MC624, Chicago, IL, 60612, USA

    Lawrence S. Chan MD

  2. Department of Dermatology, University of Alabama-Birmingham, Birmingham, AL, USA

    Young Kwak MD, CSM & Naveed Sami MD, FAAD

Authors
  1. Lawrence S. Chan MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Young Kwak MD, CSM
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Naveed Sami MD, FAAD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lawrence S. Chan MD .

Editor information

Editors and Affiliations

  1. Department of Dermatology, University of Alabama at Birmingham, BIRMINGHAM, Alabama, USA

    Naveed Sami

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Chan, L.S., Kwak, Y., Sami, N. (2016). Mucous Membrane Pemphigoid. In: Sami, N. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26728-9_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-26728-9_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-26726-5

  • Online ISBN: 978-3-319-26728-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation