Urticaria
Common condition characterised by transient, pruritic, erythematous wheals (hives) caused by mast cell degranulation and histamine release. Classified as acute (<6 weeks) or chronic (≥6 weeks). Acute urticaria is usually triggered by infection, drugs, or food. Chronic spontaneous urticaria (CSU) is often autoimmune. Non-sedating antihistamines are first-line treatment.
Key Facts
Individual wheals resolve within 24 hours (if >24 hours, consider urticarial vasculitis — biopsy) Acute urticaria (<6 weeks): triggers include infection (viral — most common in children), drugs (NSAIDs, antibiotics), food, insect stings Chronic spontaneous urticaria (CSU, ≥6 weeks): autoimmune in ~50% (anti-FcεRI or anti-IgE autoantibodies); rarely allergic First-line: non-sedating antihistamine (cetirizine 10mg OD or fexofenadine 180mg OD); up-dose to 4× licensed dose if needed Omalizumab (anti-IgE): third-line for CSU refractory to high-dose antihistamines — NICE TA339 Urticarial vasculitis: wheals lasting >24 hours, painful (not just itchy), purpuric; may leave bruising; check complement — hypocomplementaemic form associated with SLE AVOID: identifying 'allergy' triggers in CSU — extensive allergy testing is NOT recommended (most CSU is autoimmune, not allergic) Physical urticarias: dermatographism, cold urticaria, cholinergic (exercise/heat), pressure urticaria — treat with antihistamines
Overview
Key Facts
Urticaria is extremely common, affecting ~15–20% of the population at some point. The key clinical distinction is acute vs chronic, as this determines investigation and management approach.
Epidemiology
- Lifetime prevalence: ~15–20%
- Acute urticaria: very common, especially in children
- CSU: prevalence ~0.5–1%; female:male 2:1; peak age 20–40 years
- CSU duration: median 2–5 years; ~50% resolve within 5 years
Aetiology
Acute: viral infection (commonest cause in children), drugs (NSAIDs, antibiotics, opioids), food (nuts, shellfish, egg), insect stings, latex Chronic spontaneous: autoimmune (~50%; autoantibodies to FcεRI or IgE), idiopathic (~50%); NOT food allergy Physical: dermatographism (most common physical urticaria), cold, cholinergic, pressure, solar, aquagenic
Pathophysiology
- Mast cell degranulation → histamine, leukotrienes, prostaglandins → vasodilation, increased vascular permeability → wheal formation
- Autoimmune CSU: IgG autoantibodies to high-affinity IgE receptor (FcεRI) on mast cells → chronic activation
- Physical urticarias: specific physical stimulus triggers mast cell degranulation
Clinical Presentation
Typical Wheals
- Raised, erythematous, pruritic
- Variable size (mm to cm)
- Individual wheals resolve within 24 hours leaving normal skin
- Flare-and-wheal response
Angioedema
- Deep tissue swelling (lips, eyelids, tongue, hands, feet, genitalia)
- May accompany urticaria (~40% of CSU patients)
- Lasts 24–72 hours
Physical Urticarias
- Dermatographism: linear wheals from stroking/scratching skin
- Cold urticaria: wheals after cold exposure; risk of anaphylaxis in cold water
- Cholinergic: small 2–3mm wheals after exercise, heat, emotional stress
Red Flags
- Anaphylaxis (urticaria + hypotension, bronchospasm, airway compromise)
- Wheals lasting >24 hours (urticarial vasculitis)
- Angioedema without urticaria (consider hereditary angioedema — C1-INH deficiency)
- Wheals that leave bruising/purpura
Differential Diagnosis
| Diagnosis | Key Features | Investigation |
|---|---|---|
| Urticarial vasculitis | Wheals >24h, painful, leave bruising | Skin biopsy, complement |
| Anaphylaxis | Urticaria + cardiovascular/respiratory compromise | Clinical emergency |
| Hereditary angioedema | Angioedema WITHOUT urticaria, family history | C4, C1-INH level and function |
| Erythema multiforme | Target lesions, fixed, may have mucosal involvement | Clinical, biopsy |
| Contact urticaria | Wheals at contact site (e.g. latex) | Clinical history, SPT |
Diagnosis / Investigation
Acute Urticaria
- Usually NO investigations needed — clinical diagnosis
- Consider allergy testing (specific IgE, skin prick test) if clear trigger suspected and future avoidance needed
Chronic Spontaneous Urticaria
- FBC + differential: eosinophilia (parasites), basopenia
- CRP/ESR: raised in urticarial vasculitis, autoinflammatory
- TFTs: thyroid autoimmunity associated with CSU (~10%)
- Total IgE: may help predict omalizumab response
- Autologous serum skin test (ASST): research tool for autoimmune CSU
- Extensive allergy testing NOT recommended in CSU
If Urticarial Vasculitis Suspected
- Skin biopsy: leucocytoclastic vasculitis
- Complement (C3, C4): low in hypocomplementaemic urticarial vasculitis (association with SLE)
- ANA, anti-dsDNA: if SLE suspected
Management
Acute Urticaria
- Identify and avoid trigger if clear
- Non-sedating antihistamine: cetirizine 10mg OD, loratadine 10mg OD, or fexofenadine 180mg OD
- Short course oral prednisolone: 30–40mg for 3–5 days for severe acute urticaria/angioedema
- Adrenaline IM: if anaphylaxis
Chronic Spontaneous Urticaria (Stepwise — EAACI/BAD)
Step 1: Standard-dose non-sedating antihistamine (e.g. cetirizine 10mg OD) Step 2: Up-dose to 4× licensed dose (e.g. cetirizine 10mg QDS) — first escalation Step 3: Add omalizumab 300mg SC every 4 weeks (anti-IgE monoclonal — NICE TA339) Step 4: Add ciclosporin 3–5mg/kg/day (off-label; specialist only)
- Avoid: oral corticosteroids long-term; sedating antihistamines as maintenance
- LTRA (montelukast): weak evidence; can be tried as add-on
Physical Urticarias
- Non-sedating antihistamines (may need higher doses)
- Avoidance of specific trigger where possible
- Cold urticaria: desensitisation protocols (specialist)
Referral Criteria
- Dermatology/allergy: CSU not controlled with standard antihistamines
- Immunology: suspected hereditary angioedema
- Urgent: anaphylaxis, wheals >24h (biopsy for urticarial vasculitis)
Prognosis
- Acute urticaria: self-limiting in most cases (days to weeks)
- CSU: median duration 2–5 years; ~50% resolve within 5 years; ~20% persist >10 years
- Omalizumab: ~65–70% achieve good control (UAS7 ≤6)
- Autoimmune CSU: may have longer duration
- Physical urticarias: often persistent but manageable with antihistamines
- QoL: significant impact; comparable to coronary artery disease in some studies
Other Relevant Information
CSU Treatment Stepladder
| Step | Treatment |
|---|---|
| 1 | Standard-dose antihistamine |
| 2 | Up-dose antihistamine to 4× |
| 3 | Add omalizumab (anti-IgE) |
| 4 | Add ciclosporin |
Urticaria Activity Score (UAS7)
| Score | Severity |
|---|---|
| 0–6 | Well-controlled |
| 7–15 | Mild |
| 16–27 | Moderate |
| 28–42 | Severe |