J Clin Periodontol 2000; 27: 951–954 Copyright C Munksgaard 2000Printed in Denmark . All rights reserved

ISSN 0303-6979

Case ReportChrister Ullbro 1,Claes-Göran Crossner 1,Osseointegrated implants in aTord Lundgren 1, Per-Åke Stålblad 1

and Stefan Renvert 2

1Department of Dentistry, King Faisalpatient with Papillon-LefevreSpecialist Hospital & Research Centre,Riyadh, Saudi Arabia; 2Department of HealthSciences, Kristianstad University,syndrome Kristianstad, Sweden

A 41/2-year follow-up

Ullbro C, Crossner C-G, Lundgren T, Stålblad P-Å, Renvert S: Osseointegratedimplants in a patient with Papillon-Lefevre syndrome. A 41/2-year follow-up.J Clin Periodontol 2000; 27: 951–954. C Munksgaard, 2000.

AbstractBackground: The Saudi female reported in this study was diagnosed with Papil-lon-Lefevre syndrome. She had lost all her teeth, including third molars, at theage of 18 years.Method: She was wearing full dentures for 7 years, before 5 titanium implantswere installed in the mandible.Results: 41/2 years after the implant installation, the treatment was clinically andradiographically successful. Neither A. actinomycetemcomitans nor P. gingivaliswere detected at any of the implants. This very encouraging result does not differfrom what is generally reported in edentulous medically healthy patients.

Key words: dental implants; Papillon-Lefevre;Conclusion: The use of implants could considerably enhance future therapeutic syndrome.options for the severely dentally compromised patients with Papillon-Lefevresyndrome. Accepted for publication 6 December 1999

Papillon-Lefevre Syndrome (PLS) isan autosomal recessive disorder char-acterized by a diffuse transgradientpalmoplantar hyperkeratosis andrapidly progressive and devastatingperiodontitis, affecting the primary aswell as the permanent dentition. Aftereruption of the primary teeth, the gin-giva becomes inflamed. This is gener-ally followed by a rapid destruction ofthe periodontium, and most affectedchildren experience premature loss oftheir primary teeth (Haneke 1979).After exfoliation of the primary den-tition, the gingiva seems to resumenormal appearance (Hattab et al.1995). The aggressive inflammatoryperiodontal process then repeats itselfafter the eruption of the permanentteeth, and in general, all or most ofthe permanent dentition is lost duringthe teens (Haneke 1979).

Conventional periodontal treatmentusually fails in patients with PLS, andthe rapid progression of periodontitisoften results in a severe loss of alveolarbone (Rateitschak-Pluss & Schroeder1984, Van Dyke et al. 1984, Shapira etal. 1985). In order to preserve alveolarbone early extractions of periodontallyinvolved permanent teeth has been con-sidered a mode of treatment (Machteiet al. 1989).

Titanium implants have become animportant treatment alternative in or-der to replace missing teeth. The use ofimplants in patients with severe peri-odontitis has been reported (Mengel etal. 1996, Nevins & Langer 1995), andthe results indicate that periodontallycompromised patients can be success-fully treated with implants (Ellegaard etal. 1997).

In the present study, the use of osseo-

integrated implants in a patient withPLS is reported.

Previous Case History

This female patient, born 1969, was ini-tially referred to King Faisal SpecialistHospital and Research Centre(KHSH&RC), Riyadh, Saudi Arabia in1979. She was diagnosed to have PLSand revealed several loose permanentteeth due to advanced periodontitis.Multiple permanent teeth were ex-tracted over the following years, and in1987 the last remaining teeth (. 18, 28,38 and 48) were removed. Although shehad lost considerable amounts of al-veolar bone, she was able to wear fulldentures in both the maxilla and themandible. The dentures were deliveredin November 1987.

In June 1994, the patient was once

952 Ullbro et al.

Fig. 1. Panoramic radiograph taken 3 months after implant instal- Fig. 2. Periapical radiographs taken 41/2 years after implant instal-lation (October 1994), showing extensive resorption of alveolar bone. lation (January 1999), verifying successful osseointegration.

again referred to the Department ofDentistry at KFSH&RC. The maxillarydenture was well functioning, while theretention of the denture in the mandiblewas insufficient due to minimal bonesupport (Fig. 1). The oral mucosashowed normal appearance. Dermato-logical examination revealed minor pal-mo-plantar hyperkeratosis, without fis-suring, of hands and feet. No othermedical problems were found. Com-plete blood count and the levels of IgG,IgA, and IgM were within normal lim-its. Test for lymphocyte blastogenesis aswell as chemotaxis and phagocytosis ofpolymorphonuclear leukocytes (PMN)showed normal values.

In July 1994, 5, 10-mm titanium im-plants ad modum Brånemark (NobelBiocare, Gothenburg, Sweden) were in-stalled in the mandible at the areas ofteeth . 42, 41, 31, 32 and 33. Recoverywas uneventful, and abutment surgerywas performed 3 months later. The im-plants were clinically stable and con-sidered osseointegrated. A cast goldbridge was installed as a superstructure,and a new full denture was made in themaxilla. No further dental treatment orpreventive maintenance care have oc-curred. The patient lives far away fromRiyadh and has only showed up for re-call examinations every other year.

Examination in January 1999Clinical recordings

Registrations were made mesially, buc-cally and distally of each implant.Plaque was recorded as absent or pres-ent at the gingival margin. Bleeding atthe gingival margin, within 30 s follow-ing probing, was recorded.

Radiographic recordings

1 panoramic and 3 periapical radio-graphs were taken.

Microbiological sampling

Samples for microbiological analyseswere collected from the sulci around all5 implants. The supragingival areaswere cleaned with sterile gauze. 3 sterilemedium paper points (Johnsson &Johnsson, Windsor, NJ, USA) were in-serted into each sulcus until resistancewas met and left in place for 15 s. Thepaper points were transferred to sterile,dry containers and transported to thelaboratory of Oral Microbiology, Göte-borg University, Gothenburg, Sweden.Digoxigenin-labelled, whole genomicDNA-probes were used to examine anypositive occurrence of 12 different bac-terial species (Table 1) in the samplesaccording to a ‘‘checker-board’’ DNA-DNA hybridization method (Lundgrenet al. 1998).

Results

Clinical examination in January 1999revealed that all implants were clinicallystable, and that the superstructure wasfunctioning well. The gingiva aroundthe implant placed in the area of

Table 1. Prevalence of the 12 selected bacterial species assessed by means of ‘‘checkerboard’’hybridizations at the 5 implant sites (areas 42, 41, 31, 32 and 33). 0Ωno bacteria found, 1Ω,105 , 2Ω105 , 3Ω.105, 4Ω106 and 5Ω.106 bacterial cells per sample

Prevalence

Bacterial Species 42 41 31 32 33

P. gingivalis 0 0 0 0 0P. intermedia 1 1 1 1 1P. nigrescens 0 1 1 1 1B. forsythus 2 2 2 2 2A. actinomycetemcomitans 0 0 0 0 0F. nucleatum 1 1 1 1 1T. denticola 1 1 1 1 1P. micros 1 1 1 1 1C. rectus 0 0 0 0 0E. corrodens 0 0 1 1 0S. noxia 1 1 1 1 1S. intermedius 1 1 1 1 1

tooth .42 was hyperplastic withpseudopockets of 4–6 mm. No supra-gingival plaque was registered. Bleedingon probing occurred around the im-plant with hyperplastic gingiva.

The radiographs revealed a normalappearance of the bone tissue sur-rounding the 5 implants (Fig. 2). Incomparison to radiographs from Octo-ber 1994, no bone loss could be de-tected.

The microbiological analysis demon-strated the presence of all 12 testedmicro-organisms except P. gingivalis, A.actinomycetemcomitans and C. rectus(Table 1). However, none of thesampled areas demonstrated levelsabove 105 bacterial cells per sample forany micro-organism.

Discussion

The outcome of the implant treatmentin the patient reported in the presentstudy was successful 41/2 years after in-stallation, and the result did not seemto differ from what is normally re-ported on implant treatment in edentu-lous medically healthy patients.

Osseointegrated Implants in PLS 953

The moderate gingival hyperplasiaoccurring around one of the implantscould be related to lack of sufficient at-tached gingiva. It is sometimes rec-ommended to replace the unattached,nonkeratinized mucosa with a gingivalautograft (Han et al. 1995). However,Adell et al. (1986) did not find attachedgingiva to be a prerequisite for gingivalhealth. Our patient elected not to haveany surgical adjustment of the gingivaltissue in the hyperplastic area.

After having been edentulous formore than 7 years, it is most likely thatour patient, at the time of implant in-stallation, harboured a predominantlygram-positive flora. The colonizationand establishment of the microfloraaround implants seem to follow thesame pattern as on teeth (Danser et al.1997), and consequently, the presence ofimplants may facilitate the growth ofgram-negative micro-organisms. Thebacteria samples from our patient, how-ever, demonstrated the presence of peri-odontal pathogens only in low num-bers.

Dysfunction of PMN cells has beenreported in patients with PLS. If thePMN cell dysfunction is a major reasonfor the aggressive periodontal diseaseor secondary to the infection remainsunclear (Tinanoff 1995). There are re-ports suggesting that defects in thePMN cells in patients with peri-odontitis may be caused directly or in-directly, by specific periodontal bacteria(Van Dyke et al. 1982, Slots & Genco1984, Crossner et al. 1990). Other sug-gested alternatives explaining this dys-function might be an underlying cellu-lar defect of PMN cells (De Nardin1996), or an inherent hyperactive im-mune response during the host patho-gen interaction (Agarwal et al. 1996).Regardless of the reason, the impair-ment of the immune system in patientswith PLS occurs at an early age andmanifests clinically in the primary den-tition. However, the function of the im-mune system, and thereby the oral con-dition, seems to improve with age(Prabhu et al. 1979, Ullbro et al. 1997).In most cases, the PMN cell function isnormalized once the periodontal infec-tion has been successfully treated (Tin-anoff et al. 1986, Bullon et al. 1993).After having been edentulous andthereby periodontally healthy for manyyears, chemotaxis and phagocytosis ofthe PMN cells in our patient showednormal values at the time of implant in-stallation.

The primary objective for dental carein patients with PLS, is to treat the dev-astating periodontitis. When such treat-ment efforts fail, early extraction ofteeth with progressive periodontitis willpreserve alveolar bone facilitating fu-ture prosthodontic rehabilitation, in-cluding the use of osteointegrated im-plants. Dental implants are consideredcontraindicated in a growing individualsince they act like ankylosed teeth (Os-terle et al. 1993) resulting in infraposi-tioning of implants (Ödman et al. 1991,Brugnolo et al. 1996). However, the ap-position of alveolar bone and the in-crease of alveolar height, are by andlarge, completed during the teens(Behrendts 1985). Infrapositioning ofimplants in teenagers with PLS willmost probably be of little importance,in comparison to the bone-saving effectof early implant installation. The suc-cessful result in the presented case, to-gether with 2 additional patients withPLS, ages 24 and 26, where implantshave been equally well osseointegrated,have encouraged us to try implants alsoon much younger patients with PLS. Ifsuccessful, this treatment approach willin the future considerably enhancetherapeutic options in this group of se-verely dentally-compromised patients.

Zusammenfassung

Osseointegrierte Implantate bei einem Patien-ten mit Papillon-Lefevre-Syndrom. Eine vie-reinhabl-jahrige NachuntersuchungBei der saudischen Patientin, uber die hierberichtet wird, wurden die Diagnose Papil-lon-Lefevre-Syndrom gestellt. Sie hatte imAlter von 18 Jahren alle ihre Zahne ein-schließlich der dritten Molaren verloren. Be-vor ihr 5 Titanimplantate im Unterkiefer ein-gesetzt wurden trug sie fur 7 Jahren eineVollprothese. 41/2 Jahre nach Implantationkann die Behandlung als klinisch und rontge-nologisch erfolgreich bezeichnet werden. Beikeinem der Implantate wurde weder A. acti-nomycetemcomitans noch P. gingivalis nach-gewiesen. Diese sehr ermutligenden Ergeb-nisse unterscheiden sich nicht von den Resul-taten, die im Allgemeinen bei zahnlosenallgemeinmedizinisch gesunden Patienten be-richtet werden. Bei schwer dental einge-schrankten Patienten mit Papillon-Lefevre-Syndrom konnte die Verwendung von Im-plantaten die zukunftigen therapeutischenMoglichkeiten verbessern.

Resume

Implants osteointegres chez une patiente at-teinte du syndrome de Papillon Lefevre. Suivisur un an et demi

La patiente saoudienne impliquee dans cetteetude est atteinte du syndrome de PapillonLefevre. Elle a perdu toutes ses dents, y com-pris les troisiemes molaires a l’age de 18 ans.Elle portait des protheses completes depuis 7ans, jusqu’a ce que 5 implants en titanesoient poses a la mandibule. 41/2 ans apresl’installation de ces implants, le traitementetait un succes d’un point de vue clinique etradiologique. Ni l’Actinobacillus actinomyce-temcommitans, ni le Porphyromonas gingiva-lis n’etaient detectes sur aucun des implants.Ces resultats tres encourageants ne sont pasdifferents de ce qui est generalement rappor-te pour les patients edentes en bonne santegenerale. L’utilisation des implants peutconsiderablement augmenter les futures op-tions therapeutiques pour des patients auxsequelles dentaires severes atteints du syn-drome de Papillon-Lefevre.

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Address:

Christer UllbroKing Faisal Specialist Hospital & ResearchCentreMBC .70P.O. Box 3354Riyadh 11211Kingdom of Saudi Arabia

e-mail: cullbro/hotmail.com