Chronic Urticaria in Adults

1. Background Information / Important Principles

  • Chronic idiopathic urticaria has a prevalence of 1-2%.
  • It can be alarming and distressing for the patient, both because of symptoms and appearance.
  • Chronic urticaria is not due to an allergy. The cause is not fully understood but in many patients, an autoimmune aetiology is likely.
  • It is a clinical diagnosis. In the absence of other symptoms, no tests are required. No cause can be found in 80-90% of patients.
  • A careful history is important and should include atopy (25% of patients), auto-immune conditions, infections, drugs, connective tissue diseases and malignancy.
  • Chronic urticaria can be exacerbated by factors such as stress and some medication such as aspirin, NSAIDS and codeine.
  • Ask for additional physical triggers such as heat, cold, water, sunlight, exercise, vibration and pressure (dermographism).
  • 50% of patients have associated angioedema, which rarely affects the airway – see referral criteria for further information
  • Angioedema without urticaria can be drug induced (ACE inhibitors) or caused by hereditary or acquired C1 esterase inhibitor deficiency. There may also be episodic abdominal pain in these patients. A small percentage of patients with chronic spontaneous urticaria have angioedema only
  • Chronic urticaria is associated with auto-immune diseases, most commonly thyroid disease. The association with malignancy is less clear, and investigations should only be performed if there are other symptoms.
  • Lesions appear and resolve within 24 hours. Where individual lesions last longer than a day, the diagnosis may be urticarial vasculitis. Brown patches may indicate urticaria pigmentosa. See referral criteria for further information.
  • Chronic urticaria is  self-limiting in the majority of patients.30-50% will have resolution in one year, the average of disease duration is 2-5 years, up to 30% persist for longer than 5 years. Those with more severe symptoms are less likely to resolve early.

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2. Information Resources for Patients and Carers

British Association of Dermatologists patient information – Urticaria and Angioedema

3. Development and Updates to this Pathway

Developed: June 2017

4. Training and Further Resources

https://www.uptodate.com/contents/search?search=chronic+Urticaria&x=5&y=0

http://www.pcds.org.uk/clinical-guidance/urticaria-and-angioedema

http://www.dermnetnz.org/topics/chronic-urticaria/

http://emedicine.medscape.com/article/1050052-overview

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5. Patient Presents with Chronic Urticaria

Chronic idiopathic urticaria, also called spontaneous urticaria, is defined by episodic symptoms lasting for more than six weeks. Although idiopathic, there may be additional physical triggers. Angioedema may be present, affecting the face, genitals and extremities, but rarely the airway. Chronic urticaria is intensely pruritic, interferes with sleep and all daily activities and adversely affects the patient’s daily functioning and quality of life.

Photos (provided by Primary Care Dermatology Society - PCDS)


Figure 1 Chronic urticaria – wheal and flare (wheal (black arrow) and flare (blue arrow))

 


Figure 2 Chronic urticaria with dermographism (urticarial plaque (black arrow) with dermographism (blue arrow))



Figure 3
Chronic urticaria



Figure 4
Chronic urticaria



Figure 5
Chronic urticaria



Figure 6
Chronic urticaria

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6. Red Flags

Airway issues – send to casualty

Angioedema without urticaria - ? ACE inhibitor - stop!

7. Management

Reassure patient and encourage self-management.

Investigations:

  • Chronic urticaria is a clinical diagnosis.
  • Careful history: investigations if indicated by other symptoms.
  • Consider thyroid function test if autoimmune background and/or family history.
  • H-pylori if history of dyspepsia.
  • There is no place for patch or RAST testing

Management:

  • Drugs such as aspirin, NSAIDS and codeine can trigger symptoms and should be stopped. Remember to consider over the counter medications.
  • ACE inhibitor induced angioedema can be life threatening. Stop ACE inhibitor!
  • Avoid physical triggers if relevant from history.
  • Keep the skin cool and try to reduce stress.

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8. Treatment

Step 1:

  • Start with standard dose of non-sedating antihistamine.  Use cetirizine or loratadine as first line choices. If response is inadequate, increase dose on alternate days up to 4 x standard dose (unlicensed use but established practice - European guidelines state safety in adults).
  • Caution with higher doses in underlying renal and liver disorders - select antihistamine accordingly.
  • 1%, 2% or 5% menthol in aqueous cream (Dermacool) as required (topical antipruritic and cooling cream).

Step 2:

  • If inadequate response to one non-sedating antihistamine, substitute with another (may still work). Fexofenadine may be considered as a second line choice.

Step 3:

  • Add a sedating antihistamine if symptoms cause sleep disturbance. Hydroxyzine, promethazine or chlorphenamine are all suitable choices.
  • Caution with hydroxyzine. The maximum adult daily dose of hydroxyzine is now 100mg and 50mg for the elderly (if use cannot be avoided). Do not prescribe hydroxyzine to people with a prolonged QT interval or risk factors for QT interval prolongation.

Step 4:

  • Add leukotriene receptor antagonist such as montelukast 10mg daily (unlicensed use).

 
Adjustment to timing of medication can be helpful to ensure that the highest drug levels are obtained when urticaria is anticipated.

Management of Acute Symptoms
Even if control is generally achieved, patients can still have flares. For very severe flares, a short course of oral steroid can occasionally be used, e.g. prednisolone 30mg OD for 4 days, 20mg OD for 3 days, then 10mg OD for 3 days.

Rebound worsening may occur with tapering or discontinuation of systemic steroids. In this case, return to the last effective dose and then taper again more gradually. Some authors suggest that once the dose has been reduced to 20mg daily, the frequency of administration may be changed to every other day, then the dose tapered further. Long term steroids must be avoided

Ongoing Management with Antihistamines
Continue with effective treatment until symptom free for 6 weeks. Cautiously reduce/withdraw. If symptoms recur, return to last effective dose and taper more gradually.

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9. Referral Criteria

  • Problems with diagnosis or treatment as outlined below.

10. Referral to Community Clinic

Criteria for referral to Community Dermatology clinic:

  • If there is diagnostic uncertainty and none of the secondary care referral criteria (listed below) are present.

11. Referral to Secondary Care

Criteria for referral to Secondary Care clinic:

  • Failure to respond to treatment consisting of steps 1-4 outlined in box 8 – referral to immunology or dermatology for consideration of 2. line treatments, eg. immunosuppressive / biological agents.
  • Individual lesions lasting longer than a day: consider urticarial vasculitis – refer to secondary care dermatology.
  • Lesions leaving brownish marks: consider urticaria pigmentosa – refer to secondary care dermatology.
  • Urticaria with other inflammatory skin lesions – if not clearly eczema, consider lupus erythematosus – refer to secondary care dermatology.

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