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Urticaria and Angioedema 101 Scot Laurie, MD Allergy and Asthma Center at NorthPark Assistant professor, University of Texas Southwestern Medical Center.

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Presentation on theme: "Urticaria and Angioedema 101 Scot Laurie, MD Allergy and Asthma Center at NorthPark Assistant professor, University of Texas Southwestern Medical Center."— Presentation transcript:

1 Urticaria and Angioedema 101 Scot Laurie, MD Allergy and Asthma Center at NorthPark Assistant professor, University of Texas Southwestern Medical Center

2 Case Presentation Jim S. is a 45 y/o who presented for evaluation of his urticaria. He has been suffering with hives for the past 4 months. He is unable to tell what triggers his hives. Jim S. is a 45 y/o who presented for evaluation of his urticaria. He has been suffering with hives for the past 4 months. He is unable to tell what triggers his hives. His hives are generalized and an individual hive will last a few hours; he has had several episodes of lip swelling as well. His hives are generalized and an individual hive will last a few hours; he has had several episodes of lip swelling as well. He might have had a similar episode 10 years ago and his doctor told him he was allergic to penicillin He might have had a similar episode 10 years ago and his doctor told him he was allergic to penicillin

3 Case presentation He has visited his primary care physician who suggested he take Claritin He has visited his primary care physician who suggested he take Claritin He returned when his hives persisted and the doctor told him that he was allergic to something and suggested an allergy evaluation. He returned when his hives persisted and the doctor told him that he was allergic to something and suggested an allergy evaluation.

4 Case presentation Past medical history Past medical history –Hypothyroidism Medications Medications –levothyroxine –Ibuprofen prn Review of systems Review of systems –Occasional headaches; otherwise negative Physical exam Physical exam

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6 Case presentation How would his hives be classified? How would his hives be classified? What is causing his hives? What is causing his hives? Are his medical conditions or medications contributing to his hives? Are his medical conditions or medications contributing to his hives? What tests should be done to evaluate his hives? What tests should be done to evaluate his hives? How are his hives best treated? How are his hives best treated?

7 URTICARIA & ANGIOEDEMA DESCRIPTION DESCRIPTION –Urticaria Raised, erythematous, blanching Raised, erythematous, blanching Pruritic Pruritic Lesions well-circumscribed; typically coalesce Lesions well-circumscribed; typically coalesce –Angioedema Subcutaneous swelling Subcutaneous swelling Predilection to areas of loose connective tissue, such as the face or mucus membranes involving the lips or the tongue Predilection to areas of loose connective tissue, such as the face or mucus membranes involving the lips or the tongue

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9 Urticaria or “Hives”

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11 Urticaria and Angioedema Clinical features: Urticaria Clinical features: Urticaria –Repeated occurrence of short-lived cutaneous wheals accompanied by erythema and pruritus Wheals range in size from a few millimeters to several centimeters Wheals range in size from a few millimeters to several centimeters Wheals may coalesce to form larger lesions Wheals may coalesce to form larger lesions Individual wheals typically last less than 24 hours Individual wheals typically last less than 24 hours Urticaria may occur anywhere on the skin Urticaria may occur anywhere on the skin –Mucus membrane involvement is rare Lesions should resolve without any residual marking Lesions should resolve without any residual marking

12 Urticaria and Angioedema Clinical features: Angioedema Clinical features: Angioedema –Approximately 50% of patients with chronic urticaria have angioedema as well –Episodes of short-lived deep dermal and subcutaneous or submucosal edema –Like urticaria, symptoms generally last less than 24 hours Larger swellings may take longer to resolve Larger swellings may take longer to resolve –Pruritus does not consistently accompany angioedema, and may not occur at all.

13 Mediators of hives and swelling SourceFactor Mast cells (cutaneous) Histamine Prostaglandin D 2 Leukotrienes C and D Platelet Activating Factor Complement system Anaphylatoxins C3a, C4a, C5a: histamine Hageman factor- dependent pathway Bradykinin Mononuclear cells Histamine-releasing factors, chemokines

14 Urticaria and Angioedema Classification Classification –Acute: < 6 weeks Allergic Allergic Infectious Infectious Idiopathic Idiopathic –Recurrent acute –Chronic: > 6 weeks Idiopathic Idiopathic Autoimmune Autoimmune Physical Physical

15 URTICARIA CLASSIFICATION Acute: < 6 weeks Acute: < 6 weeks –Affects as many as 10-20% of the population at some point in their lives –Etiology frequently identified Food allergy Food allergy Drug allergy Drug allergy Stings/venoms Stings/venoms Infection Infection –Viral infection leading cause of urticaria in children

16 Urticaria Classification Recurrent acute (intermittent) Recurrent acute (intermittent) –Episodes of urticaria lasting days or weeks with intervals of days, weeks, or months in between episodes Chronic: > 6 weeks Chronic: > 6 weeks –Idiopathic –Physical urticarias

17 URTICARIA ETIOLOGIES Common Common –Idiopathic –Medications –Stings –Foods –Infection –Physical urticarias Rare Causes Rare Causes –Neoplasms –Collagen vascular disease –Endocrine –Urticarial vasculitis

18 Urticaria etiologies Urticaria is rarely, if ever the presenting or sole symptom of an underlying disease Urticaria is rarely, if ever the presenting or sole symptom of an underlying disease A complete Review of Systems will suggest or identify any systemic disease in which the urticaria occurs A complete Review of Systems will suggest or identify any systemic disease in which the urticaria occurs

19 URTICARIA ETIOLOGIES Medications Medications –Any drug has the potential to elicit an allergic reaction Antibiotics in general, and penicillins specifically, are most often indicated Antibiotics in general, and penicillins specifically, are most often indicated Aspirin/NSAID’s Aspirin/NSAID’s Considered second most common cause of acute drug allergic reactions Considered second most common cause of acute drug allergic reactions Frequently exacerbate chronic urticaria and angioedema Frequently exacerbate chronic urticaria and angioedema

20 URTICARIA ETIOLOGIES Foods Foods Important cause of acute urticaria Important cause of acute urticaria –Primary allergens are peanuts, tree nuts, shellfish, fish, eggs, milk Chronic urticaria typically unrelated to food allergy Chronic urticaria typically unrelated to food allergy Infection Infection Common cause of acute urticaria Common cause of acute urticaria –Viral infection most common cause in children –Episodes are self-limited Rare cause of chronic urticaria Rare cause of chronic urticaria

21 INSECT BITES & STINGS Generalized urticaria/angioedema Generalized urticaria/angioedema –Indicates systemic reaction –Requires allergist evaluation for possible immunotherapy Urticaria in children does not require immunotherapy Urticaria in children does not require immunotherapy –Hymenoptera bees, wasps, yellow jackets, hornets bees, wasps, yellow jackets, hornets Fire ants Fire ants

22 URTICARIA ETIOLOGIES Aeroallergens Aeroallergens –Rarely, if ever, cause urticaria Animals may cause contact urticaria Animals may cause contact urticaria Inhaled latex may result in systemic allergic reaction Inhaled latex may result in systemic allergic reaction ? seasonal pollens ? seasonal pollens Contact Urticaria Contact Urticaria –Nonimmunologic cinnamic acid, benzoic acid cinnamic acid, benzoic acid Diagnosed by open patch test Diagnosed by open patch test –Immunologic (Allergic) Latex, fruits, vegetables Latex, fruits, vegetables Diagnosed by applying material to eczematous or scratched skin Diagnosed by applying material to eczematous or scratched skin

23 Urticaria etiologies Endocrine/autoimmune Endocrine/autoimmune –Thyroid disease Urticaria and angioedema has been associated with hypo- and hyperthyroidism Urticaria and angioedema has been associated with hypo- and hyperthyroidism Possible association with the presence of thyroid autoantibodies (antithyroid peroxidase and antithyroglobulin) Possible association with the presence of thyroid autoantibodies (antithyroid peroxidase and antithyroglobulin) –Thyroid autoimmunity has been demonstrated in % of subjects with chronic urticaria –Thyroid autoimmunity occurs in 3-6% of the population

24 URTICARIA ETIOLOGIES Chronic urticaria Chronic urticaria –Most common etiology is idiopathic –30-60% of patients exhibit a wheal-and-flare with autologous serum skin testing Thought to be due to a complement-activating, histamine-releasing autoantibody (IgG) against the α- chain of the high-affinity IgE receptor (FcεRI) Thought to be due to a complement-activating, histamine-releasing autoantibody (IgG) against the α- chain of the high-affinity IgE receptor (FcεRI) –These autoantibodies are able to trigger mast cell or basophil histamine release through direct crosslinking of adjacent receptors –Can cause histamine release in healthy subjects Treatment implications: urticaria may be more difficult to control Treatment implications: urticaria may be more difficult to control

25 Plasma of patients with chronic urticaria shows signs of thrombin generation, and its intradermal injection causes wheal-and-flare reactions more frequently than autologous serum J Allergy Clin Immunol 2006;117:

26 Chronic urticaria: etiologies 51/96 (53%) patients had positive ASST 51/96 (53%) patients had positive ASST 61/71 (86%) patients had positive APST 61/71 (86%) patients had positive APST Prothrombin fragment F(1+2) (marker of thrombin generation) was higher in patients than in controls Prothrombin fragment F(1+2) (marker of thrombin generation) was higher in patients than in controls –Levels directly related to severity of urticaria

27 Chronic urticaria: etiologies Conclusions Conclusions –Suggests role of the activation of the extrinsic coagulation pathway with thrombin generation in chronic urticaria Thrombin increases vascular permeability (edema) Thrombin increases vascular permeability (edema) May trigger mast cell degranulation May trigger mast cell degranulation –Possible therapeutic use of anticoagulants (heparin/warfarin)

28 Natural history: Chronic Urticaria Up to 50% patients resolve within 3-12 months Up to 50% patients resolve within 3-12 months Another 20% of patients resolve in months or months Another 20% of patients resolve in months or months Up to 1.5% of patients persist for 20+ years Up to 1.5% of patients persist for 20+ years 50% of patients with chronic urticaria will have recurrences 50% of patients with chronic urticaria will have recurrences Physical urticarias tend to last longer, as do more severe forms of chronic urticaria Physical urticarias tend to last longer, as do more severe forms of chronic urticaria

29 PHYSICAL URTICARIAS Symptomatic dermographism Symptomatic dermographism Cholinergic Cholinergic Delayed pressure Delayed pressure Cold Cold Aquagenic Aquagenic Solar Solar Vibratory Vibratory adrenergic adrenergic

30 PHYSICAL URTICARIAS Dermographism Dermographism –Very common- affects 2-5% of population Small fraction of these patients will seek treatment Small fraction of these patients will seek treatment –Stroking of the skin results in linear wheals which may persist as long as 30 minutes patients may complain of generalized pruritus or “skin crawling” patients may complain of generalized pruritus or “skin crawling”

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32 PHYSICAL URTICARIAS Cholinergic urticaria Cholinergic urticaria –Likely the most common of the physical urticarias- 30% of the physical urticarias –Occurs primarily in teenagers and young adults –Pruritic, small macules and papules occur in response to heat, exercise, or emotional stress May occur with wheezing May occur with wheezing May occur without visible skin lesions (cholinergic pruritus) May occur without visible skin lesions (cholinergic pruritus)

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34 Physical urticarias Cold urticaria Cold urticaria –Characterized by the rapid onset of pruritus, erythema, and swelling after exposure to a cold stimulus Holding cold objects: hand swelling Holding cold objects: hand swelling Eating cold items: lip swelling/ oropharyngeal edema Eating cold items: lip swelling/ oropharyngeal edema Swimming, with total body immersion, can result in massive mediator release, resulting in hypotension Swimming, with total body immersion, can result in massive mediator release, resulting in hypotension –Risk factor: oral symptoms with ingestion of cold items

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37 URTICARIA EVALUATION Acute urticaria and angioedema Acute urticaria and angioedema –History to ascertain for possible triggers: food, drug, sting, infection –Exam to confirm diagnosis –May refer to board-certified allergist for select skin testing/challenge tests to suspected agents

38 Urticaria evaluation Chronic urticaria Chronic urticaria –History and physical exam Confirm diagnosis of urticaria/angioedema Confirm diagnosis of urticaria/angioedema –Laboratory studies Usually none required Usually none required –No relationship has been found between the number of identified diagnoses and the number of laboratory tests performed Consider thyroid evaluation (TSH, thyroid autoantibodies) in patients who fail initial therapy Consider thyroid evaluation (TSH, thyroid autoantibodies) in patients who fail initial therapy If urticarial vasculitis suspected: If urticarial vasculitis suspected: –ANA, complement levels –Referral for skin biopsy

39 Skin biopsy Indications Indications –Individual urticarial lesion persists for >48 hours –Urticaria are less than moderately pruritic –Lack of significant response to “maximum” doses of antihistamines

40 URTICARIA MANAGEMENT Goals Goals –control symptoms & keep patient comfortable search for and treat underlying etiologies search for and treat underlying etiologies exclude serious diseases exclude serious diseases Avoidance Avoidance –causative factor if identified –NSAID’s & ASA –excessive heat Supportive therapy Supportive therapy –Reassurance –Patient education is most important

41 Urticaria management Chronic idiopathic urticaria Chronic idiopathic urticaria –Because there is no one specific causative agent that can be withdrawn, the hives cannot be “cured”. –Treatment is considered palliative, until the condition resolves on its own Goal is to maintain a patient’s quality of life, despite condition Goal is to maintain a patient’s quality of life, despite condition

42 INITIAL URTICARIA PHARMACOTHERAPY Antihistamines: H 1 receptor antagonists Antihistamines: H 1 receptor antagonists –Second generation (“Non-sedating”) equal in efficacy to first generation without as many side effects equal in efficacy to first generation without as many side effects –cetirizine, levocetirizine, desloratadine, fexofenadine, loratadine –First generation Generally administered on a daily basis for preventative therapy Generally administered on a daily basis for preventative therapy –hydroxyzine, diphenhydramine, chlorpheniramine, etc. –dose at qhs initially to reduce daytime somnolence –May be used on a prn basis

43 SECONDARY URTICARIA PHARMACOTHERAPY H 2 antagonists H 2 antagonists –15% of histamine receptors in the skin are H 2 –May use in combination with H 1 antagonists –Inhibits metabolism of hydroxyzine, resulting in higher plasma concentration of hydroxyzine Doxepin Doxepin –Very potent H 1 antagonist –H 2 antagonist as well –May be very sedating- generally use at night Leukotriene antagonists Leukotriene antagonists –Zafirlukast and montelukast superior to placebo in the treatment of chronic urticaria

44 Urticaria management Antihistamine “cocktail” Antihistamine “cocktail” –Begin with 2 nd generation antihistamine once a day; if response unsatisfactory, –Double the dose (either split-dose twice daily, or full dose once daily); if response unsatisfactory, ADD –Doxepin qhs (titrate over time to reduce sedation) Levocetirizine/cetirizine>fexofenadine>desloratadine/loratadine

45 SECONDARY URTICARIA PHARMACOTHERAPY Oral corticosteroids Oral corticosteroids –Role of systemic steroids in the treatment of chronic urticaria is limited –Short-term use in special situations (e.g. control of symptoms prior to an important event.) –Prolonged treatment complicated by severe side effects along with worsening of urticaria upon withdrawal

46 Alternative agents for refractory chronic urticaria DrugLevel of evidence Leukotriene modifiersIb DapsoneIIb SulfasalazineIII HydroxychloroquineIb ColchicineIII Calcineurin inhibitorsIb MycophenolateIIb OmalizumabIII

47 SECONDARY URTICARIA PHARMACOTHERAPY Immunomodulatory agents Immunomodulatory agents –Limited studies demonstrate efficacy of cyclosporine in improving urticaria along with decreasing dependence on prednisone. Suppressive effect on basophil and mast cell activation Suppressive effect on basophil and mast cell activation Requires monitoring of a patient’s blood pressure and renal function Requires monitoring of a patient’s blood pressure and renal function

48 Cyclosporine Patients with chronic, severe urticaria with positive autologous skin test Patients with chronic, severe urticaria with positive autologous skin test –3-month course of treatment resulted in 80% totally or almost clearing their symptoms –Upon medication withdrawal at 3 months: 1/3 remained clear 1/3 remained clear 1/3 relapsed mildly 1/3 relapsed mildly 1/3 relapsed to baseline 1/3 relapsed to baseline Br J Dermatol 2000;143:368.

49 Urticaria and angioedema Pearls Pearls –Urticaria and angioedema frequently is not an allergic condition –Urticaria does not respond to topical treatment –Urticaria in the setting of antibiotics use might be due to the infection, rather than the antibiotic –Almost all urticaria is responsive to antihistamines; if your initial dose does not work, use more –When all else fails, refer to your favorite fellowship-trained allergy and immunology specialist –Treatment references: N Engl J Med 2002;346:175-9 or Allergy and Asthma Proc 2004;25: or Allergy and Asthma Proc 2004;25:

50 I Need an Allergist!!


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