Is fever a typical symptom of angioedema?

Is Fever a Typical Symptom of Angioedema?

Fever is NOT a typical symptom of angioedema, and its presence should prompt evaluation for alternative diagnoses, particularly autoinflammatory syndromes or urticarial vasculitis. 1

Key Clinical Distinctions

Standard Angioedema Presentations (Without Fever)

• Hereditary angioedema (HAE) presents with discrete episodes of nonpruritic, nonpitting swelling without fever, affecting extremities, abdomen, face, oropharynx, or larynx 1
• ACE inhibitor-induced angioedema causes asymmetric nondependent swelling that is generally not pruritic, without systemic symptoms like fever 1
• Histaminergic angioedema (the most common form) occurs with or without urticaria but does not typically present with fever 1, 2

When Fever IS Present: Consider Alternative Diagnoses

The presence of fever with urticaria-like lesions strongly suggests autoinflammatory syndromes rather than typical angioedema. 1

• Autoinflammatory syndromes characteristically present with spontaneous wheals, pyrexia (fever), and malaise 1
• These include:
  • Schnitzler syndrome (acquired) 1
  • Cryopyrin-associated periodic syndromes (CAPS) including Muckle-Wells syndrome (hereditary) 1
  • Adult-onset Still disease and systemic-onset juvenile idiopathic arthritis 1

Diagnostic Approach When Fever Is Present

• Test for elevated inflammatory markers (C-reactive protein and erythrocyte sedimentation rate) 1
• Test for paraproteinemia in adults 1
• Look for neutrophil-rich infiltrates on skin biopsy 1
• Perform gene mutation analysis for hereditary periodic fever syndromes if strongly suspected 1

Clinical Pitfall to Avoid

Do not assume fever is part of typical angioedema. The combination of recurrent swelling with fever should redirect your diagnostic workup away from standard angioedema causes (HAE, ACE inhibitor-induced, histaminergic) toward autoinflammatory conditions that require entirely different management strategies. 1