What is the recommended treatment for a patient with cold urticaria?

Treatment for Cold Urticaria

Start with a second-generation H1-antihistamine at standard dosing and escalate up to 4 times the standard dose if needed; cyproheptadine is specifically FDA-approved for cold urticaria and should be considered when second-generation agents fail. 1, 2, 3

First-Line Treatment: Second-Generation Antihistamines

• Begin with a single second-generation H1-antihistamine (cetirizine, fexofenadine, desloratadine, levocetirizine, or loratadine) at standard dosing for 2-4 weeks 1, 2
• Cetirizine is preferred when rapid symptom relief is needed due to its shortest time to maximum concentration 1, 4
• Over 40% of patients with urticaria show good response to antihistamines alone 1
• If one agent is ineffective, trial at least two different second-generation antihistamines, as individual responses vary significantly 2, 4

Dose Escalation Strategy

• If standard dosing provides inadequate control after 2-4 weeks, increase the antihistamine dose up to 4 times the standard dose 1, 2, 4
• This dose escalation is recommended when potential benefits outweigh risks 2

FDA-Approved Option: Cyproheptadine

• Cyproheptadine is specifically FDA-approved for cold urticaria and should be considered as an alternative or adjunct, particularly when second-generation agents are insufficient 3
• This first-generation antihistamine has documented efficacy in cold urticaria, though it causes more sedation than newer agents 3, 5

Adjunctive Second-Line Therapies

• Add H2-antihistamines (famotidine or ranitidine) for resistant cases 1
• Consider adding leukotriene receptor antagonists (montelukast), particularly beneficial for aspirin-sensitive and autoimmune urticaria 1
• First-generation antihistamines like hydroxyzine can be added at night for additional symptom control and to aid sleep 2, 5
• The combination of hydroxyzine plus cimetidine (H2-antagonist) showed statistical significance in suppressing erythema in cold urticaria 5

Third-Line Treatment for Severe Refractory Cases

• Omalizumab 300 mg subcutaneously every 4 weeks is recommended for severe antihistamine-resistant chronic urticaria, with up to 6 months allowed for response 1, 2, 4
• Resolution of cold urticaria has been documented in a patient treated with omalizumab 6
• Cyclosporine 4 mg/kg daily for up to 2 months is effective in approximately two-thirds of patients with severe autoimmune urticaria unresponsive to antihistamines 6, 1, 2, 4

Corticosteroids: Limited Role

• Use short courses of oral corticosteroids (prednisolone 50 mg daily for 3 days in adults, maximum 3-10 days) only for severe acute urticaria or life-threatening angioedema 1
• Restrict corticosteroid courses to 3-4 weeks maximum 1
• Avoid long-term corticosteroid use except in very selected cases under specialist supervision due to cumulative toxicity 1

Emergency Management for Life-Threatening Reactions

• Administer intramuscular epinephrine 0.5 mL of 1:1000 (500 µg) immediately for anaphylaxis or severe laryngeal angioedema 1, 2
• Fixed-dose epinephrine auto-injectors (300 µg for adults) should be prescribed for patients at risk of life-threatening attacks, particularly those with history of anaphylaxis or who engage in aquatic activities 2, 7, 8
• Cold water immersion poses the highest risk for systemic reactions and anaphylaxis in cold urticaria patients 7

Critical Avoidance Measures

• Avoidance of cold exposure is the most effective prophylactic measure 9, 8
• Avoid aspirin and NSAIDs, as they inhibit cyclooxygenase and can exacerbate urticaria symptoms 1, 2, 4
• Minimize non-specific aggravating factors including overheating, stress, and alcohol 1, 2, 4
• Patients should be counseled to avoid swimming in cold water due to risk of systemic reactions 7, 8

Symptomatic Relief Measures

• Apply cooling antipruritic lotions (calamine or 1% menthol in aqueous cream) for symptomatic relief 2, 4
• Use emollients regularly for any associated dry skin 4

Monitoring and Follow-Up

• Regularly assess disease activity, impact on quality of life, and treatment response 1
• Consider stepping down therapy after achieving complete control for at least 3 consecutive months 1
• The primary goal in mild to moderate cases is to improve quality of life 9