Dermatology Made Easy is based on the most popular topics from DermNet NZ's vast array of material. The book combines the essential focus of the ‘Made Easy’ book series with the authority and knowledge base of DermNet NZ's unparalleled resources.
Author and Chief Editor: Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, January 2015.
Urticaria is characterised by weals (hives) or angioedema (swellings, in 10%) or both (in 40%). There are several types of urticaria. The name urticaria is derived from the common European stinging nettle 'Urtica dioica'.
A weal (or wheal) is a superficial skin-coloured or pale skin swelling, usually surrounded by erythema (redness) that lasts anything from a few minutes to 24 hours. Usually very itchy, it may have a burning sensation.
Angioedema is deeper swelling within the skin or mucous membranes, and can be skin-coloured or red. It resolves within 72 hours. Angioedema may be itchy or painful but is often asymptomatic.
Chronic urticaria is urticaria with daily or episodic weals or angioedema that is present for more than 6 weeks.
Chronic urticaria may be spontaneous or inducible. Both types may co-exist.
Inducible or physical urticaria includes:
Chronic spontaneous urticaria affects 0.5–2% of the population; in some series, two-thirds are women. Inducible urticaria is more common. There are genetic and autoimmune associations.
Urticarial weals can be a few millimetres or several centimetres in diameter, coloured white or red, with or without a red flare. Each weal may last a few minutes or several hours, and may change shape. Weals may be round, or form rings, a map-like pattern or giant patches.
Urticaria can affect any site of the body and tends to be distributed widely.
In chronic inducible urticaria, weals appear about 5 minutes after the stimulus and last a few minutes or up to one hour. Characteristically, weals are:
The weals are more persistent in chronic spontaneous urticaria, but each has gone or has altered in shape within 24 hours. They may occur at certain times of day.
Visual analogue scales can be used to record and compare the degree of itch.
The activity of chronic urticaria can be assessed using the UAS7 scoring system. The daily weal/itch scores are added up for 7 days; the maximum score is 42.
The emotional impact of urticaria and its effect on quality of life should also be assessed. The Dermatology Life Quality Index (DLQI) and CU-Q2oL, a specific questionnaire for chronic urticaria, have been validated for chronic urticaria, where sleep disruption is a particular problem.
Weals are due to release of chemical mediators from tissue mast cells and circulating basophils. These chemical mediators include histamine, platelet-activating factor and cytokines. The mediators activate sensory nerves and cause dilation of blood vessels and leakage of fluid into surrounding tissues. Bradykinin release causes angioedema.
Several hypotheses have been proposed to explain urticaria. The immune, arachidonic acid and coagulation systems are involved, and genetic mutations are under investigation.
Chronic spontaneous urticaria is mainly idiopathic (cause unknown). An autoimmune cause is likely. About half of investigated patients carry functional IgG autoantibodies to immunoglobulin IgE or high-affinity receptor FcεRIα.
Chronic spontaneous urticaraia has also been associated with:
Weals in chronic spontaneous urticaria may be aggravated by:
Inducible urticaria is a response to a physical stimulus.
|Type of inducible urticaria||Examples of stimuli inducing wealing|
|Delayed pressure urticaria||
Chronic urticaria is diagnosed in people with a long history of daily or episodic weals that last less than 24 hours, with or without angioedema. A family history should be elicited. A thorough physical examination should be undertaken to evaluate the cause.
There are no routine diagnostic tests in chronic spontaneous urticaria apart from blood count and C-reactive protein (CBC, CRP), but investigations may be undertaken if an underlying disorder is suspected.
The autologous serum skin test is sometimes carried out in chronic spontaneous urtciaria, but its value is uncertain. It is positive if an injection of the patien's serum under the skin causes a red weal.
Investigations for a systemic condition or autoinflammatory disease should be undertaken in urticaria patients with fever, joint or bone pain, and malaise. Patients with angioedema without weals should be asked if they take ACE inhibitor drugs and tested for complement C4; C1-INH levels, function and antibodies; and C1q.
Biopsy of urticaria can be non-specific and difficult to interpret. The pathology shows oedema in the dermis and dilated blood vessels, with variable mixed inflammatory infiltrate. Vessel-wall damage indicates urticarial vasculitis.
The main treatment for chronic urticaria in adults and in children is with an oral second-generation antihistamine chosen from the list below. If the standard dose (eg 10 mg for cetirizine) is not effective, the dose can be increased fourfold (eg 40 mg cetirizine daily). There is not thought to be any benefit from adding a second antihistamine.
Although systemic treatment is best avoided during pregnancy and breast feeding, there have been no reports that second-generation antihistamines cause birth defects. If treatment is required, loratidine and cetirizine are currently preferred.
Conventional first-generation antihistamines such as promethazine or chlorpheniramine are no longer recommended for chronic urticaria:
In addition to antihistamines, the triggers for urticaria should be avoided where possible. For example:
The physical triggers for inducible urticaria should be minimised; see examples below. However, symptoms often persist.
Patients with chronic urticaria that has failed to respond to maximum-dose second generation oral antihistamines taken for 4 weeks should be referred to a dermatologist, immunologist or medical allergy specialist.
Other treatments that are sometimes used off-label in chronic urticaria include:
Long-term systemic corticosteroids are not recommended, as high doses are required to reduce symptoms of urticaria and they have inevitable adverse effects that can be serious.
Although chronic urticaria clears up in most cases, 15% continue to have wealing at least twice weekly after 2 years.
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