JURNALREADING

LEPROSYAMONGCHILDRENUNDER15YEARSOFAGE

MarcelaBahiaBarrettodeOliveira1,LuciaMartinsDiniz1
*WorkconductedattheFederalUniversityofEspritoSanto(UFES),Vitria,ES,Brazil.
UniversidadeFederaldoEspritoSanto(UFES)Vitria(ES),Brazil.2016byAnaisBrasileiros
deDermatologia
An Bras Dermatol. 2016
 Presentedby:
N.DearasiDebyNadhifanny
1518012183

Perceptor:
dr.ResatiNandoPanonsih,M.Sc,Sp.KK

KEPANITERAAN KLINIK ILMU PENYAKIT KULIT DAN KELAMIN

FAKULTAS KEDOKTERAN UNIVERSITAS LAMPUNG
RSUD DR. A. DADI TJOKRODIPO
BANDAR LAMPUNG
Introduction
Leprosyisaninfectiousdiseasecausedby
Mycobacteriumleprae,whichtoday
representsaseverepublichealthissue.In
2011,threecountriesmadeup83%ofthe
newcasesdetectedworldwide,withIndia
responsiblefor58%ofthecases,Brazil
for16%,andIndonesiafor9%.
Leprosyismostcommoninadults;however,the
outbreakofcasesinchildrenandadolescents
showstheactivecirculationofbacillus,withits
continuedtransmissionandthefailureofthe
healthsystemtocontrolthisdisease.In
countriesinwhichleprosycanbeconsidered
endemic,suchasBrazil,despitethedropin
prevalenceandincidencerates,thehigh
detectionrateofcasesinchildrenofunder15
yearsofagehelpstomonitortheendemic.
Epidemiology

Attheendof2011,Brazilpresented33,735newcasesof
leprosy,withageneraldetectioncoefficientof17.39cases
perevery100,000inhabitants,whichiscon-sideredtobe
anextremelyhighendemicity.Asregardschildrenof
under15yearsofage,2,287newcasesofleprosyhave
beenreported,representing6.7%ofthetotalnumberof
casesreportedthroughoutthecountry,withadetection
coefficientof4.89casesper100,000inhabitants,
reflectinganaverageendemicityindex

Nevertheless,Santosetal.identifieda
Asregardsgender,accordingtothe
discretepredominanceinthe5to9
BrazilianHealthMinistry,children
yearoldagegroup,in54.54%(42/77),
under15yearsofageareequally
withthenextmostaffectedagegroup
distributed,with1.163casesinboys
being10to14yearolds,44.15%
and1.123casesingirlsin2012
(34/77)
Transmission

Themaintransmissionandacquisitionpathways
forleprosyaretheupperairways,giventhat
multibacillarypatientsarethelargestsourceofthe
eliminationofbacillus.Itisacceptedthat
householdcontactswithpatientswithleprosyare
themostpronetocatchingthedisease.
 Onepeculiarityofleprosyisthatthemajorityofthepeople(about90%)donot
becomeillduetoonesownnaturaldefenseagainstMycobaterium leprae, whichis
relatedtogeneticinfluences.Thegenes involvedarestillnotfullyknown;however,it
iswellacceptedthatbothgenesfromthehumanleukocyteantigen(HLA)andthenon-
HLAareinvolvedinonespredispositiontothisdisease.
Thedevelopmentofthediseasehasalreadybeenassociatedwiththegenesthatcontrol
themacrophageresponsetobacillus,suchasvariationsinthePRAK2andPARCRG
genes.Morerecently,Zhangetal,inastudyusingagenomescanofaChinese
population,identifiedvariationsinsevengenes(CCDC122,C13orf31,NOD2,
TNFSF15,HLA-DR,RIPK2,andLRRK2)associatedwithpredispositiontoleprosy
Classification

WHO :

PB (Pauci Bacilarry) MB (Multi Bacilarry)

Ridley Jopling :

TT
BT (Boderline
(Tuberculoid
Leprosy)
Leprosy)

BB (Boderline BL (Boderline
Leprosy) Lepromatous)

LL
(Lepromatous
Leprosy)
Clinical Manifestations Of Leprosy

Indeterminateleprosyischaracterizedbyspots,generallyhypochromic,
sometimeserythematous,ofunclearouterlimits,diversesizes,with
changeinthethermaland/orpainsensitivity,reductionorabsenceofhair
(alopecia),andreductionorabsenceofexcessivesweating(hypoor
anhydrous)

Itisnormallyrep-resentedasinglelesionbutcanreachuptofivelesions
andparticularlyaffecttheexposedareasofthebody.Nocompromiseof
theperipheralnerveoccurs.Leprosycanlastbetweentwoandfiveyears,
evolvingintospontaneoushealingorintoadiseaseinoneofitspolar
forms(TTorLL)
 TTleprosyisaformofgreaterresistancetoMycobacterium leprae,witha
predominanceoftheTh1 cellularimmuneresponse,thusallowingtheconfinementof
thebacillusandthemanifestationoffewcutaneouslesions,generallysinglelesions.
TTleprosyischaracterizedbyalesioninafullyinfiltratedplaqueoroferythematous
papularsharpborders,whichareexternallydefined,withchangeinthethermal,pain,
andtactilesensitivity(inthisorder),hypooranhydrousareas,andalopecia

TheLLforminitiallyappearsashypochromic,diffuse,symmetricspotsthat
graduallyundergotheinfiltrationofbacillusrichmacrophages,clinicallyleadingto
themanifestationoferythematousorerythematousbrownishlesions,infiltrated,
shiny,coalescent,poorlydefinedlesionsofsymmetricaldistribution
Brownishpapulescanbeseenisolatedandfullofbacillus,called
leproma,inanyregionofthetegument;infiltrationoftheface,
lossoftheexternalthirdoftheeyebrow(madarosis);andthe
infiltrationoftheearlobes(Figure1).
Borderlineleprosyisaninterpolarform,asit
presentsclinicalbacilloscopicandhistopathological
characteristics,whethertendingtowardtheTTpole
(borderlineTT),whethertowardtotheLL
(borderline borderline or borderline LL),believing
thatthisdichotomyistheproductofimmunological
instability
 Asregardsthenumberoflesions,moststudiesonchildrenand
adolescentsshowapredominanceofasinglelesionandinexposed
areas.However,Ganapatietalfound8.8%(84/346)ofthelesionsinthe
glutealregion,highlightingtheimportanceofthefullbodyskinexam.

Itisimportanttoremembertheexistenceofmusculoskeletal
manifestations,suchasarthralgia,arthritis,andmyalgia,alsoplacing
leprosywithinthedifferentialdiagnosesofjuvenileidiopathicarthritis,
systemiclupuserythematosus,amongotherautoim-munediseases.
Nederetalreportedamainfindingof14%oftheexaminedchildren
withmusculoskeletalmanifestationswithasymmetricpolyarthritisofthe
smallarticulationsofthehands
Leprosy Reactions
Leprosyreactionsareacute/subacuteinflammatoryprocesses,
mediatedbyimmunecellsorbyantibodies,presentinthe
evolutionofleprosy.Thesecanbeunleashedbyinfections(dental,
urinary,etc.),parasiteinfestations,vaccinations,physicaland
emotionalstress,amongothers.Thesearesubdividedintotype1
reaction,whichismediatedbythecellularimmuneresponse,and
type2reaction,determinedbyimmunecomplexesandimmune
cellcytokines
Thetype1reactionmostcommonlyoccursintheBT,BB,and
BLforms.Thisischaracterizedbyerythemaandswellinginthe
preexistinglesionsandthemanifestationofnewlesions
(papulesandplaques)(Figure3).Thiscanevolvethough
outbreaks,anyoneofwhichcanlastforfourtosixmonths.The
moreseverereactionscanbefollowedbyfever,malaise,an-
orexia,swellingofthefaceandbodyextremities,andneurites.
Thesemaybetheonlyappearanceofareaction,calledan
isolatedneurite,whosemanifestationischaracterizedby
spontaneouspainorthecompressionoftheperipheralnerve
trunk,followedornotbyneuralthickeningandbythe
involvementoftheneurologicalfunction(sensory,motor,and
autonomic).
 Themostcommonclinicalpresentationoftype2reactionistheerythemanodosum,whichis
mostcommonlyfoundinBLandLL,characterizedbythesuddenmanifestationofnodules
anderythematous,deep,painful,shinypapules,whichcanremainfor15to20daysinthe
face,upperlimbs,lowerlimbs,andtrunk,normallyaccompaniedbyfever,malaise,myalgia,
arthralgia,iritis,lymphadenopathy,swellingandpaininthehandsandfeet,neurites
(sometimestheonlymanifestation),amongothers.Thismedicalconditionoccursinepisodes
oritcanbecontinuous

Leprareactionsarethemaincausesofneuraldamageanddeformities.Jainetal.foundthe
involvementofperipheralnervesin186patientsofatotalof306evaluatedchildren,with
morethanonethickenednerveinmostcases,theulnarnervebeingthemostaffected.In2009,
Raoreportedneuralthickeninginmultiplenervesin59.38%ofthecases,whichalsoshowed
apredominanceofulnarnerveinvolvement.FindingsfromSingaletal.showed70%ofthe
patientswiththickening,nearlyhalfofwhichhpresentedmultiplethickenednerves,
predominatelyintheulnarnerve
 AccordingtoKarandJob,childrenwithneuralthickeninghave6.1timesmore
chancestodevelopdeformitiesascomparedtothosewithoutneuralwidening.
Theseauthors,uponstudying275childrenunder15yearsofage,founda10.5%
incidenceofdeformities,withthefollowingriskfactors:latediagnosis,multiple
cutaneouslesions,multibacillarydisease,positivebascilloscopy,variousaffected
nerves,andreactionalstateatthetimeofthediagnosis.
Diagnosis
Thediagnosisofleprosyisessentiallyclinical,
butitcanbecomplementedbytheskinsmear
bacilloscopyandbiopsywithahistopathological
studyofthecutaneouslesion,duetothe
difficultyinconductingthethermalsensitivity
test,primarilyinchildrenunder10yearsofage,
andtheneedfordifferentialdiagnoseswithother
dermatosescommonlyfoundinchildhood.The
youngerthechild,themoredifficultthe
evaluationofsensitivity
Thefindingofpositivebacilloscopyisrarein
children,havingbeenreportedin10%of
childrenunder15yearsofage.However,
Imbiribaetalfoundpositivebacilloscopyin
18.3%(71/387)ofchildrenunder15yearsofage
whoweresubmittedtotheexam,whileRao
found25%(8/32)ofpositivebacilloscopy,and
Singal,19.8%(34/172),giventhatin17
children,thebacilloscipyindexwasgreateror
equaltofour.
 Currently, studies have been conducted using the
ML-FLOW test (anti PGL-I) to evaluate the sero
prevalence of household and school contacts in
hyperendemic

The importance of the study on these school

children in hyperendemic areas was the early
diagnosis of the disease, in turn avoiding disabilities
and deformities, preventing infection in the
community, and blocking the transmission chain
BCG Vaccination

Thisprocedureprovedtobemoreeffective
regardingthedevelopmentofmultibacillary
forms.Althoughtheeffectdiminishedwith
Inametaanalysis,theprotectorrateof
age,novariationwasobservedintheBCG
revaccinationwithBCGwasreportedinseven
actioninrelationtotheageuponapplication
experimentalstudiesandnineteen
ofthefirstdoseofthevaccine,butthe
observationalstudies.Theprotectionvaried
reinforcementincreaseditsresponseand
between26%and61%,respectively.
shouldbeencouragedinhouseholdcontacts
withmultibacillarypatients,astheserepresent
thehighestriskofinfection
Treatment

TheBrazilianHealthMinistryrecommendsa
multidrugtherapyregimenforthetreatmentof
childrenaccordingtoageandthesubdivisionofthose
casesinpaucibacillaryandmultibacillaryforms, TheBrazilianHealthMinistryprovidestheblisterfor
accordingtothatobservedintables1and2. paucibacillaryandmultibacillarytreatmentwith
capsulesofrifampicin(15mg)andclofazimine
Thedosesshouldbepreferentiallycalculated (50mg),andtabletsofdapsone(50mg).
accordingtotheweightofthechild:dapsone1.5mg/
kg/day,rifampicin10mg/kg/day,andclofazimine1
mg/kg/day.
Ininternationalmedicalliterature,onlyafewcasesreportonseveresideeffects
thatharmchildrenunder15yearsofage.However,Kaluarachchietalstudied
hepaticandhematologicalreactionscausedbytheuseofmultidrugtherapy
regimenforleprosy,througharetrospectivestudyconductedbetween1991and
1995inSriLanka.Ofthetotalof3,333newcasesofleprosyfound,81(2.4%of
thepatients)withanemia(mainlyhemolytic),jaundicewithtoxichepatitisor
methemoglobinemia,with14casesinchildrenunder15yearsofage,but
discardedtheageasariskfactorforadversereactionstothemultidrugtherapy
regimenandemphasizedtheimportanceofdapsoneasthedrugthatmost
determinesmedicinalchangesintheregimen.Theseauthorsreportedonlyone
caseofa12yearoldboythatpresentedseverehematemesis,asevereandrare
complicationcausedbyclofazimine.

Inadults,thealternativeregimensuse
ofloxacin(quinolone)andminocycline
(tetracycline),whicharecontraindicatedin
childrenunder10yearsofage,duetotherisk
oftheearlyclosureoftheepiphysisaswellas
dentalandbonealterations,respectively
Leprareactionsshouldbetreatedwithoral
corticotherapy,especiallyprednisoneatadose
of1mg/kg/dayuntiltheclinicalcondition
hasbeenbroughtundercontrol,withaslow
andgradualremovalofthemedicationuntil
itscompletesuspension.Sevalsekaretalupon
treating33childrenwithleprareaction,the
majoritywithtype1leprareaction,obtained
fullhealingthroughtheuseoforal
corticotherapyinallcases
Chemoprophylaxis In High-Risk Contacts

Chemoprophylaxisinhigh-riskcontacts,
thatis,contactswithmultibacillaryand
seropositivetoanti-PGL-Icases,using
rifampicin(600mg)foradultsofabove35
kgofbodyweight,450kgforchildren
abovenineyearsofage,andadultsofupto
35kg,and300mgforchildrenbetweenfive
andnineyears
Inasystematicreviewofsixrandomized
clinicaltrialsonchemoprophylaxisin
contactswithleprosy,Reveizetal.reported
astudywithrifampicin(singledose),three
studieswithdapsone(onedoseeverytwo
weeksfortwoyears,andonedosetwicea
weekforthreeyears,oronedoseaweekfor
twoyears),andtwowithacedapsone(one
doseevery10weeksforsevenmonthsin
both).

Themetaanalysisshowedareductioninthe
incidenceofleprosyamongthecontacts
withpatients,varyingfrom30%to72%,
withtheuseofdapsone,acedapsone,or
rifampicin.