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Cold urticaria in Infant/Neonate
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Cold urticaria in Infant/Neonate

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Contributors: Jeffrey M. Cohen MD, Christine Hunt MD, Sylvia Hsu MD, Susan Burgin MD
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Synopsis

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 is less common among the pediatric population than among adults, but has been seen as early as 6 months of age. Girls are more commonly affected than boys, and approximately 25% have been found to have angioedema. Primary acquired cold urticaria is the most common form of cold urticaria seen in children. Cold urticaria due to underlying conditions, such as infection, cryoglobulinemia, cryofibrinogenemia, or lymphoproliferative diseases, is especially rare in children.

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. Systemic findings such as conjunctivitis, myalgia, and nausea may also be seen. Rarely, patients may experience headache, diaphoresis, drowsiness, and amyloidosis. Symptoms begin 10 minutes to 8 hours after cold exposure and generally subside within 24 hours. The time to resolution may be longer in cases of more prolonged cold exposure.

Codes

ICD10CM:
L50.2 – Urticaria due to cold and heat

SNOMEDCT:
74774004 – Cold urticaria

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

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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References

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Last Reviewed: 03/10/2017
Last Updated: 03/10/2017
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Cold urticaria in Infant/Neonate
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Cold urticaria : Cold exposure, Erythema, Pruritus, Hives
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