What Is Cold Rash (Cold Urticaria)?

Cold rash—medically coined cold urticaria (hives)—is a rare disorder involving an immune response to cold exposure. When a person has cold urticaria, raised wheals or bumps called hives appear if the skin is exposed to cold temperatures.

Cold rash comprises approximately 3% of all cases of urticaria (hives). It is more prevalent in younger adults than in the elderly population and affects women and men equally. Treatment often includes employing preventative measures, such as avoiding exposure to cold (including cold air, icy cold drinks or food, and cold water). 

Types of Cold Rash

Cold rash can include two different forms, including:

• An acquired (essential) form
• A hereditary (familial) form

Acquired (Essential Form)

Acquired (essential) form of cold rash involves symptoms that become obvious within two to five minutes of exposure to the cold. Symptoms last approximately one to two hours in a person with the essential form of cold urticaria.

Acquired cold urticaria is said to have several subcategories including:

• Primary acquired urticaria: Symptoms may begin in 5 to 30 minutes after cold exposure; symptoms usually start when the exposed skin is rewarming, but they sometimes begin beforehand. Itching and reddening may be the initial symptoms, followed by a burning sensation. Hives usually last around 30 minutes, other symptoms may include headaches, wheezing, heart palpitations, or fainting.
• Delayed cold urticaria: Symptoms may not start until several hours after a person is exposed to the cold.
• Localized cold urticaria: A reaction to cold exposure that occurs in areas of the skin where bug bites have previously occurred or where injections for allergies have been administered (specifically, injections of ragweed to desensitize a person with a ragweed allergy).
• Reflex cold urticaria: A bodily response involving a widespread outbreak of hives that occur as a response to a localized area of cold application (such as applying an ice pack). 

Hereditary (Familial) Form

Hereditary (familial) form of cold rash involves symptoms that:

• Usually take approximately 24 to 48 hours to appear
• Last longer (usually around 24 hours, up to 48 hours) than symptoms last in the acquired, essential form of cold urticaria.

Cold Rash Symptoms

Common symptoms of cold rash include:

• An abnormal reaction of the skin in the area exposed to cold (including cold water, ice application, cold food, icy drinks, or cold weather)
• Reddened skin (erythema)
• Pruritic (itchy) hives (welts or wheals) with or without angioedema (swelling of the tissue)
• Itching
• Fever
• Headache
• Anxiety
• Fatigue
• Arthralgia (joint pain)
• Leukocytosis (an elevation in white blood cells)
• Uncommon symptoms may include: fainting, heart palpitations, wheezing

Severe Symptoms

A severe reaction is possible and usually occurs when a person has full skin exposure (such as immersion in cold water).

Severe, systemic symptoms may include:

• Swelling of the tongue and throat
• Difficulty breathing
• Anaphylaxis (which may include fainting; fast heart rate; swelling of the arms, legs, or torso; and shock).

Severe reactions, such as anaphylaxis, are usually linked with full skin exposure, such as when swimming in cold water. 

Causes

Although the exact cause of cold rash is not completely known, scientists have some clues about possible underlying contributing factors.

When the body reacts with any type of hives (including cold rash) it’s due to a cellular process called degranulation, in which mast cells release histamine into the dermis (skin).

The cold temperature triggers the mast cells to release histamine and other chemicals into the blood; these chemicals (including histamine) are responsible for causing the symptoms of cold rash, including redness and itchiness, as well as a more severe and serious reaction called anaphylaxis.

Genetic Causes

Scientists have identified a genetic mutation that causes the immune system to respond with allergic symptoms, such as those that occur in cold rash.

Cold rash can either be caused by unknown reasons, or it may be a genetic, autosomal dominant disease. This means that a person only needs to inherit a single copy of the disease gene (from either the mother or the father).  Humans have 23 pairs of chromosomes (strands of DNA encoded with genes). The familial form of cold rash has been linked to chromosome number 1.

Autoimmune Disorders

Some forms of cold rash are also diseases of the autoimmune system.

Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.”

Underlying Diseases

In some instances, cold rash can be caused by an underlying condition that has an impact on the immune system, such as cancer, or an infection.

When this is the case, you will need treatment for the underlying cause, in addition to implementing preventative measures to prevent recurring outbreaks of urticaria. 

Risk factors

Although the exact cause of cold urticaria (cold rash) may not be well known, some risk factors make you more likely to have the condition, these include:

• Being a young adult: This applies to the primary acquired form of cold rash.
• Having another type of health problem: This could be cancer or hepatitis.
• Having inherited traits: This is related to the familial type of cold rash that causes painful hives and symptoms similar to the flu when exposed to the cold.

Diagnosis

A raised red welt (hive) that appears after an application of ice is placed on the skin for approximately five minutes is considered a positive response to having cold rash. The hive may appear in the shape of the ice cube within around 10 minutes after the ice is removed

Sometimes there is a false positive to the ice test because in some instances it takes longer than five minutes for cold exposure to result in a skin reaction.

Treatment

There is no cure for cold rash; treatment is primarily aimed at implementing preventative measures (such as avoiding cold exposure) and the use of antihistamines.

Medications used as a preventative measure to treat symptoms before cold exposure include:

• Loratadine (Claritin)
• Desloratadine (Clarinex)
• Epinephrine
• Cyproheptadine
• Cetirizine (Zyrtec)

Omalizumab (Xolair) is an asthma medication that is sometimes used to treat cold rash when other medications are ineffective.

Reports show that inducing tolerance by repeat exposure to cold has had variable results, but this treatment should only be done under close medical supervision (such as an inpatient hospitalization).

Prevention

Tips for preventing episodes of cold rash from recurring include:

• Taking over-the-counter (OTC) antihistamines before exposure to cold
• Protecting the skin from cold temperatures
• Avoiding cold drinks
• Carrying an EpiPen autoinjector to prevent serious reactions (such as anaphylaxis )

A Word From Verywell

At Verywell Health we know that having a condition such as cold rash is much more complex than simply breaking out in a rash. The adverse symptoms can be challenging or they could even cause serious life-threatening danger in certain circumstances.

Even though the symptoms may resolve on their own in a relatively short amount of time, it’s important to seek medical advice and consult with a healthcare professional about taking precautionary measures.