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Dr. Kessel Aharon , Department of Allergy and Clinical Immunology Bnai-Zion,2007

CHRONIC AND ACUTE URTICARIA IN CHILDREN. Dr. Kessel Aharon , Department of Allergy and Clinical Immunology Bnai-Zion,2007. Urticaria , is characterized by transient, itchy, elevated edematous wheals or red papules. Wheal typical features # A central swelling, surrounded by erythema.

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Dr. Kessel Aharon , Department of Allergy and Clinical Immunology Bnai-Zion,2007

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  1. CHRONIC AND ACUTE URTICARIA IN CHILDREN Dr. Kessel Aharon , Department of Allergy and Clinical Immunology Bnai-Zion,2007

  2. Urticaria, is characterized by transient, itchy, elevated edematous wheals or red papules.

  3. Wheal typical features # A central swelling, surrounded by erythema. # Itching or burning sensations # The wheal disappear usually within 1-24 h.

  4. Angioedema # Pronounced swelling of the lower dermis and subcutis. # Most often found in the lips, eyelids or genitalia. # Itching and sometimes pain. # Resolution can take up to 72h. # It is associated with urticaria in about 40% of cases.

  5. The histology of the lesions of chronic idiopathic urticaria Venule and capillary dilatation tissue edema –upper and mid dermis. predominantly perivascular cellular infiltrate – primarily mononuclear cells T cell are the predominant cell type (80%) Mild to moderate increase of mast Cell numbers

  6. Clinical classification of urticaria and angioedema • Ordinary urticaria- acute , chronic, episodic. • Physical urticaria • Angioedema • Contact urticaria • Urticarial vasculitis

  7. Clinical classification of urticaria and angioedema Ordinary urticaria Acute- up to 6 weeks of continuous activity Chronic – 6 weeks or more of continuous Episodic (intermittent)

  8. מה היא הסיבה השכיחה ביותר שניתן למצוא כגורמת לאורטיקריה חריפה ? • אלרגיה לתרופות • מחלות זיהומיות • עקיצות חרקים • חומרי ניגוד • אלרגיה למזון

  9. 57 children age range 1-36 months Presumptive cause Viral infection 18 cases (12 associated with drug intake) Adenovirus EBV Enterovirus RSV ROTA virus Varicella-Zoster virus BACTERIAL INFECTION 1 CASE– E.COLI FOOD -6 cases Probable cause (27 cases)– viral infections Mortureux P, et al Archives of dermatology 1998;143:319-23.

  10. Acute urticaria • Infections- viral :herpes simplex, hepatitis B, coxsackie A and B, upper respiratory infections. • Bacterial- associated with certain infectious foci: dental caries/abscesses, pharyngitis /tonsillitis, otitis media, occult abscesses, UTI. • Parasitic : ascaris, strongyloides, echinococcus, toxocara, fasciola, filaria, schistosoma. • Fungal?: candida

  11. Acute urticaria • Exposure to food allergens- milk, eggs, peanuts, sesame ,soy wheat, shellfish, fish. • Medications-β-lactam antibiotics, sulfonamides, aspirin. • Radiocontrast media

  12. ילדה בת 4 שנים מגיעה לחדר מיון עם אוטיקריה, היא מטופלת ע"י רופא מטפל באנטיהיסטמינקה למרות כך עדיין הפריחה קיימת. האם מתן קורטיקוסטרואידים בנוסף לאנטיהיסטמיניקה עוזר בטיפול באורטיקריה חריפה ?

  13. Treatment of acute urticaria Ann Emerg Med. 1995 Nov;26(5):547-51. Short Outpatient management of acute urticaria: the role of prednisone. CONCLUSION: The addition of a prednisone burst improves the symptomatic and clinical response of acute urticaria to antihistamines. Patients' conditions improved more quickly and more completely when prednisone was administered, without any apparent adverse effects.

  14. ALGORITHM FOR ACUTE URTICARIA/ANGIOEDEMA DETAILED HISTORY: infection drug food physical triggers PHYSICAL EXAMINATION SPECIFIC EXAMINATION- CBC , URINE, ESR SKIN TESTS

  15. IgE-dependent (type -1)

  16. Skin Prick Test (SPT)

  17. Positive reaction

  18. SYNDROMYES THAT CAN BE ASSOCIATED WITH URTICARIA Muckle-Wells syndrome Familial -dominantly inherited. urticaria progressive nerve deafness Limb pain arthritis Recurrent fever hypergammaglobulinemia amyloidosis

  19. Gleich's syndrome - the episodic angioedema with eosinophilia syndrome Schnitzler syndrome (SS), first reported in 1972, is characterized by chronic, nonpruritic urticaria in association with recurrent fever, bone pain, arthralgia or arthritis, and a monoclonal immunoglobulin M (IgM) gammopathy in a concentration that is usually less than 10 g/dL.

  20. Chronic Urticaria – 6 weeks or more of continuous urticaria.

  21. מה היא הסיבה השכיחה ביותר לאורטיקריה כרונית ? • אלרגיה לתרופות • גורמים זיהומיים • אידיופטי • מנגנון אוטואימוני • אלרגיה למזון • דלקת מפרקים של גיל הילדות

  22. 93 ילדים (52-זכרים , 41 נקבות , גיל ממצוע 7.8 שנים ) אורטיקריה-אידיופטית 31% אורטיקריה אוטואימונית 29% אורטיקריה עקב גורם זיהומי 15% אורטיקריה פיזיקאלית 12% אורטיקריה מולטיפקטוריאלית 11% אורטיקריה אלרגית – 2% Brunetti et al. J Allergy Clin Immunol 2004;114:922-7

  23. Autoimmune urticaria In 1986 Grattan first described that: Autologous serum test produces a Wheal and Flare reaction In 30-50% of CIU cases

  24. Autoimmune (autoantibodies against FcεRI or IgE) In 1993 M. Greaves first found the presence of autoantibodies in CIU. N Engl J Med 1993;328:1599-604

  25. In vitro studies demonstrated that 75% of autoAb are IgG against FceR (causing in-vitro human basophil degranulation) 15% were IgG against IgE (cross-linking two IgEs on mast cells)

  26. Autoimmune basis for CIU Thyroid autoimmunity in pts with CIU anti-thyroid Ab. in 14-20% increased / decreased thyroid function in 5-10%

  27. Immune aberrations- basis for CIU Activated T cell express increased expression of CD40L B and T cell lymphocytes derived from active CU patients demonstrated an increase expression of bcl-2. Toubi et al. J Clin Immuno 2000;20:371-378

  28. Physical urticaria The physical urticarias are characterized by the development of wealing and itching promptly after application of the appropriate physical stimulus. Weals typically fade within 30-60 minutes. The exception is delayed pressure urticaria when the weals take several hours to appear after sustained pressure and can last up to 48 hours.

  29. Physical urticaria-cholinergic Itchy, monomorphic pale or pink wheals on trunk, neck, and limbs – after exercise or a hot shower Prevalence of 11% in the age group of 16-35 years.

  30. Physical urticaria-pressure Large painful or itchy red swelling at sites of pressure (soles, palms, or waist) lasting 24 hours or more- application of pressure perpendicular to skin produces red swelling after a latent period of 1 to 4 hours.

  31. Physical urticaria- dermographic urticaria Itchy, linear wheals with surrounding bright-red flare at sites of scratching or rubbing.

  32. Physical urticaria- dermographic urticaria # The most frequent form of physical urticaria. # Affecting mainly young adults # Mean duration 6.5 years

  33. Physical urticaria- heat A rare form of urticaria. nduced by direct contact of the skin with warm objects or warm air. The eliciting temperature ranges from 38º C to more than 50 º C .

  34. Physical urticaria- cold Itchy pale or red swelling at sites of contact with cold surfaces or fluids- ten minutes application of an ice pack causes a wheal within five minutes of the removal of ice.

  35. Physical urticaria- cold More frequent in women than men. Majority is idiopathic, some can also occur as a result of infections, neoplasia or autoimmune diseases. Infectious: syphilis, measles hepatitis ,mononucleosis, HIV.

  36. Other forms of physical urticaria Solar Vibratory Adrenergic aquagenic

  37. Role of nonallergic hypersensitivity reactions in children with chronic urticaria ~ 5 % Food –symptoms clears within 48 hours if relevant food allergens are eliminated. Food additives Elimination pseudoallergen diet - for at least 2-3 weeks before beneficial effect seen. Allergy 1998:53:1074-7

  38. The prevalence in different studies ~ 5-10% Viruses- hepatitis B,C , EBV , CMV Bacteria- helicobacter pylori ? streptococci/staphylococci parasites -Anecdotal series of cases documented a link between parasites and chronic urticaria such as: toxocara canis, giaardia lamblia, strongyloides stercoralis and even blastocytis hominis.

  39. Urticarial vasculitis

  40. Urticarial vasculitis

  41. IDIOPATHIC URTICARIA • Do not have a predominantly physical trigger. • Are not caused by underlying vasculitis. • Are not caused by direct contact with the causative agent. • This heterogeneous group includes: cases for which no cause can be identified.

  42. CU Duration ?? * Patients ask, how long CU will last ?? * More than 60% still visit the clinic after 6 months. * 40% of patients still suffer from CU after one year.

  43. Clinical and Laboratory Parameters in Predicting Chronic Urticaria Duration: A Prospective Study of 139 Patients 94% 75% CU% 52% 43% 14% 60 36 24 12 6 Urticaria duration (months) Toubi et al Allergy:2003

  44. Rates of urticaria duration in relation to disease severity

  45. TREATMENT OF CHRONIC URTICARIA • Daily non-sedating antihistamine • Combination of H1 and H2 receptors antagonists • Corticosteroids

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