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Cutaneouslupuserythematosus.DermNetNZ

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Cutaneouslupus
erythematosus

Whatislupuserythematosus?
Lupuserythematosus(LE)isanautoimmuneconnectivetissuedisorderthatcanaffectoneorseveralorgans.Circulatingautoantibodies
andimmunecomplexesareduetolossofnormalimmunetoleranceandarepathogenic.ClinicalfeaturesofLEarehighlyvariable.LE
nearlyalwaysaffectstheskintosomedegree.

Whatiscutaneouslupuserythematosus?
CutaneousLEcomprisesseveralchronicandrelapsingLEspecificandLEnonspecificinflammatoryconditions.Therecanbesomeoverlap.
LEspecificcutaneousLEhasbeenclassifiedasacute,subacute,intermittentandchronic.Lesionsmaybelocalisedorgeneralised.In
LEspecificcutaneousLE,lesionsareofteninducedbyexposuretosunlight.
LEnonspecificcutaneousLEmayrelatetosystemicLEorotherautoimmunedisease.

Whogetscutaneouslupuserythematosus?
CutaneousLEmostoftenaffectsyoungtomiddleagedadultwomen(aged2050years)butchildren,theelderly,andmalesmaybe
affected.
ImportantpredisposingfactorsforcutaneousLEinclude:
Femalegender
Genes:25risklocihavebeenidentifiedandthereareHLAassociations
Skinofcolour

Whatcauseslupuserythematosus?
LEisclassifiedasautoimmune,asitisassociatedwithpathogenicantibodiesdirectedagainstcomponentsofcellnucleiinvarioustissues.
UVBirradiationcauseskeratinocytenecrosis,immunesystemactivationandantibodyformation.
FactorsthataggravateLEinclude:
Sunexposure
Cigarettesmoking
Hormones
Viralinfection
Certaindrugs

Whatarethespecificfeaturesofcutaneouslupuserythematosus?
TherearevarioustypesofcutaneousLE,classifiedasacute,subacute,intermittentandchroniccutaneousLE.TherevisedCutaneous
LupusErythematosusDiseaseAreaandSeverityIndex(RCLASI)canbeusedtoassessdiseaseactivityanddamage.
Acutecutaneouslupuserythematosus
AcutecutaneousLEaffectsatleast50%ofpatientswithsystemiclupuserythematosus(SLE).Manyaresick,young,fairskinned
females.
SpecificfeaturesofacutecutaneousLEmayinclude:

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Malareruptionorbutterflyrash(erythemaandoedemaofcheeks,sparingnasolabialfolds)lastinghourstodays
Erythematouspapularrashonarms,sometimesforminglargeplaquesandspreadingwidely
Photosensitivity(arashonallrecentlysunexposedskin)
Cheilitisandmouthulcers
Blisters(bullousLE)anderosions

SLEmayalsoaffectjoints,kidneys,lungs,heart,liver,brain,bloodvessels(vasculitis)andbloodcells.Itmaybeaccompaniedby
antiphospholipidsyndrome.

SystemicLE

Moreimagesofcutaneouslupuserythematosusandsystemiclupuserythematosus...
Subacutecutaneouslupuserythematosus
About15%ofpatientswithcutaneousLEhavesubacutecutaneousLE.Onethirdofcasesareduetopreviousdrugexposure.
FeaturesofsubacutecutaneousLEinclude:
Precipitationoraggravationbysunexposure
Nonitchypsoriasislikepapulosquamousrashontheupperback,chestandupperarms
Annularorpolycyclicplaquesthatclearcentrally
Absenceofscarringonresolution

Upto50%ofpatientswithsubacutecutaneousLEmayalsohaveamildformofSLE,resultinginarthralgia(painfuljoints)orarthritis
(jointdisease)andlowbloodcounts.SevereSLEisrareinpatientswithsubacutecutaneousLE.

SubacuteLE

DruginducedsubacutecutaneousLE
Morethan100drugshavebeenassociatedwiththeonsetofsubacutecutaneousLE.Theyinclude:
Terbinafine
Tumournecrosisfactoralpha(TNF)inhibitors(biologics)
Anticonvulsants
Protonpumpinhibitors

Druginducedlupus

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Neonatalcutaneouslupuserythematosus
NeonatalcutaneousLEariseswithin2monthsofbirthtomotherswithknownorsubclinicalsubacutecutaneousLE.
FeaturesofneonatalcutaneousLEmayinclude:
Anannularerythematousrash,whichslowlyresolvesover6months
Rashismostoftenperiorbital
Photosensitivity
Bloodcountabnormalities:haemolyticanaemia,leukopenia,thrombocytopenia
Hepatobiliarydisease
Persistentcongenitalheartblock

ApaediatricianshouldassessallbabiesborntomotherswithsubacuteLE(orcarryingantiRo/La)atbirth.Mortalityinbabieswithheart
blockisupto20%,despitepacemakerimplantation.

Neonatallupus

Intermittentcutaneouslupuserythematosus
IntermittentcutaneousLE,moreoftenknownaslupustumidus,isadermalformoflupus.
Featuresoflupustumidusinclude:
Affectssunexposedsitessuchascheeks,neck,anteriorchest
Erythematous,urticarialikepatchesandplaqueswithasmoothsurface
Roundorannularshapes
Clearsduringthewintermonths
Nonscarring

LupustumidusissimilartoJessnerlymphocyticinfiltrate,inwhichdiagnosticcriteriaforlupusareabsent.

Lupustumidus

Chroniccutaneouslupuserythematosus
ChroniccutaneousLEaccountsfor80%ofpresentationswithcutaneousLE.About25%ofpatientswithchroniccutaneousLEalsohave
systemicLE.

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DiscoidLE
DiscoidLEisthemostcommonformofchroniccutaneousLE.Itismoreprevalentinpatientswithskinofcolour,whoareatgreaterrisk
ofpostinflammatoryhyperpigmentationandhypertrophicscarring.
DiscoidLEisconfinedtotheskinabovetheneckinmostpatients,butcanspreadbelowthenecktoaffectupperback,Vofneck,
forearmsandbacksofhands.
Scalp,ears,cheeks,nosearethemostcommonsites.
Mostpatientshavephotosensitivity.
Newlesionsaredestructive,erythematousscalyplaqueswithfollicularprominence.
ScalpdiscoidLEpresentsasred,scalyandbaldplaques.
Slowhealingleadstopostinflammatorypigmentationandwhitescars.
Hairgrowthmaypartiallyorcompletelyrecoverwithtreatment.Cicatricial(scarring)alopeciacanbepermanent.

HypertrophicLE
HypertrophicLEisavariantofdiscoidLEinwhichtherearethickenedandwartyplaquesresemblingviralwartsorskincancers.
HypertophicLEcanoccuronpalmsand/orsoles.ThisisalsocalledpalmoplantarLE,andisaformofacquiredkeratoderma.
MucosalLE
MucosalLEpresentswithplaques,ulcersandscaling.Mucosallesionsmaypredisposetosquamouscellcarcinoma.
Lipsandinsidethemouth
Lowereyelidwithmadarosis(lossofeyelashes)
Rarely,vulva/penis

Discoidlupuserythematosus

Moreimagesofcutaneouslupuserythematosus...
Lupusprofundus
Lupusprofundusaffectssubcutaneoustissueandmayalsobecalledlupuspanniculitis.
Lupusprofundusmaydevelopatanyage,includingchildhood.
Itmayinvolveface,buttocks,limbsoranywhere.
Firmdeepandtendernodulespersistforsomemonths.
Lesionsresolveleavingdented,atrophicscars(lipoatrophy).

Lupusprofundus

Whatarethenonspecificcutaneousfeaturesoflupuserythematosus?
LEnonspecificcutaneousfeaturesaremostoftenassociatedwithSLE.Theyinclude:
Diffusehairthinning

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Urticaria
Raynaudphenomenon:abnormalblanchingoffingersandtoesinresponsetocoldweather,followedbynumbnessandslowrewarming
bythefingerswhichgobluethenred.
Lupuschilblains:painfulerythematousnodulesonfingersandtoesduringcoolermonths.
Dilatedperiungualtelangiectasia,raggedcuticlesandnaildystrophy
Digitalulcersandpittingscars
Thrombophlebitis
Papularandnodularmucinosisoncheeks,upperchest,upperarmsorback.
Vasculitis:smallvesselvasculitis,urticarialvasculitisandlessoften,vasculitisofmediumandlargevessels
Livedoreticularisandantiphospholipidsyndrome

Chilblainlupus

Complicationsofcutaneouslupuserythematosus
ChroniccutaneousLEcausesfacialdeformityandscarring.Activeandburnedoutdiseasecanleadtosocialisolationanddepression.
SystemicLEmayinvolveheart,lungandbrainwithsignificantmorbidityandmortality.Vasculitisandantiphospholipidsyndromeinvolving
internalorganscanbeserious.

Howiscutaneouslupuserythematosusdiagnosed?
SLEisassociatedwithhightitreantinuclearantibodies
About70%ofpatientswithsubacuteLEhaveantiRo/Laextractablenuclearantigens(ENA)
TheseverityofLEmaybereflectedinthetitreofANAand/orENA.
ANAandENAareoftennegativeinapatientwithchroniccutaneousLE.
MildanaemiaorleukopeniamaybepresentinpatientsthatdonothaveSLE

Skinbiopsymaybediagnostic,showingalichenoidtissuereactionandfeaturesspecifictothekindofcutaneousLE.Direct
immunofluorescencetestsmayshowpositiveantibodydepositionalongthebasementmembrane(lupusbandtest).
DiagnosticfeaturesonbiopsyaremorelikelytobefoundinLEspecificskinlesionsthaninLEnonspecificcutaneousLE.

Howcancutaneouslupuserythematosusbeprevented?
CarefullyprotectallexposedskinfromsunexposurewithcoveringclothingandSPF50+broadspectrumsunscreen(seesun
protection)
SmokingcessationisessentialitisbesttoavoidnicotinereplacementasnicotineinanyformmayexacerbatecutaneousLE
IfsubacuteLEisdruginduced,stoptheresponsiblemedication.

Whatisthetreatmentforcutaneouslupuserythematosus?
TheaimoftreatmentforcutaneousLEistopreventflares,improveappearanceandtopreventscarring.
Localtherapy
PotentorultrapotenttopicalsteroidsareappliedtochronicdiscoidLEplaques
Calcineurininhibitors,pimecrolimuscreamortacrolimusointmentcanbeusedinsteadoftopicalsteroids
Intralesionalcorticosteroidcanbeinjectedintosmalllesionsresistanttotopicaltherapy
Topicalretinoids,calcipotriolandimiquimodhavealsobeenreportedtobehelpfulinafewpatients.
Cosmeticcamouflagemaybeusedtodisguiseunsightlyplaques.

Systemictherapy

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TreatmentforcutaneousandsystemicLEmayinclude:
Antimalarialsespeciallyhydroxychloroquine
Immunemodulatorssuchasmethotrexate,mycophenolate,dapsone,ciclosporin
Retinoids,ieacitretin,isotretinoin
Systemiccorticosteroids
Ifsunprotectionisstrict,vitaminDsupplementation.

Severediseasemayrequiremoreaggressivetreatment:
Cyclophosphamide
Thalidomide
Photopheresis
Intravenousimmunoglobulin
MonoclonalantibodiestargetingTandBcellsandcytokines:rituximab

Procedures
PhototherapyusingUVA1maybeusefultotreatskinlesionsofcutaneousLE
Photodynamictherapy(PDT)hasbeenreportedtoclearchroniccutaneousLE
Vascularlasercanreducetelangiectasia
Surgerymayimproveappearanceofdisfiguringscars

Whatistheoutlookforcutaneouslupuserythematosus?
TheprognosisforcutaneousLEisvariable.
TheskininvolvementinSLEtendstomirrorsystemicinvolvement.
DruginducedSCLEclearswithinafewweeksofwithdrawaloftheresponsibledrug.
UntreatedchroniccutaneousLEtendstopersist,butseverityislessenedbystrictsunprotectionandavoidanceofnicotine.
Relatedinformation
References:
1:KuhnA,MeuthAM,BeinD,AmlerS,BeissertS,BhmM,BrehlerR,EhrchenJ,GrundmannS,HaustM,RulandV,SchillerM,SchulzP,
StnderS,SauerlandC,KpckeW,LugerTA,BonsmannG.RevisedCutaneousLupusErythematosusDiseaseAreaandSeverityIndex
(RCLASI):amodifiedoutcomeinstrumentforcutaneouslupuserythematosus.BrJDermatol.2010Jul163(1):8392.doi:10.1111/j.1365
2133.2010.09799.x.Epub2010Apr12.
MouraFilhoJP,PeixotoRL,MartinsLG,etal.Lupuserythematosus:considerationsaboutclinical,cutaneousandtherapeuticaspects.Anais
BrasileirosdeDermatologia.201489(1):118125.doi:10.1590/abd18064841.20142146.
GrnhagenCM,NybergF.Cutaneouslupuserythematosus:Anupdate.IndianDermatologyOnlineJournal.20145(1):713.
doi:10.4103/22295178.126020.
GordonSpratt,E.A.,Gorcey,L.V.,Soter,N.A.andBrauer,J.A.(2015),Phototherapy,photodynamictherapyandphotophoresisinthe
treatmentofconnectivetissuediseases:areview.BritishJournalofDermatology,173:1930.doi:10.1111/bjd.13544

OnDermNetNZ:
Discoidlupuserythematosus
Cutaneouslupusimages
Systemiclupuserythematosus
Systemiclupuserythematosusimages
Jessnerlymphocyticinfiltrate
Mixedconnectivetissuedisease
Facialrashes

Otherwebsites:
Doyouhavesystemiclupuserythematosus(lupus,SLE)andareover18yearsofage?Ifso,pleaseparticipateinAmeriderm'sSLE
symptomsurvey
LupusFoundationofAmerica
LupusSupportGroup(NewZealand)
Skincareguide.com
Lupuserythematous:variouschaptersinMedscapeReference
DiscoidLupusErythematosusBritishAssociationofDermatologists
SubacuteLupusErythematosusBritishAssociationofDermatologists
Patientinformation:Discoidlupus(TheBasics)UpToDate(forsubscribers)

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IrishLupussupportgroup
LupusUK
AustralianRheumatologyAssociation

Books:
SeetheDermNetNZbookstore

Author:AmandaOakley,Dermatologist,Hamilton,NewZealand,2001.UpdatedinJanuary2016.

DermNetNZdoesnotprovideanonlineconsultationservice.
Ifyouhaveanyconcernswithyourskinoritstreatment,seeadermatologistforadvice.
Created2001.Lastmodified15Feb2016.2016DermNetNewZealandTrust.Youmaycopyforpersonaluseonly.Seedisclaimerandcopyright
policy.

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