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World Allergy Organization
WAO's mission: To be a global resource and advocate in the field of allergy, advancing excellence in clinical care through education, research and training as a world-wide alliance of allergy and clinical immunology societies.

Systemic Manifestations of Atopic Urticaria

Allen P. Kaplan, MD
Medical University of South Carolina

Charlestown, SC, USA

IgE antibody is a requisite mediator of acute urticaria due to food or drug allergy, but less well appreciated is its role in physical urticarias or chronic spontaneous urticaria (idiopathic or autoimmune). A subpopulation of patients with cold urticaria and dermatographism have a disorder that can be passively transferred with plasma that contains IgE, while IgE antibody directed to an antigen in sweat has been reported in a subpopulation of patients with cholinergic urticaria and a positive methacholine skin test. Systemic symptoms can be associated with cold urticaria, particularly hypotension, due to submersion causing a temperature change involving a large surface area. Familial auto-inflammatory cold-dependent disease presents with an urticarial rash associated with fever, myalgia and arthralgia, and leucocytosis. Patients with chronic spontaneous urticaria (idiopathic or autoimmune) have a 40% incidence of accompanying angioedema, but it is rare for additional symptoms to be present. There are exceptions, however, where myalgia and arthralgia (but not true arthritis) accompany urticarial episodes. There is a 25% incidence of antithyroid antibodies; although most are euthyroid, some present with thyroid dysfunction. There is also a high incidence of low titer, positive ANA’s with a speckled pattern in the absence of other signs or symptoms of systemic lupus erythematosis. Yet systemic connective tissue disorders can have urticaria as a manifestation; these include systemic lupus erythematosis, the vasculitis associated with serum sickness, Sjogren’s syndrome, polyarteritis nodosa, Churg-Strauss syndrome, and Wegener’s granulomatosis. However it is rare to have urticaria alone as a presenting symptom of these disorders and the incidence of urticarial vasculitis diagnosed by skin biopsy of patients presenting with chronic urticaria is less than 1 percent. Thus a serologic evaluation searching for connective tissue disorders (rheumatoid factor, anti double-stranded DNA, anti Sm, anti RNP, anti Scl 70, anti Ro, anti La, etc., etc.) is not recommended for routine evaluation of patients with chronic Urticaria. For those with hives lasting over 24 hours, or with myalgia or arthralgia, a C-Reactive Protein, complement C4 and a skin biopsy to rule out vasculitis, should be performed.

References:
Garofalo J, Hauber T, Kaplan AP. Idiopathic cold urticaria : In vitro demonstration of histamine release upon challenge of skin biopsies. New England J Med. 1981; 305: 1074-1077
Kaplan AP , Joseph K, Maykut RJ, Gaba GP, Zeldin RK. Treatment of chronic urticaria with Omalizumab. J. Allergy Clin Immunol. 2009; 123: 713-717
Kaplan AP, Greaves MW. Pathogenesis of chronic urticaria. Clin Exp. Immunol. 2009; 39: 777-787

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