Urticaria Angioedema Alwi

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    URTICARIAURTICARIA

    &&

     ANGIOEDEMA ANGIOEDEMA

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    IntroductionIntroduction

    As clinical manifestation ofAs clinical manifestation of

    various immunologic &various immunologic &

    inflammatory mechanism occurinflammatory mechanism occur

    after :after :

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    • IgE-or IgE receptor dependentIgE-or IgE receptor dependent

    reaction.reaction.• Assc with abnormalities of theAssc with abnormalities of the

    C sys other plasma effectorC sys other plasma effectorsys.sys.

    • Activation of cellularActivation of cellular

    arachidonic acid metabolismearachidonic acid metabolisme

    pathway after mast cell deg.pathway after mast cell deg.

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    Epidemiology

    Epidemiology

    • 1 to ! have noted" most after1 to ! have noted" most after

    adolescence" with highestadolescence" with highest

    incidence in young.incidence in young.

    • Adult patients with clinicalAdult patients with clinical

    problem #$! both urticaria &problem #$! both urticaria &

    angioedema.angioedema.

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    • %ost common at college%ost common at college

    students 1# $!.students 1# $!.

    #$! of patients with urticaria#$! of patients with urticaria

    alone within 1 year" $!alone within 1 year" $!

    continue for $ years.continue for $ years.

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    • In children" urticaria withoutIn children" urticaria without

    angioedema occur in '$!"angioedema occur in '$!"

    both occur in 1#!.both occur in 1#!.

    • #$! for more 1 year" with#$! for more 1 year" with

    mean duration of 1( months.mean duration of 1( months.

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    ClassificationClassification

    • Immunologic IgE-and IgEImmunologic IgE-and IgE

    receptor dependent urticaria )receptor dependent urticaria )

    angioedema.angioedema.

    • *rticaria ) angioedema*rticaria ) angioedema

    mediated by complementmediated by complementsystem & other plasma effectorsystem & other plasma effector

    system.system.

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    • *rticaria ) angioedema after*rticaria ) angioedema after

    direct mast cell degranulator.direct mast cell degranulator.

    • *rticaria ) angioedema relating*rticaria ) angioedema relating

    to abnormalities ofto abnormalities of

    arachidonic acid metabolism.arachidonic acid metabolism.

    • Idiopathic urticaria )Idiopathic urticaria )

    angioedema.angioedema.

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     The pathogenesis of urticaria The pathogenesis of urticaria

    • +asopermeability activities :+asopermeability activities :

    ,istamine.,istamine.

    rotaglandin .rotaglandin .

    /eu0otrine C./eu0otrine C.

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    • Chemotactic factors :Chemotactic factors :

    Eosinophil chemotacticEosinophil chemotactic

    activities.activities.

    2etrophil chemotactic2etrophil chemotactic

    activity.activity.

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    • En3ymes :En3ymes :

    4ryptase.4ryptase.

    Chymase.Chymase.

    Carbo5ypeptidase A.Carbo5ypeptidase A.

    Catepsin 6.Catepsin 6.

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    • Cyto0ines :Cyto0ines :

    4umor necrosis factor4umor necrosis factor .. Interleu0in "#"( & '.Interleu0in "#"( & '.

    roteolicans :roteolicans :,eparin.,eparin.

    Chodroitin.Chodroitin.

    7ulfate E.7ulfate E.

    ,yaluron.,yaluron.

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    • 70in chamber model70in chamber model mastmast

    cell products appearing.cell products appearing.

    Identification of mastIdentification of mast

    cell products in tissuescellproducts in tissuesor biologic fluidsor biologic fluids

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    • 7canning laser roppler7canning laser roppler

    imagingimaging biochemicalbiochemical

    mediator in IgE-meadiatedmediator in IgE-meadiated

    cutaneus reactions.cutaneus reactions.

    • Intracutaneus in8ection ofIntracutaneus in8ection of

    specific antigenspecific antigen role IgE & itsrole IgE & its

    interaction with the mast cell.interaction with the mast cell.

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    ,/A-9" 9 #; & ,/A-,/A-9" 9 #; & ,/A-

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    Clinical manifestationsClinical manifestations

    • /esions are circumscribed/esions are circumscribed

    erythematous" pruritic" edemaerythematous" pruritic" edema

    that involve the superficialthat involve the superficial

    portion of the dermis areportion of the dermis are

    0nown as urticaria" when into0nown as urticaria" when intothe deep dermis it is 0nown asthe deep dermis it is 0nown as

    angioedema.angioedema.

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    • oth urticaria & angioedemaoth urticaria & angioedema

    may occur in any locationsmay occur in any locations

    together.together.

    • Angioedema commonly effectAngioedema commonly effect

    the face ) an e5tremity isthe face ) an e5tremity is

    painful or pruritic.painful or pruritic.

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    • /esion of urticaria arise/esion of urticaria arise

    suddenly" rare persist longersuddenly" rare persist longerthan ' h and maythan ' h and may

    continue.continue.• Episodes less than ( ' wee0sEpisodes less than ( ' wee0s

    duration are acute" whereasduration are acute" whereas

    those persisting longer arethose persisting longer are

    termed chronic.termed chronic.

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    Immunologic IgE-and IgEImmunologic IgE-and IgE

    receptor-dependent urticariareceptor-dependent urticaria/ angioedema/ angioedema

    • Atopic diathesisAtopic diathesis  occuring inoccuring in

    individual with personal ) familyindividual with personal ) family

    history of asthma" rhinitis orhistory of asthma" rhinitis or

    ec3emaec3ema IgE dependent.IgE dependent.

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    • 7pecific antigen sensitivity7pecific antigen sensitivity 

    foods >shellfish" nuts &foods >shellfish" nuts &

    chocolate?" drugs &chocolate?" drugs &

    therapeutic agents >penicilin?"therapeutic agents >penicilin?"

    helminthic infestation.helminthic infestation.

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    • hysical urticaria ) angioedemahysical urticaria ) angioedema 

    mechanical traumamechanical trauma

    >elevation in blood histamin>elevation in blood histamin

    level?" prossure >IgE-mediatedlevel?" prossure >IgE-mediated

    has not demonstrated" howeverhas not demonstrated" however

    histamine" leu0otriene " I/(histamine" leu0otriene " I/(have been detected?.have been detected?.

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    4emperature4emperature  IgE"IgE"

    histamine" chemotactichistamine" chemotactic

    factors" 6" leucotrinefactors" 6" leucotrine

    have been documented.have been documented.

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    • /ight/ight  systemic lupussystemic lupus

    erythematous" '# ;$ nm &erythematous" '# ;$ nm &

    $$ #$$ nm" histamine &$$ #$$ nm" histamine &

    chemotactic factors detectedchemotactic factors detected

    after e5posure *+A & *+" #after e5posure *+A & *+" #

      1$$ 0a.  1$$ 0a.

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    E5erciseE5ercise  elevation of bloodelevation of blood

    histamine" chemotactichistamine" chemotactic

    factors" acetylcholinefactors" acetylcholine

    receptors increased.receptors increased.

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    • 7tress7tress  IC in8ection ofIC in8ection of

    nonadrenalinnonadrenalin adrenergicadrenergic

    urticaria.urticaria.

    • @ater @ater   blood histamine levelblood histamine level

    were elevated & mast cellwere elevated & mast celldegranulation was presented.degranulation was presented.

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    Urticaria / angioedemaUrticaria / angioedemamediated by the complementmediated by the complement

    system & other plasmasystem & other plasmaeffector systemeffectorsystem

    • ,ereditary & ac=uired,ereditary & ac=uired

    angioedemaangioedema  there is athere is a

    fungtional deficiency of thefungtional deficiency of theinhibitor of the activated ofinhibitor of the activated of

    C1I2, & C;b.C1I2, & C;b.

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    • 2ecroti3ing venulitis2ecroti3ing venulitis 

    abnormalities of C1= & Ig%abnormalities of C1= & Ig%component.component.

    • 9eactions to the administration9eactions to the administration

    of blood productof blood product  may arisemay arise

    after imuni3ation by transfusionafter imuni3ation by transfusion

    or by plasental tranferor by plasental tranferactivated Ig6 & ,ageman factoractivated Ig6 & ,ageman factor

    fragments.fragments.

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    • InfectionsInfections  hepatitis virushepatitis virus

    infectioninfection

    immune comple5-immune comple5-

    mediated necroti3ing vasculitismediated necroti3ing vasculitis

    with cryoglobulinemia.with cryoglobulinemia.

    • Angiotensin converting en3ymeAngiotensin converting en3yme

    inhibitorsinhibitors  the mechanism notthe mechanism not

    to be immunologic because theto be immunologic because thelesions occur within hours oflesions occur within hours of

    the first dose.the first dose.

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    Laboratory findindsLaboratory findinds

    • In all patient :In all patient :

    ,istory & physical,istory & physical

    e5amination.e5amination.

    rovocative test for physicalrovocative test for physical

    urticarias.urticarias.

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    • In selected patients :In selected patients :

    Complete blood count withComplete blood count with

    differential analysis.differential analysis.

    Erytrocyte sedimentationErytrocyte sedimentation

    rate.rate.

    *rinalysis.*rinalysis.

    lood chemistry pofile.lood chemistry pofile.

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    7tool e5amination for ova &7tool e5amination for ova &

    parasites.parasites.

    Antinuclear factor.Antinuclear factor.

    ,epatitis & C virus surface,epatitis & C virus surface

    Ag & Ab.Ag & Ab.

    70in test for IgE-mediated70in test for IgE-mediated

    reactions.reactions.

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    9adioallergosorbent test9adioallergosorbent test

    >9A74? for specific IgE.>9A74? for specific IgE.

    C,#$.C,#$.

    Cryoproteins.Cryoproteins.

    lasma & erythrocytelasma & erythrocyte

    protoporphyris.protoporphyris.

    70in biopsy.70in biopsy.

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    HistopathologyHistopathology

    • olymorphous perivascularolymorphous perivascular

    infiltrate.infiltrate.

    •2eutrophils.2eutrophils.

    • Eosinophils.Eosinophils.

    • %ononuclear cells.%ononuclear cells.

    • 7parse perivascular7parse perivascular

    lymphocytes.lymphocytes.

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    PathologyPathology

    • Chronic idiopathic urticariaChronic idiopathic urticaria 

    C" C'" 4 lymphocytes"C" C'" 4 lymphocytes"

    neu" eusi detected by directneu" eusi detected by direct

    immunofluorescence" %" -immunofluorescence" %" -

    selectinselectin biopsy.biopsy.

    • Acut urticariaAcut urticaria  %" EC.%" EC.

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    • hysical urticariahysical urticaria  C BC B

    C'" neu" eosi with %" E-C'" neu" eosi with %" E-

    selectin" +CA%selectin" +CA% by histologicby histologic

    result.result.

    7olar urticaria7olar urticaria

      %" EC.%" EC.

    • apular urticariaapular urticaria  4 lymp"4 lymp"

    macrophages" eosi" neu.macrophages" eosi" neu.

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    • Chollergic urticariaChollergic urticaria  %"%"

    EC.EC.

    • Chronic urticariaChronic urticaria  E-E-

    selectin" -selectin"selectin" -selectin"

    intercellular adhesion mol 1"intercellular adhesion mol 1"

    +CA%.+CA%.

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    Diagnosis &Diagnosis &Differential diagnosisDifferential diagnosis

    • *rticaria & angioedema*rticaria & angioedema 

    episode & evanescent" seldomepisode & evanescent" seldom

    persist for B ' h for severalpersist for B ' h for several

    days.days.

    • 7everal disorder are included in7everal disorder are included in

    ..

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    • Erythema multiformeErythema multiforme 

    edematous" papulovesicular"edematous" papulovesicular"bullous eruptions with mucosalbullous eruptions with mucosal

    involvement" typical iris orinvolvement" typical iris or

    target.target.

    • /yme borreliosis/yme borreliosis  annulerannuler

    edematous" urticarial pla=uesedematous" urticarial pla=ues

    may e5pand in diameter.may e5pand in diameter.

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    • ullous pemphigoidullous pemphigoid 

    edematous & erythematousedematous & erythematous

    pla=ues.pla=ues.

    • *rticaria pigmentosa*rticaria pigmentosa 

    generali3edgenerali3ed red-brown"red-brown"

    macular" papular.macular" papular.

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     Treatment Treatment

    4he ideal treatment for urticaria )4he ideal treatment for urticaria )

    angioedema is identification &angioedema is identification &

    removal of its cause.removal of its cause.

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    • ,1-type antihistamine drugs,1-type antihistamine drugs 

    mainstays.mainstays.

    • 2ewer 2ewer   low-sedating" reducelow-sedating" reduce

    sedative" anticholinergic.sedative" anticholinergic.

    • If this drug fails to be effectiveIf this drug fails to be effective 

    combination of agent fromcombination of agent from

    different classes may be used.different classes may be used.

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    • Combination of ,1 & , may beCombination of ,1 & , may be

    beneficial & 0etotifen has beenbeneficial & 0etotifen has been

    used succesfullyused succesfully  chronicchronic

    urticariaurticaria reported >colchine inreported >colchine in

    combination with ,1to becombination with ,1to be

    benefit? & also cycsporine.benefit? & also cycsporine.

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    7ystemic glucocorticoid have no7ystemic glucocorticoid have no

    place in regular therapy.place in regular therapy.

    rednisone in management ofrednisone in management of

    acute urticaria has beenacute urticaria has been

    advocated in emergency rooms.advocated in emergency rooms.

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    • ral disodium cromoglycate isral disodium cromoglycate is

    effectiveeffective  food allergy.food allergy.• 4erbutaline" a4erbutaline" a -agonist in-agonist in

    combination with an ,1-typecombination with an ,1-type

    has been reported to be ofhas been reported to be of

    benefitbenefit  idiopathic urticariaidiopathic urticaria

    failed from conventionalfailed from conventional

    therapytherapy nefidipine.nefidipine.

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    • Immunoglobulin >I+? wasImmunoglobulin >I+? was

    limited benefit & also trials waslimited benefit & also trials wasof was of benefit with >7C?of was of benefit with >7C?

    interferon-interferon- 

    ..

    • Epineprine is widely usedEpineprine is widely used

    particularly in hospitalparticularly in hospital 

    laryngeal edema" cardivascularlaryngeal edema" cardivascular

    collapse.collapse.

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    • Antihistamine prophylacticAntihistamine prophylactic 

    physical urticaria.physical urticaria.

    • 1D-1D- 

    -al0ylated androgen-al0ylated androgen

    dana3oldana3ol  reduction inreduction in

    e5ercise provo0ed e5perimentale5ercise provo0ed e5perimental

    whealswheals cholinergic urticaria.cholinergic urticaria.

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    • 27AIs" cetri3ine"27AIs" cetri3ine"

    sulfasala3aline & syst.sulfasala3aline & syst.

    6lucocorticoid6lucocorticoid  delayeddelayed

    pressure urticaria.pressure urticaria.

    • 4erbutaline & aminophylline4erbutaline & aminophylline

    combination" ,1 & ,1 comb.combination" ,1 & ,1 comb. 

    cold urticaria.cold urticaria.

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    • ropanolol hydrochlorideropanolol hydrochloride 

    adrenergic urticaria.adrenergic urticaria.

    *+A phototherapy & *+A*+A phototherapy & *+A

    photochemotherapyphotochemotherapy 

    physical urticaria.physical urticaria.

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    *rticaria ) angioedema may be a*rticaria ) angioedema may be a

    source of frustration to bothsource of frustration to both

    physician & patients" mostphysician & patients" most

    patients can achieve acceptablepatients can achieve acceptable

    symptomatic control of theirsymptomatic control of their

    disease without identification ofdisease without identification ofthe cause.the cause.

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