Cold urticaria

Cold urticaria
Synonyms	Cold hives
Pronounce
Field	Dermatology
Symptoms	Itchy hives, swelling of hands, feet, or face, angioedema, wheezing
Complications	Anaphylaxis, pharyngeal edema, drowning, hypothermia
Onset	Usually in childhood or early adulthood
Duration	Variable; may last months to years
Types	Primary, secondary, reflex, familial
Causes	Exposure to cold stimulus (air, water, food, objects)
Risks	Swimming, cold climates, ingestion of cold food or beverages
Diagnosis	Cold stimulation test, clinical evaluation
Differential diagnosis	Cholinergic urticaria, aquagenic urticaria, cryoglobulinemia
Prevention	Avoidance of cold exposure, protective clothing
Treatment	Antihistamines, epinephrine (for anaphylaxis), immunosuppressants (severe cases)
Medication	Cetirizine, loratadine, hydroxyzine
Prognosis	Often improves with age, but may persist
Frequency	Rare
Deaths	Rare; associated with anaphylactic reactions

Hives on the back from exposure to cold air on an individual with cold allergy. The hives were induced by riding a stationary bike shirtless for an hour next to a door cracked open on a cool day. The temperature of the air flowing in was around 10C (50F). The lighter band at chest height was covered by a heart rate monitor strap.

Cold urticaria (also known as cold hives) is a rare allergic condition characterized by the development of hives or urticaria after exposure to cold temperatures. These welts are often itchy, red, and raised, and may appear on the face, hands, feet, or other areas of the body exposed to a cold stimulus. Cold urticaria can range from mild localized discomfort to systemic, life-threatening anaphylaxis in severe cases.

Overview

Cold urticaria occurs when the body's immune system reacts abnormally to cold exposure. The release of histamine and other inflammatory mediators causes vasodilation, capillary leakage, and mast cell activation, resulting in redness, swelling, and itching. In some individuals, more severe symptoms such as angioedema, low blood pressure, or even anaphylactic shock can occur, especially with full-body cold exposure such as swimming.

Types

Primary cold contact urticaria

This is the most common form and is triggered by direct contact with cold objects or environments. Exposure to cold air, water, ice, or even handling chilled items can provoke localized wheals or generalized urticarial rash. Onset typically occurs within 5–10 minutes of exposure.

Secondary cold contact urticaria

This rare type is associated with underlying conditions such as cryoglobulinemia or cryofibrinogenemia, where abnormal proteins precipitate at low temperatures. It may present alongside other symptoms such as Raynaud’s phenomenon, purpura, or vasculitis.

Reflex cold urticaria

This form involves widespread welting and rash triggered by a general drop in body temperature, such as immersion in cold water or exposure to a cold environment, without direct contact with cold substances.

Familial cold urticaria

Also known as familial cold autoinflammatory syndrome (FCAS), this is a rare inherited disorder that follows an autosomal dominant pattern. It is associated with mutations in the CIAS1 gene (also known as NLRP3), which affects the innate immune system. Symptoms include recurrent fever, arthralgia, conjunctivitis, and widespread urticaria after generalized exposure to cold.

Signs and Symptoms

• Localized or generalized hives
• Swelling of exposed areas
• Pruritus (itchiness)
• Headache, fatigue
• Fever and chills (in familial cases)
• Angioedema of the face, lips, or throat
• Anaphylaxis in severe cases (rare)

Diagnosis

Diagnosis is typically clinical and confirmed by a **cold stimulation test**, which involves applying a cold object (such as an ice cube) to the skin for several minutes and observing for a reaction. Additional tests may include:

• Blood tests to rule out secondary causes
• Serum cryoglobulin or cryofibrinogen levels
• Genetic testing for suspected familial cases

Complications

Cold urticaria can lead to serious and potentially life-threatening complications such as:

• Pharyngeal edema from ingestion of cold drinks
• Anaphylaxis following generalized cold exposure
• Drowning due to sudden hypotension or unconsciousness while swimming
• Hypothermia in prolonged cold environments

Treatment and Management

General measures

• Avoid cold exposure and swimming in cold water
• Wear warm clothing and cover exposed skin
• Avoid consuming ice-cold foods or beverages

Medications

• Second-generation antihistamines (e.g., cetirizine, loratadine)
• First-generation antihistamines (e.g., hydroxyzine) for more sedative effect
• Montelukast or other leukotriene receptor antagonists
• Omalizumab, a monoclonal antibody, in severe or refractory cases
• Epinephrine auto-injector (e.g., EpiPen) for emergency use in patients at risk of anaphylaxis

Prognosis

The course of cold urticaria varies. In many cases, symptoms gradually diminish or resolve over several years. However, some patients may experience chronic symptoms requiring long-term management. Familial cases may persist for life and require more aggressive intervention.

Epidemiology

Cold urticaria is a rare condition, more common in younger individuals and those with a history of other atopic disorders. It affects both sexes but may be slightly more prevalent in females.

See also

• Urticaria
• Angioedema
• Anaphylaxis
• Mast cell
• Histamine
• Cryoglobulinemia
• Autoinflammatory syndrome