Cold-Induced Urticaria - Causes, Symptoms, Diagnosis, and Treatments

What Is Cold Induced Urticaria?

It is a chronic skin condition that occurs after being exposed to anything cold in nature, for example- eating ice cream, swimming in a cold pool, being exposed to cold weather, etc. Symptoms include hives, erythema, and itching.

There are two types of cold-induced urticaria based on the duration it takes for the onset of symptoms, they are-

1. Hereditary Cold-Induced Urticaria- Also known as a familial cold autoinflammatory syndrome (FCAS), it is an inherited inflammatory disorder characterized by irregular episodes of rash, fever, joint pain, and other symptoms of systemic inflammation triggered by cold exposure.

The symptoms appear somewhere between 30 minutes to 24 hours after being exposed and last typically for 48 hours. It is one of the complications of the cryopyrin-associated periodic syndrome (CAPS) that is caused due to mutation in the CIAS1/NLRP3 gene.

2. Acquired Cold-Induced Urticaria- The symptoms of acquired cold urticaria start within two to five minutes after the exposure and last for 30 minutes. It can be further subdivided into the following types-

• Primary Acquired Cold-Induced Urticaria- The symptoms appear in the cold phase which later on become prominent in the rewarming phase. They start within 30 minutes of exposure and stay for 30 minutes post-exposure. Along with redness and itching, the patient will also develop headaches, wheezing, palpitation, and fainting.

• Delayed Cold-Induced Urticaria- As the name suggests, symptoms appear after several hours after exposure to cold conditions.

• Localized Cold-Induced Urticaria- It is different from the other types in the aspect that, after the cold exposure the symptoms develop only at the location that was previously injected for ragweed allergies or ladybug bites.

• Reflex Cold-Induced Urticaria- It is characterized by the appearance of hives that develop as a response to the drop in the body temperature after being exposed to cold or cold applications like an ice pack.

• Secondary Cold-Induced Urticaria- It occurs due to underlying hematological conditions that are seen in association with viral infections like mononucleosis.

What Causes Cold Induced Urticaria?

The FCAS form of cold-induced urticaria is inherited as an autosomal dominant trait and the rest of the forms of cold-induced urticaria are caused due to autoallergy, autoimmunity, neurological pathways, and aberrant temperature sensing.

Human traits, including genetic conditions like FCAS, are a product of the interaction between two genes- one from the mother and the other from the father. In genetically transferred dominant disorders, one copy of the deceased gene (either from the mother or the father) will dominate the normal gene and express itself resulting in the abnormality. In this mode of transmission, there is a 50 percent chance of the offspring being affected for each pregnancy regardless of the sex.

In some acquired forms of cold-induced urticaria, exposure to cold will act as an antigen (foreign stimulant) and trigger immunoglobulin E (IgE) mediated autoimmunity, this activates the mast cells (cells responsible for immediate allergic reaction) which then release histamine (the chemical responsible for symptoms of allergies) and other inflammatory mediators.

In a few patients with cold-induced urticaria, topical application of capsaicin has been shown to prevent cold-induced urticarial responses, suggesting the role of nerve fibers in cold-induced urticaria, this, however, needs to be researched further.

Regardless, of the underlying cause, the symptoms of all types of cold-induced urticaria are instigated by exposure to cold; the temperature at which cold-induced urticaria patients start showing symptoms varies from four degrees to 27 degrees.

What Are the Symptoms of Cold Induced Urticaria?

Apart from the dermatological symptoms, patients with cold-induced urticaria will develop the following symptoms depending on the duration of exposure-

• Constitutional Symptoms- Include fever and fatigue.

• Skin and Mucous Membrane Symptoms- Comprise of itchy wheals with or without affecting lips, tongue, and pharynx.

• Respiratory Symptoms- The commonly seen ones are dyspnoea (difficulty in breathing), hoarseness, laryngeal angioedema (neck swelling), and nasal congestion.

• Gastrointestinal Symptoms- They are nausea, abdominal pain, and diarrhea.

• Cardiovascular Symptoms- Includes tachycardia (increased heartbeat), hypotension, and shock.

• Reproductive Symptoms- Can be noticed in the form of uterine contractions.

• Central Nervous System Symptoms- This includes headache, disorientation, fatigue, and vertigo.

How Is Cold Induced Urticaria Diagnosed?

Diagnosis of cold-induced urticaria relies on the patient's history, CST (cold stimulating test), and laboratory workup.

Cold Stimulating Test (CST)- Patients who are suspected to have cold-induced urticaria are advised to stop taking any prescription antihistamines or systemic glucocorticoids at least three days to seven days prior to the procedure. A melting ice cube in a thin plastic bag or non-latex medical glove is applied to the forearm of the patient for five minutes, followed by a test reading ten minutes after the end of the cold stimulation.

In patients with cold-induced urticaria, a positive result is demonstrated by whealing with or without itching in the contact area with ice.

The laboratory workup for cold-induced urticaria patients includes a differential blood countand erythrocyte sedimentation rate or C-reactive protein (CRP). Additional diagnostic workups should include a search for underlying infections. However, there is no guidance on the clinical relevance of positive viral serology in cold-induced urticaria, these tests should only be done if indicated for the differential diagnosis.

What Is the Treatment for Cold Induced Urticaria?

Although there is no permanent cure for cold-induced urticaria, following a few simple steps can help manage the symptoms, they are-

• Avoiding Triggers- In some patients avoiding triggers is enough to reduce the impact of cold hives on their lives. This involves identifying the triggers and then seeing if it is possible to reduce exposure to them.

• Antihistamines- These are prescribed to patients in whom avoiding triggers is not a possibility. These medications reduce the amount of histamine produced in the body which in turn reduces the associated symptoms.

• Other Medications- This includes Omalizumab, which is an asthma drug that can be used to reduce symptoms in antihistamine-resistant cold-induced urticaria.

• Epinephrine- This is an emergency medication that is used to manage serious allergic reactions (anaphylaxis) when exposed to the cold. Patients who have had this experience should carry an epinephrine pen with them at all times to prevent a recurrence.

Apart from the above-mentioned recommendations, patients with cold-induced urticaria are to wear loose cotton clothing to reduce further irritations and use a topical itch cream to soothe itching.

Conclusion

Cold-induced urticaria is a rare condition that causes allergic reactions when exposed to the cold. People with cold-induced urticaria will develop raised welts when they come in contact with cold air, water, drinks, food, or objects. Individuals are advised to seek medical help if hives occur alongside swelling in the mouth and the airway.