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Cold urticaria in Adult
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Cold urticaria in Adult

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Contributors: Jeffrey M. Cohen MD, Christine Hunt MD, Sylvia Hsu MD, Susan Burgin MD
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Cold urticaria is a chronic physical urticaria that occurs upon rewarming after exposure to cold air, liquids, or objects. It is due to IgE-mediated mast cell degranulation. Cold urticaria is a heterogenous group of conditions comprised of primary cold contact urticaria, secondary cold contact urticaria, reflex cold urticaria, and familial cold urticaria. Angioedema may also be seen. Wheals are usually localized to the area of contact. The presentation of cold urticaria may be more generalized in the setting of extensive cold exposure. Systemic symptoms of flushing, headache, syncope, and abdominal pain may also develop if large areas are affected. Systemic symptoms most commonly occur with aquatic activities, and those with a history of angioedema are at the greatest risk. There appears to be an inverse relationship between the minimum time of cold stimulus necessary to induce a confluent wheal and the clinical severity of symptoms. Individuals with hypotensive episodes usually have a lag time of less than 3 minutes.

Cold urticaria lasts an average of 4-5 years; 50% of patients show improvement or remission within 5 years.

Primary cold contact urticaria is the most commonly encountered form (95% of cases). It is often idiopathic but may follow respiratory infections or arthropod bites / stings, or occur in association with human immunodeficiency virus (HIV). Primary cold contact urticaria may occur at any age, but it is most frequently seen in young adults. Wheals lasting less than 24 hours are observed.

Secondary cold contact urticaria is seen with serum abnormalities such as cryoglobulinemia, cryofibrinogenemia, or circulating cold agglutinins. It is rare and is seen in association with Raynaud phenomenon and purpura. Wheals may last longer than 24 hours.

Reflex cold urticaria occurs after generalized cooling of the body, which induces widespread wheals. Patients may experience life-threatening anaphylaxis from exposures such as diving into a cold lake. Hives can be observed if patients are placed in a very cold room, but this should not be performed as it may induce anaphylaxis.

Familial cold autoinflammatory syndrome (FCAS) is a subtype of cryopyrin-associated periodic syndrome (CAPS). FCAS typically presents within the first 6 months of life with recurrent outbreaks of fever, arthralgia, fatigue, and nonpruritic urticaria that occurs after cold exposure.


L50.2 – Urticaria due to cold and heat

74774004 – Cold urticaria

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Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed: 03/10/2017
Last Updated: 03/10/2017
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Cold urticaria in Adult
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Cold urticaria : Cold exposure, Erythema, Pruritus, Hives
Clinical image of Cold urticaria
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