Angioedema Summary

Definitions

  • Urticaria: Superficial swelling with itching
  • Angioedema: Deep swelling without itching
  • Common sites: Face, genitalia, hands, feet

Emergency Signs

  • Airway involvement (stridor)
  • Bowel involvement (abdominal pain)
  • Progressive symptoms

Classification

  • Allergic (90%): IgE-mediated, with urticaria
  • Non-allergic: Bradykinin-mediated
  • Drug-induced: ACE inhibitors most common
  • Hereditary (HAE): C1-esterase inhibitor deficiency

Allergic Treatment

  • IV/IM chlorphenamine + hydrocortisone
  • Non-sedating antihistamines
  • Prednisolone for severe cases

HAE Treatment

  • Resistant to standard allergy treatments
  • Icatibant (SC)
  • C1 esterase inhibitor (IV)
  • Fresh frozen plasma

Key Causes

  • ACE inhibitors (#1 drug cause)
  • Food allergens
  • Insect stings
  • Latex
  • Idiopathic (unknown cause)

Differential Diagnosis

  • Anaphylaxis
  • Cellulitis/erysipelas
  • Lymphoedema
  • SLE
  • Contact dermatitis

Angioedema

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Definitions

Urticaria

  • Superficial swelling (epidermis/mucous membranes)
  • Red, raised, intensely itchy rash with pale center

Angioedema

  • Deeper swelling (dermal, subcutaneous, submucosal)
  • Commonly affects face (lips, tongue, eyelids), genitalia, hands, feet
  • Often occurs with urticaria but may be isolated

Clinical Features

  • Common sites: Face, genitalia, hands, feet
  • Bowel involvement:
    • Abdominal pain, nausea/vomiting
    • Distension, diarrhea/constipation
  • Airway involvement:
    • Stridor → potential complete obstruction
    • Medical emergency

Classification

Acute Allergic Angioedema (90%)

  • IgE-mediated mast cell degranulation
  • Usually with urticaria within 1-2h of allergen exposure
  • Self-limiting (1-3 days) but relapses common

Non-Allergic (Bradykinin-Mediated)

  • Drug-induced:
    • ACE inhibitors most common
    • Days-months after starting drug
    • May persist months after stopping
  • Hereditary Angioedema (HAE):
    • Autosomal dominant C1-esterase inhibitor deficiency
    • Presents post-puberty with recurrent episodes
    • GI symptoms may mimic acute abdomen
  • Acquired Angioedema (AAE):
    • Secondary to lymphoma or connective tissue disorders
    • Similar presentation to HAE

Idiopathic Angioedema

  • Chronic, relapsing with unknown cause
  • Associated with autoimmune disorders (SLE, thyroiditis)

Diagnosis

With Urticaria

  • Allergic: Transient, within 1-2h of known allergen
  • Idiopathic: Recurrent with no identifiable cause

Without Urticaria

  • Drug reaction: ACE inhibitor use
  • HAE: Family history
  • AAE: Known lymphoma/connective tissue disorder

Differential Diagnosis

  • Evolving anaphylaxis
  • Cellulitis/erysipelas
  • Lymphoedema
  • SLE
  • Contact dermatitis

Emergency Department Management

Initial Assessment

  • ABC approach - assess for life-threatening conditions
  • If anaphylaxis suspected → treat as anaphylaxis

Allergic Angioedema

  • Rapidly developing (non-anaphylactic):
    • IV/IM chlorphenamine + hydrocortisone
    • Emergency admission
  • Stable cases:
    • Identify/avoid triggers
    • Mild cases may not need treatment
    • Symptomatic treatment:
      • Non-sedating antihistamine (cetirizine/fexofenadine/loratadine) for ≤6 weeks
      • Severe cases: Add prednisolone 40mg daily ≤7 days
    • Advise emergency care if symptoms progress

Hereditary Angioedema (HAE)

  • Key points:
    • Bradykinin-mediated → resistant to adrenaline/antihistamines/steroids
    • Requires specific treatment
  • Treatment options:
    • Icatibant (bradykinin B2 receptor antagonist) - SC injection
    • C1 esterase inhibitor concentrate (IV)
    • Fresh frozen plasma (2 units IV)

Clinical Comparison

Feature Allergic Non-Allergic Anaphylaxis
Onset Acute Gradual Rapid
Skin Urticaria + pruritus No pruritus Variable
Systemic Localized, normotensive Localized, normotensive Respiratory/cardiovascular collapse
Other - Abdominal pain, previous episodes Progressive symptoms

Key Clinical Pearls

  • ACE inhibitors are the most common cause of drug-induced angioedema
  • HAE often presents with recurrent abdominal pain episodes
  • Airway involvement requires immediate intervention
  • Bradykinin-mediated angioedema won't respond to standard allergy treatments