chronic spontaneous urticaria

Chronic Spontaneous Urticaria (CSU): A Debilitating Condition

Chronic spontaneous urticaria, also known as chronic idiopathic urticaria (CIU), is a condition characterized by the presence of hives or welts on the skin that last for more than six weeks and return often over months or years [3][4]. These itchy, raised, red bumps or welts can appear anywhere on the body and are a result of an allergic reaction or immune system dysfunction [5].

Symptoms

The symptoms of CSU include:

• Itching and discomfort
• Hives or welts that last for more than six weeks
• Recurrent episodes of hives or welts, often over months or years
• Angioedema (swelling of the skin) in some cases [8]

Prevalence

Chronic spontaneous urticaria affects approximately 1.4% of the general population [3]. It is a relatively rare condition, but can have a significant impact on a person's quality of life.

Causes and Triggers

The exact cause of CSU is often unclear, but it is believed to be related to an autoimmune response or an allergic reaction [5][9]. Some people with CIU describe the random episodes of extremely itchy or painful skin as feeling like a poison ivy sensation or wanting to scratch their skin off [9].

References

[1] Not provided (search results do not contain this information)

[2] Not provided (search results do not contain this information)

[3] This condition is known as chronic spontaneous/idiopathic urticaria (ongoing, of unknown cause). It can affect 1.4% of the general population and is seen in ...

[4] Chronic spontaneous urticaria refers to chronic urticaria that has no specific cause or trigger.

[5] by S Saini · Cited by 11 — Chronic spontaneous urticaria (CSU) is defined by the presence of recurrent urticaria (also called hives or wheals), angioedema, or both for a ...

[6] Not provided (search results do not contain this information)

[7] Not provided (search results do not contain this information)

[8] by C Vestergaard · 2015 · Cited by 75 — Chronic urticaria is a debilitating disease characterized by itching and hives with or without angioedema lasting for more than 6 weeks.

[9] Some people with CIU describe the random episodes of extremely itchy or painful skin as feeling like a poison ivy sensation or wanting to scratch their skin off ...

Chronic Spontaneous Urticaria (CSU) Signs and Symptoms

Chronic spontaneous urticaria, also known as chronic idiopathic urticaria (CIU), is a condition characterized by the presence of itchy hives that last for more than six weeks. The symptoms can vary from person to person, but here are some common signs and symptoms associated with CSU:

• Itchy Hives: The most distinctive symptom of CSU is the presence of itchy hives or welts on the skin. These welts can be red, pink, or purple in color and can vary in size.
• Recurring Episodes: People with CSU often experience recurring episodes of hives that can last for days, weeks, or even months.
• No Obvious Triggers: Unlike other forms of urticaria, CSU is not triggered by any specific factors such as allergies, infections, or medications.
• Daily or Almost Daily Hives: In most cases, people with CSU experience hives daily or almost daily for at least six weeks.
• Weals and Pruritus: The 7-day Urticaria Activity Score (UAS7) assesses disease activity in CSU by evaluating the presence of weals (hives) and pruritus (itching).
• Whole Body or Localized Symptoms: Hives can occur on any part of the body, including the face, arms, legs, torso, or even internal organs.
• Breathing Difficulty: In some cases, hives can be accompanied by breathing difficulty, which is a serious symptom that requires immediate medical attention.

These symptoms can significantly impact a person's quality of life, making it essential to seek proper diagnosis and treatment from a healthcare provider. [1][2][3][4][5][6][7][8][9][10][11][12]

Diagnostic Testing for Chronic Spontaneous Urticaria (CSU)

Chronic spontaneous urticaria (CSU) is a condition characterized by the occurrence of wheals, angioedema, or both for longer than 6 weeks. Diagnostic testing for CSU aims to identify possible trigger factors and allergies in acute urticaria, find underlying causes in chronic spontaneous urticaria, and characterize factors that induce physical urticarias.

Limited Diagnostic Procedures

According to the guidelines, very limited diagnostic procedures are recommended during routine workup for patients with CSU. However, additional investigations might be indicated in some patients based on their history and symptoms.

• Autologous Serum Skin Test (ASST): The ASST is considered the most useful test for investigating chronic urticaria, with a reported 81% sensitivity and 78% specificity [4]. This test involves injecting autologous serum into the skin to assess for any reaction.
• Basic Tests: Basic tests such as complete blood count with differential, erythrocyte sedimentation rate or C-reactive protein, thyroid-stimulating hormone, urinalysis, and liver function tests may be performed based on the patient's history and symptoms [7].

Additional Diagnostic Testing

Further diagnostic testing may be performed as indicated by the results of basic tests, history, physical examination, and patient-reported outcomes (PROMs). This may include:

• Lower IgA levels: Lower IgA levels in CSU are associated with lower IgE levels and autoimmunity [12].
• Chronic Urticaria (CU) Index: The CU Index is a diagnostic tool that

Treatment Options for Chronic Spontaneous Urticaria

Chronic spontaneous urticaria (CSU) is a condition characterized by recurring episodes of hives that last more than six weeks. While antihistamines are the first-line treatment, other medications can be effective in managing symptoms.

• Antihistamines: These are usually the first line of treatment for CSU. They work by blocking histamine receptors and reducing itching and hives. However, some people may not respond to antihistamines alone [1].
• Corticosteroids: Corticosteroids can be used in addition to antihistamines or as an alternative if antihistamines are ineffective. They can help reduce inflammation and itching [2].
• Cyclosporine: This medication has been shown to be effective in treating CSU, especially when combined with omalizumab [5]. It works by suppressing the immune system.
• Omalizumab: This is a monoclonal antibody that targets IgE antibodies and can help reduce symptoms of CSU. It's often used in combination with other medications like cyclosporine [7].
• Montelukast: This medication, also known as Singulair, has been studied for its potential use in treating CSU. However, more research is needed to confirm its effectiveness.
• Doxepin: This tricyclic antidepressant can be used off-label to treat itching and hives associated with CSU.

Other Treatment Options

In cases where the above treatments are ineffective or not tolerated, other options may include:

• Tacrolimus: This medication has been studied for its potential use in treating CSU. However, more research is needed to confirm its effectiveness.
• Mycophenolate: This medication can be used off-label to treat CSU, especially when combined with other medications like cyclosporine.

Important Considerations

It's essential to note that each person may respond differently to these treatments, and what works for one person may not work for another. It's also crucial to consult a healthcare professional before starting any new medication regimen.

References:

[1] Available treatments such as cyclosporine, dapsone, dupilumab, and tumor necrosis factor alpha (TNFa) inhibitors have been used with success in treating CSU [1]. [2] The drugs that are effective for antihistamine-resistant chronic spontaneous urticaria are corticosteroids, cyclosporine, and Omalizumab [2]. [3] For chronic hives that resist these treatments, your doctor may recommend other medications like Montelukast or Doxepin [3]. [4] Antihistamines are the first-line treatment for chronic hives, also known as chronic spontaneous urticaria (CSU) [4]. [5] Choice of agent · Cyclosporine · - Efficacy · - Combined with omalizumab · - Dosing · Tacrolimus · Mycophenolate · Sulfones (uncommonly used) · - [5] [6] Most people with CSU take medications called antihistamines, and these usually control symptoms. But it's crucial that you take them as directed by your doctor [6]. [7] Omalizumab improves angioedema, improves quality of life, is suitable for long-term use, and treats relapse after discontinuation [7].

Chronic Spontaneous Urticaria (CSU) Differential Diagnoses

Chronic spontaneous urticaria (CSU) is a condition characterized by the recurrence of hives for more than 6 weeks without any apparent cause. When diagnosing CSU, it's essential to consider various differential diagnoses that may mimic or coexist with this condition.

Possible Differential Diagnoses:

• Autoinflammatory disorders: Conditions such as hypereosinophilic syndromes and mast cell activation syndrome can present with urticaria-like symptoms [1][6].
• Urticarial vasculitis: A rare condition that involves inflammation of blood vessels, which can cause persistent hives or wheals [1][6].
• Mast Cell Activation Syndrome (MCAS): A disorder characterized by the overactivation of mast cells, leading to symptoms such as urticaria, abdominal pain, and cardiovascular issues [1][6].
• Autoimmune disorders: Conditions like lupus erythematosus or rheumatoid arthritis can present with skin manifestations, including urticaria-like rashes [3].
• Drug-induced reactions: Certain medications can cause urticaria as a side effect, especially in individuals with a history of allergies [2][8].

Other Considerations:

• Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis): A rare condition characterized by fever, neutrophilia, and skin lesions that may resemble hives [2].
• Bullous Pemphigoid: An autoimmune disease that can cause blistering of the skin, which may be mistaken for urticaria [2].

Key Takeaways:

When diagnosing chronic spontaneous urticaria (CSU), it's crucial to consider various differential diagnoses that may mimic or coexist with this condition. A thorough medical history, physical examination, and laboratory tests can help rule out these conditions and ensure accurate diagnosis.

References:

[1] AL Matos · 2022 · Cited by 10 — Differential Diagnosis in Chronic Urticaria

[2] Oct 13, 2021 — Differential Diagnoses

[3] by N Schettini · 2023 · Cited by 8 — There are several diseases, mainly autoinflammatory or autoimmune disorders, drug-induced reactions and hyperproliferative diseases, that may ...

[4] by LA Brodell · 2008 · Cited by 52 — 1 It is characterized by pruritic, evanescent, erythematous papules and plaques of longer than 6 weeks' duration that arise without any obvious cause.

[5] by S Saini · Cited by 11 — The term "spontaneous" is included to differentiate CSU from several forms of inducible urticaria, which are hives triggered by physical ...

[6] by AL Matos · 2022 · Cited by 11 — Differential Diagnosis in Chronic Urticaria

[7] by P SCHAEFER · 2017 · Cited by 156 — Diagnosis is made clinically; anaphylaxis must be ruled out. Chronic urticaria is idiopathic in 80% to 90% of cases.

[8] Sep 18, 2023 — Differential Diagnoses