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Diagnostik und Ausschluss des hereditären Angioödems

Ein standardisierter Ansatz für die Praxis

Diagnostics and exclusion of hereditary angioedema

A standarized approach for the practice

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An Erratum to this article was published on 07 October 2012

Zusammenfassung

Beim Auftreten von Angioödemen erweist sich die Unterscheidung zwischen Mastzellmediator-vermittelten und Bradykinin-vermittelten Angioödemen oft als schwierig. Das Bradykinin-induzierte hereditäre Angioödem ist eine seltene, autosomal-dominant vererbte Erkrankung, die durch rezidivierende Ödemattacken unterschiedlicher Ausprägung gekennzeichnet ist. Die Ödeme treten an Haut und Schleimhäuten auf und können passager entstellend, sehr schmerzhaft und bei Attacken im Kehlkopfbereich lebensbedrohend sein. Aufgrund der zahlreichen möglichen Differenzialdiagnosen dauert die Diagnosestellung im Durchschnitt  > 10 Jahre. Die hier vorgestellten anamnestischen und labordiagnostischen Algorithmen sollen helfen, die verschiedenen Angioödeme einfacher differenzieren und die richtige Diagnose schneller stellen zu können.

Abstract

The differentiation between mast cell mediator-mediated and bradykinin-mediated forms of angioedema can be difficult. Bradykinin-mediated hereditary angioedema is a rare autosomal dominant hereditary disease which is characterized by recurrent edema attacks of varying magnitude. The edema occurs in the skin and mucous membranes and can be temporarily disfiguring, very painful and life-threatening by attacks in the laryngeal region. Because of the multitude of differential diagnoses, a final diagnosis is only achieved after an average duration of more than 10 years. The anamnestic and laboratory diagnostic algorithm presented here is designed to assist a simpler differentiation of the various forms of angioedema and to reach the correct diagnosis more quickly.

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Interessenkonflikt

Der korrespondierende Autor weist für sich und seine Koautoren auf folgende Beziehungen hin: Markus Magerl ist/war Vortragender/Berater für: CSL Behring, Shire/Jerini, Pharming, Viropharma. Marcus Maurer ist/war Vortragender/Berater für und/oder erhielt Forschungsförderung von: CSL Behring, Shire/Jerini, Viropharma, Biocryst. Jana Prässler: Teilnahme am Novartis Study Board Urticaria. Regina Treudler erhielt Unterstützung für den Besuch von Kongressen/Tagungen von der Fa. Shire Deutschland. Robert Vetter hat Reisegelder von Shire erhalten. Volker Wahn hat von den Firmen CSL Behring, Octapharma, Danone, UCB, AstraZeneca und MSD Honorare für Vorträge erhalten. Die Arbeit des ImmundefektCentrums der Charité (IDCC) wird unterstützt durch Grifols. Vasiliki Zampeli hat von der Firma Shire Teilnahmegebühren, Reisekosten und Übernachtungskosten (Istanbul 2011, EAACI) erhalten. Die übrigen Autorinnen/Autoren geben keine Interessenkonflikte an.

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Authors and Affiliations

  1. Klinik für Dermatologie, Venerologie und Allergologie, Allergie-Centrum, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland

    M. Magerl & M. Maurer

  2. Universitäts-Hautklinik Kiel, Kiel, Deutschland

    J. Brasch & B. Mohr

  3. Hals- Nasen- Ohrenklinik, Charité Universitätsmedizin Berlin, Berlin, Deutschland

    U. Förster

  4. Klinik und Poliklinik für HNO, Universitätsklinikum Carl Gustav Carus,TU Dresden, Dresden, Deutschland

    B. Hauswald

  5. Klinik für Dermatologie und dermatologische Allergologie, Friedrich-Schiller-Universität, Jena, Deutschland

    J. Präßler

  6. Klinik für Dermatologie, Venerologie & Allergologie, Universitätsklinikum Leipzig, Leipzig, Deutschland

    R. Treudler

  7. Universitätsklinik für Dermatologie und Venerologie, Magdeburg, Magdeburg, Deutschland

    R. Vetter & M. Ziemer

  8. Klinik für Pädiatrie m. S. Pneumologie und Immunologie, Charité Universitätsmedizin Berlin, Berlin, Deutschland

    V. Wahn

  9. Dermatologie, Venerologie und Allergologie, Immunologisches Zentrum, Städtisches Klinikum Dessau, Dessau, Deutschland

    V. Zampelli

Authors
  1. M. Magerl
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  2. J. Brasch
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  3. U. Förster
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  4. B. Hauswald
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  5. B. Mohr
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  8. R. Vetter
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  9. V. Wahn
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  10. V. Zampelli
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  11. M. Ziemer
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  12. M. Maurer
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Correspondence to M. Magerl.

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Magerl, M., Brasch, J., Förster, U. et al. Diagnostik und Ausschluss des hereditären Angioödems. Hautarzt 63, 567–572 (2012). https://doi.org/10.1007/s00105-012-2388-x

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  • DOI: https://doi.org/10.1007/s00105-012-2388-x

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