Rare Pediatrics News

Disease Profile

Cold urticaria

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

no.svg

Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

no.svg

X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

no.svg

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

no.svg

Not applicable

no.svg

Other names (AKA)

Cold contact urticaria; Primary idiopathic cold urticaria; Urticaria idiopathic cold

Summary

Cold urticaria is an allergic condition that affects the skin. Symptoms usually start in early adulthood. The most common symptom is a red, itchy rash that appears on the skin when it is exposed to the cold (e.g. cold weather or cold water). This reaction usually occurs within 5-10 minutes after exposure and can last for 1-2 hours. The exact cause of cold urticaria is unknown. In some cases, it may be associated with an infection, insect bite, or blood cancer. It is diagnosed based on the symptoms and confirmed through tests that look for a specific reaction to cold. Treatment may consist of avoiding exposures that may trigger a reaction and medications that prevent allergic reactions like antihistamines.[1][2][3][4]

Symptoms

The following list includes the most common signs and symptoms in people with cold urticaria. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition. 

Signs and symptoms include:

  • Red, itchy rash after exposure to cold
  • Swelling in the area exposed to cold
  • Headache
  • Severe allergic reaction (anaphylaxis)

Cold urticaria usually affects young adults, but can appear at any age. Females are more likely to have it than males. Symptoms usually appear within 5-10 minutes of being exposed to cold and last 1-2 hours. A few people have very severe reactions that can lead to trouble breathing, shock, or passing out. In some people, cold urticaria goes away after several years.[1][3][5]

Cause

The cause of most cases of cold urticaria is unknown. In some cases, cold urticaria is triggered by an infectious disease, an insect bite, certain medications, or blood cancers.[1][3]

Diagnosis

Cold urticaria is typically diagnosed based on the presence of characteristic signs and symptoms. A cold stimulation test may be done to confirm the diagnosis. This involves placing a cold object (such as an ice cube) against the skin of the forearm for 1-5 minutes. In people with cold urticaria, a distinct red and swollen rash will generally develop within minutes of exposure. Additional blood tests may be done to determine if there are associated diseases. Diagnostic guidelines for cold urticaria and other forms of urticaria are available.[1][4][6]

Treatment

Cold avoidance is the primary treatment for cold urticaria. Antihistamines are recommended for prophylactic treatment when exposure to cold can not be avoided, and for treating the rash. People with cold urticaria may carry an epinephrine autoinjector due to the increased risk of anaphylaxis. Other medications that may help include antibiotics and omalizumab. Treatment guidelines for cold urticaria have been published.[1][4][6]

Specialists who may be involved in the care of someone with cold urticaria include:

  • Allergist
  • Immunologist

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Mayo Clinic has an information page on Cold urticaria.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Cold urticaria. Click on the link to view a sample search on this topic.

References

  1. Maurer M. Cold urticaria. UpToDate. Updated Apr 2020; https://www.uptodate.com/contents/cold-urticaria.
  2. Maurer M, Fluhr JW, Khan DA. How to Approach Chronic Inducible Urticaria. J Allergy Clin Immunol Pract.. 2018; 6(4):1119-1130. https://pubmed.ncbi.nlm.nih.gov/30033913.
  3. Stepaniuk P, Vostretsova K, Kanani A. Review of cold-induced urticaria characteristics, diagnosis and management in a Western Canadian allergy practice. Allergy Asthma Clin Immunol.. 2018; 14:85:Published 2018 Dec 18. https://pubmed.ncbi.nlm.nih.gov/30574166.
  4. Magerl M, Altrichter S, Borzova E, Giminez-Arnau A, Grattan CEH et al. The definition, diagnostic testing, and management of chronic inducible urticarias The EAACI/GA(2) LEN/EDF/UNEV consensus recommendations 2016 update and revision.. Allergy. 2016; 71(6):780-802. https://pubmed.ncbi.nlm.nih.gov/26991006.
  5. Kulthanan K, Hunnangkul S, Tuchinda P, Chularojanamontri L, Weerasubpong P et al. Treatments of cold urticaria: A systematic review. J Allergy Clin Immunol. 2019; 143(4):1311-1331. https://pubmed.ncbi.nlm.nih.gov/30776418.
  6. Zuberbier T, Aberer W, Asero R, Abdul Latiff AH, Baker D et al. The EAACI/GA²LEN/EDF/WAO guideline for the definition, classification, diagnosis and management of urticaria. Allergy. 2018; 73(7):1393-1414. https://pubmed.ncbi.nlm.nih.gov/29336054.

Rare Pediatrics News