Cold Urticaria

Cold urticaria is a rare condition where exposure to cold temperatures triggers an allergic-like reaction on the skin, causing red, itchy hives or welts that can appear within minutes and may sometimes lead to serious complications.

Cold hives, Cold-induced urticaria, Essential cold urticaria, Acquired cold urticaria, Familial cold urticaria, Hereditary cold urticaria

Table of contents

• What is cold urticaria?
• Types of cold urticaria
• Who can get cold urticaria?
• Symptoms
• What causes cold urticaria?
• How is cold urticaria diagnosed?
• Treatment options
• Prevention and management tips
• What to expect over time

What is cold urticaria?

Cold urticaria is a condition that causes your body to have an allergic reaction when your skin is exposed to cold temperatures^[1]. The word urticaria is the medical term for hives, which are raised, itchy bumps on the skin. This reaction can happen after your skin touches something cold, such as ice, cold water, or cold air^[2].

Cold urticaria is a rare disorder^[1]. It affects about 0.05% of the population, meaning roughly 1 in every 2,000 people^[12]. The condition is more common in areas with colder climates^[8].

Types of cold urticaria

There are two main types of cold urticaria, and they differ in how quickly symptoms appear and how long they last^[1].

Acquired cold urticaria (also called essential cold urticaria) is the most common type. It occurs in people with no family history of the disease. Symptoms usually appear a few minutes after exposure to cold, typically within two to five minutes. The symptoms tend to go away within an hour or two^[1][4].

Familial cold urticaria (also called hereditary cold urticaria) is passed down in families through genes. This type is especially rare^[1]. Symptoms may take much longer to appear after exposure to cold, anywhere from 30 minutes to 48 hours. The symptoms can also last much longer, from one day up to two days^[1][4].

Who can get cold urticaria?

Cold urticaria can affect anyone at any age^[1]. The condition occurs most often in young adults, typically those between 18 and 30 years of age^[2][8]. However, it can also develop in children as young as 5 years old or even in elderly people^[4][8].

The condition affects females more than males^[4][12]. Although most cases occur in people with no family history of the disease, some people inherit the condition from their parents^[1].

Cold urticaria is sometimes associated with other health conditions. These may include^[1]:

• Chronic lymphocytic leukemia, a type of blood cancer
• Cryoglobulinemia, a blood disorder involving reaction to cold
• Lymphosarcoma, cancer in the lymphatic system
• Other types of hives or urticaria
• Syphilis, a sexually transmitted disease
• Viral infections such as mononucleosis or chickenpox

Symptoms

The symptoms of cold urticaria can be very different from person to person. They can be mild or severe and may affect just a small area of your skin or your entire body^[1].

The main symptom is a skin rash that appears after exposure to something cold. The rash generally occurs after the cold exposure is over, as your skin’s temperature rises and starts to warm up again^[1]. Cold urticaria symptoms begin soon after the skin is exposed to a sudden drop in air temperature or cold water. Damp and windy conditions may cause symptoms to get worse^[2].

The rash may include^[1]:

• Hives, bumps, or welts on the skin
• Itchiness
• Redness
• Swelling

Other symptoms that may occur include^[1]:

• Fatigue
• Fever
• Headache or lightheadedness
• Joint pain
• Burning feeling in the affected area

Specific symptoms can also occur depending on what type of cold exposure happens. Swelling of the hands may occur while holding cold objects. Swelling of the lips may happen from eating cold food or drinking cold beverages^[2].

In very rare, severe cases, a person may experience a systemic reaction, which means the entire body is affected. This can include^[1][2]:

• Fainting
• Heart palpitations or rapid, irregular heartbeat
• Anaphylaxis, which is a massive allergic reaction that can be life-threatening
• Shortness of breath or wheezing
• Swelling of the tongue and throat that can make it hard to breathe
• Low blood pressure and shock

The worst reactions generally occur when all of the skin is exposed to cold, such as when swimming in cold water. These reactions could lead to fainting and drowning^[2]. Swimming in cold water is the most common cause of a severe cold urticaria reaction^[8].

Each episode of symptoms may last for about two hours^[2]. It is important to note that cold urticaria is not contagious and cannot spread from person to person^[1].

What causes cold urticaria?

Cold urticaria occurs when the body releases histamines in reaction to cold. Histamines are chemicals made by your immune system in response to something it sees as harmful, called an allergen^[1]. However, the exact reason why the body releases histamines in response to cold is unknown^[1][2].

You could have sensitive skin cells due to a virus or an illness^[6]. Some cases of cold urticaria may be a symptom of autoimmune disorders, which are conditions where your body’s immune system attacks healthy cells or tissue for unknown reasons^[1].

Known triggers that can cause symptoms include^[1][4]:

• Walking outdoors in cold weather
• Eating or drinking something cold
• Swimming or bathing in cold water
• Putting ice on your skin
• Physical exercise
• Stress and anxiety
• Exposure to direct sunlight
• Cold air from air conditioning

Some people may be more likely to get cold urticaria due to age, since it is more common in young adults. In rare cases, you may inherit the condition from a close family member. Having some medical conditions like hepatitis and certain types of cancer can also increase the chances of getting some form of cold urticaria^[4].

How is cold urticaria diagnosed?

If you have skin reactions after being exposed to the cold, you should see your healthcare professional. Even if the reactions are mild, your healthcare professional will want to rule out other conditions that may be causing the symptoms^[2].

A healthcare professional can usually diagnose cold urticaria with a quick test called the cold urticaria test or ice cube test^[1][8]. The provider places an ice cube on your skin, such as on your arm, for a few minutes, then removes it. If you develop a hive or rash several minutes later after the ice is removed and your skin rewarms for 5 to 10 minutes, that is considered a positive test^[1][8].

The test may be repeated at shorter intervals to find out the minimum time needed to trigger a reaction. A negative test means no hive develops after 5 to 10 minutes, and this has been described in unusual forms of cold urticaria^[8]. Sometimes a rash might not appear at the test site right away, and you may have to wait 20 to 30 minutes for a reaction^[4].

However, diagnosing familial urticaria may require exposure to cold air for a longer period of time^[1].

Your doctor may also ask you questions such as^[4]:

• When did you first see symptoms?
• Have you had a recent infection?
• Does any other family member have the same symptoms as you?
• Have you started any medication recently?
• Have you tried new foods?
• Have you been to a new place recently?
• Does anything relieve or worsen your symptoms?

Your healthcare provider might also recommend blood tests to identify any underlying disease or infection^[1]. In some cases, another condition such as an infection or cancer causes cold urticaria, and this condition affects your immune system. If your healthcare professional thinks you have another condition that could be causing cold urticaria, you may need other tests^[9].

Treatment options

In some people, cold urticaria goes away on its own after weeks or months. In others, it lasts longer^[9]. The average duration of this type of chronic urticaria is about 5 to 9 years, with 50% of cases improving within approximately 5 years^[8]. While there is no cure for the condition, treatment and taking steps to avoid cold urticaria can help^[9].

The most effective way to manage cold urticaria is to stay warm and avoid cold^[5]. Your healthcare professional may recommend that you try to keep symptoms from happening or reduce those symptoms using treatments^[9].

Medicines used to treat cold urticaria include^[9][12]:

Antihistamines: These medications block histamines in your body. Non-drowsy antihistamines are commonly used. If you know you are going to be exposed to cold, take an antihistamine ahead of time to help keep a reaction from happening. Examples include loratadine (Claritin, Alavert), cetirizine (Zyrtec Allergy, Zerviate), and desloratadine (Clarinex). Some of these medicines are available without a prescription, while others require prescriptions^[9]. Regular doses of these antihistamines may be helpful, and if needed, high doses can be used, such as cetirizine up to 40 mg daily^[12].

Omalizumab (Xolair): This prescription medicine, which is typically used for people with asthma, has been used successfully to treat cold urticaria that does not respond to other medicines^[9]. Studies have shown that omalizumab in addition to antihistamines may be helpful in difficult cases, initially at 150 mg every 4 weeks, but the dose can be doubled if there is not enough response^[12][15].

Other treatment options that have been used with varying degrees of success include^[12]:

• Cyproheptadine
• Doxepin
• Ketotifen
• Leukotriene antagonists
• Ciclosporin
• Systemic corticosteroids
• Dapsone
• Oral antibiotics
• Danazol, a synthetic hormone

Desensitization: This means exposing your body to increasingly cold temperatures, like cold showers that get colder over time. This approach should be done with a loved one nearby who can monitor reactions^[5]. Cautious induction of cold tolerance may be successful by gradually hardening the skin to cold conditions and then exposing the skin to it regularly^[12].

Epinephrine injection: If you have a history of severe responses or anaphylaxis, you should carry emergency epinephrine at all times^[9][12].

If you have cold urticaria because of another condition, you may need medicines or treatments for that condition as well^[9]. Antibiotics may be added if specialists suspect an infectious cause, but not for the cold urticaria directly^[5].

Prevention and management tips

Here are some strategies that can help you cope with cold urticaria:

Plan ahead for weather and environments: Check the weather forecast, especially the wind factor. Know the indoor temperature of your workplace and always have layers of clothing on hand. Scarves, gloves, thermal socks, and even pocket hand warmers can be very helpful^[19].

Stay warm inside and out: Avoid cold drinks and foods, even in summer. Bring your own meals if needed. Use a desk heater or ask to sit away from cold drafts. For breathing in cold air, wear a thermal mask or scarf to warm the air before it reaches your lungs^[19].

Protect yourself during activities: Patients with cold urticaria should learn to protect themselves from a rapid drop in body temperature. Water activities such as swimming and surfing should always be done under supervision^[12]. Avoid full-body immersion in cold water^[22].

Communicate your needs: Be clear about your condition with your colleagues and leadership. Asking for a warmer meeting space or avoiding certain duties is not complaining—it is advocating for your health^[19].

Build recovery time into your day: Cold exposure can take a toll. Give yourself space to rest, hydrate, and recover after any exposure, even if you have taken precautions^[19].

Track symptoms: Keeping a journal or using a health app can help you notice patterns and triggers. You might discover that certain temperatures, times of day, or environments affect you more than others^[19].

Notify all your doctors: Make sure you notify all of your doctors if you have the condition, especially if you have to schedule surgery. Since operating rooms are typically cold, some people with cold urticaria have reactions while under anesthesia^[5].

Be prepared for emergencies: Patients and their caregivers should be educated on recognizing anaphylaxis and proper use of epinephrine autoinjectors^[22].

What to expect over time

Cold urticaria can come back after treatment^[5]. The condition can last for varying lengths of time. Among patients studied, approximately 14% of adults had resolution of cold urticaria at 5 years, and 43% had resolution of the condition at 10 years^[8].

The number of patients experiencing anaphylaxis due to cold urticaria ranges broadly from 0% to 34% depending on the study. Fortunately, there were no reported deaths from cold-induced symptoms in the studies reviewed^[8].

Under-diagnosis or misdiagnosis of this type of urticaria could lead to a significant impact on the quality of life of patients^[8]. Getting a proper diagnosis from an allergy specialist can be a turning point, as it confirms what you are experiencing and opens the door to treatment options^[19].

If you have gotten rash or hives after being exposed to something cold, reach out to your healthcare provider for an exam to see if you need treatment for or protection from cold urticaria^[5]. Prompt referral to an allergist to discuss symptoms and possible treatment options may be necessary in cases that do not respond to initial treatments^[22].