Chronic Urticaria

Chronic urticaria is a condition where itchy, raised welts appear on your skin at least twice per week for more than six weeks. For many people, the cause remains unknown, but treatments can help control symptoms and improve quality of life.

Table of contents

• What is chronic urticaria?
• Symptoms
• Causes and related conditions
• Diagnosis
• Treatment
• Outlook
• Living with chronic urticaria

chronic spontaneous urticaria, chronic idiopathic urticaria, chronic hives

What is chronic urticaria?

Chronic urticaria, also called chronic hives, is a skin condition where itchy, raised welts appear on your skin repeatedly over an extended period. The medical term urticaria (pronounced ur-tik-CARE-ee-uh) simply means hives^[1].

This condition is defined as the occurrence of hives and sometimes swelling that persists for six weeks or more. The welts typically appear at least twice per week^[1][4]. While individual hives usually fade within 24 hours, new ones continue to appear, creating an ongoing cycle of symptoms^[3].

Chronic urticaria is different from acute hives. Acute hives start to fade within 24 hours and are completely gone within six weeks, often caused by a recent viral infection. Chronic hives, on the other hand, last much longer and the cause is frequently unknown^[1].

This condition affects about 0.5% to 5% of the general population. It occurs approximately twice as often in women as in men, and most commonly begins between ages 20 and 50^[5][6]. Some people experience these welts every day for a year or longer^[1].

The term chronic idiopathic urticaria is sometimes used when no clear cause can be found. The word idiopathic means the condition occurs for no known reason and comes on suddenly^[1]. Another term you may hear is chronic spontaneous urticaria, which refers to hives that occur without identifiable external triggers^[4].

Symptoms

The main symptom of chronic urticaria is the repeated appearance of welts, also called wheals, on the skin. These welts can vary greatly in appearance from person to person and even on the same person at different times^[1].

Hives can appear anywhere on your body and have different shapes and sizes. They may be as small as a pinprick or larger than a softball. The welts are typically raised and itchy, and can be red, purple, or the same color as your skin depending on your skin tone^[1][3].

A characteristic feature of hives is blanching, which means the center of the hive turns white when you press on it^[1][9]. The welts can change shape, appear and fade repeatedly, and vary in size. They are intensely itchy, which can significantly interfere with sleep and daily activities^[3][12].

Some people with chronic urticaria also experience angioedema, which is swelling that occurs in deeper layers of tissue. This painful swelling can affect the face, lips, throat, hands, feet, and genitals^[1][3].

For some individuals, symptoms are triggered by specific factors such as heat, exercise, or stress. Others find that symptoms persist for more than six weeks and recur often and anytime, sometimes continuing for months or years^[3].

Causes and related conditions

For most people with chronic urticaria, there is no known cause. The exact reason why chronic hives develop remains unclear in 80% to 90% of cases^[4][12].

The prevailing theory suggests that chronic urticaria relates to autoimmune dysfunction. This means the body’s immune system mistakenly attacks its own tissues. Up to 40% of patients with chronic urticaria show signs of an autoimmune process involving autoantibodies (proteins made by the immune system) that target certain receptors on immune cells, causing them to release histamine, a chemical that triggers hives symptoms^[4].

Although the disease process involves mast cells and basophils releasing histamine and other inflammatory substances, the exact mechanism of how this results in improvement or worsening of symptoms is not completely understood^[6][14].

Related autoimmune diseases

About 1 in 5 people who develop chronic hives also have an autoimmune disease, where the body’s immune system attacks healthy tissue. Associated conditions include^[1]:

• Thyroid disease (the most common association, found in up to 9.8% of chronic urticaria patients)
• Rheumatoid arthritis
• Systemic lupus erythematosus
• Celiac disease
• Diabetes (Type 1)
• Dermatomyositis
• Polymyositis
• Vitiligo

Other associated conditions

Other health conditions that may be linked to chronic urticaria include^[1][4]:

• Asthma
• Infections, such as H. pylori bacterial infections and sinus infections
• Liver disease
• Lymphomas, including Hodgkin and non-Hodgkin’s lymphoma
• Vasculitis

Possible triggers

While not causes themselves, certain factors can trigger hives in people with chronic urticaria. These include physical factors such as heat, cold, or physical activity causing rapid body temperature changes. Pressure on your skin from tight clothing may also trigger symptoms^[1].

About 20% of chronic urticaria cases are classified as inducible or physical urticaria, where specific triggers can be identified. The most common type is symptomatic dermographism (skin writing), followed by pressure and cold urticaria^[6].

Stress, both physical and emotional, is one of the biggest known triggers. Other triggers include non-steroidal anti-inflammatory drugs, infections, and extreme temperatures^[6][21].

Importantly, food allergies and medication allergies rarely cause chronic hives. While many patients believe their condition is due to food allergies, this has been found to be true for almost no patients with chronic spontaneous urticaria^[9].

Diagnosis

Chronic urticaria is primarily diagnosed through a careful examination of your skin and a detailed discussion of your symptoms. Your healthcare provider will ask about when the welts appear, how long they last, what they look like, and whether anything seems to trigger them^[1][10].

One important feature that helps with diagnosis is that in chronic hives, individual welts come and go at random, with each spot usually lasting less than 24 hours^[10]. You may be asked to keep a diary tracking your activities, medications, foods and drinks, and when and where hives appear^[10].

Healthcare providers who diagnose chronic urticaria

Several types of healthcare providers can diagnose and treat chronic hives, including^[1]:

• Primary care physicians
• Allergists (doctors who specialize in allergies and immune system disorders)
• Dermatologists (doctors who specialize in skin conditions)

Diagnostic tests

An extensive laboratory workup is not generally recommended for chronic urticaria. The diagnosis is mainly clinical, based on your history and physical examination^[12].

However, if your healthcare provider suspects an underlying cause, some tests may be performed to pinpoint or rule out specific conditions. These might include^[1][6]:

• Complete blood count with differential
• Erythrocyte sedimentation rate or C-reactive protein (to check for inflammation)
• Thyroid-stimulating hormone (to check thyroid function)
• Urinalysis
• Liver function tests

A tryptase level may be considered if your history raises concern for anaphylaxis (a severe, life-threatening allergic reaction) or a mast cell disorder. This test should be obtained during an acute event and compared to a baseline level^[6].

Testing for food allergens or environmental allergens is not indicated in the general workup of chronic spontaneous urticaria^[6].

If individual lesions last longer than 24 to 48 hours and leave behind discoloration, or if the welts are painful rather than itchy and don’t blanch when pressed, your provider may perform a skin biopsy. This helps rule out a condition called urticarial vasculitis, which requires different management^[6][10].

Treatment

Treatment for chronic urticaria focuses on controlling symptoms and improving quality of life. The mainstay of treatment is avoiding triggers when possible and using medications to reduce itching and hives^[1].

First-line treatment: Antihistamines

The primary treatment for chronic urticaria is antihistamines, which are medications that block the effect of histamine, the chemical in the skin that causes allergy symptoms including hives. Second-generation, non-sedating antihistamines are preferred because they have fewer side effects and last longer than older antihistamines^[1][9][12].

Common second-generation antihistamines include^[23]:

• Cetirizine
• Loratadine
• Desloratadine
• Fexofenadine
• Bilastine
• Rupatadine

Daily use of antihistamines is recommended rather than taking them only when symptoms appear. Studies have shown that regular daily use leads to greater quality-of-life improvements^[23].

If standard doses of antihistamines don’t control symptoms adequately, your healthcare provider may increase the dose. These medications can be safely increased up to two to four times the standard daily dose^[10][12][23]. About 50% of patients respond to antihistamines at approved dosages, while higher doses may be needed for others^[6].

First-generation antihistamines like diphenhydramine and hydroxyzine are no longer recommended as the main treatment because they cause drowsiness, affect thinking and movement, and don’t last as long as newer antihistamines^[23].

Additional treatments

If antihistamines alone don’t provide enough relief, other medications can be added^[10][15]:

• Leukotriene receptor antagonists such as montelukast may be added to antihistamine treatment
• H2 antihistamines like ranitidine, normally used for heartburn, may help some people when added to standard antihistamines
• Short courses of corticosteroids like prednisone can help control severe outbreaks, typically given for 1 to 2 weeks. However, long-term use is not recommended due to serious side effects

Treatment for difficult cases

For chronic hives that don’t respond to antihistamines even at higher doses, your healthcare provider may prescribe^[9][10]:

• Omalizumab, a biologic medication approved by the U.S. Food and Drug Administration for chronic spontaneous urticaria. This is given as an injection once a month
• Immunosuppressive medications such as cyclosporine, tacrolimus, hydroxychloroquine, or mycophenolate
• Other medications including doxepin (which should be used carefully in elderly patients)

These treatments are typically prescribed by allergists or dermatologists with experience managing difficult-to-treat hives^[9].

Outlook

Chronic urticaria is typically a self-limiting condition, meaning it eventually goes away on its own. The average duration is between 2 to 5 years, although symptoms can persist longer in about one-fifth of patients^[6].

More than half of patients with chronic urticaria will have resolution or improvement of symptoms within a year^[12]. However, symptoms can come and go, and some people experience long periods without hives followed by relapses weeks or even longer later^[7].

While chronic hives can be very uncomfortable and frustrating, they are not typically life-threatening. However, they can significantly impact quality of life by interfering with sleep, work, and daily activities^[3][12].

It’s important to note that chronic hives themselves do not put you at sudden risk of a serious allergic reaction called anaphylaxis^[3].

Living with chronic urticaria

Managing chronic urticaria involves both medical treatment and lifestyle adjustments. Making changes in your daily life can help reduce the frequency and severity of flare-ups^[21].

Avoid known triggers

If you’ve identified specific triggers for your hives, avoiding them is an important part of management. Common things to avoid include^[10][21]:

• Foods, medicines, pollen, or pet dander that trigger symptoms
• Alcohol
• Non-steroidal anti-inflammatory drugs (if they trigger your hives)
• Extreme temperatures (very hot or very cold)
• Tight-fitting garments or scratchy fabrics

Manage stress

Stress is one of the biggest triggers for chronic hives. While reducing stress may seem difficult, acknowledging stress in your life and seeking help to manage it can make a significant difference^[21].

Strategies that may help include^[21]:

• Yoga
• Walking or other exercise
• Progressive muscle relaxation
• Breathing exercises
• Meditation or mindfulness
• Therapy or counseling

Your healthcare provider can also prescribe anti-depressants or anti-anxiety medications if needed^[21].

Other helpful tips

Additional self-care measures include^[21]:

• Getting adequate sleep, as sleep is your body’s natural reset system
• Using lukewarm water for bathing instead of very hot or cold water
• Wearing loose, cotton clothing instead of tight or scratchy fabrics
• Practicing sun protection with sunscreen and UV protection
• Applying lotions with menthol and phenol, which may provide relief from itching

Chronic hives can be frustrating because the cause is often unknown and symptoms can continue for months or years. However, with appropriate treatment and lifestyle adjustments, most people can manage their symptoms effectively. Working closely with your healthcare provider, being patient, and maintaining a symptom diary can all help in managing this condition^[1][6].