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Last updated: 12/4/2024
Years published: 1988, 1989, 2004, 2024
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders, for assistance in the preparation of this report.
Summary
Cold urticaria (ColdU) also called cold contact urticaria or acquired cold urticaria, is a subtype of physical urticaria and also considered a relatively uncommon form of chronic inducible urticaria. In ColdU, exposure to cold air, water, or objects leads to itchy hives (urticaria or wheals), redness, swelling and in some people, systemic reactions such as anaphylaxis.
The physical urticarias are forms of inducible urticaria in which there is a specific and definite stimulus that triggers the development of signs and symptoms. Chronic inducible urticaria is chronic urticaria that has a specific cause or trigger and is classified according to the stimulus that provokes wheals to develop.
There are different ways to classify cold urticaria. It can be classified in typical and atypical forms and also into primary (idiopathic) and secondary to other diseases.
What causes ColdU remains unknown. Researchers consider autoallergy, autoimmunity, neurogenic pathways and aberrant temperature sensing as underlying mechanisms.
Treatment basically consists in avoiding the triggers and managing the symptoms.
Introduction
Acquired cold urticaria is now understood to be a completely different disorder from familial cold autoinflammatory syndrome (FCAS), previously known as hereditary cold urticaria. FCAS is one of the cryopyrin-associated periodic syndromes (CAPS).
Signs and symptoms of cold urticaria become evident within 2–5 minutes after exposure and last for 1–2 hours. They include:
Depending on the affected organ or system the following symptoms may be present:
Classification of Cold Urticaria
While the cause is still not known, cold exposure triggers mast cell degranulation and the release of histamine and other inflammatory mediators. When the skin is exposed to cold, mast cells, a type of immune cell, become activated and release inflammatory substances like histamine, prostaglandins and leukotrienes. This process, known as mast cell degranulation, causes the common symptoms of cold urticaria, including redness, swelling, itching and hives (wheals).
In some people, cold exposure may also result in the formation of new (de novo) autoantigens, or molecules the immune system mistakenly identifies as harmful. In susceptible individuals, this can lead to an IgE-mediated response, where specific antibodies (IgE) bind to mast cells, making them more sensitive to cold. Subsequent exposure triggers these mast cells to degranulate, amplifying the symptoms. This process is known as IgE-mediated autoimmunity, and it has been implicated as one potential mechanism underlying cold urticaria.
This results in itching, burning, redness and swelling, usually confined to the area exposed to cold.
Triggers include cold air, water, objects and even ingestion of cold foods or beverages. Systemic reactions are more likely after extensive cold exposure, such as swimming in cold water.
Cold urticaria can be primary (Idiopathic) when there is no identifiable underlying cause, or secondary, when it occurs as part of other health conditions
Cold urticaria affects about 0.05% of the population (about 5-6 people in 10,000) and accounts for 5–34% of physical urticarias. It is more common in regions with colder climates. It typically affects young adults, with onset usually between the 2nd and 4th decades of life
Males and females are affected with similar frequency in most studies, although one study reported that females were affected twice as often as males. Up to one-half of patients with cold urticaria are atopic (tend to develop allergic diseases) and one-fourth have other types of inducible urticaria, most commonly symptomatic dermographism and cholinergic urticaria.
The diagnosis of cold urticaria should be suspected in a person who develops hives and/or angioedema after the skin is exposed to cold air, liquids, or objects. Symptoms usually develop some minutes after cold exposure and are usually limited to cold-exposed skin areas. Diagnosis is confirmed by a cold stimulation test (CST), a reproducible test using an ice cube applied to the skin. A positive response is indicated by the appearance of a “hive” in the shape of the ice cube, within 10 minutes after the stimulus is removed. This two-step process suggests that exposure to cold is required, but hive formation occurs as the skin temperature rises. A modified CST may be needed for atypical forms. If the CST is negative, the medical history should be revisited and an atypical form of cold urticaria should be considered.
Treatment
There is no cure for cold urticaria. Treatment includes avoidance of triggers and symptom management.
As soon as the diagnosis has been made, the doctor should begin to educate the affected person about the activities and procedures that may trigger systemic reactions or serious localized reactions:
General cold avoidance is recommended since it is the most logical way to prevent symptoms. However, avoidance is not always possible. Knowledge of their temperature threshold can help patients to recognize and avoid critical cold exposure in their daily lives.
Medications for cold urticaria may include:
It should be emphasized to the affected person that they should carry an epinephrine auto-injector and review its use.
Cold urticaria is self-limiting in about 50% of cases, with remission occurring within 5–6 years. Atypical forms, including familial types, may persist for a lifetime
Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
JOURNAL ARTICLES
Prosty C, Gabrielli S, Le M, et al. Prevalence, management, and anaphylaxis risk of cold urticaria: A systematic review and meta-analysis. J Allergy Clin Immunol Pract. 2022;10(2):586-596.e4. doi:10.1016/j.jaip.2021.10.012
Maltseva N, Borzova E, Fomina D, et al. Cold urticaria – What we know and what we do not know. Allergy. 2021;76(4):1077-1094. doi:10.1111/all.14674
INTERNET
Saini S. Cold Urticaria. UpToDate. November 8, 2023. https://www.uptodate.com/contents/cold-urticaria Accessed Dec 4, 2024.
Ngan V. Cold Urticaria. DermNet. July 2024. https://dermnetnz.org/topics/cold-urticaria Accessed Dec 4, 2024.
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