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GLORIA Module 12: Urticaria

GLORIA Module 12: Urticaria. an educational program of. Updated: June 2011. Sponsored by an unrestricted educational grant from. Global Resources in Allergy (GLORIA™).

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GLORIA Module 12: Urticaria

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  1. GLORIA Module 12:Urticaria an educational program of Updated: June 2011

  2. Sponsored by an unrestricted educational grant from

  3. Global Resources in Allergy (GLORIA™) Global Resources In Allergy (GLORIA™) is the flagship program of the World Allergy Organization (WAO). Its curriculum educates medical professionals worldwide through regional and national presentations. GLORIA modules are created from established guidelines and recommendations to address different aspects of allergy-related patient care.

  4. World Allergy Organization (WAO) The World Allergy Organization is an international coalition of 89 regional and national allergy and clinical immunology societies.

  5. WAO’s Mission WAO’s mission is 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

  6. GLORIA Module 12: Urticaria Authors: Allen P Kaplan Malcolm W Greaves

  7. Learning objectives Following this presentation you should be able to: • Distinguish the various forms of physical urticaria • Formulate a differential diagnosis and treatment plan for acute urticaria • Describe the role of autoimmunity as a pathogenic mechanism for chronic urticaria • Describe a therapeutic approach for patients with severe chronic idiopathic or chronic autoimmune urticaria. • Distinguish urticarial vasculitis from other forms of chronic urticaria

  8. Urticaria and angioedema Definition: A wheal and flare reaction initiated at the level of the small venules of the skin in response to substances that cause vasodilatation, increase vascular permeability, and for histamine, stimulate type C unmyelinated afferent cutaneous neurons to release neuropeptides (axon reflex)

  9. Definition of urticaria (also called hives, nettle rash) and epidemiology Urticaria affects up to 2% of the population at some time in a lifetime Transitory (individual episodes < 24h duration) red skin swellings with itching No desquamation, rarely affects mucous membranes Associated with angioedema in about 40% of cases

  10. Pathophysiology of urticaria Most types of urticaria are due to promiscuous activation of dermal mast cells, although basophils may also be involved Release of histamine and other mediators (including eicosanoids, proteases, cytokines) causes local vasodilation, vasopermeability, fibrin deposition, perivascular infiltration by lymphocytes, neutrophils, and eosinophils, and pruritus There is minimal endothelial swelling and no leukocytoclasis

  11. Substances that cause hive formation when injected into the skin include: Histamine Leukotrienes C and D Platelet activating factor (PAF) Bradykinin Substance P

  12. Skin rashes which mimic urticaria (“pseudourticaria”) Maculopapular exanthems (viral, drug rashes) Urticarial dermatitis Erythema multiforme Insect bite reactions (“papular urticaria”) Leukocytoclastic vasculitis (including urticarial vasculitis) Polymorphic light eruption Some autoinflammatory syndromes (e.g., Muckle-Wells)

  13. Classification of urticaria into acute and chronic • “Urticaria” is an umbrella term inclusive of diverse clinical entities • Conventionally (eg European guidelines: Allergy, 2004) it is broadly divided into acute and chronic • Chronic urticaria is conventionally defined as “daily or almost daily urticarial eruptions occurring for 6 weeks or more” • Chronic urticaria is further subclassified into several distinct entities

  14. Ordinary chronic urticaria

  15. Classification of chronic urticaria Chronic urticaria Ordinary chronic urticaria Urticarial vasculitis Physical urticaria Contact urticaria Schnitzler’s syndrome Autoimmune urticaria Idiopathic chronic urticaria

  16. Acute urticaria All ages; common in childhood Abrupt onset of urticarial eruption usually pruritic and widespread Angioedema common Systemic symptoms (fever, malaise) also common, depending on cause Duration: usually hours or days Zuberbier T, Ifflander J, Semmler C, et. al. Acta Derm Venereol 76:295-297, 1996.

  17. Causes of acute urticaria Viral infections; particularly in children. In adults: prodrome of Hepatitis B, infectious mononucleosis (EBV) Drugs (NSAIDS, penicillins and derivatives, radiocontrast media) Foods non–allergic (e.g., scombroid fish poisoning) and allergic (IgE–mediated) (e.g., nuts, shellfish) Immunization vaccines e.g., MMR, tetanus toxoid

  18. Investigation of acute urticaria • Many cases require no investigation - the cause is evident to patient and doctor alike • Skin prick tests may support the diagnosis (but avoid SPT in severely affected patients, and in patients with current angioedema or a history of angioedema) • Serum IgE testing may also help confirm the culprit

  19. Acute urticaria: prognosis and treatment Many attacks of acute urticaria are solitary, and the cause is evident and avoidable Facial / labial / buccal angioedema should respond to Primatene mist spray and / or subcutaneous adrenaline administered every 10-15 min Severe oropharyngeal angioedema should prompt overnight admission Chlorpheniramine 4 mg or diphenhydramine 50 mg by injection or by mouth is usually sufficient to suppress even widespread urticaria Zuberbier T, Greaves MW, Juhlin L, et. al. J Invest Dermatol Symp Proc 6:128-131, 2001.

  20. Food allergy Mediated by binding of allergens that survive digestion, and delivered to the skin to interact with IgE on cutaneous mast cells Can be diagnosed by skin test or RAST assay – result must be correlated with history and be reproducible Double-blind oral challenge represents the definitive test for diagnosis

  21. Drug reactions - 1 Drug or drug metabolite causing hives by interaction with IgE antibody on cutaneous mast cells Example: Penicillin allergy Non-IgE mediated reactions that depend on drug metabolism with resultant mast cell activation or direct interaction with resultant mast cell activation or direct interaction with small venules Example: NSAID reactions

  22. Drug reactions - 2 Direct mast cell degranulation by drugs Example: Opiates Osmotic cell degranulation and alternative complement pathway activation Example: Radiocontrast reactions

  23. Physical urticarias: classification Common: • Symptomatic dermographism (also called factitious urticaria) • Delayed pressure urticaria • Cholinergic urticaria Less common: • Cold contact urticaria Rare: • Solar urticaria • Heat contact urticaria • Aquagenic urticaria • Vibratory angioedema

  24. Characteristics of physical urticarias (except delayed pressure) Hives last less than 2 hours Stimulus (e.g., ice cube test, exercise, scratching) has no late phase response Treated readily with antihistamines but may require high doses Do not respond to corticosteroids Soter N. Physical urticaria/angioedema. Seminar Dermatology 6:302, 1987.

  25. Symptomatic dermatographism Common physical urticaria, frequently overlooked Generalized pruritus and red wheals, aggravated by scratching, rubbing, tight or coarse clothing Mucous membranes unaffected, no angioedema Greaves MW. Chronic Urticaria. J Allergy Clin Immunol 105:664, 2000. Greaves M, Sundergaard. Arch Dermatol 101:418-425, 1970.

  26. Symptomatic dermatographism, cont. • Diagnosis: firm stroking of uninvolved skin causes almost immediate linear red wheal and itch. A variable pressure dermographometer which can be calibrated is commercially available • Investigations: none indicated • Treatment: low sedation H1 antihistamines (off-label dosage if necessary)

  27. Dermatographism 27

  28. Characteristics of dermatographism Patients complain of itch (even if hives not present), skin “crawling” and worsening hives with scratching Particularly prominent over pressure points where clothing is tight or rubbing Can be associated with lip swelling without other evidence of angioedema When severe, may be confused with other types of chronic urticaria

  29. Therapy of dermatographism Non-sedating antihistamines; fexofendadine, cetirizine, desloratidine, levocetirizine May combine agents for more severe cases, e.g., fexofenadine in the morning, levocetirizine midday and bedtime For unresponsive cases to the above: hydroxyzine or diphenhydramine at 25-50 mg q.i.d. Shiarpe GR, Shuster S. Br J Dermatol 129:575-579, 1993.

  30. Delayed pressure urticaria Concurrent with chronic ordinary urticaria in about 40% of cases Common distribution sites: shoulders, waist, soles, palms Swellings are frequently of long duration (> 24h), often tender and painful; arthralgia common Estes S, Yung C. J Am Acad Dermatol 5:25-31, 1981. Dover JS, Kobza Black A, Milford WA, et. al. J Am Acad Dermatol 18:1289-1298, 1988.

  31. Delayed pressure urticaria, cont. • Diagnosis: firm application of tip of a 3mm diameter rod to uninvolved skin for 2 min; positive result – persistent firm red papule developing in 3-5 hours • Investigations: none indicated • Treatment: antihistamines disappointing; salazopyrene, dapsone, hydroxycholorquine should be tried. High dose prednisolone is effective but the condition is chronic

  32. Cholinergicurticaria Grant RT, Pearson RS, Comeau WJ. Clin Sci 2;253-272, 1936. Soter NA, Wasserman SI, Austen KF, et. al. N Eng J Med 302:604-608, 1980. Very common in older children, young adults Transitory pruritic symmetrical red maculopapular rash on neck trunk, limbs after exercise, heat, emotion Associated bronchospasm in more severe cases, rarely angioedema

  33. Cholinergicurticaria, cont. • Diagnosis: exercise challenge eg treadmill or jogging in place usually elicits a positive response. Heat challenge e.g., hot bath to evoke the rash • Investigation: none indicated • Treatment: usually responds well to H1 antihistamines, anabolic steroids eg, danazol effective in severely affected cases • Prognosis: usually resolves in months / a year or two Grant RT, Pearson RS, Comeau WJ. Clin Sci 2;253-272, 1936. Soter NA, Wasserman SI, Austen KF, et. al. N Eng J Med 302:604-608, 1980.

  34. Cholinergic (generalized heat) urticaria

  35. Characteristics of cholinergic urticaria - 1 Hives begin on neck, trunk, and spread to face and extremities Triggered by exercise, sweating, hot showers, strong emotion; Exercise induction is reproducible; requires increase in core body temperature Small punctate urticarial lesions a few mm in diameter with prominent erythema Occasional confluence of lesions and associated angioedema Histamine release demonstrated within circulation after exercise challenge

  36. Characteristics of cholinergic urticaria - 2 Sub-groups: A. Positive skin test with autologous sweat, positive in vitro histamine release with autologous sweat; positive methacholine skin test with satellite lesions; non-follicular distribution of wheals B. Negative skin tests and in vitro histamine release with autologous sweat; negative methacholine skin test; wheals tend to be follicular Kaplan A, Gray L, Shaff R, et. al. J Allergy Clin Immunol 55:394-402, 1975. Fukunaga A, Bito T, Tsura K, et. al. J Allergy Clin Immunol 116:397-402, 2003.

  37. Histamine release in serum of patient with cholinergic urticaria challenged by exercise

  38. Cold contact urticaria Redness, whealing itching on skin exposed to cold surfaces, water, air Angioedema can occur e.g., lips, tongue after sucking an iced-lolly If generalized (e.g., sea bathing), can be life threatening (syncope)

  39. Cold contact urticaria, cont. • Diagnosis: place icepack on uninvolved skin for 15 min, remove and inspect site for cold–evoked wheal 5 min after removal • Investigations: cryoglobulins and cold agglutinins commonly sought but rarely found • Treatment: usually responds to avoidance + H1 antihistamines. Cold tolerance treatment (“cold desensitization”) is effective in selected cases

  40. Cold urticaria 40

  41. Cold urticarias - 1 Hives due to contact with cold stimulus / object Predominance on unclothed areas – hands, face Can be generalized with anaphylactic-like symptoms (hypotension) with swimming

  42. Cold urticarias - 2 A sub-group is IgE-mediated and can be passively transferred Treated with oral antihistamines: a) Try fexofenadine, cetirizine, levocetirizine, desloratadine first b) Cyproheptedine 4 mg t.i.d. up to 8 mg q.i.d. may be particularly effective in resistant cases Houser D, Arbesman C, Ito K, et. al. Am J Med 49:23-33, 1970. Wanderer A, St-Pierre J, Ellis E. Arch Dermatol 13:1375-1377, 1977. Kaplan A, Garofalo J, Sigler R, et. al. N Eng J Med 305:1074-1077, 1981.

  43. Histamine release in cold urticaria

  44. Rare physical urticarias: diagnosis, treatment Kobza-Black A. In: Urticaria and Angioedema. Eds. M.W. Greaves and A.P. Kaplan. Marcel Dekker Inc. New York, 2004, P. 171-214. • Solar urticaria: Diagnosis: expose skin to direct sunlight, slide projector lamp; a local pruritic wheal and flare reaction denotes a positive result Treatment: avoidance, H1 antihistamines, light tolerance treatment in selected patients • Heat contact urticaria: Diagnosis: place warm beaker base (45o C) on clinically uninvolved skin for 5 min; a local pruritic wheal and flare reaction denotes a positive result Treatment: avoidance and H1 antihistamine

  45. Rare physical urticarias: diagnosis, treatment, cont. • Aquagenic urticaria: Diagnosis: expose face, neck upper trunk skin to tepid water (eg squeezing a sponge); elicits a transitory pruritic erythematous maculopapular eruption • Vibratory angioedema: Diagnosis: vibrate forearm with a laboratory vortex or rub a towel vigorously across the back (assuming no dermatographism). Treatment: avoidance and H1 antihistamines Kobza-Black A. In: Urticaria and Angioedema. Eds. M.W. Greaves and A.P. Kaplan. Marcel Dekker Inc. New York, 2004, P. 171-214.

  46. Chronic urticarias 1) Chronic ordinary urticaria: a) Idiopathic b) Autoimmune 2) Cutaneous vasculitis: a) Idiopathic b) Connective tissue diseases c) Hypocomplementemic urticarial vasculitis syndrome 3) Genetic autoinflammatory syndromes 4) Miscellaneous, e.g., Schnitzler’s Syndrome

  47. Chronic ordinary urticaria The cause of many cases of chronic ordinary urticaria still remains unclear, but the weight of available evidence indicates that the following are notcausative: • Food allergy • Chronic infections including Helicobacter pylori • “Stress” • Drug allergy • Environmental pollution

  48. Certain factors do exacerbate pre-existing chronic ordinary urticaria Non-steroidal anti-inflammatory drugs (NSAIDS) Certain “pseudoallergens” in foods (controversial) Consumption of alcohol Intercurrent viral infections Stress / overtiredness

  49. There are recognized associations with chronic ordinary urticaria Leznoff A, Sussman G. J Allergy Clin Immunol 84:66-71, 1989. Greaves M. N Eng J Med 332:1767-1772, 1995. Kaplan A. J Allergy Clin Immunol 114:465-474, 2004. • Angioedema: occurs in 40-80% of patients in different series, mainly affecting the eyelids, lips or tongue. Although alarming it is never fatal • Physical urticarias: (usually symptomatic dermatographism, or delayed pressure urticaria) occur in about 50% • Functional thyroid disease: (hypo- or hyperthyroidism) occurs in about 20% and Hashimoto’s disease is found in about 15%

  50. Chronic urticaria: what if the patient has more than one type concurrently? • Patients with chronic urticaria frequently have both chronic ordinary urticaria and a physical urticaria (usually delayed pressure urticaria or symptomatic dermatographism) • Which of these is the principle cause of the patient’s handicap needs to be established by taking a detailed history, as the patient’s investigations and treatment will depend on this Barlow RJ, Warburton F, Watson K, et. al. J Am Acad Dermatol 29:954-958, 1993.

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