Urticaria, also known as hives, is a skin condition characterized by the sudden appearance of wheals, angioedema, or both, accompanied by intense burning and itching at the affected sites. This condition often presents with a sense of overall physical discomfort.

Chronic spontaneous urticaria is a complex and enigmatic disorder with an unclear etiology. It typically presents with wheals and/or angioedema, accompanied by widespread itching that can affect various parts of the body.

Notably, the deeper layers of the skin, as well as areas like the lips, eyelids, hands, feet, and in rare instances, even the genital region, can be involved.

It is crucial to distinguish urticaria from other conditions that may present with similar symptoms such as wheals or angioedema, including anaphylaxis, auto-inflammatory syndromes, bradykinin-mediated angioedema, and hereditary angioedema. Proper diagnosis and differentiation are essential for appropriate management and treatment of the underlying condition.

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In urticaria, wheals have three typical characteristics:

1. Skin swelling of variable size, surrounded by an area of surrounding redness.

2. Itching sensation, sometimes burning sensation.

3. Short duration. The skin returns to normal appearance within 30 minutes - 24 hours.

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Angioedema in patients with urticaria has the following characteristics:

• Sudden onset of a usually asymmetric reddened swelling of the skin, subcutaneous tissue or mucous membranes. It generally affects the eyelids, lips or limbs.

• Sometimes it is associated with a sensation of pain as well as itching.

• The resolution of the wheals is slow and angioedema can persist for up to 72 hours.

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Acute urticaria is spoken of if the duration of the manifestations and symptoms is less than 6 weeks. This type of urticaria usually lasts a short time and responds to short-term therapy with corticosteroids.

If symptoms persist for more than six weeks, urticaria is defined as chronic.

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Chronic urticaria is classified into:

• Chronic spontaneous urticaria: characterized by the appearance of urticaria with or without angioedema for more than 6 weeks in the absence of a triggering cause.

• Inducible urticaria: urticaria and/or angioedema develops in the presence of a triggering factor (e.g., cold, sun exposure, heat, contact, water, or physical activity).

 

Urticaria related to other diseases: numerous diseases are associated with urticaria at onset or in the clinical course.

These include: vasculitic urticaria, pigmentosa urticaria associated with systemic mastocytosis, bradykinin-mediated angioedema, periodic syndromes associated with cryopyrin, Schnitzler syndrome, Gleich syndrome, Wells syndromeand bullous pemphigoid.

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It is estimated that ca.1% of the Italian and world population suffers or has suffered from chronic urticaria at least for a period of their lives. This means at least 1 person in 5. Women are more affected than men, and a family history of autoimmune or allergic diseases is a risk factor.

Urticaria is a disease caused by the activation of skin cells called mast cells which, in response to stimuli of various kinds, release molecules (primarily histamine) responsible for the appearance of wheals, angioedema and itching.

The stimuli capable of activating mast cells can come from outside (inducible urticaria) or can be related to independent immunological mechanisms (chronic spontaneous urticaria).

 

Diagnosis:

The diagnostic pathway first of all involves the careful collection of the patient's medical history, paying attention to the characteristics of the urticaria (duration, distribution and frequency of wheals, any triggering factors such as drugs, heat, stress), the presence of a family history of autoimmune or allergic diseases and of gastrointestinal or joint symptoms. 

First-level blood tests are represented by the blood count and CRP. Further in-depth tests may be requested by the specialist based on the symptoms complained of by the patient and the clinical presentation.

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Clinical evaluation:

After receiving a diagnosis of chronic spontaneous urticaria, the patient is asked to complete two questionnaires to assess the severity of the urticaria (UAS7, weekly urticaria activity score) and the impact of this disease on the patient's life

(CU-Q2oL, questionnaire on quality of life of the patient with urticaria).

The UAS7 is a diary in which the patient assigns a score from 0-3 to the number of wheals

(<20 wheals/day, 20-50 wheals/day, >50 wheals/day) and the intensity of the itching (mild, moderate, severe).

The UAS7 allows to evaluate the severity of the urticaria and above all to monitor the therapeutic response over time.

The specialist will then ask the patient to complete a questionnaire on the quality of life of the patient with urticaria that takes into account the interference of urticaria with normal activities carried out during the day. 

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Source: The EAACI/GA²LEN/EDF/WAO Guideline for the Definition, Classification, Diagnosis and Management of Urticaria. The 2017 Revision and Update.