Referral

Urticaria, Angioedema and Mastocytosis

Scope

This guideline refers to children under 18 who may require further investigation and management of urticaria, angioedema or mastocytosis.

Chronic Urticaria occurs in 3% children, and acute urticaria occurs in 4.5-15% children.

Angioedema is much rarer occurring in 1/5000 patients.

Urticaria alone occurs in 50% of patients, angioedema with urticaria in 40% patients and angioedema alone in 10%.

The true incidence of mastocytosis is unknown

Out of scope

See the following pathways for other allergic or atopic conditions:

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History and Examination

History and examination of the child suggestive of urticaria or angioedema:

Urticaria is characterised by fluctuating weals and/or angio-oedema. A weal consists of three typical features:

  • a central swelling of variable size (from a few millimetres to hand-sized lesions), almost invariably surrounded by a reflex erythema
  • associated itching or, sometimes, burning sensation
  • a fleeting nature, with the skin returning to its normal appearance, usually within 1–24 hours

Angio-oedema is characterised by:

  • a sudden, pronounced swelling of the lower dermis and subcutis, which is pale rather than pink and may be painful rather than itching
  • frequent involvement below mucous membranes
  • resolution that is slower than for weals and can take up to 72 hours

The history must rule any possible triggers such as food (see food guideline), latex, stings, and drugs.

Please ensure you check for infections:

  • childhood viral illnesses
  • ebstein Barr virus
  • viral hepatitides
  • parasites

Consider physical causes

  • cold, heat, pressure, vibration, exertion

Check

  • the drug history including NSAIDs and opiates.
  • Any treatment(s) tried and response to the treatment(s)
  • A family history of urticaria or atopy

Differential Diagnosis - include:

  • Atopic eczema
  • Contact dermatitis
  • Chronic pruritis
  • Erythema multiforme minor
  • Insect bite or sting
  • Pemphigoid (bullous) and dermatitis herpetiformis
  • Urticaria Pigmentosa
  • Urticarial vasculitis – see red flag section

  • Airway compromise with angioedema +/- urticaria
    • treat as anaphylaxis following the resuscitation council guidelines
  • Consider an underlying vasculitic process:
    • in vasculitis, lesions remain for longer than 24 hours and are painful, non-blanching, and palpable (leaving a residual pigmented lesion, such as petechial haemorrhage, purpura, or bruising).
    • there may be systemic symptoms, such as fever, malaise, and arthralgia. Causes include infection (hepatitis B or C, glandular fever, or streptococcal infection), certain drugs (for example penicillins, fluoxetine, thiazide diuretics, allopurinol, quinolones, or carbamazepine), autoimmune disease, paraproteinaemia, and malignancy.
  • Consider urticarial pigmentosa (commonest presentation of cutaneous mastocytosis)
    • the skin becomes inflamed and red when stroked (Derier's sign) and has hyperpigmented macules and papules.

Urticaria pigmentosa - British Association of Dermatologists

Baseline investigations can include

  • Full blood count (FBC)
  • Erythrocyte sedimentation rate and C-reactive protein (ESR +/- CRP)
  • Liver Function Tests
  • Renal Function Tests
  • Thyroid function and thyroid antibodies

A non-sedating antihistamine should be started (syrup or tablet, as per BNFc doses)

Chapter 13 - Skin

If this is insufficient to control the symptoms after a month the dose can be doubled.

The BSACI management of chronic urticaria and angioedema algorithm may be helpful.

Referral Criteria

Please refer in to the paediatric allergy clinic if:

  • Urticaria last for more than 4 weeks
  • Urticaria does not respond to regular daily double doses of non-sedating antihistamine
  • Angioedema occurs alone
  • Wheals fade to leave pigmentary changes
  • Wheal occurs on rubbing freckles or macules

Referral Instructions

e-Referral Service Selection
  • Specialty: Children & Adolescent
  • Clinic Type: Allergy
  • Service: DRSS-S-C&A (not for patient attendance)-Devon ICB-15N

Referral Forms

DRSS referral form

Torbay and South Devon seeking advice form

Patient Information

NHS.UK - Hives
AllergyUK – Urticaria

NHS.UK - Angioedema

GP Information

NICE - Urticaria
NICE - Angio-oedema and anaphylaxis

NICE – CKS Urticaria

NICE – Angio-oedema without anaphylaxis

Pathway Group

This guideline has been signed off on behalf of NHS Devon.

Publication date: August 2017

Updated: January 2025