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Urticaria: Overview, Diagnosis, and Treatment

About Urticaria

Urticaria, commonly known as hives, is actually the name for a diverse group of diseases with many subtypes. While the symptoms of urticaria—raised patches of skin surrounded by a red area of skin (wheals) and/or swelling of the skin (angioedema)—may be easily identified, further classifying the disease can be challenging.There are two types of urticaria:

  • Acute (<6 weeks)
    • Lifetime prevalence: 8% to 20% of adults globally2,3
  • Chronic (>6 weeks)
    • Lifetime prevalence: 0.1% to 1.8% of adults globally2,3

Urticaria occurs when mast cells and basophils in the superficial dermis release histamine and other vasoactive substances.1 This can be either an immune- or nonimmune-mediated response.1

  • Immune-mediated mast cell activation
    • Allergic reactions
    • Autoimmune disorders
  • Nonimmune-mediated mast cell activation
    • Certain drugs
    • Drug-induced cyclooxygenase inhibition
    • Physical or emotional stimuli

Understanding the instigating factor involved in your patient’s urticaria is one piece of the puzzle. Discovering whether it is acute or chronic and if it happens alone or in association with other conditions is also important. These clues can be obtained through a physical exam, detailed patient history, and possibly even the results of specific IgE blood testing. The more information you have, the better you may be able to put together a comprehensive management plan to address your patient’s symptoms.1

8% to 20%

Lifetime prevalance of acute (<6 weeks) urticaria is 8 to 20 percent globally.2,3

The triggers for acute urticaria are well documented. It is possible for there to be no immune involvement in acute urticaria, as the symptoms may result from any of the following:1,7

  • Adverse medication reactions (e.g., penicillin, sulfa, opiates, angiotensin-converting enzyme [ACE] inhibitors, non-steroidal anti-inflammatory drugs [NSAIDs])
  • Contact urticaria (e.g., plant, animal)
  • Contact dermatitis (e.g., poison ivy, nickel)
  • Exacerbation of physical urticaria (e.g., dermographism, cholinergic urticaria)
  • Physical stimuli (e.g., pressure, cold, heat, exercise, sun exposure)
  • Bacterial or viral infections (e.g., Parvo virus B19, Epstein-Barr virus)

 

Symptoms of urticaria: The hallmarks of urticaria are itchy, red skin plaques that can occur alone or in groups on any part of the skin.1 Urticaria may also present as wheals that vary in size, change shape, and appear and fade repeatedly as the reaction runs its course.1 Patients with urticaria may also have swelling under the skin (angioedema), which results from mast cell and basophil activation deep in the dermis and subcutaneous tissues.1 This symptom may present on the lips, eyelids, and inside the throat.1 Patients with chronic urticaria may have periods of exacerbation and remission that may persist for years.1

Understanding the underlying triggers of these symptoms is critical.1 Especially when autoimmune diseases are at the root of the disease, shortening the time from initial presentation to autoimmune diagnosis could mean you and your patients are spared visits, ineffective treatments, and frustration.

Urticaria diagnosis: Ruling in or out allergy as the cause 

Diagnosing urticaria will rely on information from a variety of sources, including a physical exam, patient history, and possibly test results.

In the case of acute urticaria, your diagnostic efforts will largely be focused on identifying the possible triggers and allergies. You can acquire these insights through a thorough history, which should cover medications (e.g., non-steroidal analgesic drugs) and foods.4 Your patient history should also cover infections (e.g., acute viral upper respiratory infections), as they are one of the most frequent causes of acute urticaria. However, taking blood samples to determine antiviral antibodies may not be recommended due to low specificity.4

If your allergy-focused patient history finds strong symptoms or a history of sensitizations, you may wish to follow the current guidelines and conduct diagnostic tests.1 Skin prick testing (SPT) and specific IgE blood testing can help you rule in or rule out allergen sensitization, which may give you the ability to correctly diagnose and improve clinical management.5,6

Not sure which allergy or autoimmune disease could be behind your patient’s symptoms? Use this interactive tool to take the next step in making your differential diagnosis. 

Common allergens

Allergy-related acute urticaria can be difficult to identify and manage as polysensitization is common. Up to 80 percent of patients are sensitized to more than one allergen.8 Furthermore, identifying the obvious allergen is not always enough. Allergic responses can develop to previously tolerated substances, and sensitization to seasonal and perennial allergens often add up to trigger symptoms.

It is known that urticaria can be an immune-mediated response to certain chronic disorders.9 For example, chronic urticaria is sometimes associated with connective tissue disorders, particularly systemic lupus erythematosus (SLE) or Sjögren’s Syndrome and thyroid disorders.10 Urticaria pigmentosa is a subvariant of cutaneous mastocytosis and mastocytosis should be evaluated.1,11

 

The following types of allergens may trigger an immune-mediated response:

  • Contact or inhaled allergen (e.g., latex, animal saliva, dust, pollen, mold, dander)
  • Ingested allergens (e.g., peanuts, tree nuts, shellfish, fish, wheat, eggs, milk, soybeans)
  • Insect bites or stings (e.g., Hymenoptera venom)

Testing Confidence

Testing Increases Diagnostic Confidence

Adding diagnostic testing to aid in a differential diagnosis has been shown to increase confidence in diagnosis to 90 percent.i,ii Conventionally, a diagnosis of allergic or autoimmune disease relies on the case history and a physical examination. However, adding diagnostic testing to aid in a differential diagnosis has been shown to increase confidence in diagnosis.i,ii Diagnostic testing can also help to improve the patient’s quality of life and productivity, reduce costs associated with absenteeism, and optimize use of medication, in addition to decreasing unscheduled healthcare visits.iii,iv 

i. Duran-Tauleria E, Vignati G, Guedan MJ, et al. The utility of specific immunoglobulin E measurements in primary care. Allergy. 2004;59 (Suppl78):35-41.
ii. NiggemannB, Nilsson M, Friedrichs F. Paediatric allergy diagnosis in primary care is improved by in vitro allergen specific IgE testing. Pediatr Allergy Immunol. 2008;19:325-331
iii. Welsh N, et al. The Benefits of Specific Immunoglobulin E Testing in the Primary Care Setting. J Am Pharm Assoc. 2006;46:627.
iv. Szeinbach SL, Williams B, Muntendam P, et al. Identification of allergic disease among users of antihistamines. J Manag Care Pharm. 2004; 10 (3): 234-238

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Management and care for patients with urticaria

Skin disorders, including atopic dermatitis (eczema) and urticaria (wheals) are commonly seen by healthcare providers and managing patients with these conditions can often be challenging. Results from studies have demonstrated that atopic dermatitis and urticaria have a profoundly negative impact on health-related quality of life (HRQoL), particularly impacting social functioning and psychological well-being. And many patients with one or both conditions report problems attributable to their skin in facets of everyday life including home management, personal care, mobility, sleep, rest, school, and work.12,13

The management of acute urticaria involves antihistamines, oral corticosteroids, and eliminating suspected triggers. The management of chronic urticaria involves nonpharmacologic and pharmacologic approaches, and including, but not limited to, antihistamines, anti-inflammatory agents, and avoidance of triggers.14

Practice parameters have been developed to help guide the management and treatment of patients with skin allergies. 

References

 

  1. Zuberbier T, Aberer W, Asero R, et al. The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update. Allergy. 2014;69(7):868-887. 
  2. Sanchez-Borges M, Asero R, Ansotegui IJ, et al. Diagnosis and treatment of urticaria and angioedema: a worldwide perspective. World Allergy Organ J. 2012;5(11):125-147.  
  3. Gaig P, Olona M, Muñoz Lejarazu D, et al. Epidemiology of urticaria in Spain. J Invest Allergol Clin Immunol. 2004;14(3):214-220.  
  4. Schoepke N, Doumoulakis G, Maurer M. Diagnosis of urticaria. Indian J Derm. 2013;58:211-218. 
  5. Duran-Tauleria E, Vignati G, Guedan MJ, et al. The utility of specific immunoglobulin E measurements in primary care. Allergy. 2004;59 (Suppl 78):35-41.  
  6. Niggemann B, Nilsson M, Friedrichs F. Paediatric allergy diagnosis in primary care is improved by invitro allergen specific IgE testing. Pediatr Allergy Immunol. 2008;19:325-331.  
  7. Deacock SJ. An approach to the patient with urticaria. Clinical and Experimental Immunology. 2008;153(2):151-161. doi:10.1111/j.1365-2249.2008.03693.x.
  8. Ciprandi G, Alesina R, Ariano R, et al. Characteristics of patients with allergic polysensitization: the POLISMAIL study. Eur Ann Allergy Clin Immunol. 2008;40(3):77-83.  
  9. Wai YC, Sussman GL. Evaluating chronic urticaria patients for allergies, infections, or autoimmune disorders. Clin Rev Allergy Immunol. 2002;23(2):185-193. 
  10. Confino-Cohen R, Chodick G, Shalev V, et al. Chronic urticaria and autoimmunity: associations found in a large population study. J Allergy Clin Immunol. 2012;129(5):1307-1313. 
  11. Valent P, Akin C, Metcalffe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017 Mar 16;129(11):1420-1427
  12. Lifschitz C. The Impact of Atopic Dermatitis on Quality of Life. Ann Nutr Metab. 2015;66(suppl 1):34–40 
  13. O'Donnell BF, et al. The impact of chronic urticaria on the quality of life. Br J Dermatol. 1997 Feb;136(2):197-201.
  14. Bernstein, JA; Lang, DM; Khan, DA; et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol 133 (2014):1270-1277e66.