8.5 Urticarial syndromes

Grading & Level of Importance: B




Urticarial eruptions, urticarial rash.


There are many dermatoses mimicking urticaria.. In contrast to “urticaria sensu strictu”, “urticarial syndromes” is a set of clinical images which reflect or mimic skin lesions that look like wheals or hives.

Aetiology & Pathogenesis

There are many dermatoses with an urticarial appearance that correspond to different pathogenic mechanisms such as type 1 or 3 immune reactions or are non-allergic (pharmacologic, toxic, chemical)  Many of them with an unknown aetiology. They include:


  • External pathogens
    • Insect bites and epizoonoses
    • Early stage of contact dermatitis 
    • Infectious diseases/rashes


  • Immune dermatoses 
    • Immunobullous diseases 
    • Autoinflammatory syndromes  
    • Neutrophilic dermatosis and autoimmune disorders
    • Angioedema


  • Semantic misnomers
    • Urticarial vasculitis 
    • Urticaria pigmentosa (not primarily urticarial, may evolve urticarial appearance)

Signs & Symptoms

Urticarial lesions last days, itch is variable, sometimes leave a mark with persisting erythema, blood extravasation, hematoma-like or small pigmentation, Sometimes they show scaling. Rarely angioedema is associated, fever sometime is present and is possible that systemic disturbances are key diagnostic features.


Urticarial lesions can develop at any body location.  


There is no nosologic classification because of the heterogeneity of the conditions represented.

Laboratory & other workups

There are no specific  general laboratory requirements. Some of the diseases need specific diagnostic tests and immunologic and molecular approach. 


A skin biopsy may be helpful in order to confirm a causative underlying dermatoses. 


Depends on the underlying disorder; acute, subacute or chronic persistent or relapsing.  


Depends on the underlying disorder.


The clinico-pathological correlation is nearly always essential for the diagnosis of urticarial lesions.

Differential diagnosis

See aetiology & pathogenesis.

Prevention & Therapy

Depends on the underlying disorder.

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