5 Questions About Cold-induced Hives
Who gets them and why? We'll tell you everything we know about this unusual skin condition.
Ever since she was a young girl Charlotte Levins has loved to swim. But when she developed cold-induced chronic hives three years ago at age 23, the pool suddenly was out of bounds. “I’d had hives a few times before, but they’d always gone away within a couple of days,” Levins says. “This time, they came and went over several weeks.”
She didn’t know it at the time, but her hives—itchy, red, and swollen welts on the skin—were triggered by swimming in cold water. Cold-induced hives (also known as cold urticaria) is a chronic condition, meaning there’s no cure. Most people experience recurrent episodes when their skin is exposed to cold objects, like cold water or ice, or simply from being in a cold environment, be it a freezing outdoor climate or really chilly indoor air-conditioning. “The cold temperature in contact with the skin causes the release of histamine inside the body, which leads to the hives,” says Tanya Nino, M.D., a dermatologist with St. Joseph Hospital in Orange County, CA.
It wasn’t until she’d gone swimming again—and checked in with her doctor—that Charlotte was finally diagnosed with cold urticaria. “I’d never heard of it,” she admits.
Not so surprising, considering it’s an extremely rare condition. The exact number of people with cold urticaria is unknown, but the incidence is thought to be around 0.05% of the total population, according to a review in The Journal of Allergy and Clinical Immunology. To give that figure some context, regular hives (known as plain old urticaria) affects about 20% of people at some point in their lives, according to the American College of Allergy, Asthma and Immunology.
So how do you know, exactly, if you have symptoms of cold urticaria or just run-of-the-mill hives? And, more important, why do you get them and how can you prevent and/or treat your next attack?
What Are the Types of Cold-Induced Hives?
According to the National Organization for Rare Disorders (NORD), there are two types of cold-triggered hives. They are:
With the essential kind, symptoms become obvious immediately after exposure to an external cold trigger, and they tend to last for only a couple of hours. “Usually, people with cold-induced chronic hives will notice raised welts after even just a few minutes of touching frigid air, liquid, or items.” says Milna Rufin, M.D. in internal medicine at NYU Langone Health in New York City.
With familial cold-induced urticaria, it can take up to 48 hours for symptoms to appear. But when they do, your hives typically linger for much longer—sometimes for days.
What Are the Symptoms of This Condition?
If you have either type of cold urticaria, you might experience itching and reddening of the skin, followed by a burning sensation and the appearance of hives. Charlotte, who was told she has the essential type of cold-induced hives, says hers look puffy and raised, and are pink with sharp borders.
“Hives can take multiple shapes and sizes,” Dr. Rufin says. “Cold-induced hives usually appear within a few minutes of cold exposure and can sometimes wax and wane. The amount of time they are present can really vary, and can last hours to days.”
Cold-induced hives are typically found only on the parts of the body that are exposed to the external cold trigger—so they might appear on your hand if you touch ice, or on your face if you’re out in very cold weather. “Sometimes, if people have extended cold exposure, like when swimming in icy waters, you can see hives develop all over,” adds Dr. Rufin.
According to the Genetic and Rare Diseases Information Center (GARD), such reactions normally first occur in early adulthood, although the condition can present at any age, and is more common in females than males.
Less common symptoms of acquired or familial cold urticaria include shortness of breath, wheezing, abdominal pain, rapid heartbeat (palpitations), low blood pressure, or even anaphylactic shock. The severity of the symptoms depends on the amount of exposure. “Swimming in a cold lake is more likely to trigger an anaphylactic response than holding an ice cube,” Dr. Nino says. If you feel your mouth, lips, tongue, or throat swelling, or are having trouble breathing, seek emergency medical care right away. NORD also lists fever, headache, anxiety, tiredness, and fainting as possible symptoms of cold urticaria.
So, What Causes Cold-Induced Hives?
There’s no single cause of essential cold-induced hives. However, the familial form of the condition can occur when an abnormal gene (known as CIAS1/NLRP3) is present. Such a mutation triggers increased activity of a protein complex known as the inflammasome, whose job is to regulate inflammation in the body. Basically, an uptick in inflammasome activity causes an increased release of a protein called interleukin (IL) 1B, which then lead to symptoms of inflammation.
According to NORD, this abnormal gene can be inherited from either parent, or can be the result of a new gene mutation caused by an environmental factor, such as exposure to ultraviolet radiation. The risk of passing this abnormal gene is 50% for each pregnancy, and the risk of getting it is the same for males and females.
In some people, cold urticaria (whether acquired or familial) is thought to be triggered by an underlying health concern affecting the immune system, such as a viral infection or cancer. This is connected to the body’s immune response and inflammatory cells—basically, the immune system is working on overdrive already, going after “threats” that aren’t truly threats, like cold water. “All the immune cells work together and recruit each other , including the mast cells which release histamine,” Dr. Nino explains.
If your doctor suspects that your cold urticaria is triggered by an underlying health issue, they will probably carry out additional tests, including blood tests to check for antibodies to a specific viral infection, or cancer cells, proteins or other substances created by cancer.
How, Then, Are They Diagnosed?
To determine whether or not cold is triggering your hives, your doctor might perform what’s called a cold-stimulation test, per guidelines published in Allergy in 2018. This involves placing a cold object, like an ice cube or an ice pack, against your skin for a few minutes. (Not everyone gets this test. “My doctor said it was clear from my symptoms alone that I had cold urticaria,” Charlotte says.)
If you have cold urticaria, a red, swollen rash will develop on your skin where it came into contact with the cold object within 10 minutes after the object is removed. NORD details what’s actually going on here: While exposure to cold is required for this condition, the hive actually forms on the skin as your body’s temperature rises—after the cold object is taken away.
Can Cold-Induced Hives Be Cured?
Because cold urticaria is a chronic condition, there is no cure. If you’re lucky, it might go away on its own after a few weeks or months. Otherwise, a range of treatment and preventive steps might help. Unsurprisingly, avoiding cold temperatures and anything that might cause a rapid drop in body temperature is one of the most important measures to take.
“We generally recommend that people with cold-induced urticaria should avoid the cold, but for some people, that may be totally impractical,” says Dr. Rufin before adding: “If you are in the hospital, it’s very helpful to tell your doctors that you have cold-induced chronic hives, so that your providers can warm any medications or treatments before it touches your skin.”
Over-the-counter antihistamines, like Clarinex (desloratadine), Claritin (loratadine), and Zyrtec (cetirizine) can help in mild cases of cold urticaria, Dr. Rufin says. In more serious cases, a prescription med might be needed. Xolair (omalizumab), which is normally prescribed to treat asthma, has been used successfully to treat people with cold urticaria who didn't respond to other medications. And if you’ve had a severe systemic reaction to the cold—such as anaphylactic shock—Dr. Nino and Dr. Rufin recommend talking to your doctor about carrying an epinephrine pen with you, especially during the winter if you live in a cold climate.
Charlotte is proof that if you make certain changes—and follow your doctor’s advice—you don’t have to let cold urticaria control your whole life, or prevent you from doing your favorite cool activities. “Cold-water swimming is out, but I get my swim fix in a heated indoor pool,” she says. “I take an antihistamine before I get in the water, which reduces the redness and swelling. And if we’re having a cold weather spell, I stay home—and warm—as much as possible.”
- Incidence of Cold Urticaria (1): The Journal of Allergy and Clinical Immunology: In Practice. (2018). “How to Approach Chronic Inducible Urticaria.” sciencedirect.com/science/article/abs/pii/S2213219818302654
- Incidence of Cold Urticaria (2): American College of Allergy, Asthma & Immunology. (2018). “Hives (Urticaria.)” acaai.org/allergies/types-allergies/hives-urticaria
- Cold Urticaria Subtypes: National Organization for Rare Disorders. (n.d.). “Urticaria, Cold.” rarediseases.org/rare-diseases/urticaria-cold/
- Onset of CU Symptoms: Genetic and Rare Diseases Information Center. (2020). “Cold Urticaria.” rarediseases.info.nih.gov/diseases/6131/cold-urticaria
- Diagnosis: Allergy. (2018). “The EAACI/GA²LEN/EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria.” onlinelibrary.wiley.com/doi/full/10.1111/all.13397
- Familial Cold Urticaria: National Organization for Rare Disorders. (n.d.). “Familial Cold Autoinflammatory Syndrome.” rarediseases.org/rare-diseases/familial-cold-autoinflammatory-syndrome/