• Life-threatening autoimmune blistering disorders include bullous pemphigoid and the different subtypes of pemphigus: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus.

  • Major extent of skin and mucosal lesions results in the failure of main skin functions.

  • Severe infections, including septicaemia and pneumonia and cardiovascular disorders, are the major cause of death in these patients.

  • The aim of treatment is to stop the production of autoantibodies. Corticosteroids and immuno-suppressants are widely used in the treatment of autoimmune blistering disorders.

  • New biologic agents such as rituximab are currently proposed for the treatment of the most severe, life-threatening types of these disorders.


Bullous Pemphigoid Clobetasol Propionate Pemphigus Foliaceus Epidermolysis Bullosa Acquisita Cicatricial Pemphigoid 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Giudice GJ, Emery DJ, Diaz LA (1992) Cloning and primary structural analysis of the bullous pemphigoid autoantigen BP180. J Invest Dermatol 99:243–250PubMedCrossRefGoogle Scholar
  2. 2.
    Stanley JR (1989) Pemphigus and pemphigoid as paradigms of organ-specific, autoantibody-mediated diseases. J Clin Invest 83:1443–1448PubMedCrossRefGoogle Scholar
  3. 3.
    Stanley JR (1993) Cell adhesion molecules as targets of autoantibodies in pemphigus and pemphigoid, bullous diseases due to defective epidermal cell adhesion. Adv Immunol 53:291–325PubMedCrossRefGoogle Scholar
  4. 4.
    Diaz LA, Ratrie H, Saunders WS et al (1990) Isolation of a human 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 86:1088–1094PubMedCrossRefGoogle Scholar
  5. 5.
    Ahmed AR, Spigelman Z, Caracini LA, Posner MR (2006) Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 355:1772–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Joly P, Mouquet H, Roujeau JC et al (2007) A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med 357:545–552PubMedCrossRefGoogle Scholar
  7. 7.
    Bernard P, Vaillant L, Labeille B et al (1995) Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol 131:48–52PubMedCrossRefGoogle Scholar
  8. 8.
    Gudi VS, White N, Gruickshank et al (2005) Annual incidence and mortality of bullous pemphigoid in the Crampian region of North-East Scotland. Br J Dermatol 153:424–427PubMedCrossRefGoogle Scholar
  9. 9.
    Zillikens D, Wever S, Roth A, Hashimoto T, Brocker EB (1995) Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol 131:957–958PubMedCrossRefGoogle Scholar
  10. 10.
    Joly P, Roujeau JC, Benichou J et al (2002) A comparison of oral and topical cortisosteroids in patients with bullous pemphigoid. N Engl J Med 346:321–327PubMedCrossRefGoogle Scholar
  11. 11.
    Guillaume JC, Vaillant L, Bernard P et al (1993) Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 129:49–53PubMedCrossRefGoogle Scholar
  12. 12.
    Stanley JR, Tanaka T, Mueller S, Klaus-Kovtun V, Roop D (1988) Isolation of complementary DNA for bullous pemphigoid antigen by use of patients' autoantibodies. J Clin Invest 82:1864–1870PubMedCrossRefGoogle Scholar
  13. 13.
    Vaillant L, Bernard P, Joly P et al (1998) Evaluation of clinical criteria for diagnosis of bullous pemphigoid. Arch Dermatol 134:1075–1080PubMedCrossRefGoogle Scholar
  14. 14.
    Joly P, Courville P, Lok C et al (2004) Clinical criteria for the diagnosis of bullous pemphigoid: a reevaluation according to immunoblot analysis of patient sera. Dermatology 208:16–20PubMedCrossRefGoogle Scholar
  15. 15.
    Courville P, Kupfer I, Gilbert D, Thomine E, Metayer J, Joly P (2000) Evaluation of histological criteria for bullous pemphigoid. Correlation with antigens recognized by immunoblotting of antiepidermal autoantibodies. Ann Pathol 20:564–569PubMedGoogle Scholar
  16. 16.
    Gammon WR, Kowalewski C, Chorzelski TP, Kumar V, Briggaman RA, Beutner EH (1990) Direct immunofluorescence studies of sodium chlorideseparated skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. J Am Acad Dermatol 22:664–670PubMedCrossRefGoogle Scholar
  17. 17.
    Joly P, Gilbert D, Thomine E et al (1997) Relationship between the in vivo localization and the immunoblotting pattern of antibasement membrane zone antibodies in patients with bullous pemphigoid. Arch Dermatol 133:719–724PubMedCrossRefGoogle Scholar
  18. 18.
    Bernard P, Bedane C, Bonnetblanc JM (1997) Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients. Br J Dermatol 136:694–698PubMedCrossRefGoogle Scholar
  19. 19.
    Roujeau JC, Lok C, Bastuji-Garin S, Mhalla S, Enginger V, Bernard P (1998) High risk of death in elderly patients with extensive bullous pemphigoid. Arch Dermatol 134:465–469PubMedCrossRefGoogle Scholar
  20. 20.
    Cordel N, Chosidow O, Hellot MF et al (2007) Neurological disorders in patients with bullous pemphoid. Dermatology 215:187–191PubMedCrossRefGoogle Scholar
  21. 21.
    Rzany B, Partscht K, Jung M et al (2002) Risk factors for lethal outcome in patients with bullous pemphigoid. Arch Dermatol 138:903–908PubMedCrossRefGoogle Scholar
  22. 22.
    Fine JD (1995) Management of acquired bullous skin diseases. N Engl J Med 333:1475–1484PubMedCrossRefGoogle Scholar
  23. 23.
    Joly P, Roujeau JC, Benichou J et al (2002) A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. New Engl J Med 346:326–327CrossRefGoogle Scholar
  24. 24.
    Lebrun-Vignes B, Roujeau JC, Bernard P et al (1999) Prednisone is more effective than prednisolone metasulfobenzoate in the treatment of bullous pemphigoid. Arch Dermatol 135:89–90PubMedCrossRefGoogle Scholar
  25. 25.
    Westerhof W (1989) Treatment of bullous pemphigoid with topical clobetasol propionate. J Am Acad Dermatol 20:458–461PubMedCrossRefGoogle Scholar
  26. 26.
    Grundmann-Kollmann M, Korting HC, Behrens S et al (1999) Mycophenolate mofetil: a new therapeutic option in the treatment of blistering autoimmune diseases. J Am Acad Dermatol 40:957–960PubMedCrossRefGoogle Scholar
  27. 27.
    Dereure O, Bessis D, Guillot B, Guillhou JJ (2002) Treatment of bullous pemphigoid by low-dose methotrexate associated with short-term potent topical steroids: an open prospective study of 18 cases. Arch Dermatol 138:1255–6.PubMedCrossRefGoogle Scholar
  28. 28.
    Anhalt GJ, Kim SC, Stanley JR et al (1990) Paraneoplastic pemhigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 323:1729–1735PubMedGoogle Scholar
  29. 29.
    Bastuji-Garin S, Souissi R, Blum L et al (1995) Comparative epidemiology of pemphigus in Tunisia in France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol 104:302–305PubMedCrossRefGoogle Scholar
  30. 30.
    Amagai M, Klaus-Kovtun V, Stanley JR (1991) Autoanti-bodies against a novel epithelial cadherin in pemphigus vul-garis, a disease of cell adhesion. Cell 67:869–877PubMedCrossRefGoogle Scholar
  31. 31.
    Amagai M, Tsunoda K,Suzuki H et al (2000) Use of autoantigen-knockout mice in developing an active autoimmune disease model for pemphigus. J Clin Invest 105:625–631PubMedCrossRefGoogle Scholar
  32. 32.
    Joly P, Richard C, Gilbert D et al (2000) Sensitivy and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemhigus. J Am Acad Dermatol 43:619–615PubMedCrossRefGoogle Scholar
  33. 33.
    Beutner EH, Lever WF, Witebsky E et al (1965) Autoantibodies in pemphigus vulgaris. Response to an intercellular substance of epidermis. JAMA 92:682–688Google Scholar
  34. 34.
    Ishii K, Amagai M, Hall RP et al (1997) Characterization of autoantibodies in pemphigus using antigenspecific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins. J Immunol 159:2010–2017PubMedGoogle Scholar
  35. 35.
    Amagai M, Tsunoda K, Zillikens D et al (1999) The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol 40:167–170PubMedCrossRefGoogle Scholar
  36. 36.
    Lever WF, Schaumburg-Lever G (1984) Treatment of pemphigus vulgaris: results obtained in 84 patients between 1961 and 1982. Arch Dermatol 120:44–47PubMedCrossRefGoogle Scholar
  37. 37.
    Bystryn JC, Steinman NM (1996) The adjuvant therapy of pemphigus: an update. Arch Dermatol 132:203–212PubMedCrossRefGoogle Scholar
  38. 38.
    El Tal AK, Posner MR, Spigelman Z, Ahmed AR (2006) Rituximab: a monoclonal antibody to CD20 used in the treatment of pemphigus vulgaris. J Am Acad Dermatol. 55:449–459PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2009

Authors and Affiliations

  • Pascal Joly
    • 1
  1. 1.Clinique DermatologiqueHôpital Charles NicolleRouen cedexFrance

Personalised recommendations