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Part of the book series: Fortschritte der praktischen Dermatologie und Venerologie ((DERMATOLOGIE,volume 14))

Zusammenfassung

Vor mehr als 25 Jahren beschrieben Jordon und Beutner [2] zirkulierende Autoantikörper bei Pem phigus vulgaris und bullösem Pemphigoid, denen aufgrund ihrer In-vivo-Fixation an Epidermis bzw. Basalmembranzone und damit dem Ort der für die se Krankheiten typischen Läsionen eine pathogenetische Relevanz zuzukommen schien. Es wurde da mit eine Entwicklung in Gang gesetzt, die heute zu der Erkenntnis geführt hat, daß die meisten der be kannten erworbenen blasenbildenden Dermatosen antikörpermediierte, organspezifische Autoimmun krankheiten darstellen [32]. Der Verlust der Gewebskohärenz und die daraus resultierende Blasen bildung beruht bei diesen Krankheiten auf einem Verlust der Adhäsionskräfte entweder zwischen den Epidermalzellen oder zwischen Epidermalzellen und Basalmembran oder zwischen Basalmembran und diese in der Dermis verankernden Gewebskomponenten. Da die meisten der dafür verant wortlichen molekularen Strukturen bekannt sind, verstehen wir nun, warum z. B. die akantholytische Blasenbildung bei Pemphigus vulgaris suprabasal in der Epidermis und die Spaltbildung bei Epidermolysis bullosa acquisita im obersten Korium erfol gen. Wir können also heute zwischen klinischem Bild und der molekularen Zielstruktur des Auto immunprozesses einen Bogen spannen und haben damit ein wesentlich klareres Verständnis der Pa thogenese dieser Erkrankungen, als das noch vor wenigen Jahren der Fall war.

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Literatur

  1. Amagai M, Karpati S, Prussick R, Klaus-Kovtun V, Stanley JR (1992) Autoantibodies against the aminoterminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic. J Clin Invest 90:919–926.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  2. Anhalt GJ, Bahn CF, Labib RS et al (1981) Pathogenic effects of bullous pemphigoid autoantibodies on rab bit corneal epithelium. J Clin Invest 68:1097–1101.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  3. Anhalt GJ, Labib RS, Voorhees JJ, Beals TF, Diaz LA (1982) Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease. N Engl J Med 306:1189–1196.

    Article  CAS  PubMed  Google Scholar 

  4. Anhalt GJ, Kim SC, Stanley JR et al (1990) Paraneoplastic pemphigus. An autoimmune mucocutaneous dis ease associated with neoplasia. N Engl J Med 323:1729–1735.

    CAS  Google Scholar 

  5. Beutner EH, Jordon RE, Chorzelski TP (1968) The immunopathology of pemphigus and bullous pemphigoid. J Invest Dermatol 51:63–80.

    Article  CAS  PubMed  Google Scholar 

  6. Briggaman RA, Gammon WR (1991) Epidermolysis bullosa acquisita and other acquired blistering dis eases manifesting autoimmunity to type VII collagen. Curr Probl Dermatol 3:47–74.

    Article  Google Scholar 

  7. Buxton RS, Cowin P, Franke WW et al (1993) Nomen clature of the desmosomal cadherins. J Cell Biol 121:481–483.

    Article  CAS  PubMed  Google Scholar 

  8. Diaz LA, Marcelo CL (1978) Pemphigoid and pemphi gus antigens in cultured epidermal cells. Br J Derma tol 98:631–637.

    Article  CAS  Google Scholar 

  9. Diaz LA, Sampaio SAP, Rivitti EA et al (1989) Endemic pemphigus foliaceus — »Fogo Selvagem«: 1. Clinical features and immunopathology. J Am Acad Derma tol 20:657–669.

    Article  CAS  Google Scholar 

  10. Födinger D, Anhalt G, Boecskoer B et al (1995) Autoantibodies to desmoplakin I and II in patients with erythema multiforme. J Exp Med 181:169–175.

    Article  Google Scholar 

  11. Gammon WR, Briggaman RA (1993) Bullous SLE: A phenotypically distinctive but immunologically hete rogeneous bullous disorder. J Invest Dermatol 100:28S-34S.

    Article  Google Scholar 

  12. Gammon WR, Merritt CC, Lewis DM et al (1982) An in vitro model of murine complex-mediated basement membrane zone separation caused by pemphigoid an tibodies, leukocytes and complement. J Invest Derma tol 78:285–290.

    Article  CAS  Google Scholar 

  13. Garrod DR (1993) Desmosomes and hemidesmosomes. Curr Opin Cell Biol 5:30–40.

    Article  CAS  PubMed  Google Scholar 

  14. Hashimoto K, Shafran KM, Webber PS, Lazarus GS, Singer KH (1983) Anti-cell surface pemphigus autoantibody stimulates plasminogen activator activity of hu man epidermal cells. J Exp Med 157:259–272.

    Article  CAS  PubMed  Google Scholar 

  15. Holubar K, Wolff K, Konrad K, Beutner EH (1975) Ultrastructural localization of immunoglobulins in bul lous pemphigoid skin. Employment of a new peroxidase-antiperoxidase multistep method. J Invest Der matol 64:220–227.

    CAS  Google Scholar 

  16. Ishiko A, Shimizu H, Kikuchi A et al (1993) Human an tibodies against the 230-kD bullous pemphigoid anti gen (BPAG1) bind only to the intracellular domain of the hemidesmosome whereas those against the 180-kD bullous pemphigoid antigen (BPAG2) bind along the plasma membrane of the hemidesmosome in normal human and swine skin. J Clin Invest 91:1608–1615.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  17. Jordon RE, Bushkell LL (1979) The complement system in pemphigus, bullous pemphigoid and herpes gestationis. Int J Dermatol 18:271–281.

    Article  CAS  PubMed  Google Scholar 

  18. Karpati S, Amagai M, Prussick R, Cehrs K, Stanley JR (1993) Pemphigus vulgaris antigen, a desmoglein type of cadherin is localized within keratinocyte desmo somes. J Cell Biol 122:409–415.

    Article  CAS  PubMed  Google Scholar 

  19. Korman NJ, Eyre RW, Klaus-Kovtum V, Stanley JR (1989) Demonstration of an adhering-junction mole cule (plakoglobin) in the autoantigens of pemphigus foliaceus and pemphigus vulgaris. N Engl J Med 321:631–635.

    Article  CAS  PubMed  Google Scholar 

  20. Li K, Sawamura D, Giudice GJ et al (1991) Genomic or ganization of collagenous domains and chromosomal assignment of human 180-kDa bullous pemphigoid antigen-2, a novel collagen of stratified squamous epi thelium. J Biol Chem 266:24064–24069.

    CAS  PubMed  Google Scholar 

  21. Liu Z, Diaz LA, Troy JL et al (1993) A passive transfer model of the organ-specific autoimmune disease, bul lous pemphigoid, using antibodies generated against the hemidesmosomal antigen, PB180. J Clin Invest 92:2480–2488.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  22. Liu Z, Diaz LA, Giudice GJ (1994) Autoimmune re sponse against the bullous pemphigoid 180 autoantigen. Dermatology 189 (Suppl 1):34–37.

    Article  PubMed  Google Scholar 

  23. Morioka S, Lazarus GS, Jensen PJ (1987) Involvement of urokinase-type plasminogen activator in acantholysis induced by pemphigus IgG. J Invest Dermatol 89:474–477.

    Article  CAS  PubMed  Google Scholar 

  24. Oursler JR, Labib RS, Ariss-Abdo L et al (1992) Human autoantibodies against desmoplakins in paraneoplastic pemphigus. J Clin Invest 89:1775.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  25. Rappersberger K, Tschachler E, Tani M, Wolff K (1989) Bullous disease in SLE. J Am Acad Dermatol 21:745–752.

    Article  CAS  PubMed  Google Scholar 

  26. Rappersberger K, Roos N, Stanley JR (1992) Immunomorphological and biochemical identification of the pemphigus foliaceus autoantigen within desmosomes. J Invest Dermatol 99:323–330.

    Article  CAS  PubMed  Google Scholar 

  27. Roscoe JT, Diaz L, Sampaio SA et al (1985) Brazilian pemphigus foliaceus autoantibodies are pathogenic to BALB/c mice by passive transfer. J Invest Dermatol 85:538–541.

    Article  CAS  PubMed  Google Scholar 

  28. Sawamura D, Nomura K, Sugita Y et al (1990) Bullous pemphigoid antigen (BPAG): cDNA cloning and map ping of the gene to the short arm of human chromo some 6. Genomics 8:722–726.

    Article  CAS  PubMed  Google Scholar 

  29. Scharf SJ, Friedmann A, Brautbar C et al (1988) HLA class II allelic variation of susceptibility to pemphigus vulgaris. Proc Natl Acad Sci USA 85:3504–3508.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  30. Schiltz JR, Michel B (1976) Production of epidermal acantholysis in normal human skin in vitro by the IgG fraction from pemphigus serum. J Invest Dermatol 67:254–260.

    Article  CAS  PubMed  Google Scholar 

  31. Sinha AA, Brautbar C, Szafer F et al (1988) A newly characterized HLA DQ beta allele associated with pemphigus vulgaris. Science 239:1026–1029.

    Article  CAS  PubMed  Google Scholar 

  32. Stanley JR (1989) Pemphigus and pemphigoid as para digms of organ-specific, autoantibody-mediated dis eases. J Clin Invest 83:1443–1448.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  33. Stanley JR (1993) Cell adhesion molecules as targets for autoantibodies in pemphigus and pemphigoid, bullous diseases due to defective epidermal cell adhe sion. Adv Immunol 53:291–323.

    CAS  PubMed  Google Scholar 

  34. Wolff K, Schreiner E (1971) Ultrastructural localization of pemphigus auto-antibodies within the epidermis. Nature 229:59–60.

    Article  CAS  PubMed  Google Scholar 

  35. Woodley DT, Briggaman RA, O’Keefe EJ et al (1984) Identification of the skin basement membrane autoan tigen in epidermolysis bullosa acquisita. N Engl J Med 310:1007–1013.

    Article  CAS  PubMed  Google Scholar 

  36. Woodley DT, Burgeron RE, Lundstrum G (1988) The epidermolysis bullosa acquisita antigen is the globular carboxy terminus of type VII procollagen. J Clin In vest 81:683–687.

    Article  CAS  Google Scholar 

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© 1995 Springer-Verlag Berlin Heidelberg

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Wolff, K. (1995). Pathogenese bullöser Autoimmunkrankheiten. In: Plewig, G., Korting, H.C. (eds) Fortschritte der praktischen Dermatologie und Venerologie. Fortschritte der praktischen Dermatologie und Venerologie, vol 14. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-79156-7_12

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  • DOI: https://doi.org/10.1007/978-3-642-79156-7_12

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