What are the other Names for this Condition? (Also known as/Synonyms)

• Urticaria, Chronic type

What is Chronic Urticaria? (Definition/Background Information)

• Urticaria is a common inflammatory condition of skin that results in weals (red skin sign with itching) 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
• Chronic Urticaria is a skin condition that usually lasts over 6 weeks. The signs and symptoms are observed long-term; a daily or periodic occurrence of weals may be noted. It is reported that between 25-30% of the individuals with acute urticaria may develop Chronic Urticaria
• The condition may be triggered by factors that include bacterial infections, certain medications, autoimmune conditions, and physical contact with certain substances. In many cases, no cause is established for Chronic Urticaria
• Any region of body skin may be affected, but usually the head and neck, chest and back, and arms and legs are involved. Intense itching may lead to skin ulceration and scarring, which can cause emotional stress from cosmetic concerns
• Chronic Urticaria is treated symptomatically, when required. The treatment may involve the use of topical ointments and creams, antihistamines, and immunosuppressive medication, in case of severe signs and symptoms. The prognosis with adequate treatment is usually good
• However, in a sizeable number of individuals, Chronic Urticaria is a long-term duration skin condition that may recur for years. This can affect the quality of life resulting in severe emotional stress

Who gets Chronic Urticaria? (Age and Sex Distribution)

• Chronic Urticaria is less common than acute form of urticaria. 0.5 to 2% of the population can get affected by this condition
• It is more often seen in adults than children. The maximum incidence is observed in the 4^th and 5^th decade (among 30-50 year’ age groups)
• Some indicates indicate a female predominance with a female-male ratio of 2:1
• There is no race or ethnic preference observed

What are the Risk Factors for Chronic Urticaria? (Predisposing Factors)

The risk factors for Chronic Urticaria include:

• Infections caused by bacteria that include Helicobacter pylori infection
• Roundworm infection affecting the stomach
• Intravenous contrast materials used during radiological studies
• Certain drug reactions, such as occurring from antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs), and painkillers (opiates)
• Food allergies including peanut allergy and shellfish allergy, in rare cases
• Alcohol consumption
• Use of substances such as caffeine, certain perfumes, detergents, etc.
• Other precipitating factors include pregnancy, contact with a variety of chemicals, exposure to sun, cold, exercise, etc.
• In some individuals, the condition is known to run in the families

The following autoimmune disorders are known to be associated with Chronic Urticaria:

• Systemic lupus erythematosus (SLE)
• Vitiligo
• Rheumatoid arthritis (RA)
• Graves’ disease
• Hashimoto’s thyroiditis
• Celiac disease
• Type 1 diabetes
• B12 vitamin deficiency anemia (or pernicious anemia)

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 Chronic 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.

• Chronic Urticaria may be caused by an underlying infection, through hypersensitivity on exposure to certain substances causing an allergic reaction, or due to body’s immune system attacking one’s own body
• In about 10-30% of the Chronic Urticaria cases, the cause is clearly identified. Consequently, in a majority of the individuals, the underlying cause of Chronic Urticaria is not well-established
• It is believed that in nearly 40-50% of the cases, it may be an autoimmune reaction, when the body’s own defense mechanism is triggered following exposure to a stimulus, resulting in the skin symptoms

Chronic Urticaria may be of the following subtypes:

• Chronic Inducible Urticaria (or Chronic Physical Urticaria): It occurs following a known trigger and may be of several types based on the specific and physical stimulus (such as pressure, exposure to sunlight, heat, or cold, contact, exercise, vibrations, etc.)
• Chronic Spontaneous Urticaria: It occurs without a known trigger (idiopathic), but the potential causes may include parasitic gastrointestinal (roundworm) infections and autoimmune conditions including celiac disease, RA, SLE, insulin-dependent diabetes mellitus, pernicious anemia, etc.

What are the Signs and Symptoms of Chronic Urticaria?

The signs and symptoms of Chronic Urticaria include the following:

• Initially, localized itching of skin may be observed and 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)
• The weals may change in shape and size; weals may be spherical, arranged in a random form, or ring-like pattern
• In some individuals, there may be a burning sensation accompanying itching
• Individual weals may vary in size from a few mm to few cm; but, numerous weals covering a large area of the body skin are usually observed
• The weals may last for several minutes to even up to a day; few cases are non-itchy
• Urticaria can occur in any part of the body and can be focal or extensive
• In some cases, it can lead to hyperpigmentation (increased skin pigmentation) or hypopigmentation (decreased skin pigmentation)
• When pressure is applied on the hives, 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: It may or may not occur with Chronic Urticaria
  • Angioedema is swelling due to fluid accumulation in the mucus membranes or deep within the skin. This lasts for up to 3 days, it can be also itchy
  • Angioedema usually occurs focally; most common sites are the head and neck region, hands, feet, and genitalia. In the head and neck region, it can involve the eyelids, lips, oral cavity, or larynx
• Pain, pain on touch

The itching and symptoms may be worsened by various substances or factors such as viral infections, tight clothing, food and drug allergies, and heat.

How is Chronic Urticaria Diagnosed?

A diagnosis of Chronic Urticaria may involve the following tests and examinations:

• Physical examination of the skin and medical history evaluation
• Skin allergy test
• Radioallergosorbent test (RAST); to determine the specific allergy-causing substance 
• 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 autoimmune conditions and infections
• Physical hot and/or cold provocation test
• 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

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.

A differential diagnosis may be considered to eliminate certain skin conditions, prior to arriving at a definitive diagnosis. The following conditions may be considered:

• Chronic itching due to a variety of other causes
• Contact dermatitis
• Dermatitis herpetiformis
• Drug eruptions of skin
• Eczema
• Erythema multiforme
• Insect bites
• Pemphigoid
• Pityriasis rosea
• Polymorphous eruption of pregnancy
• Urticarial vasculitis

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 Chronic Urticaria?

The following complications of Chronic 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 Chronic Urticaria can cause disruption in sleep 
• 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 Chronic Urticaria can result in anaphylactic shock, which is a potentially life-threatening medical condition

How is Chronic Urticaria Treated?

The treatment depends upon the severity of the signs and symptoms and it includes the avoidance of triggers. The treatment may be specific to the particular trigger causing the symptoms. In severe cases, an allergy specialist may be consulted. The treatment measures for Chronic Urticaria include:

• Cooling the skin using moist towels
• Topical steroid treatment that may include moisturizing creams to soothe the condition
• Systemic steroids
• Anti-histamine therapy
• Immunosuppression therapy for severe symptoms: Use of immune modulators or medications that modulate immunity
• Patient education in understanding the condition is beneficial
• Omalizumab injections are quite effective
• Stress management for severe and chronic conditions

In some cases of idiopathic Chronic Urticaria, there is no cure and only management of the symptoms, as they occur, are undertaken.

How can Chronic Urticaria be Prevented?

Although Chronic 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:

• Appropriately treating acute urticaria
• Treating viral and bacterial infections early and appropriately
• Treating autoimmune disorders adequately
• Avoiding exposure to drugs that cause the condition
• Avoiding the use of materials that cause Chronic Urticaria; informing the healthcare provider suitably of any food, drug, or contrast material allergies (if these are identified earlier)

However, in many cases, no causative factors are identified and Chronic Urticaria is not preventable.

What is the Prognosis of Chronic Urticaria? (Outcomes/Resolutions)

• The prognosis of Chronic Urticaria is generally good with suitable treatment, although the condition is known to recur in some individuals
• Anaphylactic reaction/shock can be fatal without treatment

Additional and Relevant Useful Information for Chronic Urticaria:

Please visit our Skin Care Health Center for more physician-approved health information:

http://www.dovemed.com/healthy-living/skin-disorders/