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Urticaria in Adult
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Urticaria in Adult

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Contributors: Elyse M. Love MD, Whitney A. High MD, JD, MEng, Susan Burgin MD
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Synopsis

Urticaria, commonly known as welts or hives, refers to raised, erythematous wheals caused by the release of histamine and other vasoactive substances from mast cells. When swelling is superficial, urticaria eventuates. When the swelling is deeper, angioedema occurs.

Urticaria is defined as acute (new-onset or recurring episodes of fewer than 6 weeks' duration) or chronic (recurring episodes lasting longer than 6 weeks). Chronic urticaria is more common in women and middle-aged individuals, whereas acute urticaria is more commonly seen in children. Disease resolves within 12 months in approximately 50% of adults with chronic idiopathic urticaria; however, 20% of patients may have symptoms that persist for more than 20 years.

Urticaria can be triggered by a variety of mechanisms, both allergic and nonallergic.

In half of acute urticaria cases, the inciting factor is never identified; 40% of cases are associated with an upper respiratory infection, 9% with drugs, and 1% with foods. Drugs causing acute urticaria include aspirin, nonsteroidal anti-inflammatory agents (NSAIDs), morphine and codeine, penicillin and its derivatives, cephalosporins, sulfa, streptomycin, tetracycline, griseofulvin, blood products, radiographic contrast media, angiotensin-converting enzyme inhibitors, and sulfonylureas. Acute urticaria may also be seen as part of anaphylaxis.

Chronic urticaria may be autoimmune, induced by physical stimuli, or idiopathic. NSAIDs, foods, alcohol, stress, and infections may aggravate chronic urticarial but are rarely the cause.

Chronic spontaneous urticaria may be associated with other autoimmune conditions including autoimmune thyroid disease, vitiligo, insulin-dependent diabetes, rheumatoid arthritis, and pernicious anemia.

Other Associated Factors:
  • Systemic illnesses such as infections, collagen vascular diseases, neoplasia, endocrine disorders, and blood dyscrasias
  • Environmental stimuli such as insect stings (papular urticaria) and inhalants (pollen, spores, animal dander, perfumes, detergents)
  • Pregnancy
  • Foods such as strawberries, nuts, eggs, and shellfish
  • Inducible stimuli – mechanical stimuli (dermatographism, delayed pressure, vibratory), temperature change (heat, cold), sweating or stress (cholinergic, adrenergic, exercise-induced), contact, solar, aquagenic. Vibratory urticaria has been associated with a mutation in ADGRE2 (EMR2), which affects mast cell function.
  • Helicobacter pylori gastritis – patients may undergo remission of chronic urticaria with treatment
  • Parasitic infections – intestinal strongyloidiasis, gastric Anisakis simplex
Angioedema, which manifests as deeper, ill-defined swelling of the face, bowel, or part of an extremity, is seen in approximately 50% of urticaria cases. Angioedema tends to be painful rather than pruritic and often lasts for 2-3 days.

The presence of angioedema without wheals should raise suspicion for hereditary angioedema.

Tick bites from some Amblyomma and Ixodes (and possibly Haemaphysalis) species have been associated with the subsequent development of allergies to mammalian meat (eg, beef, pork) in a small number of patients. It is thought that the allergy is mediated by induced IgE antibodies to alpha-gal (galactose-alpha-1,3-galactose), a mammalian oligosaccharide. Individuals with elevated IgE titers to alpha-gal have experienced urticaria, angioedema, and anaphylaxis symptoms either immediately or 3-6 hours (delayed onset) after ingesting mammalian meat (alpha-gal syndrome). Exactly how the tick bite leads to development of this allergy is unclear. Implicated tick bites have been noted to itch for 2 or more weeks. A blood test for these IgE antibodies exists.

Urticaria is rarely a paraneoplastic syndrome except in the association of cold urticaria as a result of cryoglobulins associated with myeloma or lymphoma. Cold urticaria without cryoglobulins has been reported in human immunodeficiency virus (HIV)-infected individuals.

Related topics: Contact urticaria, Cholinergic urticaria, Physical urticaria, Cold urticaria, Dermographism, PUPPP, Urticarial vasculitis, Urticaria pigmentosa, Angioedema, Anaphylaxis

Codes

ICD10CM:
L50.9 – Urticaria, unspecified

SNOMEDCT:
126485001 – Urticaria

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Many serious illnesses present with urticarial lesions and should be considered with every case of urticaria.

Diseases with urticarial lesions include:
  • Serum sickness / serum sickness-like reaction – Associated with fever, lymphadenopathy, arthralgias, dusky skin lesions, and recent drug (ie, beta-lactam) or sera exposure.
  • Urticarial vasculitis – Individual lesions last longer than 24 hours and are associated with pain, purpura, and/or arthralgias or arthritis (joint swelling or refusal to use extremities).
  • Mastocytosis (urticaria pigmentosa) – Has persistent yellow-brown macules and plaques that urticate with stroking.
  • Immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura) – Associated with fever, edema, palpable purpura, and renal, gastrointestinal, musculoskeletal, and central nervous system disease.
  • Bullous pemphigoid and dermatitis herpetiformis may present with urticarial lesions, but individual lesions last longer than 24 hours and progress to vesicles or erosions. These are exceptionally pruritic as well.
  • Cryopyrin-associated periodic syndromes – Muckle-Wells syndrome, familial cold autoinflammatory syndrome (familial cold urticaria), and neonatal-onset multisystem inflammatory disease.
  • Acquired autoinflammatory syndromes – Schnitzler syndrome, adult-onset Still disease, episodic angioedema with eosinophilia (Gleich syndrome), systemic capillary leak syndrome.
Other diseases with urticaria-like lesions include:

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 04/04/2018
Last Updated: 12/14/2018
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Urticaria in Adult
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Urticaria : Erythema, Recurring episodes or relapses, Scattered many, Serpiginous configuration, Pruritus, Sudden onset in minutes
Clinical image of Urticaria
Edematous pink papules and plaques on the arm.
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