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9/5/2015 Pustularpsoriasis:Management

OfficialreprintfromUpToDate
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Pustularpsoriasis:Management

Author SectionEditor DeputyEditor


RobertEKalb,MD KristinaCallisDuffin,MD AbenaOOfori,MD

Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Aug2015.|Thistopiclastupdated:May20,2015.
INTRODUCTIONPustularpsoriasisisanuncommonsubtypeofpsoriasisthatmaypresentasageneralizedor
localizedpustularskineruption.Thegoalsoftreatmentofgeneralizedpustularpsoriasis(GPP)aretoimproveskin
manifestations,toalleviateassociatedsystemicsymptoms,andtominimizeriskforlifethreateningsystemic
complications.Treatmentoflocalizedpustularpsoriasisisindicatedtominimizethedevelopmentofbothersomeor
disablingsymptoms.

Thetreatmentofgeneralizedandlocalizedpustularpsoriasiswillbereviewedhere.Theclinicalfeaturesof
pustularpsoriasis,themanagementofpustularpsoriasisinpregnantwomen(impetigoherpetiformis),andthe
managementofotherformsofpsoriasisarereviewedseparately.(See"Pustularpsoriasis:Pathogenesis,clinical
manifestations,anddiagnosis"and"Dermatosesofpregnancy"and"Treatmentofpsoriasis"and"Guttate
psoriasis".)

GENERALIZEDPUSTULARPSORIASISGeneralizedpustularpsoriasis(GPP)ischaracterizedbythe
developmentofawidespreaderuptionofpustulesanderythematousplaques(picture1AC).Aprecedinghistoryof
psoriasismayormaynotbepresent.Theacutevariant(alsoknownasgeneralizedpustularpsoriasisofvon
Zumbusch)ischaracterizedbysuddenonsetofthepustulareruptionaccompaniedbysystemicsymptomsof
feverandmalaise.Laboratoryabnormalities,suchasleukocytosis,anelevatederythrocytesedimentationrate,
hypocalcemiaandotherelectrolyteabnormalities,hypoalbuminemia,andelevatedliverenzymesarecommon.In
addition,seriouscomplications,includingsepsisandhepatic,respiratory,orrenaldysfunctioncanoccur[14].
(See"Pustularpsoriasis:Pathogenesis,clinicalmanifestations,anddiagnosis",sectionon'Generalizedpustular
psoriasis'.)

GPPmayalsopresentasalessacutedisorderinwhichpatientsdevelopwidespreadannularorfigurate
erythematousplaqueswithperipheralpustulesandscale(generalizedannularpustularpsoriasis)[4,5].Painand
fevermayaccompanythesecutaneousmanifestations.(See"Pustularpsoriasis:Pathogenesis,clinical
manifestations,anddiagnosis",sectionon'Clinicalmanifestations'.)

ThereisnocureforGPPandrecurrencesarecommon[4].Thus,longtermtreatmentoftenisrequiredtominimize
recurrencesofdisease.

IncreasingknowledgeaboutthepathogenesisofGPPassociatedwithIL36RNmutationsmayleadtonew
therapeuticoptionsforthisformofGPP.(See'IL36RNmutations'belowand"Pustularpsoriasis:Pathogenesis,
clinicalmanifestations,anddiagnosis",sectionon'Genetics'.)

ApproachtotherapyDeterminingtheoptimalapproachtothetreatmentofGPPiscomplicatedbythelackof
highqualitydataontheefficacyoftreatmentsforthisdisease.Dataontreatmentprimarilyconsistsoffindingsof
retrospectivestudies,casereports,andexpertopinion.Interpretationoftheavailabledataisfurthercompromised
bythelackofavalidatedgradingsystemfortheseverityofGPPandtheabsenceofastandardizedmethodof
assessingtheresponsetotreatment.Theapproachdescribedinthistopicreflectsourpreferredapproachbased
uponreviewoftheliteratureandclinicalexperienceotherapproachesalsomaybereasonable.

Ourapproachtotreatmentconsistsofthefollowingkeysteps:

DetermineneedforhospitalizationandsupportivecarePatientswithacuteGPPusuallyappear
systemicallyillandadmissiontothehospitaloftenisnecessarytoensureadequatesupportivecare.The
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decisiontohospitalizeapatientismadebaseduponglobalconsiderationoftheseverityofillness,vitalsign
stability,fluidandelectrolytestatus,andconcernforsystemicinfection.

SupportiveskincaremeasuresmayhelptosootheskinsymptomsinpatientswithGPP.Useof
moisturizers,wetwraps,and/oroatmealbathscanbebeneficialinpatientswiththisdisease[1].

Identifyanddiscontinuethecausativedrug(indruginducedcases)Thewithdrawaloradministration
ofavarietyofdrugshasbeenlinkedtoGPP.Systemicglucocorticoidsarecommonlycitedcontributors[4].
Ingeneral,acausativeagentshouldbediscontinuedprovidedthiscanbedonesafely.However,thereis
insufficientevidencetoconfirmthebestwaytodiscontinuesystemicglucocorticoidtherapywhenGPPis
precipitatedbysystemicglucocorticoidwithdrawal.Optionsincludemaintainingtheglucocorticoiddoseuntil
diseasecontrolisachievedwithothertherapiesandcontinuingtotaperthesystemicglucocorticoidduring
theinitiationofGPPtherapy.(See"Pustularpsoriasis:Pathogenesis,clinicalmanifestations,anddiagnosis",
sectionon'Precipitatingfactors'.)

InitiatetreatmenttocontrolskindiseaseMedicaltreatmentoptionsforGPPconsistofsystemic
therapies,topicaltherapies,andphototherapy.Wetypicallyusesystemictreatmentfortheinitial
managementofadultswithGPPbecausephototherapytendstohaveadelayedonsetofactionandtopical
therapiesareimpracticalforthetreatmentofwidespreaddisease.

Inadditiontolimitingfactorssuchasdrugavailabilityandpatientspecificcontraindications,theselectionof
aninitialsystemictherapyforGPPisbasedupontheacuityandseverityofdisease.Whereasadultswith
slowlyprogressing,relativelystableeruptionscanbemanagedwithagentssuchasacitretinormethotrexate,
patientswithacute,severediseaserequiringrapidimprovementmaybenefitfrominitialtreatmentwithfaster
actingtherapies,suchascyclosporineorinfliximab[6,7].

Topicaltherapies,includingtopicalcorticosteroids,topicalvitaminDanalogs,andtopicaltacrolimus,
primarilyserveasadjunctstosystemictherapyinadultswithGPP.Wetypicallyfocustopicaltherapyon
areasofpersistentorrecalcitrantskininvolvement[6,8].Phototherapyisasecondlinetreatmenttypically
reservedforpatientswhohavealreadyachievedcontrolofacutedisease.

ManageextracutaneouscomplicationsExtracutaneouscomplicationssuchassepsisorinternalorgan
dysfunctionshouldbemanagedappropriately.(See"Pustularpsoriasis:Pathogenesis,clinical
manifestations,anddiagnosis",sectionon'Clinicalcourse'.)

TheapproachtotreatmentreviewedbelowfocusesonnonpregnantadultswithGPP.Thetreatmentofpregnant
womenandchildrenwithGPPisreviewedseparately.(See"Dermatosesofpregnancy",sectionon'Pustular
psoriasisofpregnancy'and'Children'below.)

FirstlinetherapyOurselectionofacitretin,methotrexate,infliximab,andcyclosporineasfirstlinetherapeutic
optionsforadultonsetGPPisinagreementwitha2012consensusstatementfromthetaskforceoftheNational
PsoriasisFoundationMedicalBoard[6].

Acitretinandmethotrexatearegenerallywelltolerateddrugsthatcanbeusedforlongtermtreatment.However,
asnotedabove,thetimetotreatmentefficacyforacitretinandmethotrexatetendstobelongerthanforinfliximab
andcyclosporine.Thus,forourpatientswithsevere,acutediseasethatwarrantsrapidstabilizationand
improvement,thefasteractingdrugsinfliximabandcyclosporineareourpreferredinitialagents.Oncecontrolof
acutediseaseisachieved,patientsmaybetransitionedtoacitretin,methotrexate,orothertherapies.

Inadditiontorapidityofonset,otherpatientspecificfactorscaninfluencetreatmentselection.Asanexample,the
requirementthatwomenabstainfrompregnancyforthreeyearsafteracitretintreatmentgivesacitretinarelative
contraindicationforuseinwomenofchildbearingage.

PatientswithrelativelystablediseaseAcitretinisourtreatmentofchoicefortheinitialmanagementof
adultswithrelativelystableGPP.Methotrexateisanalternativefirstlinetreatment.Wealsousethesedrugsfor

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longtermtreatmentofGPPfollowingcontrolofsevereacutedisease.

OralretinoidsOralretinoids(etretinate,acitretin)arewellacceptedandwidelyusedtreatmentsfor
GPP.EtretinatewaswithdrawnfromtheUSmarketin1998andreplacedbyitsmetaboliteacitretinbecauseof
concernabouttheprolongedeliminationhalflifeofetretinate.

EfficacyAlthoughoralretinoidsareconsideredastandardtreatmentforGPP,evidencetosupporttheiruse
islimited.SomesupportfortheuseoforalretinoidsstemsfromaJapanesestudythatanalyzedtreatment
datafrom385patientswithacuteGPPobtainedthroughquestionnaireresponsesfrommultiplecommunity
centerhospitals[9].Thestudyfoundthatamong188patientstreatedwithretinoids,treatmentwasreported
aseffectivein84percent.Retinoidtreatmentregimenswerenotstandardizedtypically,treatmentwaswith
etretinate(1mg/kgperdayandtaperedastolerated).Manypatientsreceivedretinoidsincombinationwith
othertherapies.

Aseparateretrospectivestudyof63patientshospitalizedatMayoClinicaffiliatedhospitalsbetween1961
and1989alsosuggestsefficacyoforalretinoidtherapy.Goodresponseswererecordedinallofsixpatients
treatedwithetretinateforacuteGPPandbothoftwopatientsgiventhedrugforannularpustularpsoriasis
[10].

Thereissomeevidencetosuggestthatisotretinoin,whichisnotconsideredeffectiveforchronicplaque
psoriasis,maybeofbenefitinGPP.Inaseriesof11adultswithGPP,isotretinointherapyseemed
beneficialforimprovingpustuleformationin10patients,althoughmonotherapywiththedrugdidnotseemto
beeffectiveforclearingalllesions[11].Isotretinoinalsoappearedusefulinanadolescentfemalewhofailed
torespondadequatelytotopicalcorticosteroidsandmethotrexate[12].

AdministrationAcitretinisourpreferredoralretinoidforGPPbecauseclinicalexperiencewithisotretinoin
forthisindicationismorelimitedandetretinateisnotavailableintheUnitedStates.Ourusualstartingdose
foracitretinis0.75to1mg/kgperday.Japaneseauthorshavesuggestedaninitialdoseofetretinateof0.5
to1mg/kgperday[13].

ImprovementinGPP(ie,cessationofnewpustuleformationandinitialimprovementinotherclinicalsigns)is
usuallynotedwithin7to10daysoforalretinoidtherapy.Afterdiseasecontrolisachieved,thedoseof
acitretincanbetaperedslowlytothelowestdosenecessarytomaintaintheresponse.Itisimpossibleto
predictforindividualpatientsbutacompleteresponseoftenrequirestwotothreemonthstoachieve.

Examplesofadverseeffectsoforalretinoidsincludexerosis,cheilitis,drymucousmembranes,
hypertriglyceridemia,hairloss,liverfunctiontestabnormalities,bonechanges,andvisualchanges.Oral
retinoidsareteratogenicandpregnancyshouldbeavoidedforthreeyearsfollowingacitretintherapy.
Therefore,thedrughasarelativecontraindicationforwomenofchildbearingage.Incontrast,pregnancyis
contraindicatedforonlyonemonthfollowingisotretinointherapy.(See"Oralisotretinointherapyforacne
vulgaris",sectionon'Isotretinoinsafety'.)

MethotrexateOralmethotrexateappearstobeeffectiveforGPPandisanalternativetoacitretintherapy
[6].

EfficacyDataontheefficacyofmethotrexateforGPParelimited.InamulticenterstudyinJapaninwhich
communityhospitalsweresentquestionnairesaboutpatientswithGPP,efficacyofmethotrexatewas
documentedfor76percentof41patientsgiventhistherapyaloneorinconjunctionwithothertherapies[9].
Inaddition,inaretrospectivestudyof63patientshospitalizedforGPPatMayoClinicaffiliatedhospitals
between1961and1989,goodresponsestomethotrexatewerereportedforthreeofeightpatientswithacute
GPPandbothoftwopatientswithannularpustularpsoriasis[10].

AdministrationAtypicaldoseofmethotrexateforadultswithGPPis15mgperweek,withamaximum
doseof25mgperweek.Methotrexateisnotgivendaily.Absorptionoforalmethotrexatemaybereducedat
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higherdoses[14,15].Thus,whendoseshigherthan15mgperweekarerequired,weoftenuse
subcutaneousorintramuscularadministration.

Myelosuppressionisapotentialseriousadverseeffectofmethotrexatetherapythatwarrantscareful
administrationoftreatmentandlaboratoryfollowup.Theamountofmethotrexateusedfortheinitialdose
variesamongexperts,withsomeexpertsinitiatingwithasmalltestdose(eg,5mgperweek)followedby
upwardtitrationofthedoseandothersinitiatingathigherdoses(eg,15mgperweek)[16].Whentreating
olderadultpatientsorpatientswithrenalinsufficiency,theinitialdoseshouldnotexceed10mgperweek.

Additionaladverseeffectsofmethotrexateincludegastrointestinaldistress,hepatotoxicity,pulmonary
toxicity,andteratogenicity.Methotrexateshouldbecombinedwithfolicacidsupplementation(eg,1mgof
folicacidperday)todecreasehematologicandgastrointestinaltoxicity[17].(See"Majorsideeffectsoflow
dosemethotrexate".)

Monitoringprotocolsformethotrexatetreatmentvaryingeneral,acompletebloodcountwithdifferentialand
plateletsshouldbeperformedatbaselineandsoonaftertheinitiationofmethotrexateandfollowingdose
increases.Whilesomecliniciansperformhematologictestingapproximatelyoneweekafterdoseinitiationor
changes,others(includingtheauthor)performtestsaftertwotofourweeksprovidedthepatientisnotan
olderadultindividualanddoesnothaverenalinsufficiency.Hematologictestingisrepeatedeverytwotofour
weeksduringthefirstfewmonthsoftreatmentandissubsequentlytaperedtoeveryonetothreemonths.
Periodiclaboratorymonitoringofkidneyandliverfunctionisalsoindicatedduringmethotrexatetherapy.

TopicaltherapyBecauseofthewidespreadnatureofGPP,topicalmedicationsareprimarilyreserved
foradjunctivetherapy.Topicaltherapiesthathaveseemedusefulforadjunctivetherapyincasereportsaresimilar
tothoseusedinchronicplaquepsoriasis,andincludetopicalcorticosteroids[18],combinationtherapywitha
topicalcorticosteroidandtopicalvitaminDanalog[19],andtopicaltacrolimus[20].Topicalcorticosteroidsarethe
topicalagentsweusemostfrequentlyinpatientsforwhomcorticosteroidsarenottheprecipitatingfactorforthe
episodeofGPP.ThedevelopmentofGPPinassociationwithuseoftopicalcorticosteroids[2123]andtopical
vitaminDanalogs[21,24,25]hasbeenreported.

Wetypicallylimituseoftopicalagentstolocalizedareasofrecalcitrantskininvolvement.However,apatientin
whomGPPappearedtorespondtototalbodyapplicationoftopicaltacrolimusastheprimarytreatmenthasbeen
reported[26].

PatientswithsevereacutediseaseCyclosporineandinfliximabareourtreatmentsofchoicefortheinitial
managementofsevereacuteGPP.

CyclosporineOralcyclosporinehasalonghistoryofuseforpsoriasisandcaninducerapid
improvementofGPP.

EfficacyDespitethecommonuseofcyclosporineforsevereacuteGPP,dataontheefficacyofthedrug
arelimited.Abeneficialeffectofcyclosporineissupportedbyretrospectivedataobtainedfromhospitalsin
Japanthatsuggestedtreatmentefficacyin60to70percentofpatientstreatedwithcyclosporinealoneorin
conjunctionwithothertherapies[9,13].Markedimprovementoftenoccurswithinthefirstfewdaysof
treatment[1,27,28].

AdministrationEffectivedosesofcyclosporineforadultswithGPPhaverangedfrom2.5to5mg/kgper
day[6].WetypicallytreatsevereacuteGPPwith4to5mg/kg(idealbodyweight)perday.Becauseside
effectsoftreatmentwithcyclosporineareaconcern,oncediseasecontrolisachieved,weattempttotaper
thedoseoverthecourseoftwotothreemonths.

Potentialadverseeffectsofcyclosporineincludehypertension,renaltoxicity,andincreasedriskfor
infectionsandmalignancy.Laboratorytestsaswellasbloodpressureshouldbemonitoredcloselyduring
therapy.Adverseeffectsofcyclosporinearediscussedingreaterdetailseparately.(See"Pharmacologyand
sideeffectsofcyclosporineandtacrolimus",sectionon'Sideeffects'and"Pharmacologyandsideeffectsof
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cyclosporineandtacrolimus",sectionon'Strategiestominimizetoxicityinrheumaticdiseases'.)

InfliximabComparedwithotherfirstlinetreatments,infliximabisanewertreatmentoptionforadults
withGPP.

EfficacyTheuseofinfliximabforGPPisbaseduponcasereportsandaretrospectivestudythatsuggest
efficacyofthistreatment[7,2936].Among10patientsinwhomflaresofacuteGPPweretreatedwith
infliximabinaFrenchmulticenterretrospectivestudy,clinicalremissionwasachievedby8patients(80
percent).Thefastonsetofinfliximabwasevidentinthetimerequiredtoachieveclearanceofpustules
pustulesclearedinamedianoftwodays(rangeonetoeightdays)[7].

AdministrationStandarddosingforinfliximabforpsoriasisis5mg/kgatweekszero,two,andsix,and
everysixtoeightweeksthereafter.Treatmentwithinfliximabmaybecontinuedforlongtermmanagementof
GPP.Alternatively,patientscanbetransitionedtoothertherapiesaftercontrolofacutediseaseisachieved.
Thepossibilitythatasingledoseofinfliximabmaybesufficientissuggestedbyacasereportdocumenting
dramaticimprovementinacuteGPPwithin24hoursfollowingasingledoseofinfliximabfollowedbythe
initiationofmethotrexate[29].Infliximabhasalsobeensuccessfullyusedincombinationwithacitretinforthe
inductionofrapidimprovementwhileawaitingtheonsetofactionofacitretin[37].

PatientsshouldbeevaluatedforlatenttuberculosisandhepatitisBpriortoinitiatingtherapy.Potential
adverseeffectsofinfliximabincludeinfusionreactionsandincreasedriskforinfection,malignancy,heart
failure,anddemyelinatingdisease.Therearealsoreportsofinfliximabinducingpsoriaticeruptions,including
GPP[38].Theadverseeffectsofinfliximabarereviewedseparately.(See"Tumornecrosisfactoralpha
inhibitors:Anoverviewofadverseeffects".)

SecondlinetherapyPatientswhodonotrespondtoorcannottoleratefirstlinetherapiesforGPPmaybenefit
fromotherapproachestotherapy,suchasphotochemotherapy,additionalbiologicTNFalphainhibitors,and
combinationtherapy.

PsoralenplusUVA(PUVA)photochemotherapyPUVAphototherapyinvolvestheadministrationof
photosensitizingpsoralenorallyortopicallypriortoexposuretoaUVAlightsource.Inanuncontrolled
prospectivestudyofeightpatientswithacuteGPP,oralPUVAphotochemotherapy(fourtimesweekly)was
associatedwithcompleteclearingofGPPinallpatientswithinameanof13.510treatmentsessions[39].
Maintenancetherapy(twiceweeklyPUVAtreatmentstaperedtodiscontinuationiftolerated)wasgivenupon
completeremission,andsevenpatientsremainedincompleteremissionduringfollowupperiodsofupto1.5
years.ThefrequentclinicvisitsrequiredforPUVAphotochemotherapymaymakethisalessfavorable
treatmentoptionforsomepatients.

OtherbiologicTNFalphainhibitorsSuccessfulcontrolofGPPduringadalimumaboretanercepttherapy
hasbeenreportedinsmallnumbersofpatients[7,4044].Inoneretrospectivestudy,twoofthreeGPPflares
treatedwithadalimumabprogressedtoclinicalremissionsandclearanceofpustulesoccurredwithin7and
28days[7].Ofnote,therearereportsofGPPinducingpsoriaticeruptionsincludingGPP[38].

CombinationtherapySeveralcasereportsdemonstrateefficacyinrecalcitrantGPPwhentwoormore
classesoftherapeuticagentsareusedincombination.Examplesincludeetanerceptandcyclosporine[28],
infliximabandmethotrexate[36],adalimumabandacitretin[45],infliximabandacitretin[37],and
cyclosporineandPUVAphotochemotherapy[27].Inaddition,childrenhaverespondedtocombination
therapywithnarrowbandUVBandsystemictherapies[46,47].

OthertherapiesAvarietyofothertherapieshavebeenusedforGPP,althoughconcernforsideeffectsor
limitedexperienceprecludesarecommendationfortheroutineuseofthesetherapies.

AlthoughsystemicglucocorticoidtherapycanleadtorapidimprovementinGPP[13],thetreatmentmustbeused
withcautionbecausesystemicglucocorticoidsareimplicatedaspotentialincitingfactorsforGPP[6].Inaddition,

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thereisconcernfortheserioussideeffectsfromlongtermglucocorticoidtherapy.Thus,wedonottypicallyuse
systemicglucocorticoidsinthetreatmentofGPP.Intheeventthatsystemicglucocorticoidtreatmentisgivento
obtaininitialcontrolofGPP,wesuggestalsoinitiatingasecondtherapyinanattempttoreducethelikelihoodofa
diseaseflareduringtaperinganddiscontinuationofthesystemicglucocorticoid.However,evidencetosupportthis
approachislacking.Theadverseeffectsofsystemicglucocorticoidsarereviewedseparately.(See"Majorside
effectsofsystemicglucocorticoids".)

Severalcasereportsdocumentthesuccessfuluseofgranulocyteandmonocyteapheresisforthetreatmentof
refractoryGPP[4852].OthertreatmentsthathavebeenreportedtobeeffectiveforGPPincasereportsinclude
anakinra[51,53,54],ustekinumab[55],mycophenolatemofetil[56],andoralzinc[57].Itremainstobeseen
whetheranakinraisprimarilyeffectiveforGPPassociatedwithdeficiencyoftheinterleukin36receptorantagonist
(DITRA).Inaddition,theonsetorworseningofpustularpsoriasisfollowingustekinumabtreatmenthasbeen
reported[58,59].(See'IL36RNmutations'below.)

Specialpopulations

ChildrenChildhoodGPPisrare,contributingtoapaucityofdataontheefficacyandsafetyoftreatments
forGPPinchildren.WeagreewiththefollowingfirstlinetreatmentapproachforacuteGPPasoutlinedbythe
taskforceoftheNationalPsoriasisFoundationMedicalBoard[6]:

Firstlinetherapy

Acitretin(<1mg/kgperday)
Cyclosporine(1to3mg/kgperday)
Methotrexate(0.2to0.4mg/kgperweek)
Etanercept(0.4mg/kgperday)

NorandomizedtrialshavebeenperformedtoconfirmtheefficacyoftheseagentsinthetreatmentofacuteGPPin
children.Theselectionofanoralretinoid[8,12,60,61],cyclosporine[47,6265],methotrexate[61,66,67],and
etanercept[68]isprimarilybasedoncasereportsthatsuggestefficacyofthesedrugsinchildrenwithGPPand
experiencewiththeseagentsforotherformsofpsoriasisinchildren.Inparticular,randomizedtrialshave
supportedtheefficacyandsafetyofetanerceptformoderatetosevereplaquepsoriasisinchildren[69,70].
However,evidenceforefficacyofetanerceptinGPPisverylimited[68].

Giventhecontraindicationforpregnancyduringacitretintreatmentandforthreeyearsafterdrugdiscontinuation,
theuseofacitretiningirlsmustbeconsideredcarefully.Successfultreatmentwithisotretinoininplaceofacitretin
hasbeenreportedinanadolescentfemale[12].Aslightriskforskeletaltoxicityhasbeenobservedinchildren
treatedwithhighdosesoforalretinoidsfordisordersofkeratinization[71].

AdditionaltherapiesthatmaybeusefulinthetreatmentofpediatricacuteGPPbaseduponcasereportsinclude
narrowbandUVBphototherapyinconjunctionwithsystemictherapy[46,47],adalimumab[40],andinfliximab[68].
Also,aninfantwithGPPassociatedwithIL36receptorantagonistdeficiencyhasrespondedtoanakinra[72].

Topicalcorticosteroidtherapymaybeeffectiveforthetreatmentofchildrenwithannularpustularpsoriasis,which
exhibitslessseveremanifestationsthanacuteGPP[5].Inaddition,topicalcompresses,wetwraps,oroatmeal
bathsmaybehelpfulforsoothingtheskinlesions[5,73].

Topicaltherapyforannularpustularpsoriasisislesspracticalwhenalargeproportionofthebodysurfaceis
affected.Patientswhocannotbemanagedonlywithtopicaltherapycanbetreatedwithsystemicagents.Case
reportssuggestthatoralretinoids,oraldapsone,andmethotrexatecanbeeffectiveforthisvariant[5].

PregnantwomenThemanagementofpustularpsoriasisinpregnancy(impetigoherpetiformis)isreviewed
separately.(See"Dermatosesofpregnancy",sectionon'Pustularpsoriasisofpregnancy'.)

IL36RNmutationsGrowingunderstandingofthemolecularbasisofGPPassociatedwithIL36RN

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mutationsmayaidintherapeuticdecisionsforpatientswiththistypeofGPP.Evidencesuggeststhatsomecases
ofGPP(oracutegeneralizedexanthematouspustulosis[AGEP])mayactuallyrepresentanewgenetic
autoinflammatorydiseasebasedonmutationsintheIL36RNgene,whichencodestheIL36receptorantagonist.
Thisdiseaseisknownasthedeficiencyofinterleukin36receptorantagonist(DITRA).(See"Pustularpsoriasis:
Pathogenesis,clinicalmanifestations,anddiagnosis".)

PatientswithIL36RNmutationsupregulateIL1inresponsetoIL36stimulation.Casereportsdocumenting
successfultreatmentofGPPinpatientswithIL36RNmutationswithanakinra,anIL1receptorantagonist,support
theimportanceofthispathway[54,72].Inaddition,preliminaryfindingsofclinicaltrialssuggestthattreatmentwith
IL1antagonistscanimproveGPP(picture2).Asmoredataaccumulate,IL1blockademaybecomethetreatment
ofchoiceforpatientswhoharborthegeneticmutation.

LOCALIZEDPUSTULARPSORIASISTheapproachtothetreatmentofpustularpsoriasisdiffersfrom
generalizedpustularpsoriasis(GPP).Localtreatmentgenerallyisusedasfirstlinetreatment,withsystemic
therapiesreservedforpatientswhofailtorespondwelltolocaltherapy.

AcrodermatitiscontinuaofHallopeauAcrodermatitiscontinuaofHallopeau(ACH)isarare,chronic,
localizedformofpustularpsoriasisthatprimarilyinvolvesoneormoreextremitydigits(picture3).ACHisoften
poorlyresponsivetotherapy.

AvarietyoftopicalandsystemictherapieshavebeenutilizedforACHwithvariableresults.Dataontreatmentof
thisrarediseaseareprimarilylimitedtocasereports.Topicalcorticosteroids,topicaltacrolimus,topicalcalcipotriol
(aloneorincombinationwithtopicalcorticosteroidsortopicaltacrolimus),topicalmechlorethaminehydrochloride,
topicalfluorouracil,PUVAphotochemotherapy,andnarrowbandUVBphototherapyareamongthelocaltreatments
documentedaseffectiveinindividualpatientswithACH[74].Systemictherapieshaveincludedoralretinoids,
methotrexate,cyclosporine,systemicglucocorticoids,methotrexateandpropylthiouracil,infliximab,adalimumab,
etanercept,andanakinra[7478].Theefficaciesoftheseinterventionshavenotbeencomparedandthebest
approachtotreatmentisunclear.

OurinitialchoicefortreatmentofACHistypicallyasuperpotenttopicalcorticosteroid(eg,halobetasolor
clobetasol),whichweinstructthepatienttoapplytotheaffectedareasoncetotwicedailyfortwotofourweeks
(table1).Preferably,thepatientshouldapplythemedicationunderanocclusivedressingatnight,particularly
duringthefirstweekoftherapy.Plasticwrapiscommonlyusedasanocclusivedressing.Alternatively,occlusion
canbeappliedusingdampclothcoveredbydrycloth.

Ifagoodresponsetotreatmentoccurs,thefrequencyofapplicationcanbetaperedastoleratedtoafrequencyas
lowasonceortwiceperweek.Alternatively,weprescribeatopicalvitaminDanalog(eg,topicalcalcitriolor
calcipotriene)inconjunctionwiththesuperpotenttopicalcorticosteroidandinstructthepatienttoapplythetopical
corticosteroidfollowedimmediatelybythevitaminDanalogoncetotwicedailyfortwotofourweeks.Once
sufficientimprovementisachieved,wetaperthetopicalcorticosteroidastoleratedandcontinuetreatmentwiththe
vitaminDanalog.Acommercialproductcontainingbothbetamethasonedipropionateandcalcipotrieneis
available.

Whenpatientsfailtorespondadequatelytotopicaltherapy,weproceedtolocalphototherapyorsystemictherapy,
althoughweusuallycontinuetousetopicalcorticosteroidsasadjunctivetherapy.BothpsoralenplusUVA(PUVA)
photochemotherapyandnarrowbandUVBhaveappearedeffectiveforACHincasereports[74].Weoftenuse
acitretin(0.5to1mg/kgperday)asourfirstlinesystemictreatment,withmethotrexateandcyclosporineas
alternatives.Ifapatientfailstoimprovewiththesetraditionalsystemictherapies,wemayattempttreatmentwith
abiologicTNFalphainhibitor,suchasadalimumab,infliximab,oretanercept.

PalmoplantarpustulosisPalmoplantarpustulosisisachronicconditioncharacterizedbythedevelopmentof
yellowtobrownpustules,scale,andpatchyerythemaonthepalmsorsoles.Itiscontroversialwhether
palmoplantarpustulosisisalocalizedvariantofpustularpsoriasis(palmoplantarpustularpsoriasis)oraseparate
entity.Thetreatmentofpalmoplantarpustulosisisreviewedseparately.(See"Palmoplantarpustulosis:

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9/5/2015 Pustularpsoriasis:Management

Treatment".)

SUMMARYANDRECOMMENDATIONS

Generalizedpustularpsoriasis(GPP)isanuncommonformofpsoriasischaracterizedbythedevelopmentof
widespreadpustulesanderythematousplaquesontheskin.Thegoalsoftreatmentaretoimproveskin
manifestations,alleviatesystemicsymptoms,andpreventseriousorlifethreateningcomplicationsofthis
disease.(See'Generalizedpustularpsoriasis'above.)

TreatmentoptionsforadultswithGPPincludetopicaltherapies,systemictherapies,andphototherapy.Most
patientsrequiresystemictherapy.Topicaltherapiesareprimarilyusedasadjunctstosystemictherapy.(See
'Approachtotherapy'above.)

AspartoftheinitialmanagementofpatientswithGPP,theneedforhospitalizationshouldbeassessed.In
addition,thepatientsmedicalhistoryshouldbereviewedfordrugsthatmaycontributetothedevelopmentof
GPP.(See'Approachtotherapy'above.)

TheapproachtothetreatmentofGPPisinfluencedbytheacuityandseverityofsymptoms.Foradult
patientswithrelativelystabledisease,wesuggestacitretinasinitialtherapy(Grade2C).Methotrexateisan
alternativefirstlinetreatment.ForadultpatientswithsevereacuteGPP,wesuggestinitialtherapywithfast
actingtherapiessuchascyclosporineorinfliximab(Grade2C).(See'Firstlinetherapy'above.)

OthertherapeuticagentsthatmaybeeffectiveinadultswithGPPincludepsoralenplusUVA(PUVA)
photochemotherapy,adalimumab,andetanercept.Combinationtherapywithmorethanonetreatmentcan
alsobeattempted.(See'Secondlinetherapy'above.)

SystemicglucocorticoidscaninducerapidimprovementinGPP,buthavebeenidentifiedaspotentialinciting
factorsforthisdisease.WedonotusuallyusesystemicglucocorticoidsforthetreatmentofGPP.(See
'Othertherapies'above.)

DataonthetreatmentofacuteGPPinchildrenareverylimited.Optionsforfirstlinetherapyincludeacitretin,
cyclosporine,methotrexate,andetanercept.Somechildrenwithannularpustularpsoriasiscanbemanaged
withtopicaltreatment.(See'Children'above.)

IncreasingknowledgeaboutthepathogenesisofGPPassociatedwithIL36RNmutationsmayleadtonew
therapeuticoptionsforthisformofGPP.(See'IL36RNmutations'above.)

AcrodermatitiscontinuaofHallopeau(ACH)isarareformoflocalizedpustularpsoriasisthatprimarily
affectstheextremitydigitsandisoftenrefractorytotreatment.DataontreatmentsforACHarelimitedto
casereports.Wesuggestasuperpotenttopicalcorticosteroidasinitialtreatment(Grade2C).(See
'AcrodermatitiscontinuaofHallopeau'above.)

UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.

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Topic93554Version3.0

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GRAPHICS

Generalizedpustularpsoriasis

Pustules,erythema,andscaleingeneralizedpustularpsoriasis.

Reproducedwithpermissionfrom:www.visualdx.com.CopyrightLogicalImages,Inc.

Graphic94789Version1.0

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Pustularpsoriasis

Widespreaderythematouspatches,desquamation,andpustulesinpustularpsoriasis.

Reproducedwithpermissionfrom:www.visualdx.com.CopyrightLogicalImages,Inc.

Graphic94790Version1.0

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Generalizedpustularpsoriasis

Diffuseerythema,numerouspustules,andscale.

Reproducedwithpermissionfrom:www.visualdx.com.CopyrightLogicalImages,Inc.

Graphic95194Version1.0

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ResponseofgeneralizedpustularpsoriasistotreatmentwithanIL1antagon

DramaticresponseofseveregeneralizedacutepustularpsoriasistotreatmentwithanIL1antagonist.

Reproducedwithpermission.ImagecourtesyofXOMA(US),LLC.Copyright2014.

Graphic95263Version3.0

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AcrodermatitiscontinuaofHallopeau

Pustules,erythema,scale,andnaildystrophyinvolvingthedistalfingers.

Reproducedwithpermissionfrom:www.visualdx.com.CopyrightLogicalImages,Inc.

Graphic94791Version1.0

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9/5/2015 Pustularpsoriasis:Management

Comparisonofrepresentativetopicalcorticosteroidpreparations
(classifiedaccordingtotheUSAsystem)

Available
Trade strength(s), Generic
Potency Vehicle
Corticosteroid names percent available
group* type/form
(US) (exceptas inUS
noted)
Super Betamethasone Ointment, Diprolene 0.05 Yes
high dipropionate, optimized
potency augmented
Lotion Diprolene 0.05 Yes
(group1)
Gel Diprolene 0.05 Yes

Clobetasol Ointment Temovate 0.05 Yes


propionate
Cream Temovate 0.05 Yes

Cream, TemovateE 0.05 Yes


emollientbase

Gel Temovate 0.05 Yes

Lotion Clobex 0.05 No

Foamaerosol OluxE 0.05 No

Foamaerosol Olux 0.05 Yes


(scalp)

Shampoo Clobex 0.05 No

Solution Temovate, 0.05 Yes


(scalp) Cormax

Sprayaerosol Clobex 0.05 No

Diflucortolone Ointment,oily Nerisone 0.3 No


valerate(not cream Forte(UK,
availableinUS) others)

Halobetasol Ointment Ultravate 0.05 Yes


propionate
Cream Ultravate 0.05 Yes

Fluocinonide Cream Vanos 0.1 No

Flurandrenolide Tape(roll) Cordran 4mcg/cm 2 No

High Amcinonide Ointment Cyclocort , 0.1 Yes


potency Amcort
(group2)
Betamethasone Ointment Diprosone 0.05 Yes
dipropionate
Cream, Diprolene 0.05 Yes
augmented AF
formulation
(AF)

Halcinonide Ointment Halog 0.1 No


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Cream Halog 0.1 No

Fluocinonide Ointment Lidex 0.05 Yes

Gel Lidex 0.05 Yes

Cream Lidex 0.05 Yes


anhydrous

Solution Lidex 0.05 Yes

Diflorasone Ointment ApexiCon , 0.05 Yes


diacetate Florone

Cream, ApexiConE 0.05 Yes


emollient

Desoximetasone Ointment Topicort 0.25 Yes

Cream Topicort 0.25 Yes

Gel Topicort 0.05 Yes

High Amcinonide Cream Cyclocort , 0.1 Yes


potency Amcort
(group3)
Lotion Amcort 0.1 Yes

Betamethasone Cream, Diprosone 0.05 Yes


dipropionate hydrophilic
emollient

Betamethasone Ointment Valisone 0.1 Yes


valerate
Foam Luxiq 0.12 No

Diflucortolone Cream,oily Nerisone 0.1 No


valerate(not cream, (Canada,
availableinUS) ointment UK,others)

Fluticasone Ointment Cutivate 0.005 Yes


propionate

Fluocinonide Cream LidexE 0.05 No


aqueous
emollient

Mometasone Ointment Elocon 0.1 Yes


furoate

Desoximetasone Cream TopicortLP 0.05 Yes

Diflorasone Cream Florone 0.05 Yes


diacetate

Triamcinolone Ointment Kenalog 0.5 Yes


acetonide
Cream Triderm, 0.5 Yes
Aristocort
HP

Medium Triamcinolone Cream Kenalog 0.1 Yes


potency acetonide
Ointment Kenalog 0.1 Yes
(group4)

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Aerosolspray Kenalog 0.2mgper2 No


secondspray

Flurandrenolide Ointment Cordran 0.05 No

Fluocinolone Ointment Synalar 0.025 Yes


acetonide

Mometasone Cream Elocon 0.1 Yes


furoate
Lotion Elocon 0.1 Yes

Solution Elocon 0.1 Yes

Hydrocortisone Ointment Westcort 0.2 Yes


valerate

Clocortolone Cream Cloderm 0.1 No


pivalate

Lower Triamcinolone Lotion Kenalog 0.1 Yes


mid acetonide
Ointment Kenalog 0.025 Yes
potency
(group5) Betamethasone Lotion Diprosone 0.05 Yes
dipropionate

Flurandrenolide Cream Cordran 0.05 No

Lotion Cordran 0.05 No

Fluticasone Cream Cutivate 0.05 Yes


propionate
Lotion Cutivate 0.05 No

Prednicarbate Cream, Dermatop 0.1 Yes


emollient

Ointment Dermatop 0.1 Yes

Desonide Ointment DesOwen, 0.05 Yes


Tridesilon

Gel Desonate 0.05 No

Betamethasone Cream BetaVal, 0.1 Yes


valerate Valisone

Hydrocortisone Cream Westcort 0.2 Yes


valerate

Hydrocortisone Ointment Locoid 0.1 Yes


butyrate
Cream Locoid, 0.1 Yes
Locoid
Lipocream

Lotion,spray Cortizone10 0.1 No


maximum

Lotion Locoid 0.1 No

Solution Locoid 0.1 Yes

Hydrocortisone Cream Pandel 0.1 No


probutate


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Fluocinolone Cream Synalar 0.025 Yes


acetonide

Low Alclometasone Ointment Aclovate 0.05 Yes


potency dipropionate
Cream Aclovate 0.05 Yes
(group6)
Triamcinolone Cream Kenalog , 0.025 Yes
acetonide Aristocort

Lotion Kenalog 0.025 Yes

Desonide Cream DesOwen, 0.05 Yes


Tridesilon

Lotion DesOwen, 0.05 Yes


LoKara

Foam Verdeso 0.05 No

Betamethasone Lotion BetaVal, 0.1 Yes


valerate Valisone

Fluocinolone Cream Synalar 0.01 Yes


acetonide
Solution Synalar 0.01 Yes

Shampoo Capex 0.01 No

Oil(scalp) Derma 0.01 No


Smoothe/FS

Oil(body) Derma 0.01 No


Smoothe/FS

Least Hydrocortisone Ointment Hytone 2.5 Yes


potent (base)
Cream Hytone, 2.5 Yes
(group7)
Nutracort

Lotion Hytone 2.5 Yes

Solution Texacort 2.5 Yes

Hydrocortisone Ointment Pramosone 1or2.5 Yes


acetatewith
Cream Pramosone, 1or2.5 Yes
pramoxine1
Analpram
percent
HC
combination
Lotion Pramosone, 1or2.5 Yes
Analpram
HC

Aerosolfoam Epifoam 1 Yes

Hydrocortisone Ointment Cortaid, 1 Yes


(base) Hytone,
Nutracort

Cream Cortaid, 1 Yes


Hytone,
Synacort

Lotion AquinilHC, 1 Yes

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SarnolHC,
Cortizone
10

Spray Cortaid 1 Yes

Solution Cortaid, 1 Yes


Noble,Scalp
relief

Ointment Cortaid 0.5 Yes

Cream Cortaid 0.5 Yes

%:percent.
*ListedbypotencyaccordingtotheUSAclassificationsystem:group1isthemostpotent,group7is
theleastpotent.Othercountriesuseadifferentclassificationsystemwithonlyfourorfivegroups.
Vehicleandbaseingredient(s)forgenericproducts,insomecases,maynotbeidenticaltotrade
version.
InactiveUStradenameforspecificproductbrandmaybeavailableoutsideUS.
48%refinedpeanutoil.

Datafrom:LexicompOnline.Copyright19782015Lexicomp,Inc.AllRightsReservedandTadicherla
S,RossK,ShenefeltD,TopicalcorticosteroidsindermatologyJournalofDrugsinDermatology2009
12:1093.

Graphic62402Version27.0

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Disclosures
Disclosures:RobertEKalb,MDGrant/Research/ClinicalTrialSupport:AbbVie[Psoriasis
(Adalimumab)]Amgen[Psoriasis(Etanercept)]Janssen[Psoriasis(Adalimumab)]LEOPharma
[Psoriasis(Taclonex)]Merck[Psoriasis].Consultant/AdvisoryBoards:AbbVie[Psoriasis(Adalimumab)]
CelgeneCorporation[Psoriasis]Janssen[Psoriasis(Adalimumab)]LEOParma[Psoriasis(Taclonex)]
Novartis[Psoriasis]Pfizer[Psoriasis]Ranbaxy[Psoriasis]TaroPharm[Psoriasis].KristinaCallis
Duffin,MDGrant/Research/ClinicalTrialSupport:Amgen[Psoriasis(Etanercept,brodalumab)]Pfizer
[Psoriasis(tofacitinib)]BristolMyersSquibb[Psoriasis(abatacept)]EliLilly[Psoriasis(ixekizumab)]
AbbVie[Psoriasis(Adalimumab)]Janssen[Psoriasis(Ustekinumab,guselkumab,infliximab)Novartis
[psoriasis(secukinumab)]Celgene[psoriasis(apremilast)]SteifelXenoport[psoriasis(calcipotriene)].
Consultant/AdvisoryBoards:Amgen[Psoriasis(Etanercept,brodalumab)]Pfizer[Psoriasis(tofacitinib)]
BristolMyersSquibb[Psoriasis(abatacept)]EliLilly[Psoriasis(ixekizumab)]AbbVie[Psoriasis
(Adalimumab)]Janssen[Psoriasis(Ustekinumab,guselkumab,infliximab)Novartis[psoriasis
(secukinumab)]Celgene[psoriasis(apremilast)]SteifelXenoport[psoriasis(calcipotriene)].AbenaO
Ofori,MDNothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseare
addressedbyvettingthroughamultilevelreviewprocess,andthroughrequirementsforreferencestobe
providedtosupportthecontent.Appropriatelyreferencedcontentisrequiredofallauthorsandmust
conformtoUpToDatestandardsofevidence.
Conflictofinterestpolicy

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