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Angioedema - Types, Causes, Symptoms, Risk Factors, Diagnosis, and Treatment

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Angioedema is a sudden, short-lived swelling of the skin and mucous membranes affecting any body part. Read this article to know more about angioedema.

Medically reviewed by

Dr. Dhepe Snehal Madhav

Published At September 5, 2022
Reviewed AtDecember 26, 2023

What Is Angioedema?

Angioedema is swelling in the inner surface of the skin. It is usually harmless and clears within a day, but it becomes life-threatening when the airway gets blocked. Hives sometimes accompany angioedema. Angioedema is often visibly seen on one side of the face. This swelling results from the accumulation of fluid in the deeper layers of the skin, causing a distinct puffiness that is noticeable, particularly on one side of the face. The swelling in angioedema can lead to a swollen face on one side, impacting the lips, eyes, or cheeks.

What Are the Symptoms of Angioedema?

In angioedema, the swollen face on one side can occur suddenly and may be accompanied by other symptoms such as redness, warmth, and discomfort. Following are the common features of angioedema.

  • Large, thick, firm swellings.

  • Mild pain, redness, and warmth in the affected areas.

  • Swellings within minutes to hours, usually around the eyes, lips, or cheeks.

  • Its common location includes the face (swollen face on one side), throat, uvula (the little piece of skin that hangs from the back of the throat), larynx (voice box), arms, hands, legs, or feet. Rare locations include around the genitals and intestines.

  • Some rare symptoms of angioedema include breathing difficulty, red, irritated eyes, stomach pain, feeling sick, loose stool, dizziness, and fainting.

What Causes Angioedema?

Angioedema is caused by a rapid increase in the permeability of capillaries beneath the skin and post-capillary venules with localized fluid discharge. Most cases of angioedema are dependent upon the release of either histamine or bradykinin. The histamines are released by antigen-dependent crosslinking of immunoglobulin E (IgE) at the mast cells or basophils surface, as in typical allergic reactions.

Bradykinin is a chemical mediator of angioedema associated with angiotensin-converting enzyme (ACE) inhibitors that prevent bradykinin destruction. This raises the bradykinin level. The bradykinin forms either by the tissue bradykinin-forming pathways or plasma. C1 inhibitor (INH) deficiency, either be inherited or acquired, led to the overproduction of bradykinin caused without inhibition of the enzymes kallikrein and activated factor, XII.

What Are the Types of Angioedema?

Angioedema is categorized into four types, each with distinct causes.

1. Allergic Angioedema: It is the most prevalent type of angioedema. Common allergens include certain foods such as shellfish, fish, peanuts, tree nuts, eggs, and milk. Allergic reactions leading to angioedema can be caused by exposure to substances like pollen, animal dander, latex, and insect stings.

2. Drug-Induced Angioedema: Angioedema can be induced by certain medications. These may include drugs such as penicillin, aspirin, and other non-steroidal anti-inflammatory drugs (NSAIDs) like Ibuprofen and Naproxen. Specific blood pressure medications known as angiotensin-converting enzyme (ACE) inhibitors can lead to sudden flare-ups of angioedema, even in individuals who have been using these medications for an extended period. It is important to be aware of the potential for abrupt reactions, even with prolonged use of ACE inhibitors.

  • Angioedema associated with Lisinopril is a condition where swelling occurs, and it is specifically linked to the use of the medication Lisinopril. Angioedema associated with Lisinopril means that the swelling is a side effect of taking Lisinopril. Lisinopril is known to cause angioedema in some individuals, leading to symptoms like swelling in areas such as the face, lips, or tongue. If someone experiences symptoms of swelling, especially in the face, while using Lisinopril, it is crucial to consider the possibility of angioedema related to Lisinopril.

3. Hereditary Angioedema (HAE): This type of angioedema is a rare condition where the body does not produce enough of a blood protein called C1 esterase inhibitor. This deficiency allows fluid from the blood to move into other tissues, causing swelling.

  • Individuals experience their first episode before the age of 12, and there is a possibility of passing the condition to their children.

  • Acquired angioedema is an exceptionally rare type with similar symptoms to HAE but occurs later in life, typically after the age of 40.

4. Idiopathic Angioedema: This type of angioedema refers to swelling without a known cause. Potential triggers may include anxiety, stress, minor infections, extreme temperatures, or excessive exercise. Allergic and drug-induced angioedema typically occur within an hour of exposure to triggers, while hereditary and acquired types often develop over several hours, though the swelling may seem sudden upon waking up.

types-of-angioedema

What Are the Risk Factors of Angioedema?

Angioedema is seen as widespread. Some risk factors of angioedema include:

  • History of angioedema.

  • History of any allergic reactions.

  • Family history of angioedema.

  • History of thyroid disease, lupus, lymphoma, HIV, hepatitis, cytomegalovirus, Epstein-Barr virus, or blood transfusion.

  • History of asthma and NSAIDs.

  • Intake of angiotensin-converting enzyme inhibitors.

  • History of recombinant tissue plasminogen activator (rtPA ) treatment for a stroke.

Foods that can worsen swelling should be avoided. Consider the following:

  • Steer clear of foods that are commonly allergenic, such as peanuts, nuts, shellfish, fish, eggs, milk, and soy.

  • Food Additives: Chemicals found in processed foods may pose a problem. Check labels and opt for foods with fewer additives.

  • Histamine-Rich Foods: Histamine, found in certain foods, can trigger allergies. Foods like aged cheese, fermented foods, processed meats, and specific vegetables (like tomatoes and eggplants) tend to have high histamine levels.

  • Excessive salt intake can lead to water retention and worsen swelling.

  • Some individuals with angioedema may experience issues with alcohol. Consider reducing alcohol intake or avoiding it altogether.

  • Foods with a high spice or hot pepper content can cause blood vessels to open up more, potentially worsening swelling. If spicy foods cause discomfort, it is best to avoid them.

  • Certain individuals may be sensitive to particular foods, even if they are not allergic. Identifying and avoiding these troublesome foods may be necessary.

How Is Angioedema Diagnosed?

Angioedema is usually diagnosed with a complete examination at adequate lighting and a good history of swelling. Identifying the specific cause of angioedema is crucial for effective management and prevention of recurring episodes of a swollen face on one side. Sometimes it may require an allergy test or a blood test for confirmation.

  • Skin Prick Test: In this test, the skin is pricked with a small amount of the doubted allergen to check for the skin reaction.

  • Blood Test: A blood test is done when a genetic inheritance is suspected. C1 esterase inhibitor level is tested for immune system response. Low C1 esterase inhibitor level shows decreased immune system response.

How Can Angioedema Be Treated?

Angioedema is usually a self-limiting reaction. Medications are not mandatory but are prescribed to control itching or any discomfort. Treatment for angioedema varies based on the specific type. For allergic angioedema, healthcare providers may suggest antihistamines or steroids, available in oral or intravenous forms. Antihistamine and oral corticosteroids help in relieving swelling, inflammation, and itching. Non-allergic drug reactions may involve finding an alternative medication to replace the one causing swelling. The common drugs used in treatment are listed below:

  • Drugs to suppress the immune system.

  • Leukotriene antagonists for pain and swelling.

  • Blood protein regulators for hereditary angioedema.

  • Epinephrine is given in a severe allergic outbreak (anaphylaxis). When the attack repeats, a pen-like device loaded with Epinephrine is carried for emergency auto-injectors. Different types of auto-injectors are available in the market.

  • If the airway passage is involved, the priority must be to maintain the airway circulation by intubation or rarely a tracheostomy and administer oxygen.

  • Medications like C1 esterase inhibitor (recombinant), C1 inhibitor (human), Ecallantide, Icatibant, Lanadelumab, and Berotralstat are used to treat or prevent hereditary angioedema.

What Is the Prognosis of Angioedema?

Angioedema usually has a good prognosis. Rarely seen as a life-threatening issue.

What Are the Complications of Angioedema?

Angioedema becomes life-threatening when it involves the tongue or throat and blocks the airway.

What Are the Preventive Measures?

Angioedema can be prevented by taking the following measures;

  • Try to avoid the identified triggers.

  • Bath and change the clothes when exposed to pollen or animals that have been a trigger in the past.

What Lifestyle Modifications Are Needed?

  • Avoiding triggers is the primary treatment.

  • Covering the affected area with a cold cloth or rubbing an ice cube on it for a few minutes can help calm the skin and prevent scratching.

  • Taking a comfortably cool bath can relieve itching. Some people have benefited from bathing in baking soda or oatmeal powder sprinkled with cold water. But this is not a solution for long-term control of prolonged itching.

  • Wear loose, smooth-textured cotton clothing, and avoid wearing rough, tight, scratchy clothes or woolen clothes.

  • Sun protection by applying sunscreen generously for about 30 minutes before going outside. When outdoors, look for a shady place to help relieve discomfort.

Conclusion:

When angioedema occurs, the individual may sometimes experience a swollen face on one side that can develop rapidly. The swelling can be accompanied by other symptoms like redness, warmth, and discomfort. Angioedema is life-threatening with multiple subtypes, each with a distinct disease pattern. Each of these subtypes of angioedema has a specific approach to diagnosis and management. The most effective treatment depends on identifying the cause and the clinician's knowledge and experience with the treatment procedure.

Frequently Asked Questions

1.

Does Stress and Anxiety Cause Angioedema?

When the cause is not known, it is termed idiopathic angioedema. It is due to a defective immune system. In idiopathic angioedema, certain triggers such as stress or anxiety, may lead to swelling.

2.

Which Food Triggers Angioedema?

Angioedema can be triggered by certain types of food such as:
 - Nuts.
 - Shellfish.
 - Milk.
 - Eggs.
 - Meat 
 - Butter.
 - Rice.

3.

What Are the Tests for Angioedema?

Angioedema test may include a skin prick test where the individual's skin is pricked with a tiny amount of the suspected allergen to check for a reaction. A blood test and allergy testing may also be done.

4.

How Do You Treat Angioedema?

Treatment involves:
 - Avoid triggers.
 - Administration of antihistamines and steroid medicines.
 - Adrenaline auto-injectors.
 - Plasma kallikrein inhibitors.
 - Androgenic hormones.

5.

What Are the Ways to Reduce Angioedema Naturally?

Natural ways to reduce angioedema involve:
 - Avoid triggers.
 - Apply cold.
 - Take a cool bath.
 - Wear loose clothing.
 - Protect skin from the sun.

6.

What Is the Time Taken for Angioedema to Develop?

Allergic edemas happen quickly, within minutes to one to two hours after the individual has made contact with the allergen.

7.

Does Angioedema Require Urgent Medical Care?

Some angioedema can be treated at home, but airway angioedema may be fatal and requires immediate medical attention.

8.

How Is Edema Different From Angioedema?

Angioedema is asymmetric, nonpitting, and has the capacity not to involve gravitationally dependent areas, unlike other forms of edema.

9.

Is Angioedema a Disability?

Hereditary angioedema may cause physical or psychological disability due to the lack of effective treatments and the unpredictability of symptom severity.

10.

What Are the Synonyms for Angioedema?

The synonyms include:
 - Giant hives.
 - Quincke’s edema.
 - Periodic edema.
 - Atrophedema.
 - Dropsy.

11.

What Is the Cause of Non-allergic Angioedema?

Non-allergic or drug-induced edema is caused due to certain medications such as blood pressure drugs and angiotensin-converting enzyme inhibitors.

12.

What Happens if Angioedema Is Not Treated?

In severe cases of allergic reactions, there may be anaphylaxis which leads to swelling of the throat, making it hard for the individual to breathe. Hence in such cases, it requires immediate medical attention.

13.

Is Angioedema Life-Threatening?

Angioedema can be life-threatening when the swelling occurs in the throat and can cause airway obstruction.

14.

Which Autoimmune Disease Causes Angioedema?

It has been reported that 30 % to 50 % of cases of idiopathic angioedema may be linked to systemic lupus erythematosus.

15.

What Does Hereditary Angioedema Mean?

Hereditary angioedema is a condition characterized by repetitive episodes of swellings. The commonly affected areas are the airway, limbs, face, and intestinal tract. Trigger factors may include stress and anxiety.
Dr. Dhepe Snehal Madhav
Dr. Dhepe Snehal Madhav

Venereology

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