If you would like to learn about symptoms and diagnosis of angio-oedema, and angio-oedema treatment, you will find the following information of interest.
Episodes of angio-oedema cause swelling of deeper skin tissues, most commonly of the eyelids, lips, genitals, hands, and feet. Sometimes the tongue and throat are affected which may affect breathing. There are various causes. Some people have recurring episodes. Each episode usually clears within a few days. Antihistamines and steroid tablets ease symptoms. If your breathing is affected then go straight to your local accident and emergency department or call for an ambulance urgently.
What is angio-oedema?
Angio-oedema is a condition that can cause swelling of:
- the deeper layers of the skin. That is, the dermis and subcutaneous tissues. Also,
- the tissues just under the lining of the airways, mouth and gut. That is, the submucosal tissues.
What happens is that cells called mast cells are triggered to release a chemical called histamine. Histamine causes the tiny blood vessels in these tissues to become 'leaky'. Fluid then leaks into the tissues which makes them swell. The swelling can occur under any part of the skin or submucosal tissues. However, it commonly affects the eyelids, lips, genitals, hands and feet. Sometimes the tongue, throat and airway are affected and become swollen. The swelling sometimes becomes bad enough to cause difficulty breathing.
What causes angio-oedema?
In most cases, there is no known cause and it is not clear why it occurs. This is called 'idiopathic angio-oedema'. Although the cause is not clear, in up to half of cases there is a link to an auto-immune disorder, for example, chronic urticaria, systemic lupus erythematosus (SLE), etc. About half of people who have chronic urticaria also have regular episodes of angio-oedema.
In some cases there are known 'triggers' that can cause the release of histamine which leads to tissue swelling of angio-oedema. These are:
In some cases there are known 'triggers' that can cause the release of histamine which leads to tissue swelling of angio-oedema. These are:
Allergic reactions
Some people develop angio-oedema as part of an allergy reaction. Something triggers the immune system which triggers mast cells to release histamine. In effect, it is a bit like the immune system 'over reacting'. For example:
- Allergy to foods, for example, nuts, shellfish, milk, eggs.
- Allergy to drugs, for example, penicillin, aspirin.
- Allergy to latex, insect bites or stings.
The symptoms that may develop with an allergic reaction can vary. For example:
- Some people develop an urticarial rash.
- Some people develop angio-oedema.
- Some people develop an urticarial rash and angio-oedema.
- Some people develop a very severe reaction called anaphylaxis. This usually includes an urticarial rash, angio-oedema, and other symptoms such as low blood pressure, severe breathing problems, and collapse. Anaphylaxis is rare, but is the most serious type of allergic reaction and can be fatal unless promptly treated.
- Various other symptoms can develop to localised allergies. For example, nasal symptoms if you are allergic to pollen (hay fever), etc.
Non-allergic drug reaction
Some drugs (medicines) can cause angio-oedema as a side-effect. For example, angiotensin converting enzyme (ACE) inhibitors and angiotension-II receptor antagonists (AIIRAs). These medicines are commonly used to treat heart problems and high blood pressure.
Hereditary
Some people inherit a tendency to develop episodes of angio-oedema. This is due to a lack of an enzyme called C1 esterase. This condition is called 'hereditary C1 esterase deficiency'. About 1 in 50,000 people inherit the gene for C1 esterase deficiency. Children born to people with this condition have a 50:50 chance of inheriting the condition. Although it is hereditary and most cases first develop in childhood, in some cases the angio-oedema may first develop in early adulthood.
How common is angio-oedema?
The number of people affected by angio-oedema is not known, but it is uncommon. Women are affected more often than men. It can occur at any age. However, it most commonly affects people aged 40-60 (apart from hereditary angio-oedema which often develops in children).
Hereditary angio-oedema is rare and accounts for about 1 in 200 cases of angio-oedema.
Hereditary angio-oedema is rare and accounts for about 1 in 200 cases of angio-oedema.
What are the symptoms of angio-oedema?
Symptoms of each episode develop quickly, over minutes or hours.
- A 'typical' episode is as follows:
- Areas of the skin become more and more swollen. Most commonly this affects the eyelids, lips, genitalia, hands and feet.
- The surface of the skin may appear normal - it is the tissues just beneath the skin that swell.
- The swellings are often more painful than itchy.
- An itchy urticarial rash often develops at the same time on various parts of the body. See separate leaflets called 'Acute Urticaria' and 'Chronic Urticaria'.
- The swelling takes up to 72 hours to ease and go.
- In some cases, in addition to the above:
- You may become short of breath, become wheezy, and have difficulty breathing due to swelling of the lining of throat, main airway, tongue and and mouth.
- You may develop abdominal pain with vomiting or diarrhoea.
- Occasionally, angio-oedema is part of a more severe anaphylaxis episode (described above).
- Hereditary angio-oedema:
- Typically causes recurring episodes. In most cases there are one or more episodes per month. These can occur 'out of the blue' for no apparent reason, but episodes may be triggered by stress, trauma, minor operations and dental surgery.
- Swellings commonly affect the hands or feet and are painless.
- Up to 1 in 4 cases have involvement of the throat, tongue or airway with some degree of wheezing or breathing difficulty.
- Abdominal pain is common.
- Episodes last 1-4 days.
What is the treatment for angio-oedema?
Treating each episode
The most important thing is to determine whether an episode of angio-oedema affects breathing, or if it is part of an anaphylaxis episode.
- If breathing is affected or if you have any symptoms of anaphylaxis:
- You should go straight to your local casualty (accident and emergency department) or call for an ambulance urgently.
- You may be given adrenaline by injection, a course of antihistamines, and a short course of steroids. These help to prevent symptoms from getting worse, and help to clear the symptoms more quickly than they would do naturally.
- You will be observed until the symptoms subside.
- Help with breathing and intensive care may be needed in severe cases.
- If breathing is not affected and you feel otherwise OK:
- You may be advised to take a short course of antihistamines and steroid tablets. These help to prevent symptoms from getting worse, and help to clear the symptoms more quickly than they would do naturally.
- Most episodes of angio-oedema will clear away within a few days.
- A cool shower or a cold compress on the affected area may ease symptoms.
- If your skin is itchy it is best to try not to scratch as it may damage the skin. If necessary, rubbing itchy skin with the palms of your hands is better than scratching. Choose clothing that does not irritate the skin. Consider whether any skin creams, soaps, or detergents are making symptoms worse.
- If symptoms get worse and breathing does become affected, then go straight to your local casualty (accident and emergency department) or call for an ambulance urgently.
Follow up and general advice
Most people who have an episode of angio-oedema will be referred to specialist (immunologist). This is to confirm the diagnosis, and where possible, to identify a cause. The severity of one episode compared to another is unpredictable. So, if you do have an allergy to something, it is best to be aware of what the allergy is.
The specialist will also advise on such things as
The specialist will also advise on such things as
- Whether it is likely to happen again.
- What to do if it does happen again.
- Whether you should carry an injection of adrenaline with you at all times in case you have a severe episode.
- Advice on avoiding the cause, if you are diagnosed as having an allergy.
What is the course and prognosis (outcome)?
- For sudden episodes of allergic angio-oedema - in most cases they are not severe or life-threatening, and will usually clear in 1-3 days. However, recurrences are common and the severity of each episode can vary. Some episodes are severe and life-threatening, especially if the angio-oedema is part of an anaphylactic episode.
- If your angio-oedema is due to a non-allergic drug reaction, then the episodes of angio-oedema can get more severe if the drug is not stopped.
- Idiopathic angio-oedema often has a 'waxing and waning' course. The severity of each episode can vary. If you have chronic urticaria, then treatment for this may help to prevent some episodes of angio-oedema. See separate leaflet called 'Chronic Urticaria'.
- Hereditary angio-oedema can vary in severity.
References
- Angio-oedema and anaphylaxis. Clinical Knowledge Service. 2007
- Nedra Dodds and Richard Sinert. Angioedema. eMedicine. Article dated April 4 2005
- WR Heymann and KM Rossy. Angioedema, Hereditary. eMedicine. Article dated February 27 2006
© EMIS and PiP 2007 Updated: 9 May 2007
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