What are the other Names for this Condition? (Also known as/Synonyms)
- Cold Contact Urticaria
- Cold-Induced Hives
- Cold-Induced Urticaria
What is Cold Urticaria? (Definition/Background Information)
- Urticaria is a common inflammatory condition of skin that results in weals (red skin sign with itching known as hives or urticaria) and/or angioedema (swelling due to fluid accumulation). It is seen in individuals of a wide age group and may be described as acute or chronic, based on the duration of the signs and symptoms
- Cold Urticaria is a common type of urticaria mostly seen in cold climates in which skin rashes develop following exposure of skin to cold environments. The exposure may be of any form - via cold air, cold water, or through direct contact of ice or cold surfaces with skin
- Cold Urticaria forms a subset of chronic inducible urticaria, a form of chronic urticaria, in which the signs and symptoms usually occur within a few minutes of exposure to the stimuli (or a known trigger). It can be a life-threatening condition in some individuals
- The condition is also known as Cold-Induced Hives and is generally seen in young adults, more so in females. Exposure to cold temperatures may take place while working in air-conditioned spaces, cold and chilly weather, or after swimming in cold waters. The skin rashes appear almost within minutes of exposure to cold
- The body skin exposed to cold is typically affected. Intense itching may lead to skin ulceration and scarring, which can cause emotional stress from cosmetic concerns. The skin rashes and other symptoms disappear within 1-2 hours (for acquired Cold Urticaria), when the body temperature returns to normal levels. However, in the case of inherited forms, they may last for about 2 days
- The prognosis of Cold Urticaria with adequate treatment is usually good, although the condition can recur. In a few individuals, complications including breathing difficulties, shock, and anaphylaxis may develop, which can even result in death
Who gets Cold Urticaria? (Age and Sex Distribution)
- Cold Urticaria is a common skin condition that can affect both children and adults, though it is more often seen in the 18-25 year’ age group
- Both males and females are affected, but the male-female ratio is 1:2 (more cases are reported in females)
- There is no race or ethnic preference observed, but Cold Urticaria is more common in the colder climates of the world (cold European and American regions). More number of cases are reported during the winters
What are the Risk Factors for Cold Urticaria? (Predisposing Factors)
The main risk factor for Cold Urticaria is exposure of the body skin to cold, freezing conditions, which may occur following:
- Working or staying in air-cooled offices or homes
- Exposure to cold water while swimming (most common cause of Cold Urticaria), while showering, or taking a bath
- Living or travelling to cold countries and regions; being exposed to cold weather, freezing temperatures, chilly winds, etc.
- Occupational exposure - handling ice blocks, using cold storage facilities, freezers
- Being administered a cold therapy or cryotherapy
- Skin contact with certain chemicals causing an endothermic reaction (loss of heat) in the body, such as rubbing alcohol
- Drinking cold water or taking ice cream
- Family history of Cold-Induced Urticaria
It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.
Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.
What are the Causes of Cold Urticaria? (Etiology)
Urticaria can be caused by a variety of factors. The condition develops due to the release of chemical substances, such as histamines, platelet activating factor, and cytokines (example bradykinin), from inflammatory cells called mast cells and basophils.
- Cold Urticaria is a form of physical urticaria that occurs following a known trigger, which is exposure to cold air or water in this case. Nevertheless, the exact reason for development of this skin condition is not well-understood. The skin rashes occur following the release of histamines in response to stimuli (in this case, exposure to cold conditions)
- There are two main forms of Cold Urticaria - primary and secondary
- The primary form is known as Primary Cold Contact Urticaria and it develops following contact of skin with chilly air, cold water, or ice. The cause of this condition is unknown (or idiopathic)
- The secondary form is known as Secondary Cold Contact Urticaria and it develops secondary to an underlying illness (i.e., in the presence of infections such as chicken pox, hepatitis infection, or infectious mononucleosis) or blood disorder (such as lymphatic tissue sarcoma, cryoglobulinemia, Raynaud's phenomenon, or chronic lymphocytic leukemia)
- Cold Urticaria is also based on whether it occurs sporadically or is inherited. Hence, it is also subdivided as acquired Cold Urticaria (most common form) and familial Cold Urticaria (very rare form) respectively
- Any temperature that is lower than normal body temperature may constitute a cold stimulus, and it need not be very cold or freezing temperatures. The threshold temperature of tolerance for each individual (which may vary from one individual to another), dictates the temperature below which hives/skin rashes and other symptoms develop in that particular individual
Lesser known forms of Cold Urticaria include reflex Cold Urticaria and Familial Cold Urticaria (or Familial Cold Autoinflammatory Syndrome, FCAS, an autosomal dominant disorder).
Note: The term cold allergy is sometimes used to describe Cold Urticaria. However, since cold is merely an absence of heat, and not a physical material, the body does not produce antibodies against cold (because the body is not exposed to any associated allergen). Thus, it is incorrect to use the term ‘cold allergy’ to describe this form of urticaria.
What are the Signs and Symptoms of Cold Urticaria?
The signs and symptoms of Cold Urticaria may be mild or severe, localized or diffused (and sometimes, extensive), and can vary from one individual to another. The onset of symptoms may be at different temperatures for affected individuals, based on their specific tolerance level. Familial Cold Urticaria symptoms may be observed even after 1-2 days of cold exposure.
The signs and symptoms of Cold-Induced Urticaria that occur within a few minutes (2-5 minutes) of stimuli exposure may include the following:
- Initially, localized itching of skin may be observed; the skin may appear swollen
- Formation of skin rashes or weals (wheals) that are associated with redness (or pinkish discoloration) and intense itching
- The skin surrounding the affected area (weal) may be normal skin color or appear red (erythema). Only the exposed portions of the skin display the weals
- Depending on the exposed area, the condition can be localized or widespread or extensive
- In some individuals, there may be a burning sensation accompanying itching
- The weals may change in shape and size and can last for a couple of hours after exposure
- Individual weals may vary in size from a few mm to few cm; but, numerous weals covering a large area of the body skin may be observed
- In some cases, it can lead to hyperpigmentation (increased skin pigmentation) or hypopigmentation (decreased skin pigmentation)
- When pressure is applied on the affected skin area, it blanches (becomes pale or white in color)
- Scratching can result in worsening of the symptoms and lead to further skin complications such as ulceration and secondary infections
- Angioedema formation, which is the swelling due to fluid accumulation in the mucus membranes or deep within the skin
- Pain, pain on touch
- Along-with skin symptoms, the following may be also noted:
- Fatigue and tiredness
- Pain in the joints, body aches
- Breathing difficulties; respiratory problems may also occur following the intake of cold water or ice cream. The throat and food-pipe may be affected and it can lead to choking sensation or respiratory obstruction
- Increased or irregular heart rate
- Decreased blood pressure (hypotension)
- Abdominal pain
- Signs and symptoms of any underlying hematological disorder or infection
- Individuals, may have other forms of inducible (physical) urticaria such as cholinergic urticaria and dermographism
Severe exposure, causing Cold Urticaria, which occurs on plunging into very cold water can result in cardiovascular shock and loss of consciousness. Due to this, the individual is at a greater risk for drowning.
How is Cold Urticaria Diagnosed?
A diagnosis of Cold Urticaria may involve the following tests and examinations:
- Physical examination of the skin and medical history evaluation
- Skin allergy test
- Radioallergosorbent (RAS) test; to determine the specific allergy-causing substance
- Ice cube test, when the healthcare professional places an ice cube on the arm to test for the presence of urticaria
- Physical cold provocation test
- Blood test to detect various antibodies against allergens including:
- Complete blood count (CBC) including eosinophil count since, there may be increased number of eosinophils (peripheral eosinophilia)
- Antinuclear antibody (ANA) test
- Erythrocyte sedimentation rate (ESR) test
- Immunoglobulin E (IgE) levels in blood
- Serum level of complement
- Serum C-reactive protein blood test
- Tests to rule-out other underlying blood disorder or infections (if necessary)
- Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
- A differential diagnosis may be considered to eliminate certain skin conditions, prior to arriving at a definitive diagnosis
Note: The healthcare provider may induce symptoms by administering the triggers in a controlled fashion. This procedure is performed under close medical supervision, either in an out-patient healthcare setting, or as an in-patient setting in a hospital. Since, potentially life-threatening symptoms may develop during this procedure, access to emergency medical resources is generally warranted. Hence, such testing should not be performed at home.
Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.
What are the possible Complications of Cold Urticaria?
The following complications of Cold Urticaria may be noted:
- Intense scratching may result in skin scarring
- Itching can lead to ulceration of skin, causing superimposed bacterial and fungal skin infections
- Severe cases can cause disruption in sleep
- In some individuals, the condition may be severe and involve the entire body and become a life-threatening emergency
- Cosmetic concerns leading to stress
- Frequent episodes of urticaria can affect the quality of life, which can result in severe stress. This severe stress can in-turn be a trigger for further worsening of the condition
- Involvement of the larynx due to angioedema, may be life-threatening and a medical emergency. If the larynx is involved, it can result in sudden breathing difficulties due to laryngeal edema
- Severe condition can result in anaphylactic shock, which is a potentially life-threatening medical condition
How is Cold Urticaria Treated?
The treatment depends upon the severity of the signs and symptoms and the type of Cold Urticaria. It primarily includes the avoidance of known triggers. In severe cases, an allergy specialist may be consulted. The treatment measures for Cold Urticaria include:
- Minimizing exposure to cold: Avoiding any situation that may lead to a rapid drop in one’s temperature, whenever possible
- Patient education in understanding the condition is beneficial
- If cold exposure is anticipated, then prophylactic measures may be considered, such as the use of antihistamines (high-dose)
- Systemic steroids
- Anti-histamine therapy to prevent skin rashes
- Cold desensitization treatment, where the body threshold temperature level is improved through controlled exposure to cold
- Use of oral antibiotics and synthetic hormones
- Stress management for severe and chronic conditions
How can Cold Urticaria be Prevented?
Although Cold Urticaria may not be prevented, further and future flare-ups may be avoided, if the triggers that cause the condition are known and recognized. In such cases, the following may be considered:
- Adequately protecting oneself against cold weather
- Limiting exposure to cold environments such as A/C work spaces and rooms
- Avoiding cold foodstuffs, drinks, and ice creams
- Informing the healthcare provider of Cold Urticaria in advance may prevent the use of cold infusions (intravenous fluids)
Some patients may be advised to use self-administered epinephrine injections for anaphylactic shock, or as and when indicated.
What is the Prognosis of Cold Urticaria? (Outcomes/Resolutions)
- The prognosis of Chronic Inducible Urticaria is generally good with suitable early treatment. It is reported that in many individuals (around 50%), the condition is known to last for 5-10 years and then disappear
- In some individuals, the condition is recurrent and chronic and may last their entire lifetime
- Severe symptoms, the development of complications, and a lack of access to proper and prompt treatment can result in fatal outcomes
- Anaphylactic reaction/shock can be fatal without treatment
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