Cold urticaria is a rare condition that affects about 0.05% of people worldwide. It’s more common in areas with cold climates.
Cold urticaria is an uncommon form of chronic inducible urticaria (CIndU), an inflammatory skin condition that causes itchy wheals or hives, and possibly angioedema (an allergic reaction that causes swelling in the tissue under the skin or mucosal membranes). CIndU usually lasts more than 6 weeks and occurs from exposure to specific physical or environmental factors.
Some people with cold urticaria also have other types of CIndU, such as symptomatic dermographism that causes hives when you scratch or apply pressure to your skin.
Your doctor may conduct tests to learn whether you have cold urticaria and can help you learn how to reduce symptoms with medication and lifestyle changes.
Read on to learn more about this condition.
What causes cold urticaria?
If you have cold urticaria, exposure to cold causes mast cell degranulation. This happens when mast cells in your immune system quickly release histamine and other chemicals that trigger inflammation.
Scientists don’t know exactly why this happens. It’s possible that cold exposure may cause your body to produce autoantigens, which are molecules that your immune system mistakes as harmful. This may cause proteins known as antibodies to bind to mast cells and make them more sensitive to cold.
Some cases of cold urticaria might be linked to other health conditions, such as
- an autoimmune disease
- a lymphoproliferative disease
- a viral or bacterial infection
Your risk of cold urticaria increases if you have an allergic disease, such food allergy.
What are the symptoms of cold urticaria?
Cold urticaria causes itchy welts known as hives. These raised bumps on your skin may appear the same color as your skin, lighter or darker, redder or deeper-colored, or have other skin changes, depending on your skin tone.
If you are outside and then immediately go indoors, this change in temperature can cause cold urticaria.
Cold urticaria may also cause angioedema. After eating cold foods, drinking cold beverages, or holding something cold, you may develop swelling of your lips, tongue, or throat.
A 2022 review of studies indicated that just under 21.5% of people with cold urticaria experienced anaphylaxis.
Anaphylaxis is a life threatening systemic reaction that may cause symptoms such as:
- swelling of your lips, tongue, or throat
- abdominal pain, vomiting, or diarrhea
- difficulty breathing
- rapid heartbeat
- dizziness
- fainting
If you have cold urticaria, the risk of anaphylaxis is highest after large areas of your body have been exposed to cold, such as swimming in cold water. This can lead to:
- swelling over the entire body
- fainting
- signs of shock
- a racing heartbeat
Symptoms usually develop within 2 to 5 minutes following cold exposure and go away within 1 to 2 hours. Sometimes delayed symptoms occur several hours after cold exposure.
How is cold urticaria diagnosed?
If you develop itchy hives or other symptoms after cold exposure, let your doctor know. They will ask about your symptoms and medical history.
They may order tests, such as:
- cold stimulation testing, in which a healthcare professional places an ice cube against your skin for several minutes and checks for a hive 10 minutes after removing the ice
- threshold testing, which helps them learn how cold something needs to be or how long it needs to be exposed to your skin to trigger symptoms
- blood tests to rule out other health conditions that may cause similar symptoms
Your doctor may conduct the tests themselves or refer you to an allergist-immunologist or dermatologist for testing.
Treatment for cold urticaria
Treatment for cold urticaria involves of combination of lifestyle changes and medication.
Your doctor will recommend lifestyle changes to limit cold exposure.
For example, they may advise:
- limiting the time you spend in cold environments and wearing warm clothing to protect your skin there
- avoiding winter sports, swimming in cold water, and exploring cold areas
- avoiding eating cold foods and drinking cold beverages
Certain types of intravenous (IV) fluids or other injections may be refrigerated. Tell your doctor or other healthcare professionals that you have cold urticaria before you get IV fluids or injections, as they may need to warm up these types of medications.
Certain kinds of surgery and other medical procedures also involve cold temperatures. Tell your healthcare team about your condition before any medical procedures so they can take steps to limit symptoms.
To treat cold urticaria, your doctor will prescribe a second-generation H1 antihistamine, such as:
- cetirizine (Zyrtec)
- desloratadine (Clarinex)
- fexofenadine (Allegra, Aller-Ease)
- levocetirizine (Xyzal)
- loratadine (Alavert, Claritin, Wal-itin)
They will likely ask you to take the antihistamine every day to prevent urticaria symptoms. Your doctor may increase the antihistamine dose if you continue to have frequent symptoms or prescribe other medication, such as omalizumab (Xolair) or cyclosporine (Sandimmune).
If an antihistamine isn’t helping manage your symptoms, your doctor may suggest additional medication. This might involve:
- Phototherapy: This type of treatment, also called light therapy, is a noninvasive treatment that exposes your skin to light in a controlled environment. The way phototherapy treats hives symptoms is still being studied, but experts know it works on your immune system. This helps reduce itching and prevent new hives from appearing.
- Omalizumab: This is an FDA-approved type of medication known as a biologic that comes in the form of an injection. Your doctor will show you how to administer the injection yourself. Omalizumab (Xolair) helps calm your immune system, which stops your body from overreacting and creating hives. You might be prescribed this medication on its own or along with an antihistamine.
- Dupilumab: Dupilumab (Dupixent) is an FDA-approved biologic medication that helps manage the inflammation that leads to hives. It can help lower the severity of itchiness and the number of hives. You might be prescribed dupilumab on its own or with an antihistamine. Your doctor will show you how to inject yourself with the medication so you can do this at home.
Your doctor might prescribe epinephrine if you’ve developed anaphylaxis. It’s important to always carry prescribed epinephrine with you and take it at the first sign of anaphylaxis.
When to seek emergency care
Get emergency medical treatment if you develop swelling in your tongue or throat or signs of anaphylaxis.
In addition to hives and skin swelling, anaphylaxis may involve any of the following:
- swelling of your lips, tongue, or throat
- respiratory symptoms, such as:
- runny nose
- wheezing
- hoarse voice
- shortness of breath
- difficulty breathing
- gastrointestinal symptoms, such as:
- abdominal pain
- nausea
- vomiting
- diarrhea
- heart or neurological symptoms, such as:
- rapid heartbeat
- low blood pressure
- headache
- dizziness
- confusion
- fainting
- uterine cramps
Take epinephrine if it’s available to treat anaphylaxis. Multiple doses of epinephrine and hospital treatment are sometimes needed.
Outlook for cold urticaria
Cold urticaria often goes away within 6 years, although some people may have it for 20 years or longer. For others, it may become a long-term condition.
Limiting your exposure to cold and taking medication that your doctor prescribes may help reduce cold urticaria symptoms and improve your quality of life.
Talk with your doctor if you develop itchy hives, skin swelling, or other symptoms after contact with cold temperatures.
They may order a cold stimulation test to determine whether you have cold urticaria. They may also request other tests to assess your condition.
Your doctor will likely encourage you to avoid exposure to the following to help limit symptoms of cold urticaria:
- cold air
- cold water
- cold foods and beverages
They may prescribe a second-generation antihistamine, omalizumab, or cyclosporine to help manage your condition. If you have a history of anaphylaxis, you might be prescribed epinephrine.
Get emergency care if you develop swelling of your tongue or throat or signs of anaphylaxis, which is a life threatening systemic reaction. Anaphylaxis requires epinephrine and sometimes hospitalization to treat.