Angioedema is a condition in which you may have swelling of the deeper layers of the skin. This swelling occurs due to fluid leakage from blood vessels into the surrounding tissues. . Angioedema most commonly affects the face, lips, eyes, hands, genitals and feet. It can be triggered by various factors, including allergic reactions, medications, insect bites, infections, or inherited genetic conditions.
Usually, angioedema comes on quickly and lasts about a day or two. It most often affects your lips and eyes. However, angioedema can be serious, even fatal, when it affects your airways.
What are the types of angioedema?
Angioedema can be classified into several types based on its underlying cause and triggers. The main types include:
Allergic Angioedema: This type is triggered by an allergic reaction to specific allergens, such as certain foods (e.g., nuts, shellfish, eggs), medications (e.g., penicillin, aspirin, nonsteroidal anti-inflammatory drugs), insect stings or bites, latex, or environmental factors (e.g., pollen, animal dander). Allergic angioedema typically occurs rapidly after exposure to the allergen.
Non-Allergic (Idiopathic) Angioedema: Non-allergic angioedema occurs without an identifiable allergic trigger. It may be associated with other underlying conditions, such as certain medications (e.g., ACE inhibitors used for hypertension), hormonal changes (e.g., estrogen fluctuations in women), infections (e.g., viral or bacterial infections), or genetic factors.
Hereditary Angioedema (HAE): HAE is a rare genetic disorder characterized by recurrent episodes of angioedema. It is caused by mutations in genes responsible for regulating a blood protein called C1 inhibitor. HAE can be further classified into three types: Type I (low levels of functional C1 inhibitor), Type II (normal or elevated levels of dysfunctional C1 inhibitor), and Type III (normal C1 inhibitor levels, but mutations in different genes).
Acquired Angioedema: Unlike hereditary angioedema, acquired angioedema occurs later in life and is not inherited. It is typically associated with underlying conditions such as lymphoproliferative disorders (e.g., lymphoma), autoimmune diseases (e.g., systemic lupus erythematosus), or certain medications.
Drug-Induced Angioedema: Some medications, particularly angiotensin-converting enzyme (ACE) inhibitors used to treat hypertension, can cause angioedema as a side effect. This type of angioedema typically occurs within the first few weeks of starting the medication but can occur at any time during treatment.
Symptoms and Causes Angioedema
Angioedema is characterized by sudden and often severe swelling beneath the skin's surface, typically in areas such as the face, lips, tongue, throat, hands, feet, or genitals. The swelling is caused by fluid accumulation in the deeper layers of the skin and mucous membranes. Symptoms and causes of angioedema can vary depending on the type:
Symptoms:
Rapid swelling: Swelling typically occurs within minutes to hours and may be accompanied by redness, warmth, or itching in the affected area.
Location: Angioedema commonly affects the face (especially around the eyes and lips), but it can also occur in other areas such as the hands, feet, throat, or genitals.
Pain or discomfort: Swelling may cause discomfort, pain, or a tingling sensation, especially if it affects mobility or pressure-sensitive areas.
Potential complications: Severe swelling in the throat or tongue can lead to difficulty breathing, swallowing difficulties, or hoarseness. In such cases, angioedema can become a life-threatening emergency requiring immediate medical attention.
Causes:
Allergic triggers: Allergic angioedema is often triggered by exposure to allergens such as certain foods (e.g., nuts, shellfish), medications (e.g., antibiotics, NSAIDs), insect stings or bites, latex, or environmental factors (e.g., pollen, animal dander).
Non-allergic triggers: Non-allergic angioedema can be caused by various factors, including certain medications (e.g., ACE inhibitors used for hypertension), hormonal changes (e.g., estrogen fluctuations), infections (e.g., viral or bacterial infections), or genetic factors.
Hereditary factors: Hereditary angioedema (HAE) is a rare genetic disorder caused by mutations in genes responsible for regulating blood proteins, such as C1 inhibitor. HAE can be further classified into Type I (low levels of functional C1 inhibitor), Type II (normal or elevated levels of dysfunctional C1 inhibitor), and Type III (normal C1 inhibitor levels, but mutations in different genes).
Acquired conditions: Acquired angioedema can occur later in life and is often associated with underlying conditions such as autoimmune diseases (e.g., systemic lupus erythematosus), lymphoproliferative disorders (e.g., lymphoma), or certain medications.
Angioedema Diagnosis
Diagnosing angioedema involves a combination of medical history, physical examination, and sometimes additional tests to determine the underlying cause and assess the severity of symptoms. Here are the key steps involved in diagnosing angioedema:
Medical history: The healthcare provider will typically start by asking about the patient's symptoms, including the timing, duration, and location of swelling episodes. They may inquire about potential triggers, such as recent exposure to allergens, medications, or environmental factors. Any relevant medical history, including family history of angioedema or allergic conditions, will also be reviewed.
Physical examination: A thorough physical examination is performed to assess the extent and location of swelling. The healthcare provider will examine the affected areas, such as the face, lips, tongue, throat, hands, or feet, and check for signs of inflammation, redness, or itching. They may also evaluate the patient's vital signs, including blood pressure, heart rate, and respiratory rate.
Differential diagnosis: Since angioedema shares some symptoms with other conditions, such as allergic reactions, cellulitis, or hereditary disorders, the healthcare provider will consider other possible diagnoses and may order additional tests to rule out alternative explanations for the symptoms.
Allergy testing: If allergic angioedema is suspected, allergy testing may be recommended to identify specific allergens that could be triggering the immune response. Allergy testing methods may include skin prick tests, blood tests (such as IgE antibody tests), or oral food challenges, depending on the suspected allergens and individual patient factors.
Laboratory tests: In some cases, laboratory tests may be ordered to assess for underlying conditions or factors contributing to angioedema. These tests may include complete blood count (CBC), blood chemistry panels, liver function tests, thyroid function tests, or tests to measure complement levels in the blood.
Imaging studies: In rare cases of suspected hereditary angioedema (HAE), imaging studies such as abdominal ultrasound or computed tomography (CT) scans may be performed to evaluate for abdominal involvement or swelling of internal organs.
Genetic testing: In cases of suspected HAE or other hereditary forms of angioedema, genetic testing may be recommended to identify specific gene mutations associated with the condition.
Treatment & Management of angioedema
The treatment and management of angioedema depend on several factors, including the underlying cause, severity of symptoms, and individual patient characteristics. Here are some general strategies for managing angioedema:
Identifying and avoiding triggers: For allergic angioedema, identifying and avoiding triggers is crucial. This may involve allergy testing to pinpoint specific allergens and then taking steps to minimize exposure to them. Common triggers include certain foods, medications, insect stings or bites, latex, and environmental factors.
Medications:
Antihistamines: Antihistamine medications, such as cetirizine, loratadine, or diphenhydramine, can help reduce itching and swelling associated with allergic angioedema.
Corticosteroids: In cases of severe or persistent angioedema, corticosteroids like prednisone may be prescribed to reduce inflammation and swelling.
Epinephrine: For severe allergic reactions with difficulty breathing or swallowing (anaphylaxis), epinephrine is the first-line treatment and should be administered promptly using an epinephrine auto-injector. Individuals at risk of anaphylaxis should carry an epinephrine auto-injector with them at all times.
ACE inhibitor discontinuation: If angioedema is caused by an ACE inhibitor medication, discontinuing the medication under medical supervision is necessary. Alternative medications for managing hypertension may be prescribed.
C1 esterase inhibitor replacement therapy: For hereditary angioedema (HAE), replacement therapy with C1 esterase inhibitor concentrates, such as Berinert or Cinryze, can help prevent and treat attacks.
Supportive measures:
Cold compresses: Applying cold compresses or ice packs to the affected area can help reduce swelling and provide symptomatic relief.
Elevating affected areas: Elevating the affected limbs or areas above heart level can help reduce swelling.
Airway management: In cases of severe angioedema affecting the throat or tongue, maintaining a clear airway is crucial. Emergency medical care may be necessary to ensure adequate oxygenation and breathing support.
Patient education: Educating patients and their caregivers about angioedema triggers, emergency response techniques (such as using epinephrine auto-injectors), and when to seek medical help is essential for effective management and prevention of complications.
Regular follow-up: Individuals with angioedema should be closely monitored by healthcare providers, especially after severe episodes. Regular follow-up visits are important to assess treatment effectiveness, adjust medications if needed, and address any concerns or complications.
FAQ Angioedema?
Is angioedema curable?
There is no known cure, however it may be possible to prevent swelling with medications. Wheal like swellings on the surface of the skin are called hives (urticaria). Angioedema involves swelling deeper in the tissues. Allergy is very rarely the cause of isolated angioedema (swelling without any other symptoms)
Foods to avoid with angioedema ?
When managing angioedema, particularly allergic angioedema, it's essential to identify and avoid triggers that may lead to an allergic reaction. While specific food triggers can vary from person to person, some common allergenic foods that may exacerbate angioedema include:
Shellfish: Crustaceans (such as shrimp, crab, lobster) and mollusks (such as clams, mussels, oysters) are common allergens for many people.
Nuts: Peanuts and tree nuts (such as almonds, walnuts, cashews, pistachios) are frequent triggers for allergic reactions.
Eggs: Some individuals may be allergic to eggs or specific components of eggs, such as the proteins found in egg whites or yolks.
Dairy: Milk and dairy products, including cow's milk, cheese, yogurt, and butter, can be allergenic for some people, particularly those with lactose intolerance or a milk protein allergy.
Wheat and Gluten: Wheat and gluten-containing grains (such as wheat, barley, rye) can trigger allergic reactions or exacerbate symptoms in individuals with gluten sensitivity or wheat allergy.
Soy: Soybeans and soy products, including soy sauce, tofu, and soy milk, can cause allergic reactions in some individuals.
Fish: Certain types of fish, such as salmon, tuna, and mackerel, can be allergenic for some people.
Certain Fruits and Vegetables: Although less common, some fruits (e.g., strawberries, citrus fruits) and vegetables (e.g., tomatoes, celery) can trigger allergic reactions in susceptible individuals.
How common is angioedema?
Up to 20% of people will develop hives (urticaria) at some time in their life, and around one in three of these will have angioedema as well. Having angioedema on its own (without hives) is much less common. There are three major patterns of angioedema: • Angioedema plus hives (urticaria): the hives itch and the angioedema is itchy, hot or painful. • Angioedema alone: itchy, hot and red swellings, often large and uncomfortable. • Angioedema alone: skin-coloured swellings, not itchy or burning, often unresponsive to antihistamines. Angioedema eventually disappears in most people. It may reappear following infection, when under stress or for no particular reason that can be identified. Occasionally it is a recurrent problem that reappears throughout life. Angioedema is seldom caused by a serious underlying disease, nor does it make you sick or cause damage to organs such as kidneys, liver or lungs.
Where does angioedema occur?
The most common areas of the body involved are the face, lips, tongue, throat and genital areas. Swelling in one area usually lasts between one to three days. Occasionally, swelling of internal organs like the oesophagus, (food pipe or gullet that leads to the stomach), stomach, or bowel can trigger chest or stomach pains. Whilst angioedema may be itchy, tingling, or burning, often there are no symptoms other than the discomfort of the swelling. Sometimes the swelling can be painful, particularly when it occurs over joints. These swellings can be large and may last for days. If swelling is constant rather than coming and going, other possible causes of swelling should be considered.
Is angioedema dangerous?
Angioedema does not damage internal organs like kidneys, liver or lungs. The only danger is if the throat or the tongue swell severely, as this can cause difficulty breathing. Severe throat swelling requires early use of medication such as adrenaline for anaphylaxis or icatibant for hereditary angioedema (HAE), and transfer to hospital by ambulance. Swelling on the outside of the neck is uncomfortable but does not affect breathing. Swelling that interferes with breathing is uncommon, even in people with recurrent angioedema. People with recurrent angioedema should be referred by their doctor to a clinical immunology/allergy specialist to investigate for an underlying cause and optimise treatment.
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