How should cold‑induced urticaria presenting with itchy hives on cold exposure be managed?

Management of Cold-Induced Urticaria

For cold-induced urticaria presenting with intensely itchy hives on cold exposure, prescribe non-sedating H1-antihistamines as first-line treatment, ensure the patient carries self-injectable epinephrine due to anaphylaxis risk, and implement strict cold avoidance measures including avoiding cold water immersion. 1, 2, 3

Diagnostic Confirmation

Before initiating treatment, confirm the diagnosis through:

• Cold stimulation test (ice cube test): Apply an ice cube to the forearm for 5-10 minutes and observe for wheal development upon rewarming 1
• Detailed history: Document specific cold triggers (swimming, cold air, cold beverages, weather changes) and timing of symptom onset 2, 3
• Rule out cryopyrin-associated periodic syndromes (CAPS): A negative ice cube test in the presence of cold-induced symptoms suggests CAPS rather than typical cold urticaria, which requires different management 1

Important caveat: Atypical cold urticaria can present with negative cold stimulation tests yet still cause severe reactions including anaphylaxis with cold water exposure—diagnosis relies on clinical history in these cases 4

First-Line Pharmacologic Management

Non-sedating H1-antihistamines are the cornerstone of treatment 1, 2, 5:

• Start with standard doses of second-generation antihistamines (cetirizine, loratadine, fexofenadine, desloratadine) 1, 5
• If inadequate response, increase to up to 4 times the standard dose before adding other agents 1
• Continue daily prophylactic dosing rather than as-needed use for patients with frequent cold exposure 2, 5

Adjunctive Therapies for Refractory Cases

When H1-antihistamines alone are insufficient 1, 5:

• Add H2-antihistamines (though benefits are unclear, some patients respond) 5
• Consider leukotriene antagonists for a small subset of resistant cases 5
• Short courses of corticosteroids only for acute severe exacerbations, not for chronic management 5
• Cyclosporine or tacrolimus reserved for chronic cases completely resistant to antihistamine therapy 5

Critical Safety Measures

Anaphylaxis prevention is paramount because cold-induced urticaria carries significant risk of life-threatening reactions, particularly with whole-body cold exposure 6, 4, 2, 3:

• Prescribe self-injectable epinephrine (EpiPen or equivalent) to all patients—this is non-negotiable 2, 3
• Educate patients to recognize early warning signs: pruritus, cutaneous warmth, flushing, or initial wheals 1
• Instruct immediate cessation of cold exposure at first symptoms 1, 2
• Warn against swimming alone or in unsupervised settings 6, 4, 3

Specific Avoidance Strategies

Implement comprehensive cold avoidance 2, 3:

• Avoid swimming in cold water or sudden immersion (highest anaphylaxis risk) 6, 4, 3
• Wear protective clothing in cold weather (gloves, scarves, face protection) 2
• Avoid cold beverages and foods 6, 2
• Pre-medicate with antihistamines before unavoidable cold exposure 2
• Gradual acclimatization to cold rather than sudden exposure 2

Monitoring and Follow-Up

• Assess treatment efficacy at each visit by reviewing frequency and severity of reactions 1
• Monitor for development of systemic symptoms suggesting progression to anaphylaxis 3
• Screen for secondary causes if presentation is atypical: check for cryoglobulinemia, underlying infections, or systemic diseases 2, 5
• In children, most cases are idiopathic and may spontaneously resolve, but maintain vigilance for severe reactions 5

Common pitfall: Prophylactic antihistamines may reduce but not completely prevent reactions—patients must understand that medication does not eliminate anaphylaxis risk and cold avoidance remains essential 4, 2