Presentation on theme: "Urticaria and Angioedema 101"— Presentation transcript:
1 Urticaria and Angioedema 101 Scot Laurie, MDAllergy and Asthma Center at NorthParkAssistant professor, University of Texas Southwestern Medical Center
2 Case PresentationJim 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.He might have had a similar episode 10 years ago and his doctor told him he was allergic to penicillin
3 Case presentationHe has visited his primary care physician who suggested he take ClaritinHe 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 Medications Review of systems HypothyroidismMedicationslevothyroxineIbuprofen prnReview of systemsOccasional headaches; otherwise negativePhysical exam
6 Case presentation How would his hives be classified? What is causing his hives?Are his medical conditions or medications contributing to his hives?What tests should be done to evaluate his hives?How are his hives best treated?
7 URTICARIA & ANGIOEDEMA DESCRIPTIONUrticariaRaised, erythematous, blanchingPruriticLesions well-circumscribed; typically coalesceAngioedemaSubcutaneous swellingPredilection to areas of loose connective tissue, such as the face or mucus membranes involving the lips or the tongue
9 Urticaria or “Hives” Urticaria or “Hives” Urticaria consists of raised, erythematous skin lesions that are markedly pruritic, tend to be evanescent in any one location, and generally are worsened by scratching. Angioedema is frequently associated with urticaria except that it occurs in deeper tissues and is clinically characterized by asymmetric swelling of tissue.Metzger WJ. Urticaria, angioedema, and hereditary angioedema. In: Patterson, et al, eds. Allergic Diseases: Diagnosis and Management. 4th ed. Philadelphia, Pa: JB Lippincott Co; 1993:
11 Urticaria and Angioedema Clinical features: UrticariaRepeated occurrence of short-lived cutaneous wheals accompanied by erythema and pruritusWheals range in size from a few millimeters to several centimetersWheals may coalesce to form larger lesionsIndividual wheals typically last less than 24 hoursUrticaria may occur anywhere on the skinMucus membrane involvement is rareLesions should resolve without any residual marking
12 Urticaria and Angioedema Clinical features: AngioedemaApproximately 50% of patients with chronic urticaria have angioedema as wellEpisodes of short-lived deep dermal and subcutaneous or submucosal edemaLike urticaria, symptoms generally last less than 24 hoursLarger swellings may take longer to resolvePruritus does not consistently accompany angioedema, and may not occur at all.
13 Mediators of hives and swelling SourceFactorMast cells (cutaneous)HistamineProstaglandin D2Leukotrienes C and DPlatelet Activating FactorComplement systemAnaphylatoxins C3a, C4a, C5a: histamineHageman factor- dependent pathwayBradykininMononuclear cellsHistamine-releasing factors, chemokines
15 URTICARIA CLASSIFICATION Acute: < 6 weeksAffects as many as 10-20% of the population at some point in their livesEtiology frequently identifiedFood allergyDrug allergyStings/venomsInfectionViral infection leading cause of urticaria in children
16 Urticaria Classification Recurrent acute (intermittent)Episodes of urticaria lasting days or weeks with intervals of days, weeks, or months in between episodesChronic: > 6 weeksIdiopathicPhysical urticarias
18 Urticaria etiologiesUrticaria is rarely, if ever the presenting or sole symptom of an underlying diseaseA complete Review of Systems will suggest or identify any systemic disease in which the urticaria occurs
19 URTICARIA ETIOLOGIES Medications Any drug has the potential to elicit an allergic reactionAntibiotics in general, and penicillins specifically, are most often indicatedAspirin/NSAID’sConsidered second most common cause of acute drug allergic reactionsFrequently exacerbate chronic urticaria and angioedema
20 URTICARIA ETIOLOGIES Foods Infection Important cause of acute urticariaPrimary allergens are peanuts, tree nuts, shellfish, fish, eggs, milkChronic urticaria typically unrelated to food allergyInfectionCommon cause of acute urticariaViral infection most common cause in childrenEpisodes are self-limitedRare cause of chronic urticaria
21 INSECT BITES & STINGS Generalized urticaria/angioedema Indicates systemic reactionRequires allergist evaluation for possible immunotherapyUrticaria in children does not require immunotherapyHymenopterabees, wasps, yellow jackets, hornetsFire ants
22 URTICARIA ETIOLOGIES Aeroallergens Contact Urticaria Rarely, if ever, cause urticariaAnimals may cause contact urticariaInhaled latex may result in systemic allergic reaction? seasonal pollensContact UrticariaNonimmunologiccinnamic acid, benzoic acidDiagnosed by open patch testImmunologic (Allergic)Latex, fruits, vegetablesDiagnosed by applying material to eczematous or scratched skin
23 Urticaria etiologies Endocrine/autoimmune Thyroid disease Urticaria and angioedema has been associated with hypo- and hyperthyroidismPossible association with the presence of thyroid autoantibodies (antithyroid peroxidase and antithyroglobulin)Thyroid autoimmunity has been demonstrated in % of subjects with chronic urticariaThyroid autoimmunity occurs in 3-6% of the population
24 URTICARIA ETIOLOGIES Chronic urticaria Most common etiology is idiopathic30-60% of patients exhibit a wheal-and-flare with autologous serum skin testingThought 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 receptorsCan cause histamine release in healthy subjectsTreatment 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 serumJ Allergy Clin Immunol 2006;117:
26 Chronic urticaria: etiologies 51/96 (53%) patients had positive ASST61/71 (86%) patients had positive APSTProthrombin fragment F(1+2) (marker of thrombin generation) was higher in patients than in controlsLevels directly related to severity of urticaria
27 Chronic urticaria: etiologies ConclusionsSuggests role of the activation of the extrinsic coagulation pathway with thrombin generation in chronic urticariaThrombin increases vascular permeability (edema)May trigger mast cell degranulationPossible therapeutic use of anticoagulants (heparin/warfarin)
28 Natural history: Chronic Urticaria Up to 50% patients resolve within 3-12 monthsAnother 20% of patients resolve in months or monthsUp to 1.5% of patients persist for 20+ years50% of patients with chronic urticaria will have recurrencesPhysical urticarias tend to last longer, as do more severe forms of chronic urticaria
30 PHYSICAL URTICARIAS Dermographism Very common- affects 2-5% of populationSmall fraction of these patients will seek treatmentStroking of the skin results in linear wheals which may persist as long as 30 minutespatients may complain of generalized pruritus or “skin crawling”
32 PHYSICAL URTICARIAS Cholinergic urticaria Likely the most common of the physical urticarias- 30% of the physical urticariasOccurs primarily in teenagers and young adultsPruritic, small macules and papules occur in response to heat, exercise, or emotional stressMay occur with wheezingMay occur without visible skin lesions (cholinergic pruritus)
34 Physical urticarias Cold urticaria Characterized by the rapid onset of pruritus, erythema, and swelling after exposure to a cold stimulusHolding cold objects: hand swellingEating cold items: lip swelling/ oropharyngeal edemaSwimming, with total body immersion, can result in massive mediator release, resulting in hypotensionRisk factor: oral symptoms with ingestion of cold items
37 URTICARIA EVALUATION Acute urticaria and angioedema History to ascertain for possible triggers: food, drug, sting, infectionExam to confirm diagnosisMay refer to board-certified allergist for select skin testing/challenge tests to suspected agents
38 Urticaria evaluation Chronic urticaria History and physical exam Confirm diagnosis of urticaria/angioedemaLaboratory studiesUsually none requiredNo relationship has been found between the number of identified diagnoses and the number of laboratory tests performedConsider thyroid evaluation (TSH, thyroid autoantibodies) in patients who fail initial therapyIf urticarial vasculitis suspected:ANA, complement levelsReferral for skin biopsy
39 Skin biopsy Indications Individual urticarial lesion persists for >48 hoursUrticaria are less than moderately pruriticLack of significant response to “maximum” doses of antihistamines
40 URTICARIA MANAGEMENT Goals control symptoms & keep patient comfortable search for and treat underlying etiologiesexclude serious diseasesAvoidancecausative factor if identifiedNSAID’s & ASAexcessive heatSupportive therapyReassurancePatient education is most important
41 Urticaria management 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 ownGoal is to maintain a patient’s quality of life, despite condition
42 INITIAL URTICARIA PHARMACOTHERAPY Antihistamines: H1 receptor antagonistsSecond generation (“Non-sedating”)equal in efficacy to first generation without as many side effectscetirizine, levocetirizine, desloratadine, fexofenadine, loratadineFirst generationGenerally administered on a daily basis for preventative therapyhydroxyzine, diphenhydramine, chlorpheniramine, etc.dose at qhs initially to reduce daytime somnolenceMay be used on a prn basis
43 SECONDARY URTICARIA PHARMACOTHERAPY H2 antagonists15% of histamine receptors in the skin are H2May use in combination with H1 antagonistsInhibits metabolism of hydroxyzine, resulting in higher plasma concentration of hydroxyzineDoxepinVery potent H1 antagonistH2 antagonist as wellMay be very sedating- generally use at nightLeukotriene antagonistsZafirlukast and montelukast superior to placebo in the treatment of chronic urticaria
44 Urticaria management Antihistamine “cocktail” Begin with 2nd generation antihistamine once a day; if response unsatisfactory,Double the dose (either split-dose twice daily, or full dose once daily); if response unsatisfactory, ADDDoxepin qhs (titrate over time to reduce sedation)Levocetirizine/cetirizine>fexofenadine>desloratadine/loratadine
45 SECONDARY URTICARIA PHARMACOTHERAPY Oral corticosteroidsRole of systemic steroids in the treatment of chronic urticaria is limitedShort-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 Drug Level of evidenceLeukotriene modifiers IbDapsone IIbSulfasalazine IIIHydroxychloroquine IbColchicine IIICalcineurin inhibitors IbMycophenolate IIbOmalizumab III
47 SECONDARY URTICARIA PHARMACOTHERAPY Immunomodulatory agentsLimited studies demonstrate efficacy of cyclosporine in improving urticaria along with decreasing dependence on prednisone.Suppressive effect on basophil and mast cell activationRequires monitoring of a patient’s blood pressure and renal function
48 CyclosporinePatients with chronic, severe urticaria with positive autologous skin test3-month course of treatment resulted in 80% totally or almost clearing their symptomsUpon medication withdrawal at 3 months:1/3 remained clear1/3 relapsed mildly1/3 relapsed to baselineBr J Dermatol 2000;143:368.
49 Urticaria and angioedema PearlsUrticaria and angioedema frequently is not an allergic conditionUrticaria does not respond to topical treatmentUrticaria in the setting of antibiotics use might be due to the infection, rather than the antibioticAlmost all urticaria is responsive to antihistamines; if your initial dose does not work, use moreWhen all else fails, refer to your favorite fellowship-trained allergy and immunology specialistTreatment references: N Engl J Med 2002;346:175-9or Allergy and Asthma Proc 2004;25:
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