Diagnosing A Condition With Swelling Of The Lips, Swelling Of The Eyes, Swelling Of The Hand, Swelling Of The Feet
Angioedema: A Complete Overview
Introduction
Angioedema is a medical condition characterized by sudden swelling beneath the skin or mucosa, most commonly affecting the face, lips, tongue, throat, hands, and feet. It can be alarming due to its rapid onset and potential to obstruct the airway, posing a life-threatening emergency in some cases. Angioedema may occur alone or with other allergic conditions such as urticaria (hives).
Types of Angioedema
Angioedema is classified into several types based on its underlying cause:
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Allergic Angioedema
This is the most common form and occurs due to an allergic reaction to foods (e.g., peanuts, shellfish), medications (e.g., penicillin, aspirin), insect stings, or environmental allergens. It is mediated by histamine release. -
Hereditary Angioedema (HAE)
A rare, genetic form caused by a deficiency or dysfunction of the C1 esterase inhibitor protein, leading to unregulated inflammation. It typically begins in childhood or adolescence and does not respond to antihistamines or steroids. -
Acquired Angioedema
This form develops later in life and is similar to hereditary angioedema but not inherited. It may be associated with autoimmune diseases or certain cancers. -
Drug-induced Angioedema
Most commonly caused by angiotensin-converting enzyme (ACE) inhibitors used for hypertension. It can occur even after years of using the drug. -
Idiopathic Angioedema
In some cases, the cause cannot be determined. These cases are labeled idiopathic and may have recurrent episodes.
Causes and Triggers
- Allergens: Foods (nuts, eggs, dairy), insect bites, pollen, animal dander.
- Medications: NSAIDs, antibiotics (penicillin), ACE inhibitors.
- Infections: Viral or bacterial infections may sometimes trigger angioedema.
- Physical stimuli: Pressure, cold, heat, or vibration in rare types (e.g., vibratory angioedema).
- Genetic mutations: In HAE, mutations in the SERPING1 gene affect the C1 inhibitor protein.
Symptoms
The hallmark of angioedema is rapid swelling, usually occurring within minutes to hours. Key symptoms include:
- Swelling of the face, eyelids, lips, tongue, or throat
- Abdominal pain (in HAE cases)
- Difficulty breathing or swallowing (in severe cases)
- Skin may feel tight or painful, but usually not itchy (unlike hives)
- Voice hoarseness or stridor in airway involvement
Diagnosis
Diagnosis is primarily clinical, based on symptoms and history. Additional investigations may include:
- Blood tests for C1 inhibitor level and function (especially in suspected HAE)
- Allergy testing to identify triggers
- Complete blood count (CBC) and complement levels (C4, C1q)
- Imaging (CT, ultrasound) for abdominal attacks in HAE
Treatment
Emergency Management
If breathing is affected, immediate medical attention is required. Emergency treatments may include:
- Epinephrine (adrenaline) injection
- Antihistamines (e.g., diphenhydramine)
- Corticosteroids (e.g., prednisone)
- Airway management (intubation in severe cases)
Ongoing Management
- Allergic angioedema: Avoid known triggers, carry epinephrine auto-injector if needed.
- Hereditary angioedema:
- C1 inhibitor concentrate replacement (e.g., Berinert, Cinryze)
- Bradykinin inhibitors (e.g., icatibant)
- Prophylactic treatments (e.g., lanadelumab)
- ACE inhibitor-induced: Discontinue the offending drug.
- Idiopathic cases: May respond to antihistamines or require immunosuppressive therapy.
Complications
- Airway obstruction: The most serious complication, requiring urgent intervention.
- Recurrent attacks: Especially in HAE, may affect quality of life.
- Psychological stress: Due to unpredictability and impact on appearance and breathing.
Prognosis
The outcome depends on the type and cause of angioedema:
- Allergic forms are usually self-limiting and resolve with treatment.
- Hereditary angioedema requires lifelong management but can be well-controlled.
- Prompt treatment is key to preventing serious complications, especially in airway involvement.
Conclusion
Angioedema is a potentially serious condition involving rapid swelling under the skin or mucous membranes. Early recognition and treatment, especially in airway-involving cases, are vital to prevent fatal outcomes. Identifying and avoiding triggers, using appropriate medications, and educating patients about emergency action plans are crucial for long-term management and improving quality of life.
