What is the treatment for angioedema of the face?

Treatment for Angioedema to the Face

Immediately assess for airway compromise and determine the type of angioedema, as treatment differs fundamentally between histamine-mediated and bradykinin-mediated forms—using standard allergy treatments (epinephrine, antihistamines, corticosteroids) for bradykinin-mediated angioedema is ineffective and delays appropriate therapy. 1, 2

Immediate Airway Assessment

• Evaluate the airway first in any patient presenting with facial angioedema, looking specifically for oropharyngeal or laryngeal involvement 1
• Monitor for signs of impending airway closure: change in voice, loss of ability to swallow, stridor, or difficulty breathing 3, 1
• Patients with oropharyngeal or laryngeal involvement require observation in a facility capable of performing emergency intubation or cricothyrotomy 3, 1
• Consider elective intubation early if signs of airway compromise are developing, as waiting for complete obstruction significantly increases morbidity and mortality 1, 2

Determine Angioedema Type

Key Historical Features:

• Presence or absence of urticaria/hives: Bradykinin-mediated angioedema (hereditary angioedema, ACE-inhibitor induced) presents without urticaria, while histamine-mediated typically presents with urticaria 4, 5
• Medication history: Specifically ask about ACE inhibitors or ARBs—ACE-inhibitor angioedema can occur after years of stable use, not just in the first month 4
• Family history: Hereditary angioedema (HAE) follows an autosomal dominant pattern 5
• Response to prior treatments: Lack of response to antihistamines or epinephrine suggests bradykinin-mediated etiology 6, 5

Treatment Based on Angioedema Type

Histamine-Mediated Angioedema (with urticaria, pruritus, or allergic trigger)

• Epinephrine 0.3 mL of 0.1% solution (1:1000) intramuscularly for significant symptoms or any airway involvement 1
• Diphenhydramine 50 mg IV 1
• Methylprednisolone 125 mg IV 1
• H2 blockers: ranitidine 50 mg IV or famotidine 20 mg IV 1

Bradykinin-Mediated Angioedema

Hereditary Angioedema (HAE):

• First-line: Plasma-derived C1 inhibitor concentrate 1000-2000 U intravenously 3, 1, 2
• Alternative first-line: Icatibant 30 mg subcutaneously 1, 2, 7
• Ecallantide (plasma kallikrein inhibitor) is effective but must be administered by healthcare professionals due to anaphylaxis risk 3
• Do NOT use epinephrine, antihistamines, or corticosteroids—these are ineffective for HAE 3, 1, 2

ACE-Inhibitor Induced Angioedema:

• Immediately and permanently discontinue the ACE inhibitor 4, 2
• Consider icatibant 30 mg subcutaneously if available and symptoms are severe 1, 2
• Fresh frozen plasma (FFP) 10-15 mL/kg may be considered when bradykinin-targeted therapies are unavailable 1, 2
• Standard allergy treatments (epinephrine, antihistamines, corticosteroids) are NOT effective 4, 8
• Critical pitfall: Epinephrine use in ACE-inhibitor angioedema has caused myocardial ischemia and dysrhythmias without therapeutic benefit 8
• The propensity to swell can continue for up to 6 weeks after ACE inhibitor discontinuation 4

When Specific Therapies Are Unavailable (Resource-Limited Settings):

• Fresh frozen plasma 10-15 mL/kg for acute HAE attacks, though response is slower (median 4 hours) and carries transfusion reaction risk 4, 3, 1
• Tranexamic acid may be attempted for very early/mild attacks, though evidence is limited 4

Critical Management Considerations

Observation and Monitoring:

• All patients with facial angioedema involving the oropharynx or larynx require prolonged observation in a facility with airway management capabilities 3, 1
• Do not discharge patients with oropharyngeal involvement without adequate observation period 1
• Laryngeal attacks carry historical mortality rates of approximately 30% and are potentially life-threatening 3, 1

Common Pitfalls to Avoid:

• Delaying epinephrine in true histamine-mediated angioedema with airway compromise 1
• Using epinephrine, antihistamines, or corticosteroids for bradykinin-mediated angioedema—this is the most common error and delays appropriate therapy 3, 1, 2, 8
• Failing to permanently discontinue ACE inhibitors in patients with ACE-inhibitor induced angioedema 4, 2
• Switching ACE-inhibitor angioedema patients to ARBs carries a modest recurrence risk (2-17%), though most tolerate ARBs without recurrence 4, 2

Special Populations:

• Pregnant patients with HAE: C1-inhibitor concentrate is the only recommended treatment for both acute attacks and prophylaxis 1
• African American patients have substantially higher risk of ACE-inhibitor induced angioedema compared to white patients 4