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macchia orticaria

Urticaria

What is urticaria and how does it present itself

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.

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.

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.

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.

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.

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.

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. 

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

Chronic Urticaria Worldwide

During their lifetime, 1 in 5 patients will experience hives.

Worldwide, approximately 1% of the population suffers from hives, which translates to around 80 million people globally and nearly 1 million in Italy.

Hives are classified primarily based on their duration as acute urticaria (AU), lasting less than 6 weeks, or chronic urticaria (CU), lasting more than 6 weeks.

Urticaria is further divided into induced forms (e.g., cold-induced urticaria) and spontaneous urticaria.

Spontaneous urticaria is more common than induced urticaria, and both can coexist in the same patient.

The following graph shows the percentages worldwide:

Source: Peck, G. et al. Global epidemiology of urticaria: increasing burden among children, females and low-income regions. Acta Derm. Venereol. 101, adv00433 (2021)

From diagnosis to treatment

2 STEP

4 STEP

mappa per giungere alla terapia

1 STEP

Visit: Visit a specialist (immunologist, dermatologist, allergist). Bring photos of the hives and write down your symptoms.

3 STEP

Activation of the Therapeutic Plan:

If the antihistamine treatment does not work, the specialist will activate the treatment plan with Omalizumab (monoclonal antibody).

5 STEP

Revaluation:

After 12 months of treatment, there will be a 1-month break to assess if you are cured or if you need to resume the treatment plan with Omalizumab.

Diagnosis:

You will start your UAS and UAS7 hives diary. Specialists will prescribe you antihistamines, up to 4 per day. Follow their instructions carefully.

Therapeutic Plan:

if you respond well to Omalizumab, you will continue the medication for 12 months with specialist checkups every 4 months.

It is important to note that it is essential to follow the instructions provided by your specialist. It is inadvisable to attempt to treat yourself or rely on information that has been misinterpreted. Such actions may lead to undesirable outcomes, as they do not adhere to the guidelines that have been established on the global level and that are recognised by AIFA,

which is the Drug Agency in your country.

  • How is urticaria diagnosed?
    To diagnose urticaria accurately, it is crucial to undergo a comprehensive medical evaluation conducted by a specialized physician, such as a dermatologist, allergist, or immunologist. These healthcare professionals possess expertise in managing skin conditions like urticaria and can offer precise diagnosis and tailor-made treatment strategies.
  • The medications commonly used to treat urticaria include
    antihistamines, corticosteroids, histamine receptor antagonists, and in some cases, omalizumab. It is essential to follow the doctor's instructions to achieve the maximum benefit from the treatment.
  • Will I ever recover from urticaria?
    Urticaria can completely heal in some cases, but in others, it can become a chronic condition. Treatment aims to control symptoms and improve the patient's quality of life.
  • Will I always need therapy?
    Therapy for urticaria depends on the severity of symptoms and response to treatment. In some cases, long-term therapy may be necessary to keep symptoms under control.
  • Can I get an exemption or disability with urticaria?
    Currently, urticaria is not considered a disabling condition. However, this condition often coexists with other conditions for which specific exemptions may apply (such as asthma). Regarding exemption or disability, it's important to consult with your specialist and primary care physician to assess your specific situation and any available benefits.
  • Can I expect side effects from omalizumab?
    Omalizumab can cause side effects such as local skin reactions, headache, or fatigue or some more. It's important to report any symptoms to the doctor to evaluate any necessary adjustments to the therapy.
  • What if omalizumab doesn't work?
    If omalizumab is not effective in treating urticaria, it's important to consult the doctor to explore other possible therapeutic options, such as changing medication or adding other therapies.
  • Can I exercise?
    Generally, it's advisable to avoid exercise during periods of worsening urticaria symptoms, but it's important to consult the doctor to assess the specific situation and receive personalized recommendations. Once symptoms are controlled with therapy, the patient can resume physical activities.
  • How do I deal with urticaria during a pregnancy?
    Dealing pregnancy with urticaria requires careful management of the condition in collaboration with a specialist. Some medications may not be safe during pregnancy, so it’s crucial to discuss with the doctor the most appropriate therapeutic options.
  • Can I take omalizumab during pregnancy?
    The use of omalizumab during pregnancy should be evaluated by a specialist considering the potential risks and benefits for both the mother and the fetus. It's important to carefully follow the doctor's instructions in this particular case.
  • What therapies can be used for children under 12 years old in Italy?
     For children under 12 years old with urticaria, therapies may include age-appropriate antihistamines, topical or systemic corticosteroids in selected cases, and other therapeutic options prescribed by a pediatrician or pediatric allergist/dermatologist. It's crucial to follow the doctor's instructions carefully to ensure effective and safe treatment.

UCare Centers

What are they and where can I find them ?

In Italy, there are four UCARE centers of excellence for urticaria located within renowned hospital institutes. These centers contribute to research and treatment of urticaria thanks to the presence of teams of specialists, researchers, and cutting-edge diagnostic pathways for the diagnosis and treatment of urticaria in all its forms.

To become international reference centers for our disease in all its forms, they had to undergo a complex quality audit, which they successfully passed, receiving accreditation from GA²LEN, the Global Allergy and Asthma Excellence Network.

Clinica San Carlo

Diagnosis and therapy of urticaria

Centro UCare Paderno Dugnano

Via Ospedale, 21

Paderno Dugnano, Milan (MI), Italy

Phone Number: 02 990381

Centro UCare Milano

Diagnosis and therapy of urticaria

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Via Francesco Sforza, 28

Milan (MI), Italy

Phone Number: 02 55035146

Policlinico di Roma 

Dermatologia Allergologica

Diagnosis and therapy of urticaria

Centro UCare Roma

Viale del Policlinico, 155

Rome (RM), Italy

Phone Number: 06 49972072

Ambulatorio di Allergologia e Malattie Immunologiche rare 

Diagnosis and therapy of urticaria

UOC Medicina Interna 2

AORN Cardarelli

Centro UCare Napoli

A. Cardarelli, 9

Naples (NA), Italy

Phone Number: 081 7472122

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