Emmy Award-winning actress Vicki Lawrence woke up one morning jaded with her condition. Her palms were covered with hives and the itchiness was unbearable. She found a temporary relief by submerging her hands in cold water. But the itch went back the next morning. Later that day, the itchiness spread in her abdomen and back. The hives were unsightly. She tried bathing in cold shower and using mentholated lotion but her condition persisted.
She tried avoiding things that she thought were causing the apparent allergic reaction—from wine, her soap, and even warm water. Her initial visits to an allergist were also frustrating. She underwent several tests to determine what was causing her itch and hives. The entire ordeal was both disappointing and disabling. She was not getting any answers and relief.
After six weeks of trial and error, the allergist finally diagnosed Lawrence with Chronic Idiopathic Urticaria or CIU.
Defining and understanding chronic idiopathic urticaria
Urticaria or hives is a type of skin rash frequently caused by allergic reactions or other autoimmune responses. They appear as red, itchy bumps or welts that turn pale when pressed in the centre. Urticaria is considered as chronic if it persists for more than six weeks.
According to the website CIUandYou.com—a joint project of Lawrence, the Asthma and Allergy Foundation of America, Genetech, Inc., and Novartis Pharmaceuticals Corporation—chronic idiopathic urticaria is an unpredictable form of chronic urticaria or hives that can appear at any time with no identifiable cause. The hives may persist for months and even years and they come and go without a known cause or trigger.
Specific clinical features of CIU include the daily appearance of hives, lasting on average between six to 18 hours. Lesions or wheals can occur on any part of the body and they are extremely itchy. While the hives often manifest severely in the morning, the itching and burning sensation are often worse at night.
In approximately 50 percent of CIU cases, angioedema occurs. This condition is characterised by the rapid swelling of the dermis, subcutaneous tissue, mucosa, and submucosal tissues. Thus, this swelling manifests around the mouth, throat, tongue, and around the eye.
The gastrointestinal and pulmonary tracts may also swell because of angioedema. This occurs in severe cases of CIU. The swelling of the larynx can be fatal as it can block oxygen from entering the body, causing death by asphyxiation.
Both the presence of hives and in some rare instances, angioedema do not only cause physical discomfort. Their unsightly and to a certain extent, disfiguring manifestations can render a CIU patient withdrawn from people and the society, thus affecting his or her job, social life, and family life.
The literature review of C. L. Goh and K. T. Tan mentioned that 30 to 40 percent of CIU is autoimmune in nature in which antibodies trigger mast cell and basophil activation that results in the release of histamine and other pro-inflammatory mediators.
Prevalence and management of chronic idiopathic urticaria
For every 10 people who have chronic urticaria, at least seven of them have CIU. Estimates from AAFA revealed that around 1.5 million people in the United States suffer from this form of chronic urticaria and most cases appear between the ages of 20 and 40. A study by J. M. Negro-Alvarez and J. C. Miralles-López further mentioned that 75 percent of all chronic urticaria cases in the U.S. and Europe are considered idiopathic.
Although some mistakenly consider CIU as an allergic reaction, several studies have suggested otherwise. Researchers R. S. Demera, B. Ryhal, and M. E. Gershwin noted that the association between chronic urticaria and thyroid autoimmunity was first suggested in 1983. Follow-up studies predicted that 5 to 20 percent of patients with CIU would have positive thyroid autoantibodies.
Still, there are no known trigger for or specific cause of CIU. Researcher K. V. Godse noted that food additives might aggravate hives. In addition, aspirin and other non-steroidal anti-inflammatory drug can worsen hives in 20 to 30 percent of patients during the active phase. Alcohol can worsen the condition by the mechanism of vasodilation in which blood vessels are dilated resulting in a decrease in blood pressure.
The lack of known trigger or cause means that patients and care providers should resort to management instead of prevention. Of course, some patients continue to undergo series of tests to determine specific triggers. But this can be counterproductive, costing too much time and money.
According to CIUandYou.com, CIU can look and feel different for everyone. Some doctors might provide misdiagnosis. Hence, it is better to approach specialists, particularly allergists and dermatologists, who have an expansive understanding of CIU. It is also better for patients to have complete understanding of their symptoms, overall health condition, and medical history. As reiterated by Demera et al, the most valuable diagnostic tool in CIU is detailed history and physical examination.
The literature review of K. V. Godse mentioned that treatment plan should include treatment of identifiable cause, avoidance of aggravating factors, advice and written information about the condition, and antihistamines trial. In the actual treatment phase, there are three line of treatments to manage CIU. The first line of treatment centres on administering antihistamine. Treatment is generally started with nonsedating antihistamine in the daytime and sedating antihistamine in the night.
There are patients who do not respond to antihistamines. In such case, the second line of treatment includes administering doxepin—a tricyclic antidepressant used to treat depression, anxiety disorders, and insomnia, as well as CIU. Other options for second line of treatments include montelukast, a leukotriene receptor antagonist commonly used to mange asthma and seasonal allergies; prednisolone, a synthetic cortisol derivative used to treat a variety of inflammatory and autoimmune conditions; and sulfasalazine, long acting sulfonamide used in the treatment of ulcerative colitis, rheumatoid arthritis, and Crohn’s disease.
In cases when the second line of treatment does not work, the third line of treatment offers several options. Some specialists administer methotrexate for the treatment of resistant autoimmune urticaria. Others administer cyclosporine—a drug that inhibits the release of histamine from basophils and tumor necrosis factorαproduction by mast cells.
Another drug used to manage CIU is omalizumab. This is a recombinant humanised monoclonal antibody against immunoglobulin IgE, represents a unique therapeutic approach for the treatment of allergic diseases. This agent acts as a neutralising antibody by binding IgE at the same site as the high-affinity receptor. Subsequently, IgE is prevented from sensitising cells bearing high-affinity receptors.
The review of Godse also revealed that autologous serum therapy can reduce the severity of CIU as well as antihistamine requirement while also preventing the relapse of symptoms for durations as long as two years.
Topical medications, particularly the use of calamine lotion, menthol with aqueous cream, and crotamiton lotion are useful soothing agents integrated in the entire treatment plan.
Further details of the literature review of Goh and Tan are in the article “Chronic autoimmune urticaria: Where we stand?” published in 2009 in the Indian Journal of Dermatology.
More details of the study of Negro-Alvarez and Miralles-López are in the article “Chronic idiopathic urticaria treatment” published in 2001 the journal Allergologia et Immunopathologia. Further details of the study of Demera, Ryhal, and Gershwin are in the article “Chronic idiopathic urticaria” published in 2001 in the journal Comprehensive Therapy. Further details of the study of Godse are in the article “Chronic urticaria and treatment options” published in 2009 in the Indian Journal of Dermatology.
The dedicated website of CIU by the Asthma and Allergy Foundation of America is accessible at www.CIUandYou.com. The website provides quick facts and several critical resources to include treatment options, and a Conversation Starter and Symptoms Tracker for helping patients communicate their conditions with specialists.