1/15/2017

PediatricChickenpox:Background,Pathophysiology,Etiology

Thissiteisintendedforhealthcareprofessionals

PediatricChickenpox
Updated:Apr13,2016
Author:KirstenABechtel,MDChiefEditor:RussellWSteele,MDmore...

OVERVIEW

Background
Varicella,commonlyknownintheUnitedStatesaschickenpox,iscausedbythevaricellazoster
virus.Thediseaseisgenerallyregardedasamild,selflimitingviralillnesswithoccasional
complications.Varicellaiscommonandhighlycontagiousandaffectsnearlyallsusceptible
childrenbeforeadolescence.Althoughmostvaricellainfectionconferslifelongimmunity,varicella
clinicalreinfectionsamonghealthychildrenhavebeendescribed.[1](SeePathophysiologyand
Etiology.)
BeforevaccinationforvaricellabecamewidespreadintheUnitedStates,thisdiseaseaffected
approximately4millionchildrenperyear,causedasmanyas100deathsinchildrenannually,and
wasresponsibleforanestimated$400millioninmedicalcostsandlostwageseachyear.Since
thevaricellavaccinewasintroducedforchildrenaged1218monthsintheUnitedStatesin1995
andboostervaccinegivenatage45years,diseaseincidencehassubstantiallydecreased.(See
Epidemiology.)
Chickenpoxisusuallyabenigndiseaseinchildren,andalmostallchildrenrecoveruneventfully.
(SeePrognosis.)However,varicellaisnottotallybenigneventoday.Asignificantnumberof
varicellacasesareassociatedwithcomplications,amongthemostseriousofwhicharevaricella
pneumoniaandencephalitis.
Presentingsymptomsmayincludeahistoryoflowgradefever,abdominalpain,ahistoryofrash,
intensepruritus,headache,malaise,anorexia,coughandcoryza,andsorethroat.Rashandfever
arethetypicalfindingsduringphysicalexaminationinpediatricpatientswithvaricella.Anill
appearanceshouldraiseconcernforpulmonaryorneurologiccomplicationsorseriousbacterial
superinfection.(SeeClinicalPresentation.)
Adolescents,adults,andpregnantpatientsareatincreasedriskforseverediseaseandareoften
treatedwithantivirals.Othergroupsthatrequirespecifictreatmentarechildrenwhoare
immunocompromised,thosewhoareotherwiseatriskforseveredisease,andthosewhoalready
haveseveredisease.Othertreatmentsincludesupportivemeasures,administrationofvaricella
zosterimmuneglobulin(VZIG),andmanagementofsecondarybacterialinfection.(SeeTreatment
andManagement.)
GotoChickenpoxforcompletenonpediatricinformationonthistopic.

Pathophysiology
Thecausativeorganism,varicellazostervirus,isamemberofthehumanherpesvirussubfamily
Alphaherpesvirinaeand,likeallherpesviruses,isaDNAvirus.Thevirusentersthroughthe
respiratorysystem(conjunctivalorupperrespiratorymucosa)andcolonizestheupperrespiratory
tract.Viralreplicationtakesplaceinregionallymphnodesoverthenext24days46dayslater,a
primaryviremiaspreadsthevirustoreticuloendothelialcellsinthespleen,liver,andelsewhere.
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PediatricChickenpox:Background,Pathophysiology,Etiology

Afteraweek,asecondaryviremiadisseminatesthevirustothevisceraandskin,elicitingthe
typicalskinlesions(seetheimagesbelow).[2]Thisviremiaalsospreadsthevirustorespiratory
sitesandisresponsibleforthecontagionofvaricellabeforetheappearanceoftherash.Infection
ofthecentralnervoussystem(CNS)orliveralsooccursatthistime,asmayencephalitis,hepatitis,
orpneumonia.

Thepleomorphicrashcharacteristicofvaricella.Papules,vesicles,andpustulesareconcurrentlypresent.

ViewMediaGallery

Papulovesicularlesionsonarminvaryingstagesofhealinginthisinfantwithvaricella.Photographcourtesyof
SusanFeigelman,MD.

ViewMediaGallery
Theusualincubationperiodis1021days.Thepatientiscontagiousfrom12daysbeforethe
appearanceofrashuntilthelesionscrustover,usually56daysaftertherashfirstappears.
Varicellaisassociatedwithhumoralandcellmediatedimmuneresponses.Theseresponses
inducelonglastingimmunity.Repeatsubclinicalinfectioncanoccurinthesepersons,butsecond
attacksofchickenpoxareextremelyrareinimmunocompetentpersons.Reexposureand
subclinicalinfectionsmayservetoboosttheimmunityacquiredafteranepisodeofchickenpox.
Thismaychangeinthepostvaccineera.

Etiology
Transmission
Varicellaiscausedbythevaricellazostervirus.Theinfectiousparticlesarecellfreevirusparticles
derivedfromskinlesionsortherespiratorytract.Transmissionoccursmainlythroughrespiratory
dropletsthatcontainthevirus,makingthediseasehighlycontagiousevenbeforetherash
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appears.Directpersontopersoncontactwithlesionsalsospreadsthevirus.Papulesand
vesicles,butnotthecrusts,havehighpopulationsofthevirus.Inaddition,maternalvaricellawith
viremiacantransplacentallyspreadtothefetus.Thisleadstoneonatalvaricella.

Riskfactorsforseverevaricella
Riskfactorsforseverevaricellainneonatesareasfollows:
Thefirstmonthoflife:Aneonatesfirstmonthoflifeisasusceptibleperiodforsevere
varicella,especiallyifthemotherisseronegative.
Earlydelivery:Deliverybefore28weeksgestationalsorendersababysusceptiblebecause
transplacentaltransferofimmunoglobulinG(IgG)antibodiesoccursafterthistime.
Riskfactorsforseverevaricellainadolescentsandadultsareasfollows:
Steroidtherapy:Highdoses(ie,dosesequivalentto12mg/kg/dofprednisolone)for2weeks
ormorearedefiniteriskfactorsforseveredisease.Evenshorttermtherapyatthesedoses
immediatelyprecedingorduringtheincubationperiodofvaricellacancausesevereorfatal
varicella.[3]
Malignancy:Allchildrenwithcancerhaveanincreasedriskforseverevaricella.Theriskis
highestforchildrenwithleukemia.Almost30%ofpatientswhoareimmunocompromisedand
whohaveleukemiahavevisceraldisseminationofvaricella7%maydie.
Immunocompromisedstate(eg,malignancy,antimalignancydrugs,humanimmunodeficiency
virus[HIV],othercongenitaloracquiredimmunodeficientconditions):Defectsofcellularbut
nothumoralimmunodeficiencyarebelievedtorenderapersonsusceptibletosevere
varicella.
Pregnancy:Pregnantwomenhavehighriskofseverevaricella,especiallypneumonia.

Epidemiology
UnitedStatesstatistics
Beforevaricellavaccineusebecamewidespread,4millioncasesofchickenpoxwerereported
annually.Nationalseroprevalencedatafor19881994indicatedthat95.5%ofadultsaged2029
years,98.9%ofadultsaged3039years,andmorethan99.6%ofadultsolderthan40yearswere
immunetovaricella.[4]Thediseasewasresponsiblefor11,000hospitalizationseachyearand
approximately50100deaths.
Theadoptionofuniversalvaccinationagainstvaricellain1995reducedtheincidenceofvaricella,
aswellastheassociatedmorbidityandmortalityrates.[5,6,7,8]By2000,vaccinationcoverage
amongchildren1935monthsin3communitiesinTexas,California,andPennsylvaniahad
reached7484%,andreportedtotalvaricellacaseshaddeclined7184%.Mostofthedecline
occurredamongchildrenaged12monthsto4yearshowever,incidencedeclinedinallage
groups,includinginfantsandadults.[9]
Currently,fewerthan10deathsoccurperyear,mostoftheminunimmunizedpeople.Although
vaccinationcoveragehasexceeded80%overthepastfewyears,outbreaksofbreakthrough
varicellastilloccurinschoolsanddaycarecenters.[10,11,12]
Childrenwithvaricellaexposeadultcontactsinhouseholds,[13]schools,anddaycarecentersto
theriskofsevere,evenfatal,disease.Householdtransmissionratesare8090%.Secondcases
withinthehouseholdareoftenmoresevere.Schoolordaycarecentercontactisassociatedwith
lowerbutstillsignificanttransmissionrates.Childrenwhoaresusceptiblerarelyacquirethe
diseasebycontactwithadultswithzoster.Maximumtransmissionoccursduringlatewinterand
spring.
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PediatricChickenpox:Background,Pathophysiology,Etiology

Internationalstatistics
Varicellaaffectsnearlyallchildrenworldwidewhodonothaveimmunity.Annualincidenceis
estimatedat8090millioncases.Mostdevelopingcountrieshavelowimmunizationratesbecause
ofthecostinvolved,andvaricelladiseaseisariskfortravelerstosuchcountries.
Asurveyof1473casesinJapandemonstratedthat81.4%involvedchildrenyoungerthan6years.
[14] InJapan,theannualprevalencepeakedbetweenMarchandMay,withsubsequentlower
prevalencebetweenAugustandOctober.
Theepidemiologyofvaricelladiffersbetweencountrieswithtemperateclimatesandthosewith
tropicalclimates.Inmostcountrieswithtemperateclimates,morethan90%ofpersonsare
infectedbyadolescencebutincountrieswithtropicalclimates,ahigherproportionofinfectionsare
acquiredatolderages,whichresultsinhighersusceptibilityamongadults.

Agedistributionforvaricella
Themaximumincidenceofvaricellainunvaccinatedpopulationsisinchildrenaged16years.
Personsolderthan14yearsaccountfor10%ofvaricellacases.IntheUnitedStates,thepeakage
isnow911years.[5]
Intropicalclimates,varicellaismorecommoninolderchildren.MostcasesinJapanwerein
childrenyoungerthan6years.Approximately9.6%ofcasesinvolvedchildrenyoungerthan1year,
andalmostonethirdofthesewereinfantsyoungerthan5months.
Varicelladoesnothavearacialpredilectionorasexpredilection.

Prognosis
Otherwisehealthychildrenwithvaricellahaveexcellentprognoses.Inotherwisehealthychildren
aged114years,themortalityrateisestimatedat2deathsper100,000cases.Thecasefatality
rateinthegeneralpopulationis6.7casesper100,000population.Childrenwith
immunocompromisedstates,however,areatriskforseverediseaseanddeath.Themortalityrate
inchildrenwhoareimmunocompromisedismuchhigherthanthatinotherwisehealthychildren.
Amongchildrenwithleukemia,themortalityrateofvaricellais7%.
Onestudysuggestedthatnearly1:50varicellacasesareassociatedwithcomplications.Among
themostseriouscomplicationsarevaricellapneumoniaandencephalitisbothareassociatedwith
ahighmortalityrate.Beforeuniversalvaccination,mostvaricellarelateddeathsintheUnited
Stateswerefromassociatedencephalitis,pneumonia,secondarybacterialinfection,andReye
syndrome.(SeeComplications.)Inaddition,significantconcernshavebeenraisedaboutthe
associationofvaricellawithsevereinvasivegroupAstreptococcaldisease.[15]
Thediseasecanbeseriousinneonates,dependingonthetimingofinfectioninthemother.
Varicelladuringpregnancycancausevariousadverseoutcomesformotherandinfant,depending
onthestageofpregnancy.Neonatalvaricellamortalityratescanreach30%.

PatientEducation
Familiesofchildrenwithchickenpoxshouldbeinstructedtobathethechildregularlytoreduce
itchingandpreventsecondaryinfection.Topreventscratching,whichcanleadtosecondary
infectionandscarring,thechildsfingernailsshouldbekeptshort,mittensorsocksmaybewornon
thehandsatnight,andmedicationforitchingcanbegivenasneeded.Aspirincontaining
medicationsshouldnotbeused.

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Childrenwithchickenpoxshouldavoidnonimmunepregnantwomen,unimmunizedyounginfants,
andotherswithimmunodeficienciesorwhoaretakingprednisonelongterm.Childrenwith
chickenpoxmaynotreturntoschoolordaycareuntilalllesionsarecrustedover.
Parentsshouldtakechildrentothehospitalifthefollowingsymptomsoccur:
Unusualredness,swelling,orpainoveranareaoftherash
Refusaltodrinkfluids
Signsofdehydration,suchasscantyandyellowcoloredurine,increasingdrowsiness,dry
mouthandlips,excessivethirst,orlethargy
Confusion,irritability,drowsiness,ordifficultywaking
Inabilitytowalkorunusualweakness
Complaintsofsevereheadache,stiffneck,and/orbackpain
Frequentvomiting
Difficultybreathing,chestpain,wheezing,fastbreathing,orseverecough
Feverpersistingmorethan4daysorfeverreturnsafterdefervescence
Amoresicklyappearancethanwhenlastseenbythedoctor
Forpatienteducationresources,seetheBacterialandViralInfectionsCenter,aswellas
ChickenpoxandSkinRashesinChildren.
ClinicalPresentation
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