the abcs of oral diagnosis in the pediatric patient
TRANSCRIPT
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TheABCsofOralDIAGNOSIS
inthePediatricPatient
Juan F. Yepes DDS, MD, MPH, MS, DrPHAssociate Professor –Indiana University-
Clinical Associate Professor –University at Buffalo-Riley Hospital for Children, Indianapolis, Indiana
Diplomate ABOM, ABDPH, ABPD
The ABCs of Oral Diagnosis in the Pediatric Patient
DisclaimersI do not have any affiliation with any commercial company. My only interest is exclusively academic and for the benefit of PATIENTS, residents, RDH, dental assistants, managers, dentists, dental therapist, and physicians.
All clinical pictures were authored by myself unless otherwise listed on the slide.
Patients and/or parent consent was received for all photos and radiographs.
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Learning Objectives
1. To review the most common oral lesions in children
2. To learn how to develop a correct differential diagnosis.
3. To understand the rationale behind the current treatments.
Bestmethodtolearnoraldiagnosisinthepediatricpatient?
OralDiagnosis
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Ulcersintheoralcavityinchildren
arealltheSAME?
Yepes’s approach
AGE !!!Pre-K childKindergarten child?Teenager baby?
Ulcersintheoralcavityinchildren
arealltheSAME?
Yepes’s approach
MEDICAL HISTORY !!!Chronic GI issuesLesions in other places (skin – genitals)Periodicity (every 90 days?)Other systemic symptomsJoint pain
Not assuming the role of the PCP
Ulcersintheoralcavityinchildren
arealltheSAME ?
Yepes’s approach
Ulcer history !!!First timeOftenAlmost dailyFor a long time (more than 2 weeks)
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Ulcersintheoralcavityinchildren
arealltheSAME?
Yepes’s approach
Clinical appearance !!!Round?Irregular?Location àGum line?, NK mucosa?Keratinized mucosaCluster?
Ulcersintheoralcavityinchildren
arealltheSAME ?
NO!
RASHerpes virusHerpanginaHand foot mouth diseaseCrohn’s diseaseCyclic neutropeniaSLETrauma
Erythema multiformeMononucleosisBechet's diseaseMAGIC syndromePFAFA syndrome
Behcet’sdisease
• BDisanidiopathiccondition,chronic,relapsing,multi-systemic,characterizedbyrecurrentoralandgenitalulcers,oculardiseaseandskinlesions.
• TheprevalenceishigherincountriesaroundtheMediterraneansea
• TheprevalenceintheUSvariesbetween0.2– 5.2per100,ooo
• BDismorecommoninfemales(inNorthAmerica)
• Thediagnosisisbasedonclinicalcriteria
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Behcet’sdisease
• ThereareNOTpathognomoniclaboratorytestforBD
• Diagnosisrequirestheobservationofrecurrentoralulcerations(threeepisodeswithinany12monthperiod)plusANYtwoofthefollowing:recurrentgenitalulcers,eyelesions,skinlesionsorapositivepathergytest.
• ManagementofBDischallengingà useofanti-TNF-α,colchicinesteroids,immunomodulatorsandimmunosuppressants.
Francisco,9year-old
9yearsold,boy,Caucasian
Thisseemstohaveallstartedwithavirusthatmostofhisfamilygot.
“Hestartedwithsmallwhitebumpsonhistongue(earlyApril)verypainful.ParentstookhimtothepediatricianwhoprescribedChlorhexidine.Then,parentstookhimtothepediatricdentist.Hispediatricdentistsaidthathistonguewasinflamedandprescribedasteroidrinse(Dexamethasone).Withinthenext2days(May12)thelesion(s)weregotworse.Hisdentistthensendhimtoanoralsurgeon.Hecouldn’tgivetheparentsanyideawhatitwasandsaidsometimesthingslikethisjusthappenandifyougetridoftheopeningthenitwouldheal.ParentscalledhispediatricianagainwhoreferhimtotheENT.TheENTphysicianreferredagaintopediatricdentistry“
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9yearsold,boy,Caucasian
Neverbefore.Firsttimeofsomethinglikethis
PastMedicalHistoryPMH:UnremarkableMedications:NonePastSurgicalhistory:UnremarkableAllergies:NKDASocialhistory:Excellentfamilysupport
Reviewofsystems:Neurologic:NosymptomsGI:OccasionalGI“disturbances”(notwellexplainedbythemom)Immune:NosymptomsCardiovascular:Nosymptoms
PE: Withinnormallimits,exceptforbelowidealpercentileweightandheight
InflammatoryBowelDisease:Crohn’sDisease
• IBSisachronicrelapsingdisorderofunknownetiology(probablyimmunerelated)thatencompassestwodifferentconditions:Crohndisease(CD)andulcerativecolitis(UC)
• InCDtheinflammationoccuranywhereintheGItract(includingthemouth)
• CDcausesabdominalpain,diarrhea,weightlossandinsomecasesanorexia
• TheannualincidenceofpediatricCDintheUSisbetween0.2-8.5casesper1000,000
• Approximately10%ofpatientswithCDhaveoralmucosaulcers,andtheoralmanifestationsoccasionallyprecedeGIsymptoms
• OralulcersinCDoftenhaveinduratedbordersandarehistologicallydifferentfromRAS
Ulcersintheoralcavity?arealltheSAME (causedbyavirus!)
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RecurrentAphthousStomatitis
RecurrentAphthousStomatitis• RASisthemostcommonulcerativediseaseoftheoralmucosa.
•AGE– AGE- AGE
• Healthyindividuals.
• Involvementoftheheavilykeratinizedmucosaofthepalateandgingivaisuncommon.
• Oftencomplexdifferentialdiagnosis:neutropenia,Crohn’s,SLE,etc..
• Severalfactorshavebeenproposedasapossibleetiology.
• Extensiveresearchhasfocusedonimmunologicalfactors,butadefinitiveetiologyofRAShasnotbeenconclusiveestablished.
RAS
Major
Minor
Herpetiform
Lessthan1cmHealwithoutscars
Largerthan1cmPersistsforweeksandmonthsHealwithscar
Differentialdiagnosis
RecurrentAphthousStomatitis
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RecurrentAphthousStomatitis
Epidemiology
• Approximately20%ofthegeneralpopulationisaffectedbyRAS.
• TheepidemiologyofRASisinfluencedbythepopulationstudied,diagnosticcriteriaandenvironmentalfactors.
• Inchildren,prevalenceofRASmaybeashighas39%andisinfluencedbythepresenceofRASinoneorbothparents.
• TheonsetofRASseemstopeakbetweentheagesof10and19yearsbeforebecominglessfrequentinadvancedage,gender,orrace.
Sollecito T. Oral soft tissue lesions. Dental Clinics of North America 2005; 49: 1
RecurrentAphthousStomatitis
Etiology
Localfactors: TraumaNegativeassociationwithsmokingChangesofsalivapH
Microbialfactors HelicobacterpyloriàNostrongassociationS.sanguisàAntigenstimulant
UnderlyingMedical - Behčet’ssyndromeCondition - MAGICsyndrome:mouthandgenitalulcerswith
inflammationofthecartilage- Crohn’sdisease- Cyclicneutropenia- PFAPAsyndrome:periodicfever,RAS,pharyngitis,andcervicaladenitis
Sollecito T. Oral soft tissue lesions. Dental Clinics of North America 2005; 49: 1
RecurrentAphthousStomatitis
Etiology
HereditaryandGenetic TheroleofheredityistheFactors BESTdefinedunderlyingcause
ofRAS
ChildrenwithRAS+parentshavea90%chanceofdevelopingRAS
HLA-A2,HLA-B5,HLAB12
Sollecito T. Oral soft tissue lesions. Dental Clinics of North America 2005; 49: 1
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Recurrent Aphthous Stomatitis
Etiology
AllergicFactors HypersensitivitytofoodMicrobialheatshockproteinsSodiumSulfateà toothpaste**
Immunologicfactors - AbnormalproportionofCD4andCD8- Elevatedlevelsofinterleukin-2- ElevatedlevelsofIFNalpha- Local– dysregulatedcell-mediatedimmuneresponseà accumulationofTcells(CD8).
Nutritionalfactors Smallnumberassociationwithlowlevelsofiron,folate,zinc,VitaminsB
Recurrent Aphthous Stomatitis
Clinical Manifestations
• RASpatientsusuallyexperienceashortprodromalburningsensationthatlastfrom2to48hoursbeforeanulcerappears.
*NOGINGIVITIS
•Ulcersareround,welldefinedwitherythematousmarginsandshallowulceratedcentercoveredbyayellowpseudomembrane.
• Usuallydevelopinnon-keratinizedmucosa.
• Theylastapproximately7to10days.
• Histologicalcharacteristicsarenospecific.
RecurrentAphthousStomatitis
Treatmentà TOPICAL
• Thetreatmentdependsonthefrequency,size,andnumberofulcers.
• Patientswithoccasionalepisodesofminoraphthousulcersexperiencereliefwithtopicaltherapy
Zilactin®,Orabase®,CankerMelts®,Amlexanox®
• Patientswithmorefrequentormoreseverediseaseà Topicalsteroids(Fluocinonide0.05%)
• Topicalantibiotics:Tetracyclinemouthrinseshavebeenreportedtodecreaseboththehealingtimeandpainofthelesionsinseveraltrials.Morerecentlyà PenicillinGtroches
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RecurrentAphthousStomatitis
Treatmentà SYSTEMIC
• Shortcourseofsystemicsteroids(prednisone).
• Pentoxifylline(PTX)amethylxanthinerelatedtocaffeine,hasbeenusedformanyyearstotreatintermittentlegcrampsinpatientswithperipheralvasculardisease.PTXimprovescirculationincreasingtheflexibilityofRBC.PTXhasalsoshowntodecreaseinflammationbyitself.
• SeveralreportsoftheuseofPTX,400mgthreetimesaday.
• Othermedications:colchicine,thalidomideandDapsone.
Worthington H, et al. Interventions for preventing mucositis for patients receiving treatment. Cochrane Database Systematic Reviews 2007; 14
Thornhill MH, Baccaglini L, et al. A RCT of pentoxifylline for the treatment ofRAS. Arch Dermatol 2007; 143: 463-470
UlcersintheoralcavityarealltheSAME ?
MostCommonulcerscausedbyvirusintheoralcavityofchildren
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MostCommonViralInfectionsofTheOralCavity
RNAà CoxsackievirusgroupA
DNAà HerpesSimplexVirusHumanPapillomaVirus
MostCommonViralInfectionsofTheOralCavity
RNAàCoxsackievirusgroupA
MostCommonViralInfectionsofTheOralCavity
RNA à Coxsackievirus group A
www.bilbo.bio.purdue.edu
üHerpangina
üAcutelymphonodularpharyngitis
üHand-foot-and-mouthdisease
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Most Common Viral Infections of The Oral Cavity
RNA à Coxsackievirus group A
Pinto A. Pediatric soft tissue lesions. Dental Clinic of NA 2005; 49: 241-258
Herpangina Oral ulcerations limited to the soft palate, uvula tonsils, and fauces.
Incidence of the disease peaks during the initial months of summerand fall.
Sudden fever, sore throat, headache, dysphagia, and malaise followed in 24 to 48 hours by erythema and vesicular eruption.
Most Common Viral Infections of The Oral Cavity
RNA à Coxsackievirus group A
Pinto A. Pediatric soft tissue lesions. Dental Clinic of NA 2005; 49: 241-258
HFMD Frequently seen in epidemics outbreaks in day care or school agechildren.
Mild headache and malaise followed by skin and oral lesions.
Presence of limb lesions.
Most Common Viral Infections of The Oral Cavity
DNA àHerpes virusHuman Papilloma Virus
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Oral Herpetic Infections
• Herpes virus cause a primary infection whenthe patient initially contacts the virus and then remain latent within the nuclei of specificcells for the life of the individual.
• HSV 1, and VZV remain latent in sensorynerve ganglia.
Photo courtesy of Dr. Craig Miller
Oral Herpetic Infections
Primary herpes virus infections
• The incidence of primary infections with HSV-1 increases after 6 months.
• The incidence reaches a peak between 2 and 3 years of age.
• A significant percentage of primary herpes infections are subclinicalor cause pharyngitis difficult to distinguish from URI.
• Significant prodromal with generalized marginal gingivitis.
• Primary HSV in healthy children is usually self limiting disease.
• Treatment: palliative Antiviral ?
Oral Herpetic Infections
• After reactivation, HV can cause localizedsymptomatic or asymptomatic recurrentinfections.
•They are transmitted from host to hostby direct contact with saliva or genitalsecretions.
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Oral Herpetic Infections
Recurrent herpes simplex infections
• Following resolution of a primary HSV infection, the virus migrateto the trigeminal nerve ganglion à latent state
• Reactivation of virus may followexposure to cold, sunlight, stresstrauma, or immunosuppression
• “cold sore” or “fever blister”
Oral Herpetic Infections
Recurrent herpes simplex infections
• Several studies have been published comparing topical antiviral medications for treating RHV
•Topical penciclovir (Denavir®) reduces the duration and pain of RHV by 1 or 2 days
•Topical acyclovir has been reported to decrease duration of RHL lesionsby 12 hours and found to be more effective than N-docosanol (abreva®)
• Other topical products
• Systemic treatment
Oral Herpetic Infections
Recurrent herpes simplex infections
• Differential diagnosis:
RAS (NO prodromal symptoms and NO gingivitis)
Coxsackie viral infections (hand-foot and mouth – herpangina)
Erythema multiforme
• Laboratory testing: It may be necessary to diagnose atypical presentations.
Gold standard à tissue culture
Cytology smears àTznack smear
Immunology test à (DFA)
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• Erythemamultiforme(EM)isatypicallymild,selflimiting,andrecurringmucocutaneousreactioncharacterizedbytargetlesionsoftheskinandmucousmembranes.
• Greatvariabilitybetweenepisodes
• Typicalageisbetween7and21years.Morefemalesthanmales.
• EMischaracterizedbysymmetricallydistributedskinlesions.
ErythemaMultiforme
Leaute et al. Diagnosis, classification, an management of EM and SJS. Arch Dis Child 2000; 83: 347
Etiology
• Herpessimplexvirus(HSV)istheinfectiousagentin60%to70%ofthecases.
• HSVantigensareexpressedintheendothelialcellsofthebloodvesselsandkeratinocytesofEMlesionsà targetfortheimmuneattack.
• EM:drugsprecipitatesomecasesofEM(sulfonamides:trimethoprim-sulfamethoxazole,NSAIDs,PNC,etc.)
ErythemaMultiforme
Leaute et al. Diagnosis, classification, an management of EM and SJS. Arch Dis Child 2000; 83: 347
ClinicalPresentation
• Thelesionsareinafixedpositionwithasymmetricdistribution.
• Acentralblisterorareaofnecrosismaybepresent.
• Prodromalsymptomsarerare,andfewsystemicsymptomsarepresentduringtheEMepisode.
•Oralmucosallesionsoccurinmorethan70%ofcasesofEMà althoughlesswellrecognized,EMdoespresentasoralmucosalulcerationswithfeworNOskinlesions.
• Preferredsitesofinvolvementincludethelips,alveolarmucosa,andpalate.
•Orallesionsarepainfulandmaycompromisedspeechandeatingà healwithoutscarring
ErythemaMultiforme
Leaute et al. Diagnosis, classification, an management of EM and SJS. Arch Dis Child 2000; 83: 347
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ClinicalPresentation
•MildsymptomsassociatedwithEMaretypicallytreatedsymptomatically.
• Topicalcorticosteroidsuspensionsprovidesymptomaticreliefofpainfuloralulcers
• Systemicantiviralagents(valacyclovir500mgbidà abortiveorValacyclovir500mgbidx1yearsuppressive)
• Systemicsteroids:48to72hours
ErythemaMultiforme
Leaute et al. Diagnosis, classification, an management of EM and SJS. Arch Dis Child 2000; 83: 347
WhatwelearnedfromPatriciaFrancisco,andEdward?
1. Not all the ulcers in the oral cavity in children are the SAME !!!
2. “tips” for the differential diagnosis (not always work àuse glasses!)
3. Different treatments
Josephine….from4y.o.until12y.o.
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4years4monthsold
PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimits.Nocarieslesionswerenoted.Follow-up:6months.Excellentoralhygiene
5years2monthsold
PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimits.Nocarieslesionswerenoted.FU:6months.Excellentoralhygiene
6years2monthsold
PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitis.Nocarieslesionswerenoted.Excellentoralhygiene
LONG HISTORY…................
7yearold
PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitis.Nocarieslesionswerenoted.Excellentoralhygiene
7year8monthsold
PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitisandsome“blisters”inthelipsandgums.Nocarieslesionswerenoted.Moderateplaque.Treatment:reinforceOH
8year6monthsold
PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitis.Carieslesionswerenotedontooth#A(MO)andtooth#B(DO).Excellentoralhygiene
12yearold
PastMedicalHistory:Unremarkable Nomedications NoallergiesOralexamrevealedinflammationanderythemaonfacialgingiva(mandibleandmaxilla)withNOplaqueaccumulation.Diagnosis:PubertygingivitisTreatment:Patientreferredtoperiodonticsforassessmentandtreatment
12yearold
EmergencyAppointmentChiefComplain:“tissuesloughingfromthebackofthemouth”PE:Notedgeneralizedgingivitis.Gingivaappearstobesloughing(butgoodOH).Largeulcerationsnotedlingualto#20and#21.Theclinicalexamrevealednoprecipitatingfactorsforgingivitis
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12yearold5months
PMH:Unremarkable Nomedications NoallergiesErythemaassociatedwith“peelingoff”wasnotedoverthegingivaaroundeanteriormaxillaryandmandibularteeth.
Impression: Desquamativegingivitis
- Erosivelichenplanus(ELP)- Mucousmembranepemphigoid(MMP)- Pemphigusvulgaris(PV)
Plan:StopallOHproductsandexcisional(“punch”)biopsy
Desquamative Gingivitis ???
Des
quam
ativ
e G
ingi
vitis
(p
edia
tric
pat
ient
s)
• Clinicaltermtodescribered,painful,“peelingoff”gingiva.
• Atleastthreedifferentmucocutaneousconditionspresentasdesquamativegingivitisinchildren.
• Desquamativegingivitiscanbemistakenforplaqueinducegingivitisandthiscanleadtodelayeddiagnosisandinappropriatetreatment.
• Thereislossofstipplingandthegingivamaydesquamateeasilywithminimaltrauma
Robinson N. Desquamative gingivitis: A sing of mucocutaneous disorders.Australian Dental Journal 2003; 48: 4
Des
quam
ativ
e G
ingi
vitis
(p
edia
tric
pat
ient
s)
Robinson N. Desquamative gingivitis: A sing of mucocutaneous disorders.Australian Dental Journal 2003; 48: 4
• Gingivalerosivelichenplanus(ELP)
•MucousMembranePemphigoid(MMP)
•PemphigusVulgaris(PV)
• Crohn’sdisease,LinearIgA,plasmacellgingivitis
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Pemphigusandpemphigoidaretwoofagroupofbullousdiseasesaffectingtheoralmucosaandskin.
Theyarebothautoantibody-mediateddisease,althoughthetargetantigensarequitedifferentintypeandlocation.
Mucousmembranepemphigoid(MMP)comprisesaheterogeneousgroupofdisorderscharacterizedbysub-epithelialseparationandthedepositionofIgandcomplementalongthebasementmembrane(BMZ).
PemphigusischaracterizedbyacantholysiswithintheepitheliumowingtothebindingofIgGauto-antibodiestodesmogleins.(1)
Pemphigusaffectsbetween0.1and0.5patientsper100,000dependingonthepopulationstudied.(2)
(1) NguyenDQ.Cicatricialpemphigoid:diagnosisandtreatment.IntOphtalmol Clin1996;6:41-60(2) AhmedAR.Pemphigus:currentconcepts.AnnIntMedicine1980;92:396-405
Pemphigus Vulgaris I
Diet Garlic
Drugs sulfhydryl radicalpenillaminecaptoprilnonthiolrifampicindiclofenac
Viruses HVS
Other factors Smokingpesticides
Pemphigus Vulgaris V
Etio
logy
Pemphigus àAuto-antibodies àUnknown
Suppression Removal
Swanson DL. Pemphigus vulgaris and plasma exchange. J Am Acad Dermatology 1983; 81: 258-60
AzathioprineCyclophosphamideMethotrexateGoldCyclosporinePrednisone
Oportunistic infectionsBone marrow suppressionRenal failureSkin problemsBladder problems
Pemphigus Vulgaris VI
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WhatwelearnedfromJosephine?
The importance of medical and dental history!!!!
Theresa,10y.o.
10yearold,girl,Caucasian
A10-year-oldgirlpresentedtothepediatricdentistforaregular6monthsrecall.Theextra-oralexamwaswithinnormallimits.Theintraoralexamwaswithinnormallimits,exceptforawelllocalizederythemaatthegingivaoftooth#8.Theperiodontalexamdidnotshowincreaseinthegingivalpocketoftooth#8andnobleedingwasobserved.Fairoralhygiene.
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LocalizedJuvenileSpongioticGingivalHyperplasia
Thislesionisconsideredauniqueanddistinctiveformofinflammatorygingivalhyperplasiaseeninyoungpatients(averageage11.8years),predominantlyfemaleandgenerallyfoundinthemaxillaryanteriorregion
ThistypeoflesionwasfirstdescribedbyDarlingetal.asjuvenilespongioticgingivitis.
AftertheinvestigationofalargersamplesizebyChangetal.themoreaccuratetermLJSGHhadbeensuggested.
Itappearsasabrightredraisedovergrowthwithapapillaryorfinelygranularsurface,however,itdoesnotseemtobeaplaquerelatedlesion.
LocalizedJuvenileSpongioticGingivalHyperplasia
Thelesionpresentsasasmall(averagesizewas6mm),localizedandeasilybleedingovergrowthonthegingivaofachild.
Itisusuallygiventheclinicaldiagnosisofpyogenicgranulomaandfrequentlyseeninconjunctionwithorthodonticbrackets,whichmaybepurelycoincidentwiththepatientpopulation.
Thelesionisnotpainful,butbledeasily.
LocalizedJuvenileSpongioticGingivalHyperplasia
Thehistologicpresentationisanexophyticlesionwithasubtlepapillaryarchitecturecomposedofinterconnectingbandsofepithelialhyperplasia.
Thehistologyisuniqueandcharacterizedbyprominentintercellularedema(spongiosis)andneutrophilicexocytosis.
Thepresenceofhighlyvascularconnectivetissuecoresisseencontainingmostlyacute,butwithsomechronicinflammatorycells
LJSGHdisplaysasagingivalovergrowthratherthanapureinflammatoryprocesswithminimaltonotissueswelling.
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LocalizedJuvenileSpongioticGingivalHyperplasia
Theetiologyisunknownandthelesiondoesnotrespondtoperiodontaltreatmentshowingalackofassociationwithplaqueorcalculus.
Darlingetal.comparedjuvenilespongioticgingivitis/LJSGHwithpubertygingivitisandfoundseveraldistinguishingfeaturesincludingalackofimmunoreactivityforestrogenandprogesteronereceptorsinLJSGH
TreatmentforLJSGHisconservativesurgicalexcisionandthecarbondioxidelaserisidealfortreatingthislesion.Theyoungageofthepatientmakeslaserablationapreferredandveryefficientprocedure,welltoleratedbythispopulationofpatients.Recurrenceisrareandwhenitdoesoccur,maybeduetoincompleteremovalofthelesion.
WhatwelearnedfromTheresa?
Alex..3 year-old
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3 yearold,girl,Caucasian
Patientisa3yearoldhealthygirlwhopresentedtothepediatricdentalofficewith“whitestuff”inhermouthforthelastweek.Nocomplainofpain.
PMH:Noncontributory Allergies:NKDA
Medications:None
Reviewofsystems:Essentiallynegativeexceptforarecentepisodeofotitismedia
Basedontheclinicalpicturesandhistory,whichofthefollowingdiagnosisatthispointyouwillplacehigherinthedifferential?
1. Candidiasis
2. Candidiasis
3. Candidiasis
4. Other(IdiopathiccandidiasisL)
Case 4
RBC: 4.5 mm³
Platelet: 250,000
Hg: 14 gr/dlWBC: 6,300
Lymphocytes: 10%
Patient laboratory results
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Clinicalfeatureswhichmayindicateimmunodeficiency
• Threeormoreepisodesofotitismediain6months• Persistentpurulenteardischarge• Twoormoreserioussinusinfectionwithinoneyear• Twoormoreepisodesofpneumoniawithinoneyear• Failuretothrive• Recurrentdeepskininfections• Persistentcandidiasis• Familyhistory
Primary Immunodeficiency
Both
B-cell
T-cell
Primary Immunodeficiency Disorders
•Bcell(antibody)deficiencies
•Tcelldeficiencies
•CombinationBandTcelldeficiencies
•Defectivephagocytes
•Complementdeficiencies
•Unknown(idiopathic)
Primary Immunodeficiency
Both
BcellTcell
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Immunity to infection
The areas of the immune system to consider are:
• Humoral immunity (B cells and Immunoglobulins production
• Cell-mediated immunity (T cells), neutrophils
• Complement cascade
T cell disorders
B cell defects
Phagocyte disorders
Complement disorders
Selective IgADeficiency
stem cell
Myeloid progenitor cell
Neutrophil Monocyte
Lymphoid progenitorcell
B-cell
Ig-A
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Selective IgA deficiency
• SelectiveIgAdeficiencyisoneofthemostcommontypesofprimaryimmunodeficiency
• Manypatientsgoundiagnosedbecausetheyareneversickenoughtobeseenbyadoctor
• PatientwithselectiveIgAdeficiencydoproducealltheotherIg
• Thecauseisunknown
• ChildrenwithselectiveIgAdeficiencyareatriskofinfection.abouthalfhaverepeatedinfectionsoftheears,sinusandairway
• ChildrenwithIgAdeficiencyareatincreasedriskforanaphylacticreactions
WhatwelearnedfromAlex?
Sophia
Hillary
Bobby
Kawne
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Review of Patient I• Age:13y.o,girl.
• ChiefComplaint:“Thereissomethinginmygums”
• HPI:Asymptomaticlesionbetween10and11.Unknownduration.
pedunculated.
• PastMedicalHistory(PMH):
- Hypothyroidism
- Anemia
- Visionproblems
• PastDentalHistory(PDH):Lastvisittothedentist:18monthsago
June 9 2011
Themicroscopicsectionsrevealapapillarynoduleofmucosathatissurfacebyparakeratoticstratifiedsquamousepitheliumwhichformsseveralshortprojectionsofmucosawithconnectivetissuecores.
Theunderlinelaminapropiaconsistsoffibrousconnectivetissuewithscatteredsmallvascularchannelsandneuralbundles.
Diagnosis:Papilloma
Review of Patient III• Age:4y.o,girl.
• ChiefComplaint:“Thereissomethinginthebackofmymouth”
• HPI:Asymptomaticlesionatthejunctionbetweenhardandsoftpalateattherightside.Unknownduration.Wellpedunculated.
• PastMedicalHistory(PMH):
Unremarkable
• PastDentalHistory(PDH):Lastvisittothedentist:6monthsago
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Review of Patient IV• Age:17y.o,boy.
• ChiefComplaint:“Thereissomethinginthefrontofmymouth”
• HPI:Asymptomaticlesionbetweenmaxillarycentralincisors.Unknownduration.Wellpedunculated.
• PastMedicalHistory(PMH):
Asthmawellcontrolled.UsingsteroidsPRN
• PastDentalHistory(PDH):Lastvisittothedentist:6monthsago
July 17 2011
HPV
üSquamousPapilloma
üVerrucaVulgaris(commonwart)
üCondylomaAcuminatum
üMultifocalEpithelialHyperplasia(HeckDisease)
Oral and Maxillofacial Pathology, Neville, Dam et al , 2009
Squamouspapilloma
-Benignproliferationofstratifiedsquamousepithelium-ThelesionisinducedbyHPV-Exactmodeoftransmissionisunknown-Equalfrequencyinboysandgirls-Morecommonplaces:tongue,lipsandsoftpalate.-Thepapillomaisusuallysolitary-Histopathology:proliferationofkeratinizedstratifiedsquamousepitheliumin“fingerlikeprojections”withfibro-vascularconnectivetissuecores.
Oral and Maxillofacial Pathology, Neville, Dam et al , 2009
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CondylomaAcuminatum
CondylomaacuminatumisaSTDappearingmostfrequentlyasasoft,pinkcauliflowerlikegrowth.
Theconditionishighlycontagious
Bothgendersareaffectedequally
Thepeakincidenceisbetween17to20
Thehistologyshowsà papillarylesions
MultifocalEpithelialHyperplasia(Heck’sDisease)
-Virusinduced,localizedproliferationoforalsquamousepitheliumthatwasfirstdescribedinNativeAmericans-AssociatedwithHPV13and32-Insomepopulations,atmanyas39%ofchildrenareaffected.-Theconditionusuallyaffectsmultiplemembersofthesamefamily-Multiple,flattened,soft,nontenderpapulas,usuallyclusterandwiththesamecoloroftheoralmucosa.
O ral and M axillo facial P ath o lo gy, N eville, D am et al , 2009
Humanpapillomavirusinfectionoftheoralmucosa
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Humanpapillomavirusinfectionsoftheoralmucosa
Classifica
tion • Papillomavirusesaresmall,doublestrandedDNAviruses.
• HumancanbeinfectedonlybyHPV’s,notbypapillomavirusesfoundinanimals.
• TheHPVgenomecontainseightopenreadingframes(ORFs)whicharepotentiallycodingsitesofsixearlyproteins(E)andtwolateproteins(L).TheL1ORFisusedtoidentifythedifferenttypesofHPVbecauseitisthemostconservedoftheeightORFswithinthegenome.(1)
1. Rautava J, Syrjanen S. Human papillomaviruses infections in the oral mucosa. JADA 2011; 142(8):905-914
Humanpapillomavirusinfectionsoftheoralmucosa
Classifica
tion • Investigatorshavedescribedmorethan120differentHPVtypesonthebasis
oftheisolationandsequencingofcompletegenomes.(1)
•MostHPVthatinfectoralmucosasitebelongtothealphapapillomaviruses,whichconsistof15species.(2)
• Todate,investigatorshaveidentifies30HPVgenotypes:15high-risktypes,3typesthatprobablyarehighriskand12low-risktypes.
1.RautavaJ,SyrjanenS.Humanpapillomavirusesinfectionsintheoralmucosa.JADA2011;142(8):905-914
Humanpapillomavirusinfectionsoftheoralmucosa
Classifica
tion
RautavaJ,SyrjanenS.Humanpapillomavirusesinfectionsintheoralmucosa.JADA2011;142(8):905-914
HPV-16
LCRà Regulationandvirusgeneexpressionandvirusreplication
HPV-16
Majorcapsideprotein
Minorcapsideprotein
Membranesignalingprotein
E1
E2
E3E4
E5
E6E7
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MariaFernanda14year-old
(thisismyonlychangetogetinvitedagain)
IdiopathicBoneSclerosisvs.
Cemento-osseousdysplasia
PrinciplesofRadiographicInterpretation
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PrinciplesofRadiographicInterpretation
“Thereisaradiopacity,nottoobig,nottoosmall,looksactuallyfunny…..locatedattheapexoftooth#28”.
Condensingosteitis
PrinciplesofRadiographicInterpretation
DifferentialInterpretation
RadiographicDescription
Somedefinitions
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SummaryofInterpretationSteps
1. Radiopaque/Radiolucent/mix2. Welldefined/illdefined/mix3. Corticated/non-corticated/mix4. Location,sizeandshape5. Whathappeninthe“neighborhood”6. Differentialinterpretation
let’spracticewithsomefilms
SummaryofInterpretationSteps
1. Radiopaque/Radiolucent/mix(mymom..)2. Welldefined/illdefined/mix(mymom)3. Corticated/non-corticated/mix(mymom)4. Location,sizeandshape5. Whathappeninthe“neighborhood”6. Differentialinterpretation
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Mostcommonjawlesionsin
children
4 Mostcommonjawlesionsin
children
BoneDiso
rders 1. Idiopathic Bone Sclerosis
2. Fibrous Dysplasia
3. Simple Bone Cyst
4. Cemento-osseous dysplasia
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IdiopathicBoneSclerosis
(notcondensingosteitis!)
IdiopathicBoneSclerosis
IBSisafocalsolitaryscleroticlesionthatarisesinthelate1storearly2nddecadeoflife.
Itscauseisunknown.
Itisasymptomatic,isnotassociatedwithinflammation,andmayremainstaticordemonstrateslowgrowththatusuallystopswhenthepatientreachesskeletalmaturity.
IdiopathicBoneSclerosis
In90%ofpatientsitoccursinthemandible,usuallynearthefirstmolarorsecondmolarorpremolar.
Atimaging,IBSisradiopaque,welldefined,welllocalized,non-corticated,locatedattheapexofvitalteeth.Norootresorptionandnoteethdisplacement.
Somepatientsmayhavemultiplelesions.
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SimpleBoneCyst
-Traumaticbonecyst,hemorrhagicbonecyst,solitarybonecyst
-Simplebonecyst:Itisacavitywithinbonethatislinedwithconnectivetissue.Itmaybeemptyoritmaycontainfluid
-Itisnotatruecyst
-Etiologyunknown,howeverà Localizedaberrationinnormalboneremodelingormetabolism
-Firsttwodecadesoflife(mean17years)
-Females2:1
-Expansionispossiblebutunusual,discoverybychance
SimpleBoneCyst
àMandible
àPosteriormandible
àAssociatedwithcemento-osseousdysplasiaandfibrousdysplasia
àMargin:Welldefinedtoill-definedborder
àInternalstructure:Radiolucent,itmay appearmultilocular,althoughthelesiondoesnotcontaintruesepta
àNoeffectonthesurroundingteeth
àLaminaduraintactandvitalitypositive
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FibrousDysplasia
LocalizedchangeinnormalbonemetabolismàReplacementofallcomponentsofnormalbonebyfibroustissuecontainingvaryingamountsofabnormalappearingbone
Solitaryormultiple(monostotic70%ofallcases)
McCune-Albrightsyndrome
Asymptomatic,rareassociatedwithpain
Thelesionmaybecomeactiveà Pregnant,etc..
Theskullisinvolvedin10to25%ofcases
Patientsbetween10and30yearsold
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FocalCemento-OsseousDysplasia
Focalcemento-osseousdysplasia-LocalizedchangeinnormalbonemetabolismàReplacementofthecomponentsofnormalbonewithfibroustissueandcementum-likematerial,abnormalboneoramixtureofthetwo
-Neartheapexofatooth
-Middleage,females9:1,Afro-American
-Incidentalfinding
-Theinvolvedteetharevital
-Sometimesquitelarger
Source: Oral Radiology, Principles and Interpretation. White et al ,2000
Focalcemento-osseousdysplasia
-Epicenterusuallyattheapexofatooth
-Welldefined,Irregularlyshaped
-Internalstructure:Variesà Radiolucentarea
Mixedstage:RadiopaqueandRadiolucentareas
Maturestage:Radiopaque
-Laminaduraoftheteethinvolvedwiththelesionislost
-Rare:resorptionoftheroots
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let’scomebacktoMariaFernanda…..
Accordingtothepatient'smother“shehadherwisdomteethout2monthsagoandshehadaknotinthearearightaftertheextractionbuteventuallywentaway”.Then2monthslaterwithswellingandpain overtheleftmandible.
Momconsultedwiththeoralsurgeon.Noconcernsovertheareaofextractionoftooth#17
Painpartiallycontrolledwithibuprofenandantibiotics(?)
Accordingtothepatient,“mywholejawhurts,thosethreeteethareaching(18-19-20)”
Initiallyreferredtotheendodontistforrootcanaloftooth#20
Mydifferentialdiagnosisà Neuropathicpain ?
Differentialdiagnosisà Neuropathicpain?
Neuropathicpainischronicpainconditioncausedbyanalterationintheperipheralorcentralnervoussystem(trigeminalneuralgia,atypicalodontalgia,burningmouthsyndrome,traumaticneuropathies,post-herpeticneuralgia,andcomplexregionalpainsyndrome
OftenneuropathicpainismisdiagnosedwhichcanleadtounnecessaryTREATMENT
Vascularcompression,radiation,inflammation,trauma,infection,etc.canleadtoneuropathicpain
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Differentialdiagnosisà Neuropathicpain?
Pain:short– electrical– sharp– shooting– paresthesia– swelling
Extractionofthirdmandibularmolars,dentalinjections,implanttreatments,andendodontictreatmentsarethemostcommonproceduresindentistryassociatedwithneuropathicpain
Treatment:Amitriptyline,pregabalin,gabapentin
Suggestedtoseeaneurologist
WhatwelearnedfromMariaFernanda?
1. Radiographic interpretation (probably the MOST important goal)
2. Common bone lesions in children (just remember one: IBS)
3. Neuropathic pain in children (you can delete that from your brain immediately)
4. Use of CBCT in pediatric dentistry (who cares?)
Special thank you to Dr. Jennifer Garvey, DABPD, pediatric dentist in Greenville, SC
Mary,4year-old
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4.5year-old,girl,Hispanic
6 months ago, parents noticed intraoral swelling of thegingiva (not a lot of pain)...Dad said it was mostly aroundthe back teeth. He took them to their general dentist.
PMH: Unremarkable
Allergies: NKDA
OrofacialGranulomatosisI
Orofacialgranulomatosishasbecomeawell-acceptedandunifyingtermencompassingavarietyofclinicalpresentations(biopsy=non-specificgranulomas)
Orofacialgranulomatosis~Aphthousstomatitis(idiopathicbutappearstobeanabnormalimmunereaction)
SystemicdiseasethatmimicOG:Crohn’sdisease,sarcoidosis,tuberculosis
Severaltriggersareinvolved
Themajorityofpatientsareadults.However,whenisinchildrenà strongassociationwithasymptomaticinflammatorygastrointestinalprocess (differentfromCrohn’sdisease)anassociatedwithdietarytriggers
Clinicalpresentationishighlyvariable.Mostcommonsite:LIPS
Whenthesignsarecombinedwithfacialparalysisandfissuretongue:___________________________
Intraoral:Tongueandgingiva(swellinganderythema)
Melkersson-Rosenthalsyndrome
Orofacial Granulomatosis II
ThediagnosisofOGà histopathology:presenceofgranulomasassociatedwithnegativestainsfororganismsandnoforeignmaterial
Treatment:
• Firstgoalà identifythepossiblecause(noteasyatall)
• Inchildrenà StrongconsiderationtodietaryallergensoranassociationwithunderlyingGIdisease
• Topicaluseofsteroids(similartoRAS),TNF-α antagonist(infliximab),intra-lesionalinjectionsofsteroids
Prognosisà highlyvariable
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WhatwelearnedfromMary?
1. The importance of a detailed medical history (Most important goal)
2. Orofacial Granulomatosis (well…....not to common)
SoftTissueLesionsinChildren
andAdolescents(besideswealreadyreviewed)
IdiopathicGingivalFibromatosis
Idiopathic gingival fibromatosis (IGF) is an enlargement localized orgeneralized of the gingival tissue characterized by an expansion andaccumulation of the connective tissue, mainly collagen type I.
Gingival fibromatosis is commonly induced as a side effect ofsystemic drugs (such as phenytoin, nifedipine, and cyclosporine).
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IdiopathicGingivalFibromatosis
However, in some cases the gingival overgrowth is idiopathic. Theenlargement is more prominent during the eruption of the primary andpermanent teeth. Poor oral hygiene has been also associated with thecondition.
The diagnosis is established through history, clinical examination andhistopathology.
Surgical treatment including gingivectomy and gingivoplasty are usually thetreatment.
DermoidCyst
Dermoid cyst are malformations that are common in the oralcavity.
Dermoid cyst are development lesions found inside organs ortissues as a result of the inclusion of tissue from diverse sources(ectoderm, mesoderm or endoderm)
Dermoid cyst of the oral cavity are often relatively softunfluctuating masses, frequently adhered to the child’s hyoidbone.
DermoidCyst
The differential diagnosis includes lipoma, ranula, thyroglossal duct cyst,cystic hygroma, andmalignant tumors.
Boys aremore affected than girls by a ratio of 3:1.
Almost one in five of the dermoid cyst that occur in the head and neck areaare located in the floor of the mouth and often they cause tongueelevation.
Usually they are diagnosed at young ages. The treatment is completesurgical excision and they have a good prognosis.
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Mucocele• Commonlesionoftheoralmucosathatresultsfromruptureofsalivaryglandduct
andspillageofmucinintothesurroundingsofttissues
• Themostcommonreason:TRAUMA
• Itisnotatruecyst(noepitheliallining)
• Typicallytheyaredomeshapedswellingthatcanrangefrom1to2mm
• Mostcommonlesioninchildren
• Oftentranslucent
• Fluctuantatpalpation.Fromafewdaystofewyears:Historyofrecurrentswelling
Mucocele
• ThelowerlipisbyfartheMOSTcommonsite
• Somemucocelesareshort-livedlesionsthatruptureandhealbythemselves
• Somemucocelesarechronicinnatureandsurgicalexcisionisnecessary
• Excellentprognosis
PyogenicGranuloma
Itisacommontumorlikegrowthoftheoralcavitythattraditionallyhasbeenconsideredtobenon-neoplastic.
Unrelatedwithinfectionandgranulomas!
Itisanexuberanttissueresponsetolocalirritationortrauma.
Itisasmooth,lobulatedmass,usuallypedunculated.
Microscopicevaluationshowsahighlyvascularproliferation.
Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology 3 edition
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Pyogenic Granuloma
Etiology:
-connective tissue reaction to injury or other stimulus
-Hormonal changes/puberty
-Composed of hyperplastic granulation tissue
Treatment:
-surgical excision
-Frequently recurs
Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology 3 edition
Pyogenic Granuloma
EruptionCyst
Theeruptioncyst(orinsometextbooks,eruptionhematoma)developsfromseparationofthedentalfolliclefromaroundthecrownofatoothwhoiserupting.
Theeruptioncystisasoft,swellinginthegingivaoverlyingthecrownofaneruptingprimaryofpermanenttooth.Themajorityofcasesoferuptioncystsareseeninchildrenundertheageof10.
Thelesionismostcommonlyassociatedwiththecentralpermanentincisorsorcentralprimarymolars.
Treatmentisusuallynotrequiredbecausetheeruptioncystrupturesspontaneously.
Leiomyomas
Leiomyomas are benign tumors that originate from smoothmuscle.
The most common place that leiomyomas are found is the uterinemyometrium. However, leiomyomas are also found in the gastrointestinal tract,skin and lower extremities.
Leiomyomas are rare in the oral cavity, the most common place is the lipsfollowed by tongue, cheeks, palate and gingiva.
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LeiomyomasUsually the lesion is asymptomatic, slow-growing. In children is rare.Histopathology has a key role in establishing the diagnosis.
The differential diagnosis when is located in the oral cavity is Mucocele andfibromas. Leiomyomas in the oral cavity are characterized by a solitary, usuallyovoid andmobile mass covered by normal appearing epithelium.
The consistency is usually firm with a well-defined margins. The treatment ofleiomyomas in the oral cavity is the complete resection of the lesion.
GiantCellFibromaThegiantcellfibromaisafibroustumorthatisprobablyunrelatedwithchronictrauma(differencewiththetraumaticfibroma).
Typicallythegiantcellfibromaisasymptomaticandrepresents2%to5%ofalloralfibrousproliferationssubmittedforbiopsy.
Acommondifferentialdiagnosisispapilloma.Comparedwiththeirritationfibroma,thelesionoccursatayoungerage.
Thereisaslightlyfemalepredilection.Themandibulargingivaisaffectedtwiceasoftenasthemaxillarygingiva.Thepalateisalsoacommonplace.Fromthehistopathologyperspective,thehallmarkisthepresenceoffibroblastwithinthesuperficialconnectivetissue.Thetreatmentisconservativesurgicalexcision.
PeripheralGiantCellGranuloma
-Foundonlyingingiva
-Usuallydistaltoincisors
-Maycauseboneresorption
-Appearasredorbluebroad-basedmasses
-Morefrequentinfemales
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Peripheral Giant Cell Granuloma
Etiology:
-Hyperplastic connective tissue response to gingival tissue injury
-Histologically see multinucleated giant cells
-Similar in appearance to pyogenic granuloma
Treatment:
-Surgical excision
-Recurrence is uncommon
ThankyousoMUCH!!!!!!!!!
JuanF.YepesDDS,MD,MPH,[email protected]