Familial Cold Urticaria (FCU)

Familial Cold Urticaria (FCU) is a type of physical urticaria, which is a subcategory of hives. FCU is a rare genetic condition that affects the skin and is characterized by the development of hives or wheals (swollen, red bumps) in response to cold temperatures. This reaction is caused by the release of histamine, a chemical produced by the body during an allergic reaction. The severity of the symptoms can vary from person to person, with some individuals experiencing mild to moderate symptoms, while others may have more severe reactions, including difficulty breathing and low blood pressure. There is currently no cure for FCU, but treatment options are available to manage the symptoms and reduce the risk of serious reactions. These may include antihistamines, corticosteroids, and avoidance of cold temperatures.


The main causes of FCU are:

  1. Inheritance: FCU is an autosomal dominant disorder, meaning it is passed down from parent to child through a single mutated gene. If a parent has FCU, there is a 50% chance that their child will also have the condition.
  2. Environmental triggers: Cold exposure, such as cold air, water, or objects, can trigger an allergic reaction in individuals with FCU.
  3. Stress: Stress can increase the likelihood of an attack, making it important

The exact cause of FCU is not known, but it is believed to be related to a genetic mutation that affects the body’s ability to regulate its temperature. This leads to an overactive immune response to cold temperatures, causing the release of histamine and other chemicals that cause the symptoms associated with FCU.

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There is evidence to suggest that the condition may be linked to a deficiency in certain proteins that play a role in regulating the immune system and controlling inflammation. Other factors that may contribute to the development of FCU include changes in the structure and function of the blood vessels, increased sensitivity to histamine, and a general overactivity of the immune system.


Familial cold urticaria is a type of physical urticaria, which is an allergy to cold temperatures. The main symptoms include:

  1. Hives: Raised, red, itchy welts that appear on the skin after exposure to cold temperatures.
  2. Angioedema: Swelling of the face, lips, tongue, or throat, which can lead to difficulty breathing.
  3. Flushing: A rapid increase in body temperature, causing redness and warmth of the skin.
  4. Fatigue: A feeling of exhaustion or weariness after exposure to cold temperatures.
  5. Chills: A sudden feeling of cold and shivers that can be accompanied by a fever.
  6. Headaches: Pain or discomfort in the head that can occur after exposure to cold temperatures.
  7. Joint pain: Pain or stiffness in the joints that can occur after exposure to cold temperatures.
  8. Chest pain: Pain or discomfort in the chest that can occur after exposure to cold temperatures.

It is important to seek medical attention if you experience any of these symptoms after exposure to cold temperatures, as they can be indicative of an underlying condition that requires treatment.


Familial cold urticaria is a rare hereditary condition that causes an allergic reaction to cold temperatures. The main diagnosis of this condition is through a thorough medical history, physical examination, and specific tests such as:

  1. Cold challenge test: A doctor will expose the patient to cold temperatures, usually by placing an ice pack on the skin, and observe for any allergic reactions.
  2. Skin prick test: A small amount of allergen is placed on the skin and a small needle is used to prick the skin. The doctor will observe for any redness or swelling.
  3. Blood test: Blood is drawn and sent to a laboratory for analysis to measure the levels of antibodies that are produced in response to an allergen.
  4. Cryoglobulin test: A test that measures the levels of a type of antibody called cryoglobulins in the blood. This test is useful in diagnosing cold urticaria.
  5. Complement C4 test: A blood test that measures the levels of complement C4, a protein that helps the body fight infections. Low levels of complement C4 may indicate familial cold urticaria.
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It is important to consult with a doctor for proper diagnosis and treatment of familial cold urticaria.


Familial cold urticaria (FCU) is a hereditary condition characterized by hives and itching in response to cold temperatures. The main treatment for FCU is to avoid exposure to cold temperatures and to use medications to control symptoms when exposure is unavoidable.

  1. Anti-histamines: Over-the-counter anti-histamines such as diphenhydramine (Benadryl), loratadine (Claritin), or cetirizine (Zyrtec) can help reduce itching and hives.
  2. Corticosteroids: A short course of oral corticosteroids may be prescribed for severe symptoms. These medications help to reduce inflammation and suppress the immune response.
  3. Desensitization: In some cases, patients may undergo a process of desensitization, where they are exposed to gradually increasing levels of cold over time. This can help to reduce the severity of symptoms and increase tolerance to cold.
  4. Avoidance: The best treatment for FCU is to avoid exposure to cold temperatures. This may involve wearing warm clothing and avoiding cold drinks, showers, and outdoor activities in cold weather.
  5. Emergency epinephrine: In severe cases, an epinephrine auto-injector may be prescribed for use in the event of an anaphylactic reaction.

It is important to work with a healthcare provider to develop a comprehensive treatment plan for FCU, as treatment may vary depending on the severity of symptoms and individual needs.