© by aha! Swiss Allergy Centre - Angioedema - Info on allergies - a woman with abdominal pain

Our skin is made up of three layers: epidermis, dermis and hypodermis (or subcutaneous layer). Angioedema – also known as Quincke’s oedema or angioneurotic oedema – means accumulations of fluid in the subcutaneous tissue that lead to swelling. Two basic forms of angioedema can be distinguished: mast cell-mediated and bradykinin-mediated angioedema.

Causes and triggers

Mast cell-mediated angioedema

Most cases of angioedema that occur spontaneously are mast-cell-mediated (also known as mast cell mediator-mediated). The causes of activation of the mast cells are diverse: e.g. allergic reactions, viral or bacterial infections, medications or even stress factors.

Bradykinin-mediated angioedema

Bradykinin is a tissue hormone that increases vessel permeability. The protein C1-inhibitor regulates the formation of bradykinin. If C1-inhibitor is available in insufficient quantities, if it is deficient or too much of it is consumed, the result is an excess of bradykinin. This also happens if degradation of bradykinin is blocked. This excess leads to angioedema. Subforms of this group are:

  • hereditary angioedema (HAE)
  • acquired angioedema
  • drug-induced angioedema (caused by certain blood pressure-lowering drugs, especially  ACE-inhibitors or to some extent blood sugar-lowering drugs such as gliptins)

Symptoms

Swellings occur in both forms of angioedema, often combined with a feeling of pressure or a burning sensation in different parts of the body, e.g.

  • Head: swelling of the eyelids, lips, facial skin, headaches, dizziness
  • Genital organs: swelling of the external genital organs
  • Arms and legs: swellings on hands, feet, arms and legs; normal sequences of movement are restricted, walking impeded

In mast cell-mediated angioedema, itchy hives (urticaria) may appear on the body at the same time.

In hereditary angioedema (HAE) swellings frequently occur in the gastrointestinal tract (especially in the small and large bowel), which can result in severe abdominal pains, nausea, vomiting and diarrhoea even lasting several days. Swellings in the airways can be very dangerous. However, life-threatening situations can be averted in most cases because effective medicines are available nowadays.

Diagnosis

If the swelling is visible, the clinical diagnosis is relatively straightforward. On the other hand, searching for the cause is often a challenge, which is why it is important for the doctor to question the individual thoroughly when investigating the symptoms. In addition, specific laboratory tests (e.g. measurement of the level of C1-inhibitor) can provide further information to help clarify matters. A symptom diary kept by the sufferer may prove helpful to the doctor when making the diagnosis. This diary should detail the times when the swelling appears and contain information about pain, itching and accompanying symptoms (such as possible skin rashes, joint pains, breathlessness or abdominal pains). Photographs of the swellings are also very useful to the doctor.

Therapy and treatment

Mast cell-mediated angioedema

If the angioedema is induced by allergy, the trigger should be avoided as far as possible. If a medication is responsible for the reaction, the individual should stop taking the drug. If a bacterial infection or some other illness is the cause, it is important to treat this appropriately.

In most cases – irrespective of the cause – antihistamines and cortisone preparations are used to relieve the symptoms. In the case of life-threatening swellings, these drugs are also used as emergency medication, if necessary combined with an adrenaline pre-filled syringe.

Bradykinin-mediated angioedema

In hereditary angioedema (HAE), the primary aim should also be to avoid the triggers. In addition, there is a possibility of preventive therapy with certain drugs. The protein C1-inhibitor and icatibant (active ingredient) – a bradykinin receptor antagonist – can be used to treat an acute angioedema attack.

In acquired angioedema it is important to ensure that the underlying disease is treated. Similarly in drug-induced angioedema via bradykinin, the drugs that cause the angioedema must be withdrawn. If necessary, they can be replaced by medicines from a different class of active substance. 

Tips & Tricks

  • Drug-induced angioedema can occur for the first time even though the drug has already been taken for a lengthy period of time (weeks or months). Consult a doctor immediately if the relevant symptoms appear.
  • Hereditary angioedema (HAE) runs in families. Therefore family members should also be tested when a case of HAE is confirmed.

Facts and figures

  • Up to 20 per cent of the population are affected by an acute swelling at some time in their lives.  
  • In Switzerland there are about 160 people suffering from hereditary angioedema. The incidence is roughly 1 in 50,000. 
  • Angioedema can occur in one area or several areas of the body.


Editors: aha! Swiss Allergy Centre in co-operation with the Scientific Advisory Board.
 

aha! Swiss Allergy Centre helps

  • aha!shop: other booklets and factsheets provides detailed information and are available in German, French, Italian
  • aha!kinderlager for children with allergies aged between 8 and 12 years (in German or French)
  • aha!jugendcamp for youngsters aged between 13 and 16 years (in German)
  • Other education and courses on the subjects "Allergy, asthma, chronic bronchitis", "Anaphylaxis" (in German)
  • We value your opinion. Or would you like to exchange experiences with other sufferers? Find out more in german under "Advice and exchange"

Help us

  • Your donation will enable us to provide important services to people with allergies, asthma and neurodermatitis. Your support will be put to effective use. Many thanks.