Skip to main content
Log in

Schleimhautbeteiligung bei blasenbildenden Erkrankungen

Orogenital and conjunctival involvement in hereditary and autoimmune blistering diseases

  • Leitthema
  • Published:
Der Hautarzt Aims and scope Submit manuscript

Zusammenfassung

Die Beteiligung von oraler und genitaler Schleimhaut bzw. der Konjunktiven im Rahmen seltener („rare“ = “orphan diseases“), genetisch (Epidermolysis bullosa hereditaria) oder autoimmunologisch (z. B. Pemphigus vulgaris, Schleimhautpemphigoid, Epidermolysis bullosa acquisita) bedingter bullöser Dermatosen ist bei chronischem Verlauf durch eine signifikante Morbidität gekennzeichnet. Deshalb verdienen die Schleimhautläsionen, insbesondere bei anfänglich regionaler Beschränktheit und wegen ihrer Therapieresistenz besondere Beachtung. Dazu werden in dieser Übersichtsarbeit klinische, pathogenetische, diagnostische und jüngst forcierte therapeutische Aspekte diese Erkrankungsgruppen betreffend diskutiert.

Abstract

Chronic involvement of orogenital and conjunctival mucosa in the course of either genetically based (epidermolysis bullosa hereditaria) or auto-immunologically mediated (as for example pemphigus vulgaris, mucous membrane pemphigoid or epidermolysis bullosa acquisita) blistering diseases can cause significant morbidity. To provide accurate care, recognition of clinical, pathogenic and diagnostic features as well as awareness of recent advances in the development of new therapeutic modalities are mandatory and thus will be discussed in this review.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6
Abb. 7

Literatur

  1. Bork K (2005) Erkrankungen der Lippen und der Mundhöhle. In: Braun-Falco O, Plewig G, Wolff HH (Hrsg) Dermatologie und Venerologie. Springer, Heidelberg, S 975–976

  2. Fine JD, Hintner H (eds) (2008) Life with epidermolysis bullosa (EB): etiology, diagnosis, multidisciplinary care and therapy. Springer, Wien New York

  3. Mavilio F, Pellegrini G, Ferrari S et al (2006) Correction of junctional epidermolysis bullosa by transplantation of genetically modified epidermal stem cells. Nat Med 12:1397–1402

    Article  CAS  PubMed  Google Scholar 

  4. Ortiz-Urda S, Lin Q, Green CL et al (2003) Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue. J Clin Invest 111:251–255

    CAS  PubMed  Google Scholar 

  5. Woodley DT, Krueger GG, Jorgensen CM et al (2003) Normal and gene-corrected dystrophic epidermolysis bullosa fibroblasts alone can produce type VII collagen at the basement membrane zone. J Invest Dermatol 121:1021–1028

    Article  CAS  PubMed  Google Scholar 

  6. Woodley DT, Remington J, Huang Y et al (2007) Intravenously injected human fibroblasts home to skin wounds, deliver type VII collagen, and promote wound healing. Mol Ther 15:628–635

    Article  CAS  PubMed  Google Scholar 

  7. Woodley DT, Keen DR, Atha T (2004) Intradermal injection of lentiviral vectors corrects regenerated human dystrophic epidermolysis bullosa skin tissue in vivo. Mol Ther 10:318–326

    Article  CAS  PubMed  Google Scholar 

  8. Woodley DT, Keen DR, Atha T (2004) Injection of recombinant human type VII collagen restores collagen function in dystrophic epidermolysis bullosa. Nat Med 10:693–695

    Article  CAS  PubMed  Google Scholar 

  9. Wong T, Gammon L, Liu L et al (2008) Potential of fibroblast cell therapy for recessive dystrophic epidermolysis bullosa. J Invest Dermatol 128:2179–2189

    Article  CAS  PubMed  Google Scholar 

  10. Tamai K, Kaneda Y, Uitto J (2009) Molecular therapies for heritable blistering diseases. Trends Mol Med 15:285–292

    Article  CAS  PubMed  Google Scholar 

  11. Amagai M (2008) Pemphigus. In: Bolognia JL, Jorizzo JL (eds) Dermatology e-dition, 2nd edn. Elsevier, Mosby

  12. Yancey KB, Allen DM (2008) The biology of the basement membrane zone. In: Bolognia JL, Jorizzo JL (eds) Dermatology e-dition, 2nd edn. Elsevier, Mosby

  13. Stanley JR, Amagai M (2006) Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med 355:1800–1810

    Article  CAS  PubMed  Google Scholar 

  14. Amagai M, Ahmed AR, Kitajima Y et al (2006) Are desmoglein autoantibodies essential for the immunopathogenesis of pemphigus vulgaris, or just „witnesses of disease“? Exp Dermatol 15:815–831

    Article  CAS  PubMed  Google Scholar 

  15. Borradori L, Bernhard P (2008) Pemphigoid Group. In: Bolognia JL, Jorizzo JL (eds) Dermatology e-dition, 2nd edn. Elsevier, Mosby

  16. Bedane C, McMillan J, Balding S et al (1997) Bullous pemphigoid and cicatrical pemphigoid autoantibodies react with ultrastructurally separable epitopes on the BP180 ectodomain: evidence that BP180 spans the lamina lucida. J Invest Dermatol 108:901–907

    Article  CAS  PubMed  Google Scholar 

  17. Egan CA, Lazarova Z, Darling TN et al (2001) Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet 357:1850–1851

    Article  CAS  PubMed  Google Scholar 

  18. Pohla-Gubo G, Bauer JW, Hintner H (1997) Immunhistochemische Untersuchungen bei angeborenen und erworbenen bullösen Dermatosen. In: Korting HC, Sterry W (Hrsg) Diagnostische Verfahren in der Dermatologie. Blackwell Wissenschafts-Verlag, Berlin Wien, S 171–176

  19. Bauer JW, Scheappi H, Metze D et al (1999) Ocular involvement in IgA-epidermolysis bullosa axquisita. Br J Dermatol 141:887–892

    Article  CAS  PubMed  Google Scholar 

  20. Borradori L, Bernhard P (2008) Dermatitis herpetiformis and Linear IgA bullous dermatosis. In: Bolognia JL, Jorizzo JL (eds) Dermatology e-dition, 2nd edn. Elsevier, Mosby

  21. Schmidt E, Bröcker EB, Goebeler M (2008) Rituximab in treatment-resistant autoimmune blistering skin disorders. Clin Rev Allergy Immunol 34:56–64

    Article  CAS  PubMed  Google Scholar 

  22. Hertl M, Zillikens D, Borradori L et al (2008) Recommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseases. JDDG 6:366–373

    Article  PubMed  Google Scholar 

  23. Ahmed AR, Spigelman Z, Cavacini LA, Posner MR (2006) Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 355:1772–1779

    Article  CAS  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to M. Laimer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Laimer, M., Lanschützer, C., Emberger, M. et al. Schleimhautbeteiligung bei blasenbildenden Erkrankungen. Hautarzt 60, 881–890 (2009). https://doi.org/10.1007/s00105-009-1804-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00105-009-1804-3

Schlüsselwörter

Keywords

Navigation