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Morbus Behçet Klinische und immunpathologische Aspekte

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Neue Entwicklungen in der Dermatologie
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Zusammenfassung

Beim Morbus Behçet (MB) bzw. Behçet-Touraine handelt es sich um eine Systemvasculitis mit polytoper Organbeteiligung. Hinsichtlich der multifaktoriellen Manifestationsmechanismen der Erkrankung werden Klinik, Atiologie, Pathogenese, Epidemiologie und genetische Aspekte unter besonderer Berücksichtigung der immunpathologischen Vorgänge, der cutanen Hyperreaktivität und Therapie beschrieben. Anhand eigener Ergebnisse wird gezeigt, daß es sich beim MB nicht um eine Immundefekterkrankung, sondern um eine Krankheit mit gesteigerter Immunreaktivität handelt, wobei die Hyperchemotaxis der Granulocyten während akuter Krankheitsschübe dominiert. Zirkulierende Immunkomplexe und positive Lymphocytotoxicität gegen autologe orale Mucosazellen weisen auf die wichtige Rolle der immunpathologischen Vorgänge bei der Manifestation des MB hin. Die Untersuchung der Zellmorphologie der cutanen Pathergie mit Hilfe der Hautfenstermethode nach Rebuck zeigt semiquantitativ eine pathognomi- sche Zellkonstellation beim MB.

Summary

Behçet’s disease (BD) is a very serious systemic disorder exhibiting the clinical features of simultaneously or successively recurrent oral aphthosis, genital ulcerations and ophthalmitis.

The aetiology and pathogenesis of BD are still subject to controversial discussion. Former studies have indicated that immunological mechanisms play an essential role in the pathogenesis of BD. Vasculitic lesions, involving many organs, with hyperchemotaxis of polymorphonuclear leukocytes are characteristic of the disease.

Circulating immune complexes and lymphocytotoxicity for autologous oral epithelial cells are very significant findings in patients suffering from BD.

Through the use of the skin window technique cellular alterations in the so-called pathergy phenomenon can be continuously in vivo qualified as well as quantified in patients with BD.

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Djawari, D. (1987). Morbus Behçet Klinische und immunpathologische Aspekte. In: Hornstein, O.P., Hundeiker, M., Schönfeld, J. (eds) Neue Entwicklungen in der Dermatologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-72828-0_11

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