Acquired angioedema: what it is and how swelling is managed

NHS inform describes angioedema as swelling of the deeper layers of the skin, caused by a build-up of fluid. The swollen area may be hot or painful. The face is commonly affected, particularly the lips, tongue and eyelids, as well as the hands, feet and genitals.

Acquired angioedema is one of five types listed by the NHS. It is described as very rare and as something you can get if you have lymphoma, which is a cancer of the lymphatic system, or a condition affecting the connective tissue, such as lupus. Because it is linked to another condition, the swelling can be a clue that prompts doctors to look for an underlying cause.

The other types are acute allergic angioedema, non-allergic angioedema (most commonly a reaction to ACE inhibitor medicines), idiopathic angioedema where the cause is unknown, and hereditary angioedema, which is rare and often inherited from a parent.

The main symptom is swelling. If an allergic reaction has caused it, there is usually also a raised, itchy rash (hives).

• Swelling of the lips, tongue, eyelids or face.

• Swelling of the hands, feet or genitals.

• Stomach pain if the bowel becomes swollen.

• Swelling of the throat, which can cause breathing difficulty and is a medical emergency.

The NHS says to call 999 if your lips, mouth, throat or tongue suddenly become swollen, if you are breathing very fast or struggling to breathe, if your throat feels tight or you are struggling to swallow, if your skin, tongue or lips turn blue, grey or pale, if you suddenly become very confused, drowsy or dizzy, if someone faints and cannot be woken, or if a child is limp, floppy or not responding normally. These can be signs of anaphylaxis, which needs immediate treatment in hospital.

The NHS advises not to drive yourself to A&E. Ask for an urgent GP appointment or get help from NHS 111 if part of your body suddenly becomes swollen, such as your hands, feet or genitals.

The cause of angioedema depends on the type. The NHS summarises them as follows.

NHS inform says your healthcare professional will examine the swollen area and ask about your symptoms, which helps them work out whether you need further tests. Tell your doctor about any medications you take, including over-the-counter painkillers, herbal supplements and vitamins, and do not stop prescribed medication unless advised to by a healthcare professional.

The sources reviewed for this page do not describe the specific blood tests used to identify acquired angioedema. These are specialist investigations, normally arranged through immunology or allergy services after the initial assessment.

The NHS says you may not need treatment for angioedema if the swelling is mild or only affects a small area. If the swelling is severe or widespread, or if it affects the lips, tongue or throat, you will usually need emergency treatment in hospital.

Treatments include identifying and avoiding the underlying cause, changing a medicine thought to be responsible, antihistamines such as cetirizine, fexofenadine or loratadine, and steroid tablets such as prednisolone. If you have a type that keeps coming back, such as hereditary angioedema, you may also need medicine to stop this happening, with regular reviews. If symptoms do not improve or get worse you may need to return to hospital for further tests and treatment.

For acquired angioedema, the underlying lymphoma or connective tissue condition is part of the picture, so it is reasonable to expect that more than one specialty may be involved.

Recurrent angioedema can be unsettling because swelling can be unpredictable. Keeping a note of when swelling occurs, what you had taken or eaten, and any medicines you were using can help your clinician find patterns.

If you have been given an emergency plan or medicines by a specialist, keep them with you and make sure family or colleagues know the warning signs. Tell any doctor, dentist or pharmacist about your angioedema before you start a new medicine.

On the NHS, emergency swelling is treated in A&E, and recurrent or unexplained angioedema is usually referred by a GP to allergy or immunology services. Rare types are often looked after in specialist centres.

Some people choose a private allergy or immunology consultation to be seen faster. For a very rare type like acquired angioedema, the key is access to a team with experience of the condition and its underlying causes, whichever sector it sits in.