Introduction
Cold urticaria (ColdU, Cold hives) is a type of chronic hives that significantly impacts your daily life, especially if you live in or visit cold environments. This guide will help you understand what ColdU is, its symptoms, how it’s diagnosed, and the available treatments to manage it effectively.
What is Cold Urticaria?
Cold urticaria is a condition where your skin reacts to cold temperatures by developing itchy, red welts or hives. In severe cases, it can lead to more serious reactions like swelling (angioedema) and even anaphylaxis, a life-threatening allergic reaction.
How Common is Cold Urticaria?
Cold urticaria is more common than you might think. Studies show that it affects about 7.62% of people with chronic urticaria and 26.10% of those with chronic inducible urticaria . This means that many people experience symptoms similar to yours when exposed to cold.
Recognizing the Symptoms
The symptoms of ColdU include:
- Red, Itchy Welts: These welts appear on skin exposed to cold air, liquids, or objects.
- Swelling: Areas affected by cold exposure can swell, causing discomfort.
- Whole-Body Reactions: In severe cases, you might experience difficulty breathing, fainting, or other systemic reactions.
These symptoms can significantly impact your quality of life, making it difficult to participate in activities that involve cold weather or cold water.
Hives from Cold Urticaria on arm.
Why Does Cold Urticaria Happen?
Mechanisms of ColdU
When your skin is exposed to cold, your body releases chemicals like histamine from cells called mast cells. This release causes the redness, itching, and swelling you experience. Researchers believe that this reaction involves both known and unknown allergens triggered by the cold.
Mast Cell Activation
Mast cell degranulation is a critical event in the pathophysiology of ColdU. Upon exposure to cold stimuli, these cells release histamine and other mediators, causing vasodilation, increased vascular permeability, and the formation of wheals. Research suggests that an unknown cutaneous autoallergen becomes accessible during cold stimulation, triggering this cascade.
Genetic and Immunologic Factors
ColdU may involve genetic predispositions and immunologic factors. Research indicates a potential link to cryopyrin-associated periodic syndromes (CAPS) in some patients . This implies that ColdU might not be just an isolated skin condition but part of a broader systemic issue in some individuals.
Getting a Diagnosis
Diagnostic Tools
Diagnosing ColdU is straightforward. Your doctor will likely perform an ice cube test, where an ice cube is placed on your skin for a few minutes to see if a reaction occurs. This test is reliable and helps confirm the diagnosis.
Critical Temperature and Stimulation Thresholds
Assessment of disease severity and treatment response in ColdU often involves measuring the critical temperature threshold (CTT) and critical stimulation time threshold (CSTT). These parameters help determine the lowest temperature and the shortest duration of cold exposure required to induce a wheal response. The use of validated instruments like the TempTest allows for accurate and reproducible measurements.
- CTT (Critical Temperature Threshold): The highest temperature at which a wheal forms. Lower CTT indicates more severe disease.
- CSTT (Critical Stimulation Time Threshold): The shortest duration of cold exposure needed to cause a wheal. Shorter CSTT means more sensitivity.
Treating Cold Urticaria
Treating ColdU involves a combination of medications, lifestyle adjustments, and preventive measures. The goal is to manage symptoms effectively and prevent severe reactions. Here, we will explore various treatment options in detail.
First-Line Treatment: Antihistamines
Nonsedating second-generation H1-antihistamines (nsAHs) are typically the first-line treatment for ColdU. These antihistamines block the effects of histamine, a chemical that your body releases during an allergic reaction.
- Common nsAHs: Cetirizine, loratadine, desloratadine, fexofenadine.
- Standard Dosage: For cetirizine, the typical dosage is 10 mg once daily. Other antihistamines have similar standard dosages:
- Cetirizine: 10 mg daily.
- Loratadine: 10 mg daily.
- Desloratadine: 5 mg daily.
- Fexofenadine: 180 mg daily.
Updosing Antihistamines
In many cases, standard doses of antihistamines might not be sufficient to control symptoms. Updosing, or taking higher-than-standard doses, has been found to be effective. For instance:
- Cetirizine: Can be increased to 20-40 mg per day.
- Loratadine: Up to 40 mg per day.
- Desloratadine: Up to 20 mg per day.
- Fexofenadine: Up to 360 mg per day.
- Efficacy: Updosing has been shown to improve critical temperature thresholds (CTTs) and critical stimulation time thresholds (CSTTs), providing better control of symptoms.
Omalizumab for More Severe Cases
If antihistamines alone do not provide sufficient relief, omalizumab, a monoclonal antibody, may be considered. Omalizumab works by binding to immunoglobulin E (IgE), a molecule involved in allergic reactions, thereby preventing it from triggering the release of histamine and other inflammatory mediators.
- Dosage: 150-300 mg every four weeks.
- Efficacy: Studies have shown that omalizumab significantly reduces symptoms in patients who do not respond to antihistamines. It improves both CTTs and CSTTs, making cold exposure more tolerable.
Mechanism of Action
Omalizumab targets free IgE in the blood, reducing its levels and subsequently decreasing the number of IgE receptors on mast cells. This reduction prevents the mast cells from becoming activated and releasing histamine, thereby reducing allergic symptoms.
Other Treatment Options
For patients who do not respond adequately to antihistamines and omalizumab, other treatment options may include:
Leukotriene Receptor Antagonists
Leukotriene receptor antagonists (LTRAs) such as montelukast can be used in combination with antihistamines to enhance symptom control. LTRAs work by blocking leukotrienes, another group of chemicals involved in the allergic response.
- Montelukast: 10 mg daily has been shown to reduce the severity of symptoms when added to antihistamines.
Cyclosporine
Cyclosporine is an immunosuppressant that has been used to treat severe cases of ColdU. It works by suppressing the immune system, reducing the activity of T cells and preventing the release of inflammatory mediators.
- Dosage: Typically 3-5 mg/kg/day.
- Efficacy: Cyclosporine can be effective in patients who do not respond to antihistamines or omalizumab, but it requires close monitoring due to potential side effects like kidney toxicity and hypertension.
Cold Desensitization Therapy
Cold desensitization involves gradually exposing the skin to colder temperatures over time to build up tolerance. This therapy should be done under medical supervision to avoid severe reactions.
- Procedure: Patients are exposed to progressively colder temperatures in a controlled environment.
- Efficacy: Can help reduce sensitivity to cold and decrease the frequency and severity of reactions.
Biologics and Other Immunosuppressants
In addition to omalizumab and cyclosporine, other biologics and immunosuppressants may be considered for refractory cases:
- Anakinra: An interleukin-1 receptor antagonist that can be effective in some patients with ColdU associated with CAPS.
- Methotrexate: An immunosuppressant that has been used off-label for chronic urticaria, including ColdU.
- Hydroxychloroquine: An anti-malarial drug that has shown some efficacy in chronic urticaria and may be considered for ColdU.
Monitoring and Follow-Up
Regular monitoring and follow-up with your healthcare provider are essential when managing ColdU. This helps ensure that treatments are effective and adjusted as needed. It also allows for the monitoring of potential side effects, especially when using immunosuppressants or biologics.
- Regular Check-Ups: Schedule regular appointments to assess the effectiveness of treatment and make any necessary adjustments.
- Side Effect Monitoring: Be vigilant for potential side effects, particularly with immunosuppressants like cyclosporine and methotrexate.
Managing and Preventing Symptoms
Everyday Precautions
Avoiding cold exposure is key to managing ColdU. Here are some tips:
- Dress Warmly: Wear layers of clothing when going outside in cold weather.
- Avoid Cold Foods and Beverages: These can trigger symptoms.
- Cautious with Cold Water Activities: Swimming in cold water can induce severe reactions.
Be Prepared for Emergencies
If you have a history of severe reactions, always carry an epinephrine auto-injector and know how to use it. Regular check-ups with your doctor are important to monitor your condition and adjust treatments as necessary.
Living with Cold Urticaria
Living with ColdU can be challenging, but with the right management strategies and treatments, you can reduce your symptoms and improve your quality of life. Stay informed about your condition, follow your treatment plan, and don’t hesitate to reach out to your healthcare provider with any concerns.
Lifestyle Adjustments
- Stay Informed: Keep up with the latest research and treatments.
- Follow Your Treatment Plan: Adherence to medication and lifestyle adjustments can significantly improve your symptoms.
- Regular Doctor Visits: Regular follow-ups help in adjusting treatments and managing the condition effectively.
Conclusion
Remember, you are not alone in this. Many people manage their ColdU effectively and continue to lead active, fulfilling lives. With the right information and support, you can take control of your condition and improve your quality of life.
References
- Maltseva, Natalya, et al. “Cold urticaria–What we know and what we do not know.” Allergy 76.4 (2021): 1077-1094.
- Kulthanan, Kanokvalai, et al. “Treatments of cold urticaria: a systematic review.” Journal of Allergy and Clinical Immunology 143.4 (2019): 1311-1331.
- Singleton, Reid, and Caroline P. Halverstam. “Diagnosis and management of cold urticaria.” Cutis 97.1 (2016): 59-62.
- Prosty, Connor, et al. “Prevalence, management, and anaphylaxis risk of cold urticaria: a systematic review and meta-analysis.” The Journal of Allergy and Clinical Immunology: In Practice 10.2 (2022): 586-596.
