Mucous Membrane Pemphigoid
Mucous membrane pemphigoid (MMP) is the subgroup of pemphigoid which affects mucous membranes. Several subtypes are classified based on clinical symptoms and target antigens, such as ocular mucous membrane pemphigoid (OMMP), localized vulvar pemphigoid (LVP), and anti-laminin 332 MMP (anti-LN-332 MMP). Autoantibodies are directed against various structural proteins in the epidermal basement membrane zone (EBMZ), with the 180-kD antigen (BP180) as the main target antigen. Other antigens, such as BP230, α6β4 integrin, and laminin 332, can also be targeted by autoantibodies. Clinically, MMP is characterized by erosions and blistering of the oral mucosa (85 %), conjunctiva (65 %), and, less frequently, the nose (20–40 %), esophagus (5–15 %), pharynx (20 %), larynx (5–10 %), and genitals (20 %). Clinical severity is highly variable in the different subtypes of MMP. Progressive scar formation is a severe complication in active disease in OMMP and anti-LN-332 MMP, resulting in blindness or upper airway obstruction when not treated accurately. Previously, the term cicatricial pemphigoid was used synonymously for MMP. However, at present, the term refers to the rare clinical phenotype with scarring skin lesions. Patient’s and doctor’s delay is frequently seen in MMP. For an accurate diagnosis, direct immunofluorescence microscopy (DIF) and detection of circulating autoantibodies in serum are mandatory. Management and prognosis of MMP depends on the severity and extent of the disease and involves a stepwise approach with first-choice treatment with oral corticosteroids (CS), often used in combination with adjuvant immunosuppressive drugs to reduce the adverse effects caused by long-term CS use.
KeywordsHemidesmosome Immunoglobulin Basement membrane zone Autoimmune disease Desquamative gingivitis Pemphigoid Mucous membrane
- 1.Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, Fine JD, Foster CS, Ghohestani R, Hashimoto T, Hoang-Xuan T, Kirtschig G, Korman NJ, Lightman S, Lozada-Nur F, Marinkovich MP, Mondino BJ, Prost-Squarcioni C, Rogers 3rd RS, Setterfield JF, West DP, Wojnarowska F, Woodley DT, Yancey KB, Zillikens D, Zone JJ. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138:370–9.PubMedGoogle Scholar
- 3.Egan CA, Lazarova Z, Darling TN, Yee C, Yancey KB. Anti-epiligrin cicatricial pemphigoid: clinical findings, immunopathogenesis, and significant associations. Medicine (Baltimore). 2003;82:177–86.Google Scholar
- 4.Domloge-Hultsch N, Gammon WR, Briggaman RA, Gil SG, Carter WG, Yancey KB. Epiligrin, the major human keratinocyte integrin ligand, is a target in both an acquired autoimmune and an inherited subepidermal blistering skin disease. J Clin Invest. 1992;90:1628–33.CrossRefPubMedPubMedCentralGoogle Scholar
- 5.Terra JB, Pas HH, Hertl M, Dikkers FG, Kamminga N, Jonkman MF. Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin-332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients. Br J Dermatol. 2011;165:815–22.CrossRefPubMedGoogle Scholar