BrazJOtorhinolaryngol.2014;80(4):366---367
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
www.bjorl.org
CASE
REPORT
Oral
Langerhans
cell
histiocytosis:
case
report
with
follow-up
of
ten
years
夽
Histiocitose
de
células
de
Langerhans
oral:
relato
de
caso
com
acompanhamento
de
dez
anos
Emeline
das
Neves
de
Araújo
Lima,
Eliakim
Medeiros
Alves
de
Araújo,
Patrícia
Teixeira
de
Oliveira,
Ana
Miryam
Costa
de
Medeiros
∗UniversidadeFederaldoRioGrandedoNorte(UFRN),Natal,RN,Brazil
Received25September2012;accepted14March2013 Availableonline23May2014
Introduction
Langerhanscellhistiocytosis(LCH)ischaracterizedbyclonal proliferationofLangerhanscellsexhibitingBirbeckgranules andpositiveimmunohistochemistry forS100andCD1A.1A
malignant transformation or a functional proliferation of
Langerhanscellsrespondingtoexternalstimuliarepossible
sources.2Intheoralcavity,theycanoccasionallypresentas
hyperplasiaof the gingiva or ulcersof the cheek, palate,
or tongue mucosa.3 The diagnosis is made after careful
examination,andtheexclusionofother similardiagnostic
possibilities.
Several therapeutic modalities have been suggested
for LCH, such as intralesional corticosteroid injection,
antibiotics,steroids,radiationtherapy,andchemotherapy.
Surgicaloptionsrangingfromextensiveresectionstomore
conservativeapproachesareavailableand,inmanycases,
healinghasresultedfromasinglebiopsy.4
The present report regardsLCH in the oral cavity and
emphasizestherarityof thislesion,aswellasthe
impor-tanceofdifferentialdiagnosis,treatment,andappropriate
follow-upforthesepatients.
夽 Pleasecitethisarticleas:LimaEN,deAraújoEM,deOliveiraPT, deMedeirosAM.OralLangerhanscellhistiocytosis:casereportwith follow-upoftenyears.BrazJOtorhinolaryngol.2014;80:366---7.
∗Correspondingauthor.
E-mail:emelinelima@hotmail.com(A.M.C.deMedeiros).
Case
presentation
A ten-year-old male with leukoderma presented to the
Stomatology Clinic,withathree-monthhistoryof alesion
on the roof of the mouth. On intraoral examination, a
red ulcerated lesion of approximately 1cm size with an
orthodontic ring(Fig.1A) wasseen onthepalatal gingiva
adjacent to the first upper molar. Following periodontal
treatment, no improvement was observed, and an initial
diagnosis of paracoccidioidomycosis was suggested.
Inci-sional biopsy revealed a lesion predominantly consisting
of polygonalcells, at times exhibitinggranular cytoplasm
(Fig. 1B), consistent withLCH and confirmed by strongly
positiveimmunohistochemistryforS100(Fig.1C)andCD1A
(Fig.1D).Theconditionwastreatedbychemotherapyand
surgicalremovalofthelesion.Overthenexttenyears,the
patientwasassessedtwice ayear,and showednoclinical
signsofrelapse.Onhislastvisit,heunderwentabonescan
andtemporalbonecomputedtomographyandtherewasno
evidenceofrelapseormetastases.
Discussion
TheetiopathogenesisofLCHhasnotbeenfullydetermined,
andapossiblereactionorneoplasticphenomenahasbeen
proposed.Afewauthorsfurthersuggestanimmunesystem
regulation disorder anda familialpredisposition,5 sinceit
frequentlyaffectschildren,asinthepresentreport.
http://dx.doi.org/10.1016/j.bjorl.2014.05.003
OralLangerhanscellhistiocytosis 367
Figure1 (A)Intraoralclinicalimagingshowingredulceratedlesioninthepalatalgingiva,adjacenttothefirstuppermolar.(B) Microphotographshowingneoplasticlesioncharacterizedbypolygonalcells,attimesexhibitinggranularcytoplasmamongnumerous bloodvessels,andinflammatoryinfiltrateconsistingoflymphocytes,neutrophils,andeosinophils(HE×400).(C)
Immunohistochem-icalfindingspositiveforS100(×200).(D)ImmunohistochemicalfindingspositiveforCD1A(×400).
The clinical featuresofLCHaresimilartoseveral
con-ditions,includingperiodontaldisease,malignanciessuchas
squamouscellcarcinomaorlymphoma,aswellas
granulo-matousorulcerativelesionsthatarecharacteristicoffungal
infections.2Thus,athoroughassessmentisappropriatefor
oralcavitylesionsthatpersistaftertreatment.Intheoral
cavity,itusuallypresentsasamucosalulcerassociatedwith
underlying bone lesions,6 which was not observed in this
case.
Diagnosis may be confirmed by detecting the
charac-teristicBirbeck granules(X bodies)or specificmonoclonal
antibodiestosurfaceantigens(CD1).6Inthepresentcase,
diagnosis was confirmed after an immunohistochemical
studythatcharacterizedtheproliferatingcelltype.
Thetherapeuticapproachdependsontheextentofthe
disease and local treatment is usually effective in forms
limited to a single organ. However, a few complications,
suchaspituitaryglandmalfunction,andespeciallydiabetes
andneurodegenerativediseasescanoccur.5Aretrospective
reviewofpatientswithLCHrevealedahighten-yearsurvival
rate(93%);mostindividualsarelow-riskandhavenobone
marrow, spleen, liver, or lung involvement.1 Our patient
has hadno relapseor metastasis aftera ten-year
follow-up;however,long-termfollow-upisrequiredtodetectand
controlpossiblelate-onsetsequelae.3
Final
comments
Thepresent reportdiscussedclinical andlaboratory
infor-mationonLCH,emphasizingtheimportanceofdifferential
diagnosis,appropriatetreatment,andlong-termfollow-up
withcomplicationand/orrelapseprevention.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
References
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2.Madrigal-Martínez-Pereda C, Guerrero-Rodríguez V, Guisado-MoyaB,Meniz-GarcíaC.Langerhanscellhistiocytosis:literature reviewanddescriptiveanalysisoforalmanifestations.MedOral PatolOralCirBucal.2009;14:222---8.
3.KilicE,ErN,MaviliE,AlkanA,GunhanO.Oralmucosal involve-mentinLangerhans’cellhistiocytosis:long-termfollow-upofa rarecase.AustDentJ.2011;56:433---6.
4.LeeS-H,YoonH-J.Intralesionalinfiltrationofcorticosteroidsin thetreatmentoflocalizedLangerhanscellhistiocytosis ofthe mandible:reportoftwocases.OralSurgOralMedOralPathol OralRadiol.2012[inpress].
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