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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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slide1

CHRONIC AND ACUTE URTICARIA IN CHILDREN

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

slide3

Wheal typical features

# A central swelling, surrounded by erythema.

# Itching or burning sensations

# The wheal disappear usually within 1-24 h.

slide4

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.

slide5

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

clinical classification of urticaria and angioedema
Clinical classification of urticaria and angioedema
  • Ordinary urticaria- acute , chronic, episodic.
  • Physical urticaria
  • Angioedema
  • Contact urticaria
  • Urticarial vasculitis
clinical classification of urticaria and angioedema1
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)

slide9

מה היא הסיבה השכיחה ביותר שניתן למצוא כגורמת לאורטיקריה חריפה ?

  • אלרגיה לתרופות
  • מחלות זיהומיות
  • עקיצות חרקים
  • חומרי ניגוד
  • אלרגיה למזון
slide10

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.

acute urticaria
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
acute urticaria1
Acute urticaria
  • Exposure to food allergens- milk, eggs, peanuts,

sesame ,soy wheat, shellfish, fish.

  • Medications-β-lactam antibiotics, sulfonamides, aspirin.
  • Radiocontrast media
slide13

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 ?

treatment of acute urticaria
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.

slide15

ALGORITHM FOR ACUTE URTICARIA/ANGIOEDEMA

DETAILED HISTORY: infection

drug

food

physical triggers

PHYSICAL EXAMINATION

SPECIFIC EXAMINATION- CBC , URINE, ESR

SKIN TESTS

slide19

SYNDROMYES THAT CAN BE ASSOCIATED WITH URTICARIA

Muckle-Wells syndrome

Familial -dominantly inherited.

urticaria progressive nerve deafness

Limb pain arthritis

Recurrent fever

hypergammaglobulinemia

amyloidosis

slide20

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.

slide22

מה היא הסיבה השכיחה ביותר לאורטיקריה כרונית ?

  • אלרגיה לתרופות
  • גורמים זיהומיים
  • אידיופטי
  • מנגנון אוטואימוני
  • אלרגיה למזון
  • דלקת מפרקים של גיל הילדות
slide23

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

slide24

Autoimmune urticaria

In 1986 Grattan first described that:

Autologous serum test produces a

Wheal and Flare reaction

In 30-50% of CIU cases

slide25

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

slide26

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)

slide27

Autoimmune basis for CIU

Thyroid autoimmunity in pts with CIU

anti-thyroid Ab. in 14-20%

increased / decreased thyroid

function in 5-10%

slide28

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

physical urticaria
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.

slide30

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.

slide31

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.

slide32

Physical urticaria- dermographic urticaria

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

slide33

Physical urticaria- dermographic urticaria

# The most frequent form of physical urticaria.

# Affecting mainly young adults

# Mean duration 6.5 years

slide34

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 .

physical urticaria cold
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.

slide36

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.

slide37

Other forms of physical urticaria

Solar

Vibratory

Adrenergic

aquagenic

slide38

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

slide39

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.

slide42

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.
cu duration
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.

slide45

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

treatment of chronic urticaria
TREATMENT OF CHRONIC URTICARIA
  • Daily non-sedating antihistamine
  • Combination of H1 and H2 receptors antagonists
  • Corticosteroids
treatment of severe unremitting urticaria
TREATMENT OF SEVERE UNREMITTING URTICARIA
  • Plasmapheresis
  • Intravenous immunoglobulin
  • Cyclosporin A
cyclosporine a
Cyclosporine A
  • cyclosporine on helper T-cells to inhibit T-cell receptor- activated induction of IL-2 gene.
  • cyclosporine may also inhibit IgE-stimulated mast cell degranulation and stimulate TGF- expression.
low dose cyclosporin a in the treatment of severe chronic idiopathic urticaria
Low dose cyclosporin A in the treatment of severe chronic idiopathic urticaria.
  • 2-3mg\kg of CsA, was shown to be beneficial
  • AST Positivity could not predicted response to treatment.
  • In some cases Prolonged treatment is needed

Toubi et al Allergy 1997;52:312-316.

slide55

ANGIOEDEMA

With weals

Without weals

Idiopathic

DRUGS- NSAIDs ,ACEIs

C1 eterase deficiency

All kinds of urticaria except dermographism

slide58

אנגיואדמה תורשתית סוג 1

C4 –decreased , C1 esterase-decreased

אנגיואדמה תורשתית סוג 2

C4 –decreased , C1 esterase-normal

אנגיואדמה אידיופטית

C4 –normal , C1 esterase-normal

slide59

CONTACT URTICARIA

Contact urticaria is an important manifestation of natural rubber latex allergy.

slide60

תאור מקרה

ל.ע בת 16 שנים

הופנתה עקב אורטיקריה מזה כחצי שנה.

סקירה מערכתית : עליה במשקל של כ5 ק"ג בחצי שנה ,עייפות.

בדיקה גופנית אורטיקריה קלה בעיקר בבטן וגב – בולטת יותר באזורי לחץ .

slide61

בדיקות מעבדה : ס"ד , תפקודי כבד ,כליה, שתן כללית ,צואה לפרזיטים-ללא ממצא.

טסט עורי למוצרי מזון- שלילי

TSH- 50 ↑, נוגדנים לרקמת בלוטת התריס.

- AUTOLOGUS SERUM TEST חיובי.

טיפול – מתן אלטרוקסין

לורסטין 10 מ"ג