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The Light is an initiative by the Sri Lanka College of Paediatricians aimed at advancing paediatric knowledge in Sri Lanka, benefiting both healthcare professionals and the general public.44/1, Gnanartha Pradeepa Mawatha, Last Modified April 3rd, 2025
Tharindi Suriapperuma1,2
1Department of Paediatrics, Faculty of Medicine, University of Kelaniya
2Colombo North Teaching Hospital Ragama
What is urticaria?
Urticaria is wheal formation that can occur anywhere on the body. It is characterized by well-circumscribed superficial swelling of variable size. The swelling and redness often improve in one area and then appear in another. Urticaria can tingle and itch but is not usually painful; however, sometimes, it can cause a burning sensation. It usually resolves within 1–24 hours.
What is angioedema?
Angioedema is sudden, profound swelling within the lower dermis and subcutis. It frequently occurs under the mucous membranes, such as the eyelids, face, lips, and oropharynx. Angioedema can be asymptomatic, painful with a burning or tight sensation, or, less commonly, itchy. It is slow to resolve and usually lasts up to 72 hours.
Classification of urticaria
Acute Urticaria: Symptoms for less than 6 weeks
Chronic Urticaria: Symptoms for more than 6 weeks
Chronic Spontaneous Urticaria – No identifiable trigger
Chronic inducible urticaria – Triggered by a specific stimulus
Acute urticaria is defined as episodes of urticaria with or without angioedema lasting less than 6 weeks. Acute urticaria without a trigger is called acute spontaneous urticaria, which is the most common form of urticaria in childhood. It is usually generalized. Having haemorrhagic lesions and arthralgia is suggestive of infection-induced urticaria. Following an acute urticaria episode, 25% of them develop recurrence with intercurrent viral infections.
Chronic urticaria is defined as urticaria with or without angioedema for most of the day during a period of 6 weeks or more. All ages are affected, however common in older children. Both sexes are equally affected. Urticaria is commonly involved in children with atopy.
In some children, this process is triggered by specific stimulus. Therefore, it is called chronic inducible urticaria. Common such triggers are listed in the box below. If no trigger is identified, it is called chronic spontaneous urticaria.
| Cold – following exposure to a cold object, air, fluid or wind | Pressure – wheals arising 3-12 hours following tight clothes – can be categorized as delayed pressure |
| Cholinergic urticaria – following physical activity, hot bath or spicy food, increasing body core temperature | Vibration – vibratory force |
| Aquagenic – contact with hot or cold water | Solar – UV and/or visible light |
| Contact – contact with an urticariogenic substance like tomatoes | Delayed pressure – |
| Symptomatic dermographism – wheals arising 1-5 minutes following mechanical shearing force | Contact – contact with urticariogenic substance like tomatoes |
Isolated angioedema could appear spontaneous or as a result of C1 esterase inhibitor deficiency or drug related (NSAIDs, antibiotics, ACE inhibitors).
How common is urticaria and angioedema?
The incidence of urticaria in children has been reported at between 2% and 7%, and the incidence of chronic urticaria in children is between 0.1% and 3%. Chronic urticaria is more common in older children and adolescents. Acute spontaneous urticaria is the most common form of urticaria in childhood. Chronic spontaneous urticaria is the most common subgroup of chronic urticaria in children.
Pathophysiology
Urticaria is a mast cell-driven disease. Histamine, cytokines, and platelet-activating factor are released by the activated mast cells. They induce vasodilatation and plasma extravasation, which manifest as wheal formation/urticaria and angioedema. Pruritus is due to histamine release and sensory nerve activation.
Diagnosis
It is a clinical diagnosis. A detailed history is vital in making the diagnosis of chronic spontaneous urticaria. Try to elicit triggering factors in history. Symptoms generally appear within 2 hours after exposure to the triggering factor. In chronic spontaneous urticaria, there should not be identifiable risk factors in history. Urticaria should improve when the trigger is eliminated in chronic inducible urticaria and food allergies and is not compatible with urticaria appearing and disappearing over a period.
Urticarial vasculitis leaves pigmentation in the skin and other types of urticaria resolve without leaving pigmentation. There can be other systemic symptoms in urticarial vasculitis.
Investigations
Chronic urticarial does not generally need extensive investigations. They are indicated depending on associated symptoms or suspicion of associated disease conditions.
Every child with acute or chronic spontaneous urticaria does not need to undergo investigations. If clinically indicated, a full blood count, ESR, total IgE level, thyroid function tests, autoantibodies, coeliac screen, liver function test, C1 esterase inhibitor level, and function should be done. Rarely, coeliac disease, autoimmune thyroiditis, type 1 diabetes mellitus, and other autoimmune diseases can associate with chronic urticaria. C1 esterase inhibitor deficiency should be suspected in children with isolated angioedema without urticaria.
Skin biopsy is not routinely indicated; however, it is considered if the features are consistent with urticarial vasculitis or mastocytosis.
Assessment of disease activity
Urticaria Activity Score: disease activity assessment in chronic urticaria
| Score | Wheal | Pruritus |
| 0 | None | None |
| 1 | Mild – <20 wheals for 24 hours | Mild – present but not troublesome |
| 2 | Moderate – 20-50 wheals for 24 hours | Moderate – troublesome but does not interrupt daily living or sleep |
| 3 | Intense – >50 wheals for 24 hours or large confluent areas of wheals | Intense – severe pruritus interferes with daily living or |
Total score is 0 – 6 for each day. Maximum 42/week
Angioedema activity score: disease activity assessment in chronic urticaria
| Score | Dimension | Answer options |
| – | Have you had a swelling episode in the last 24 h? | No, yes |
| 0 – 3 | At what time(s) of day were these swelling episode(s) present? (please select all applicable times) | Midnight–8 a.m., 8 a.m.–4 p.m., 4 p.m.–midnight |
| 0 – 3 | At what time(s) of day were these swelling episodes (s) present? (Please select all applicable times) | No discomfort, slight discomfort, moderate discomfort, severe discomfort |
| 0 – 3 | How severe is/was the physical discomfort caused by this swelling episode(s) (eg, pain, burning, itching)? | No restriction, slight restriction, severe restriction, no activities possible |
| 0 – 3 | Do/did you feel your appearance is/was adversely affected by this swelling episode(s)? | No, slightly, moderately, severely |
| 0 – 3 | How would you rate the overall severity of this swelling episode? | Negligible, mild, moderate, severe |
Total score is 0-15 for each day. Maximum 105/week.
Management of chronic urticaria
The goal of treatment is to achieve symptom-free control. The avoidance of triggers is the mainstay of management in chronic inducible urticaria. Symptomatic control with medications is shown below.
Oral non-sedating H1 antihistamine – start with the BNF recommended dose for the age
If inadequate response after 2 weeks
Increase the non-sedating H1 antihistamine dose up to 4 times the standard dose
If inadequate response after 4 weeks
Add-on therapy with leukotriene receptor antagonist – montelukast
Consider an alternative diagnosis if no response to high-dose antihistamine
Consider relevant investigations
Consider a short course of corticosteroids for severe exacerbations
Inadequate response after 2-4 weeks
Omalizumab as add-on therapy – not locally available
Inadequate response after 6 months, or if symptoms are intolerable
Consideration of switch to ciclosporin or tacrolimus
Cetirizine, levocetirizine, loratadine, desloratadine and fexofenadine are commonly used second-generation antihistamines. Cetirizine and desloratadine are licensed to be used in children from 1 year old. Rupatadine has a greater efficacy as it is a combined antihistamine and platelet-activating factor antagonist. It is licensed for children above 12 years.
NSAIDs and aspirin should be avoided. 1% menthol in aqueous cream may provide symptomatic relief.
Psychological stress can induce urticaria, and chronic urticaria can induce stress. There is a place for cognitive behavioural therapy, expecting to reduce stress-related exacerbations and to improve coping skills.
Maintenance therapy
Once a patient is totally symptom-free, it is recommended to continue the medication or medications required for symptom control for a minimum of 1-3 months before tapering the medications. The maintenance therapy can be extended in patients whose symptoms are difficult to control.
Standard drug doses are mentioned below as per the BNF. These doses can be increased up to 4 times of the standard doses where appropriate as monotherapy.
| 1 year | 2 -5 years | 6 – 11 years | 12 – 17 Years | |
| Cetirizine | 250micrograms/kg | 2.5 mg BD | 5 mg BD | 10 mg once daily |
| 2 -11 years (up to 31 kg ) | 2 -11 years (> 31 kg) | 12 -17 years | |
| Loratadine | 5 mg OD | 10 mg OD | 10mg OD |
| 6 -11 years | 12 – 17 years | |
| Fexofenadine | 30 mg BD | 120 mg OD |
| 2 – 5 years | 6 – 17 years | |
| Levocetirizine | 1.25 mg BD | 5 mg OD |
| 1 – 5 years | 6 -11 years | 12 – 17 years | |
| Desloratadine | 1.25 mg OD | 2.5 mg OD | 5 mg OD |
Key messages
Chronic urticarial is a condition associated with auto antibody formation and not all of them are of IgE-mediated allergic disease.
Chronic spontaneous urticaria is the most common subtype of chronic urticaria in children compared to chronic inducible urticaria.
First-generation antihistamines should be avoided where possible. They have significant anticholinergic and sedative side effects lasting more than 12 hours; however, the antipruritic effect lasts for 4-6 hours.
Monotherapy with a non-sedating H1 antihistamine administered up to 4 divided doses per day is superior to 2 different antihistamines given twice daily. In patients who do not tolerate higher doses of antihistamine, another antihistamine up to 4 times of the standard doses considered. If symptoms are still not under control, try addon therapy with montelukast.
Omalizumab is not locally available. Refractory cases need ciclosporin for 3-6 months with careful monitoring for renal functions.
There is no role for the use of topical steroids.
Steroids are not recommended for long-term use.
Tranexamic acid can be tried for symptom control in patients with troublesome angioedema.
Dietary restrictions are only beneficial in cases when a confirmed food allergy exists.
Chronic spontaneous urticaria carries significant impact on quality of life, therefore psychological support should be considered where appropriate.
Prognosis of chronic spontaneous urticaria
Children have a favourable prognosis compared to adults. Three years following the first presentation, 25% of them are disease-free and 96% are asymptomatic following 7 years. Children who respond to a standard dose of antihistamine, have a lower disease activity score, and have lower IgE levels achieve remission faster. Chronic spontaneous urticaria can recur several months or years of disease remission.
References