Chronic cold urticaria
Encyclopedia
Cold Urticaria is an allergy
Allergy
An Allergy is a hypersensitivity disorder of the immune system. Allergic reactions occur when a person's immune system reacts to normally harmless substances in the environment. A substance that causes a reaction is called an allergen. These reactions are acquired, predictable, and rapid...

 where hives (urticaria
Urticaria
Urticaria is a kind of skin rash notable for pale red, raised, itchy bumps. Hives is frequently caused by allergic reactions; however, there are many non-allergic causes...

) or large red welts form on the skin after exposure to a cold stimulus. The welts are usually itchy and often the hands and feet will become itchy and swollen as well. Hives vary in size from about the size of a pencil eraser (7 mm diam.) to as big as a fifty pence piece (27 mm diam.) or larger. The disease is classified as chronic
Chronic (medicine)
A chronic disease is a disease or other human health condition that is persistent or long-lasting in nature. The term chronic is usually applied when the course of the disease lasts for more than three months. Common chronic diseases include asthma, cancer, diabetes and HIV/AIDS.In medicine, the...

when hives appear for longer than 6 weeks; they can last for life, though their course is often unpredictable. This disorder, or perhaps two disorders with the same clinical manifestations, can be inherited (familial cold urticaria) or acquired (primary acquired cold urticaria). The acquired form is most likely to occur between ages 18–25.

Types

Cold urticaria may be divided into the following types:
  • Primary cold contact urticaria
    Primary cold contact urticaria
    Primary cold contact urticaria is a cutaneous condition characterized by wheals, and occurs in rainy, windy weather, and after contact with cold objects, including ice cubes....

  • Secondary cold contact urticaria
    Secondary cold contact urticaria
    Secondary cold contact urticaria is a cutaneous condition characterized by wheals, due to serum abnormalities such as cryoglobulinemia or cryofibrinogenemia are extremely rare, and are then associated with other manifestations such as Raynaud's phenomenon or purpura....

  • Reflex cold urticaria
    Reflex cold urticaria
    Reflex cold urticaria is a cutaneous condition in which generalized cooling of the body induces widespread whealing....

  • Familial cold urticaria
    Familial cold urticaria
    Familial cold urticaria is an autosomal dominant condition characterized by rash, conjunctivitis, fever/chills and arthralgias elicited by exposure to cold - sometimes temperatures below 22°C .It has been mapped to CIAS1 and is a slightly milder member of the disease family including Muckle–Wells...


Symptoms

When the body is exposed to the cold, hives appear. Hives result from dilation of capillaries which allow fluid to flow out into the surrounding tissue which is the epidermis.They resolve when the body absorbs this fluid. The border of a hive is described as polycyclic, or made up of many circles, and changes as fluid leaks out and then is absorbed. Pressing on a hive causes the skin to blanch distinguishing it from a bruise or papule. Hives can last for a few minutes or a few days, and vary from person to person. Also a burning sensation occurs. During a severe reaction, hypotension
Hypotension
In physiology and medicine, hypotension is abnormally low blood pressure, especially in the arteries of the systemic circulation. It is best understood as a physiologic state, rather than a disease. It is often associated with shock, though not necessarily indicative of it. Hypotension is the...

, which can be life-threatening, can occur. A serious reaction is most likely to occur if the hives occur with less than 3 minutes of exposure (during a cold test).

Cause

The hives are a histamine
Histamine
Histamine is an organic nitrogen compound involved in local immune responses as well as regulating physiological function in the gut and acting as a neurotransmitter. Histamine triggers the inflammatory response. As part of an immune response to foreign pathogens, histamine is produced by...

 reaction in response to cold stimuli, including a drastic drop in temperature, cold air, and cold water. There are many causes for cold hives, most are idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...

 (meaning they have no known cause). Some rare conditions can cause cold hives, and it can be useful to test for these conditions if the cold hives are in any way unusual.

Diagnosis

Diagnosis is typically obtained by an allergist or other licensed practitioner performing a cold test. During the cold test, a piece of ice is held against the forearm, typically for 2–3 minutes. A positive result is a specific looking mark of raised red hives. The hives may be the shape of the ice, or it may radiate from the contact area of the ice.

Treatment

The most important treatment is to stay warm. Some air conditioned rooms and pools may be too cold for the skin. Warming up immediately after exposure to cold
Cold
Cold describes the condition of low temperature.Cold may also refer to:*Common cold, a contagious viral infectious disease of the upper respiratory system*Chronic obstructive pulmonary disease...

 temperatures usually helps control the hives before they get worse. Although, the hives do not instantaneously disappear after warming the affected area(s), warming up afterwards reduces the time it takes for the hives to go away. Some patients report that spreading butter or vegetable shortening on their affected areas reduces the risk of future eruptions.

Allergy medications containing antihistamines such as Benadryl
Benadryl
Benadryl is a brand name allergy medicine marketed over-the-counter by Johnson & Johnson subsidiary McNeil Consumer Healthcare. Prior to 2007, Benadryl was marketed by Pfizer Consumer Healthcare...

, Zyrtec
Cetirizine
Cetirizine , a second-generation antihistamine, is a major metabolite of hydroxyzine, and a racemic selective H1 receptor inverse agonist used in the treatment of allergies, hay fever, angioedema, and urticaria.-Availability:...

, Claritin, Periactin
Cyproheptadine
Cyproheptadine , sold under the brand name Periactin, is a first-generation antihistamine with additional anticholinergic, antiserotonergic, and local anesthetic properties.- Indications :...

 and Allegra
Fexofenadine
Fexofenadine is an antihistamine drug used in the treatment of hayfever and similar allergy symptoms...

 may be taken orally to prevent and relieve some of the hives (depending on the severity of the allergy). There are also topical antihistamine creams which are used to help relieve hives in other conditions but there is not any documentation stating it will relieve hives induced by cold temperature. Please note however, it is not often that antihistamines work for cases like Cold Urticaria.

There has also been evidence to show that Cold Urticaria has been linked to a gluten intolerance, and gluten free diets have helped some CU sufferers.

Cold hives can result in a potentially serious, or even fatal, systemic reaction (anaphylactic shock). People with cold hives may have to carry an injectable form of epinephrine
Epinephrine
Epinephrine is a hormone and a neurotransmitter. It increases heart rate, constricts blood vessels, dilates air passages and participates in the fight-or-flight response of the sympathetic nervous system. In chemical terms, adrenaline is one of a group of monoamines called the catecholamines...

 (like Epi-pen or Twinject) for use in the event of a serious reaction.

Contrary to popular belief, immersing the affected area in hot water or a warmer environment does not improve conditions. Immersing the affected area in warm water or a warmer environment is a means of which is used to help bring the allergic reaction under control so it does not become worse.

The best treatment for this allergy is avoiding exposure to cold
Cold
Cold describes the condition of low temperature.Cold may also refer to:*Common cold, a contagious viral infectious disease of the upper respiratory system*Chronic obstructive pulmonary disease...

 temperature
Temperature
Temperature is a physical property of matter that quantitatively expresses the common notions of hot and cold. Objects of low temperature are cold, while various degrees of higher temperatures are referred to as warm or hot...

.

Ebastine
Ebastine
Ebastine is a non-sedating H1 antihistamine.It does not penetrate the blood-brain barrier and thus allows an effective block of the H1 receptor in peripheral tissue without a central side effect, i.e not causing sedation or drowsiness.The basic patent for ebastine in Europe is EP-B-134124...

 has been proposed as an approach to prevent acquired cold urticaria.

Situations when reactions may occur

  • Cold weather: Exposure to cold or cool air for more than a few minutes can trigger a reaction. Avoid long walks outdoors during cold weather. Be prepared to bundle up and cover exposed skin as long as the weather remains cool. Experiment to find the right number of layers.

  • Sweat: A reaction may even occur on a warm day when there is sweat on the skin. If there is a breeze it will rapidly cool the skin and create hives. Getting warm quickly by drying the skin and covering up will help to control the hives before they get too bad. Using or carrying a towel while exercising and wiping off sweat before it has a chance to dry is an effective means of preventing hives from occurring.

  • Cold/Cool IV Lines: While in the hospital
    Hospital
    A hospital is a health care institution providing patient treatment by specialized staff and equipment. Hospitals often, but not always, provide for inpatient care or longer-term patient stays....

    , inserting IV
    Intravenous therapy
    Intravenous therapy or IV therapy is the infusion of liquid substances directly into a vein. The word intravenous simply means "within a vein". Therapies administered intravenously are often called specialty pharmaceuticals...

     lines that have been refrigerated or are cool into individuals who are allergic to cold
    Cold
    Cold describes the condition of low temperature.Cold may also refer to:*Common cold, a contagious viral infectious disease of the upper respiratory system*Chronic obstructive pulmonary disease...

     temperature
    Temperature
    Temperature is a physical property of matter that quantitatively expresses the common notions of hot and cold. Objects of low temperature are cold, while various degrees of higher temperatures are referred to as warm or hot...

     can/may result in pain within the limb the IV is placed and a line of hives may appear within a radius of 6-8 inches from the IV site in the pattern of a line going up the limb. Notify your doctor/nurse of your condition so they will be able to use a machine which warms IV
    Intravenous therapy
    Intravenous therapy or IV therapy is the infusion of liquid substances directly into a vein. The word intravenous simply means "within a vein". Therapies administered intravenously are often called specialty pharmaceuticals...

     lines before they enter your body

  • Swimming: Swimming can be especially dangerous as the rapid heart rate combined with the onset of hives can lead to hypotension
    Hypotension
    In physiology and medicine, hypotension is abnormally low blood pressure, especially in the arteries of the systemic circulation. It is best understood as a physiologic state, rather than a disease. It is often associated with shock, though not necessarily indicative of it. Hypotension is the...

    . Taking an allergy medication before entering the water and conserving energy until your body adjusts to the temperature may help to prevent the hypotension
    Hypotension
    In physiology and medicine, hypotension is abnormally low blood pressure, especially in the arteries of the systemic circulation. It is best understood as a physiologic state, rather than a disease. It is often associated with shock, though not necessarily indicative of it. Hypotension is the...

    . Take an antihistamine if you choose to go swimming; preferably a non-drowsy histamine, and have an individual with you at all times

  • Air Conditioning: Entering a cool building during a summer day can result in an allergic reaction to exposed areas of skin, congestion
    Congestion
    Congestion generally means excessive crowding.Congestion may refer to:* congestion in heart failure, a term to describe low cardiac output seen in heart failure.* Nasal congestion, the blockage of nasal passages due to swollen membranes...

     from breathing the cool air and possibly feeling fatigued. Carrying jackets with you on warm days is a good idea in case you enter an air conditioned building.

  • Cold Foods/Drinks: Eating or drinking cold or cool substances such as ice cream or even iced tea may result in the individuals tongue and surrounding tissues swelling
    Swelling (medical)
    In medical parlance, swelling is the transient enlargement or protuberance in the body and may include tumors. According to cause, it may be congenital, traumatic, inflammatory, neoplastic or miscellaneous....

    . If eating cold foods or having cold drinks results in swelling of tissues
    Tissue (biology)
    Tissue is a cellular organizational level intermediate between cells and a complete organism. A tissue is an ensemble of cells, not necessarily identical, but from the same origin, that together carry out a specific function. These are called tissues because of their identical functioning...

     in and around the mouth/throat it is advisable to take an antihistamine
    Antihistamine
    An H1 antagonist is a histamine antagonist of the H1 receptor that serves to reduce or eliminate effects mediated by histamine, an endogenous chemical mediator released during allergic reactions...

     before consuming them and/or refraining from eating/drinking them.

  • Cool/Cold Surfaces: Sitting on sidewalks which are cool, leaning on or grabbing a cold pole will result in hives forming on the area which had contact with the cool surfaces. The only treatment for these kinds of situations is trying to avoid cold surfaces, if you can not avoid them using gloves to touch them or using a pad to sit on cool sidewalks is helpful

  • Restriction of Blood Flow: Typing or other activities which tense muscles and reduce blood flow can cool the body parts enough to cause itching and hives.

  • Blood Flow: While putting hands or feet in hot water nails turn purple/white until the body gets used to the temperature.

See also

  • Cholinergic urticaria
    Cholinergic urticaria
    Cholinergic urticaria is a subcategory of physical urticaria that is a skin rash brought on by a hypersensitive reaction to body heat...

    , a similar hives reaction in response to heat
  • Goose bumps
    Goose bumps
    Goose bumps, also called goose flesh, goose pimples, chill bumps, chicken skin, funky spots, Dasler Bumps, chicken bumps or the medical term cutis anserina, are the bumps on a person's skin at the base of body hairs which may involuntarily develop when a person is cold or experiences strong...

    , an unrelated, normal response to cold temperatures
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