Autoimmune Blistering Diseases of the Skin and Mucous Membranes

  • Timothy PattonEmail author
  • Neil J. Korman


Autoimmune blistering diseases of the skin and mucous membranes are characterized by the presence of tissue bound and circulating antibodies that are directed against specific proteins present in the skin. The different diseases that compose this category of conditions vary in their clinical presentation, prognosis, and response to therapy.


Pemphigus vulgaris Mucous membrane pemphigoid Paraneoplastic pemphigus Desquamative gingivitis Direct immunofluorescence 


  1. 1.
    Lin MS, Mascaró JM Jr, Liu Z, España A, Diaz LA. The desmosome and hemidesmosome in cutaneous autoimmunity. Clin Exp Immunol. 1997;107(Suppl 1):9–15.PubMedGoogle Scholar
  2. 2.
    Stanley J. “Pemphigus.” Fitzpatick’s Dermatology in General Medicine. Ed. Wolff K et al. 7th ed. New York: McGraw-Hill, 2008.Google Scholar
  3. 3.
    Mimouni D, Anhalt G. Pemphigus. Derm Ther. 2002;15(4):362–8.CrossRefGoogle Scholar
  4. 4.
    Anhalt GJ, Kim SC, Stanley JR, Korman NJ, Jabs DA, Kory M et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med. 1990;323:1729–35.PubMedCrossRefGoogle Scholar
  5. 5.
    Cummins DL, Mimouni D, Tzu J, Owens N, Anhalt GJ, Meyerle JH. Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies. J Am Acad Dermatol. 2007;56(1):153–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Lo Russo L, Fierro G, Guiglia R, Compilato D, Testa NF, Lo Muzio L, Campisi G. Epidemiology of desquamative gingivitis: evaluation of 125 patients and review of the literature. Int J Dermatol. 2009;48(10):1049–52.PubMedCrossRefGoogle Scholar
  7. 7.
    Woodley DT, Remington J, Chen M. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Clin Rev Allergy Immunol. 2007;33(1–2):78–84.PubMedCrossRefGoogle Scholar
  8. 8.
    Egan CA, Lazarova Z, Darling TN et al. Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet. 2001;357(9271):1850–1.PubMedCrossRefGoogle Scholar
  9. 9.
    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(3):370–9.PubMedCrossRefGoogle Scholar
  10. 10.
    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.PubMedCrossRefGoogle Scholar
  11. 11.
    Yancey K. “Cicatricial Pemphigoid.” Fitzpatick’s Dermatology in General Medicine. Ed. Wolff K et al. 7th ed. New York: McGraw-Hill, 2008.Google Scholar
  12. 12.
    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.PubMedGoogle Scholar
  13. 13.
    Ross AH, Jaycock P, Cook SD, Dick AD, Tole DM. The use of rituximab in refractory mucous membrane pemphigoid with severe ocular involvement. Br J Ophthalmol. 2009;93(4):421–2, 548.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Department of DermatologyUniversity of PittsburgPittsburgUSA

Personalised recommendations