CartasalEditor/MedClin(Barc).2019;152(1):37–40 39 4.Bogdanovi ´cR,Cobelji ´cM,Markovi ´cM,Nikoli ´cV,Ognjanovi ´cM,Sarjanovi ´cL,etal.
Haemolytic-uraemicsyndromeassociatedwithAeromonashydrophila enteroco-litis.PediatrNephrol.1991;5:293–5.
5.GómezCampderaJ,Mu ˜nozP,LópezPrietoF,RodríguezFernándezR,RoblesM, RodríguezCreixemsM,etal.GastroenteritisporAeromonasenpediatría.AnEsp Pediatr.1996;44:548–52.
AnaCastellano-Martineza,∗,MoisesRodriguez-Gonzalezb yVirginiaRoldan-Canoa
aSeccióndeNefrologíaPediátrica,HospitalUniversitarioPuertadel
Mar,Cádiz,Espa˜na
bSeccióndeCardiologíaPediátrica,HospitalUniversitarioPuertadel
Mar,Cádiz,Espa˜na
∗Autorparacorrespondencia.
Correoelectrónico:anacastellanomart@gmail.com
(A.Castellano-Martinez).
https://doi.org/10.1016/j.medcli.2018.04.008 0025-7753/
©2018ElsevierEspa ˜na,S.L.U.Todoslosderechosreservados.
Papillon-LefevreSyndrome:Acasereport
SíndromedePapillon-Lefevre:descripcióndeuncaso
DearEditor:
Papillon-LefevreSyndrome(PLS)isarareautosomalrecessive disordercharacterizedbyrapidlyprogressiveperiodontaltissue destruction,earlylossofprimaryandpermanentteethand wides-preadhyperkeratoticareasofpalmsandsoles.1–3CathepsinCgene mutationisresponsibleforitsetiology.4Palmoplantar hyperkera-tosistypicallystartsbetween1and4yearsofage.5
A6-year-oldboywithaPLSdiagnosiswasreferredtoour cli-nicfromthedermatologydepartment.Hedidnothaveanyother systemicdisease,andhisparentshadnoconsanguineousmarriage. Thepatientunderwentextraoral,intraoralandradiographic exa-mination.
Extraoralexaminationshoweddrynessandhyperkeratosisin bilateralpalms,kneesandfootdorsum(Fig.1a–c).Hairandnails hadnormal development.Our patienthadnormal development accordingtoage;therewasnophysicalandmentalretardation.
Intraoralexaminationshoweddeepdentincariesintheupper primaryincisors,molars(55,52,51,61,62,64and65),lower pri-marymolars(74,75,84and85)androotofprimarytoothnumber 54.Itwasalsonotedthatthepatient’soralhygienewasnotgood (Fig.1d–e).
Fig.1.Hyperkeratosisofpalms(a).Hyperkeratosisofknee(b).Hyperkeratosisoffootdorsum(c).Intraoralview—maxilla(d).Intraoralview—mandibular(e).Panoramic radiograph(f).
Panoramicand periapicalradiographsfromourpatient sup-ported our intraoralfindings. Theydetected destructionin the alveolar bone at the root apical of teeth numbers 51, 75 and 84andfractureoftoothnumber75(Fig.1f).
Examinationofthebiopsyspecimentakenfromthepatient’s knee revealed microscopic findings compatible with PLS. In addition,normalbraincomputerizedtomographyfindings were observedintheresultofbraincomputerizedtomographytaken fromthepatient.CathepsinCgeneanalysiswasnotrequestedfrom thepatientagainbecausethepatientwasdiagnosedfromthe der-matologyclinic.
Basedonpatient’shistory,clinical,radiographicandbiopsy fin-dings,aPLSdiagnosiswassupportedandtreatmentplanningwas performed.Itwasdecidedtoextractprimaryteethnumbers51,64, 75and84andtherootofprimarytoothnumber54;tofillprimary teethnumbers61,62and74;andtoapplyrootcanaltreatmentto primaryteethnumbers55,52,65and85.Oralhygienetrainingof thepatientandfollow-upofthepatientwasrequested.
PLSwasoriginallydescribedin1924bytwoFrenchphysicians, PapillonandLefèvre.PLSis inheritedasanautosomalrecessive traitandhasareportedprevalenceof1–4casespermillion.PLS usuallyappearsinchildhood.Malesandfemalesareequally affec-ted, and patientsarenormal at birth.The disorderis characte-rizedbydiffusepalmoplantarkeratodermaandrapidlyprogressing periodontitisleadingtoprematurelossofbothdeciduousand per-manentteeth.Skinlesionsdevelopconcurrentlywithorallesions
40 CartasalEditor/MedClin(Barc).2019;152(1):37–40 andmayextendtodorsalsurfacesofthehandsand feet.
Anot-herformofdiseaseassociated withpalmoplantar keratosisand severeaggressiveperiodontitisisHaim-MunkSyndrome.Itdiffers fromPLSwithsymptomssuchasarachnodactyly,acroosteolysis andonychogryphosis.1
Thecauseofthedevelopmentoftheselesionsisattributedto threemainfactors,namelygenetic,immunologicand microbiolo-gic.Thegeneticfactorresponsibleforcausingthisdiseaseisdue tomutationsofthecathepsinCgene(CTSC)intheregionof chro-mosome11q14–21.TheimmunologicalfactorresponsibleforPLS mainlydepends onthe deterioration of neutrophilchemotaxis, phagocytosis, bactericidalcapabilities, decreased cell migration, lymphocyticresponseandmonocyticactivity.Impairmentin natu-ralkillercells’cytotoxicityhasbeenimplicatedinthedevelopment ofPLS.Apossiblebacterialetiologyhasalsobeenproposed.Itis believedthatActinobacillus actinomycetemcomitans, Porphyromo-nasgingivalis,FusobacteriumnucleatumandPrevotellaintermedia maybeamongsttheorganismsinvolvednotonlyinperiodontal breakdownbutalsointhecutaneouslesionsofPLS.2
InPLS,thereissevereperiodontaldestructionand earlyloss ofprimaryandpermanentteeth.Thepermanentdentitionstarts toeruptatthepropertime,butperiodontaldestructionbeginsto occurataround8–9yearsofage.Allpermanentteethareusually lostbefore14–16yearsofage.
ThemanagementofcaseswithPLSrequiresamultidisciplinary approachwiththeactiveparticipationofthedentalsurgeon, der-matologistandpediatrician.Treatmentofthedentalcomponentof thedisorderisaimedateliminatingthereservoirofcausative
orga-nisms.Oralretinoidssuchasacitretinandisotretinoinhaveproven tobebeneficialintreatingboththedentalandcutaneouslesions ofPLS.
Bibliografía
1.RathodVJ,JoshiNV.Papillon-Lefevresyndrome:areportoftwocases.JIndian SocPeriodontol.2010;14:275–8.
2.JijinMJ,JaishankarHP,NarayaranVS,RangaswamyK,PuthaswamyKA. Papillon-LefevreSyndrome inanadolescent female:acasestudy.J ClinDiagnRes. 2015;9:ZD23–5.
3.BaniM,AkalN.Papillon-LefevreSyndrome:twocasereports.ActaOdontologica Tursica.2009;26:117–23.
4.AhmadM,HassanI,MasoodQ.Papillon-LefevreSyndrome.JDermatolCaseRep. 2009;3:53–5.
5.DhanrajaniPJ. Papillon-Lefevre syndrome:clinicalpresentation and a brief review.OralSurgOralMedOralPatholOralRadiolEndod.2009;108:e1–7. GözdeDerinda˘g∗,NebihaHilalBilge,HayatiMuratAkgül, OsmanMuratBilge
DepartmentofOralandMaxillofacialRadiology,FacultyofDentistry, AtaturkUniversity,Turkey
∗Correspondingauthor.
E-mailaddress:gozde.derindag@atauni.edu.tr(G.Derinda˘g). https://doi.org/10.1016/j.medcli.2018.04.009
0025-7753/
