Der Hautarzt

, Volume 63, Issue 7, pp 567–572

Diagnostik und Ausschluss des hereditären Angioödems

Ein standardisierter Ansatz für die Praxis
  • M. Magerl
  • J. Brasch
  • U. Förster
  • B. Hauswald
  • B. Mohr
  • J. Präßler
  • R. Treudler
  • R. Vetter
  • V. Wahn
  • V. Zampelli
  • M. Ziemer
  • M. Maurer
Übersichten

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.

Schlüsselwörter

Angioödem Bradykinin C1-Inhibitor Anamnestischer Algorithmus Diagnostischer Algorithmus 

Diagnostics and exclusion of hereditary angioedema

A standarized approach for the practice

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.

Keywords

Angioedema Bradykinin C1 inhibitor Anamnestic algorithm Diagnostic algorithm 

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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • M. Magerl
    • 1
  • J. Brasch
    • 2
  • U. Förster
    • 3
  • B. Hauswald
    • 4
  • B. Mohr
    • 2
  • J. Präßler
    • 5
  • R. Treudler
    • 6
  • R. Vetter
    • 7
  • V. Wahn
    • 8
  • V. Zampelli
    • 9
  • M. Ziemer
    • 7
  • M. Maurer
    • 1
  1. 1.Klinik für Dermatologie, Venerologie und Allergologie, Allergie-CentrumCharité - Universitätsmedizin BerlinBerlinDeutschland
  2. 2.Universitäts-Hautklinik KielKielDeutschland
  3. 3.Hals- Nasen- OhrenklinikCharité Universitätsmedizin BerlinBerlinDeutschland
  4. 4.Klinik und Poliklinik für HNOUniversitätsklinikum Carl Gustav Carus,TU DresdenDresdenDeutschland
  5. 5.Klinik für Dermatologie und dermatologische AllergologieFriedrich-Schiller-UniversitätJenaDeutschland
  6. 6.Klinik für Dermatologie, Venerologie & AllergologieUniversitätsklinikum LeipzigLeipzigDeutschland
  7. 7.Universitätsklinik für Dermatologie und Venerologie, MagdeburgMagdeburgDeutschland
  8. 8.Klinik für Pädiatrie m. S. Pneumologie und ImmunologieCharité Universitätsmedizin BerlinBerlinDeutschland
  9. 9.Dermatologie, Venerologie und Allergologie, Immunologisches ZentrumStädtisches Klinikum DessauDessauDeutschland

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