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Angioedema

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Urticaria and Angioedema

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Abstract

Recurrent isolated angioedema (AE) is a diagnostic and therapeutic challenge. Only a rigorous clinical approach eliminates the most obvious diagnoses. The major clinical sign to look for is the presence or absence of recurrent hives, concomitant, or distant from the AE. The hives then lead to a mast cell origin. In the majority of cases, AE is secondary to non-specific mast cell activation. In these cases, they are benign and treated with high dose of antihistamines. Allergic AE is exceptionally isolated. They are almost always associated with extra-skin signs (digestive, respiratory, collapse) and associated with hives or generalized erythema. It should be kept in mind that some AE may reveal a life-threatening bradykinin pathology that does not respond to standard therapies. The most common bradykinin AE is those secondary to angiotensin-blocking drugs that require definitive discontinuation of treatment. Hereditary bradykinin AE (HAE) is of two types: either associated with a C1Inh deficiency or with a normal C1Inh. The diagnosis of HAE with normal C1Inh is based on the clinical and genetic mutation research on the F12, PLG, KNG1 and ANGPT1 genes. In the case of bradykinin AE, a specific treatment must be administered.

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Authors and Affiliations

  1. French National Reference Center for Angioedema (CREAK), Internal Medicine/Clinical Immunology Department, Grenoble University Hospital, Grenoble, France

    L. Bouillet

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  1. L. Bouillet
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Correspondence to L. Bouillet .

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Editors and Affiliations

  1. Institute for Allergology, Charité – Universitätsmedizin, Berlin, Germany

    Torsten Zuberbier

  2. St John’s Institute of Dermatology, Guy’s Hospital, London, UK

    Clive Grattan

  3. Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany

    Marcus Maurer

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Bouillet, L. (2021). Angioedema. In: Zuberbier, T., Grattan, C., Maurer, M. (eds) Urticaria and Angioedema. Springer, Cham. https://doi.org/10.1007/978-3-030-84574-2_9

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  • DOI: https://doi.org/10.1007/978-3-030-84574-2_9

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