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Overview

Angioedema is a swelling of the deeper layers of the skin and/or mucous membranes. It can develop in any region of the body. Most commonly affected areas include the face (often lips or eyelids), hands, feet, arms, legs or genital organs. The trunk, however, may also be affected by angioedema.

Swellings occur at irregular intervals and typically last one to three days (sometimes as long as seven days). Sometimes, though by no means always, triggers may be identified. Common triggers include surgical procedures, stressful situations, infections, certain foods, heat or cold. In women, hormonal changes have been implicated. In most cases, the swelling of the skin is accompanied by a feeling of tightness.

Angioedema is caused by the escape of fluid from the blood vessels into the surrounding tissues in the affected body region. This occurs in response to the synthesis (formation) of vasoactive substances at the site, which may occur as a result of inflammation, allergies, injuries etc. “Vasoactive” refers to these substances’ effects on the properties of the vascular wall. For example, certain vasoactive substances, such as histamine or bradykinin, may temporarily increase the permeability of the vascular wall: the result is an outflow (extravasation) of fluid into the tissue with swelling of the skin and/or mucous membrane.

In certain forms of bradykinin-mediated angioedema, such as in hereditary angioedema (HAE), the gastrointestinal tract is frequently involved. Patients experience cramping or colicky pain, sometimes accompanied by nausea, vomiting and diarrhoea. In a few patients, angioedema symptoms are (almost) exclusively restricted to the gastrointestinal tract without visible swellings of the skin. In such cases, it may be very difficult to distinguish angioedema from other diseases affecting the gastrointestinal tract. Not infrequently, patients’ complaints may be misinterpreted as acute abdomen, appendicitis or a gastric ulcer. Under certain circumstances, this may even lead to unnecessary surgical procedures.

Sometimes, angioedema may become dangerous. This is the case when swellings affect the airways. When swellings develop in the area of the larynx (voice box), or in cases of severe swelling of the tongue, respiration may be compromised. Without prompt intervention, this may result in death by asphyxiation (suffocation).

Important: There are different forms of angioedema, each of which is treated differently. With very few exceptions, bradykinin-mediated forms of angioedema do NOT respond to treatment with cortisone or antihistamines. For these forms, there are other medications that result in an effective and rapid resolution of the swelling.

In order to be able to select the appropriate treatment, it is necessary to distinguish between the different forms of angioedema. Based on the underlying mechanism, angioedema is classified as:

The Angioedema Leaflet provides a concise overview of this subject. To download, click here. 

Further information: