Case Report

Matteo D'Angeio\ Valerio

Treatment of prepubertal Margiotta\ Pietro Ammatuna^ and
Francesca Sammartano^

periodontitis Departments of 'Periodontology and

^Hygiene and Microbiology, University of
Palermo, Italy

A case report and discussion

D 'Angelo M, Margiotta V, Ammatitma P and Sammartano F: Treatmetit of prepub-

ertal periodontitis. J Clin Periodontol 1992: 19: 214-219.

Abstract. This paper reports the treatment of prepubertal periodontitis in a 3-

year-old girl with Papillon-Lefevre Syndrome. Initially, the patient was found to
have a myeloperoxidase deficiency and microbiological tests have identified Bacter-
oides and Fusobacteritttn. in 60% and 25%. respectively of the total number of
microbia] flora cultivated. The initial treatment was extration of all the primary
teeth with grade 3 mobility, scaling, root planing and daily subgingival irrigation
with a 0.2%n solution of chlorhexidine. Several months before the eruption of the
first permanent molars, the rest of the primary teeth were extracted. The patient
was treated with daily subgingiva! irrigation of chlorhexidine and weekly pro-
fessional oral hygiene. At the age 6V; years, the permanent teeth have normal Key words: PLS-periodontitis; periodcntai
therapy; chlorhexidine.
gingiva and crevice depths; microbiological investigation reveals a prevalence
of the coccoid forms, and radiographs show no evidence of periodontai pathology. Accepted for publication 3 January 1991

Prepubertal periodontitis (PP) is a (Newman et al. 1977. Jung et al, i98i, protocol chosen by us to manage a case
pathology that develops during or im- Tinanoff et al. 1986, Preus & Gjermo of PP associated with PLS in a young
mediately after the eruption of the pri- 1987). patient.
mary teeth. It is almost always associ- Immunodeficiency is frequently
ated with complex hereditary patho- found in these patients, e.g, chemotaxis
Case Report
logies involving immunological and phagocytic function of neutrophilic
deficiencies, such as: Chediak-Higashi granulocytes and decreased lymphocyte In September, 1986, we examined a
Syndrome; neutropenia (cyclic or per- response to PHA stimulation (Haneke girl aged 3 years and 3 months, sent
manent); Ehler-Danlos Syndrome; his- et al, 1975, Djawari 1978, Van Dyke et to us by a pediatrician who had ob-
tiocytosis X; hypophosphatasia; Papill- al, !984, Tinanoff et al. 1986, Preus & served a loosening of the child's pri-
on-Lefevre Syndrome. Gjermo 1987, Preus & Morland 1987), mary teeth.
The most frequent association is PP It has also been suggested that the
with the Papillon-Lefevre Syndrome pathogenesis of periodontilis may be
Family history
(PLS). PLS is hyperkeratosis palmo- linked to a dyskeratosis of the junc-
plantaris hnked to a recessive auto- tional epithelium, with reduced ef- The parents were both normally healthy
somal penetrating gene and has an in- ficiency of the decreased epithelial bar- subjects and were not blood relatives.
cidence of \-A- per million; in 33*Mi of rier (Vrahopoulos et al. 1988). An older brother aged 8 years had no
the cases the parents, are blood-rela- The therapeutic management of PP health problems. Neither the parents
tives (Gorlin et al. 1964). presents several problems; traditional nor the older child had any periodontal
Periodontitis due to PLS normally mechanical treatment associated with pathology.
occurs at the age of 3 to 4 years and drag administration does not always
causes early loss of the primary teeth, prevent the early loss of the primary
Medical history
with subsequent involvement of the teeth and involvement of the permanent
permanent teeth (Gorlin et al. 1964, teeth (Ratcitschak-PIus & Schroeder The patient's birth had been perfectly
Haneke 1979), The etiopathogenesis of 1984). In recent years, a different thera- normal. At the age of 4 months, she was
PP is believed to include both local ir- peutic approach has been proposed. treated with Ventolin and erythromycin,
ritant factors and factors instrinsic to This involves elimination of the perio- and subsequently with cortisone (beta-
the patient. In very young subjects, dontal pathogenic micro-organisms by methasone) for spastic bronchitis. At 10
there is an increase of rnobiie, anaer- extraction of the primary teeth several months she had phlyctenular conjuncti-
obic and gram-negative germs and the months before the permanent teeth ap- vitis, which recurred at the age of 2
presence of such periodontopathogenic pear, plus antibiotic treatment to avoid years. In January, 1986, she had tonsilli-
micro-organisms as Bacteroides gin- any possible involvement of the perma- tis; in July of the same year she had
givalis. Capnocytophaga, Fusobacterium nent teeth (Tinanoff et al. 1986, Preus & exudative eczema of both the upper and
nucleaturn, Eikenella corrodens, A. acti-Gjermo 1987, Preus 1988). lower limbs. In the Spring of 1986, the
nomycetemcomitans and .spirochaetes We therefore present the therapeutic child suffered from a series of multiple

abscesses involving both the maxiUary
and mandibuiar arches, with a !oose-
ning of the primary teeth. In Ju!y, !986,
5.! and 6.! were !ost; the periodonta!
pathology was treated with penici!!in.
Clinical features
The gingiva was extremely red and
edematous and there was spontaneous
gingivorrhagia, with multiple abscesses,
mostly in the mandibular arch. The
probing depth ranged from 5-% mm,
pus was discharged from pockets of
both anterior and posterior teeth. A!l
the teeth were mobile to the grade of
2-3: 5.1 and 6.1 were absent (Fig. I).
About a month after this observation,
hyperkeratosis appeared on the palm of
the left hand and the soies of the feet.
X-ray examination
An orthopantomograph revealed wide-
spread destruction of the alveolar bone
around a!! the teeth (Fig. 2).
Blood chemistry tests
B!ood tests showed a defective neutro-
phil chemotaxis (Skin Window: !5'/''o -
after 15': 20%) and a reduction of T-
lymphocytes with a normal T4/T8 ratio:
T3 - 65% T8 - 25%
T4-4i% T4/T8 - 1:64
immunohistochemical assays with ben-
zidine revealed myeloperoxidase de-
ficiency.
Microbioiogicai tests
Dark-field microscopy, cultures and
scanning electron microscopy (SEM)
were performed. The subgingival plaque
samples were obtained with a curette
and immcdiate!y resuspended in 0.5 ml
of prereduced thiog!yco!ate broth me-
dium U.S.P. (OXOID). The samp!es
were then transferred into an anaerobic
chamber (Progammed Anaerobic Con-
tro!!ed Environment, Lab-Line Instru-
ments) under an atmosphere of Hi
(10%), CO, (10%)) and N. (80%.) and
dispersed by shaking on a Vortex mixer.
They were then used within 30 min for
dark-field microscopy and culturing.
All the bacteria] cells observed by
dark-field examination were classified
into 4 groups: coccoid cells, mobile
rods, spirochaetes and other forms, fo!-
lowing the criteria proposed by Rosen-
berg et al. (1981). The proportion of
spirochaetes was significantiy higher
than of other bacteria! forms (60%).
There were no protozoa in the samp!es.
For cu!turing, the p!aque sampies
were seria!!y di!uted !0-fo!d and each
di!ution was piated (0.! m!/cu!ture
plate) on Wilkins-Cha!gren anaerobe
agar (OXOID) supplemented with 5%i
defibrinated sheep blood and on 4 dif-
ferent types of selected media based on
the same Wilkins-Chalgren agar with
5%u of defibrinated sheep b!ood.
For isolation of the non-spore form-
ing anaerobes, we used NAT medium
containing 10 /(g/ml of nahdixic acid
and 2.5 /ig/mi of vancomycin as selec-
tive agents (Wren 1977).
For selective iso!ation of A. actinom-
ycetemcomitans, a medium containing 8
/ig/ml malachite green and 128 /ig/ml
bacitracin was used (Mandell & Socran-
sky !98I). For Fusobacterium nucleat-
um, we used a cu!ture medium contain-
ing 5 /;g/m! crysta! violet and 4 /^g''m!
erythromycin (Wa!ker et al. !979), whi!e
for Eikene!!a eorrodens, 4 ^g/m! c!inda-
mycin was added to the medium (Wa!!c-
er et a!. !978). After insemination the
cu!tures were incubated under anaer-
obic conditions at 37'C for 5 days.
The various species were then iden-
tified from the type of co!onization and
the morpho!ogica!, staining and bio-
chemical features of the iso!ated micro-
organisms by means of the API 20 A
identification system. The largest
groups identified were black-pigmented
Bacieroides and Fusobacterium. 6O'/o
and 25%. respective!y, of the tota! num-
ber of microbic fiora cultivated. In spite
of using se!ective media, we were unab!e
to detect any significant numbers of A.
actinomyeetemcomitans, E. eorrodens or
Capnocylophaga.
SEM was performed on extracted
teeth which had been treated as foilows:
phosphate buffer washing; dehydration
by ethanol (70%, 80%, 95%, !00%);
transfer into Freon 1! 3 for ! 2 h; drying
with 25 m/( paUadium gold.
Observation of the sampies revea!ed
that they were comp!ete!y covered by a
!ayer of p!aque bacteria arriving to
within I mm of the apex of the tooth.
The p!aque on the crown surface and
the corona! 2/3 of the root seemed to
consist mostly of long smooth-wa!!ed
fi!aments with rounded ends, with a
mean diameter of about 0.5-0.8 /.im and
from lO's to !00's /zm long, inserted
perpendicularly into the root surface.
Although it was not possible to make a
taxonomic identification of these fila-
ments, it is quite probable that they
Prepubertal periodontitis 215
were Leptothrix buccalis or Bacter-
rionema matruchotii (Fig. 3). Blunt-
ended, slight!y concave rods, at times
simi!ar to Actinomyces, were found in
the coronal zone (Fig. 4). From the me-
dian 1 /3 to the apical 1/3 of the root,
there gradually appeared short round-
ended rods and spirochaetes. These lat-
ter species predominated in the zones
where the plaque was more developed
(Fig. 5).
Occasiona! round cel!s with short
parieta! appendices, strongly suggestive
of B-!ymphocytes, could a!so be seen
(Fig. 6).
Therapeutic management
The initia! treatment of the case in-
c!uded extraction of a!! the teeth with
grade 3 mobi!ity: 5.4, 6.2, 6.4, 7.2, 7.!,
8.!, 8.2, 8.4. The rest of the primary
teeth were then sca!ed and root-p!aned
and sub-gingiva! irrigation with 0.2
ch!orhexidine was performed daily.
60 days after the beginning of the
treatment. dark-field microscopy
showed a considerable reduction of the
spirochaetes (2%)), as compared to the
coccoid forms (80%>). Cultures showed
that the Bacteroides and Fusobacteria
had also diminished to 10%, while the
streptococc!ii were at 80%. Clinicai ob-
servation revea!ed norma! gingival color
and morphology: probing of 2 to 4 mm
produced no bleeding.
The child then began a therapeutic
phase consisting of weekly sessions of
professional oral hygiene.
Several months before the eruption
of the first permanent molars, which
occurred at the age of 5 years and 6
months, all the remaining primar}' teeth
were extracted to prevent the formation
of pockets in which periodontal patho-
genic microorganisms might develop.
The patient was then treated again with
daily sub-gingival irrigation with 0.2%
chlorhexidine and weekly professional
oral hygiene. Microbio!ogica! investiga-
tion at that time again revealed a preva-
lence of the coccoid fomis (Fig. 7).
Chemotaxis was within the normal
range and there was sti!i a myeloperox-
idase deficiency. C!inica! and radio!ogi-
ca! fol!ow-up ! year after the eruption
of the first molars did not reveai any
periodotital lesions (Figs. 8, 9).
Discussion
Therapeutic management based on
mechanical control of the local irritant
 216 D'Augelo et al.

Fig. I. Oral conditions at first examination in September 1986, Fig. 2. Orthopan tomograph at the start ofthe study.

Fig, 3. Scanning electron microscopy; subgingivai plaque in the cor- Fig. 4. Scanning electron microscopy: subgingivai plaque in the cor-
onal 2/3 ofthe root (original magnification x 5700), Long Hianients onai zone of the root (originai magnification x 10,000), Presence of
with rounded ends inserted perpendicuiariy into the root surface, rods with biunt-ended and slightiy concave.

Fig. 5. Scanning eieetron microscopy: subgingivai piaque in the apical Fig. 6, Scanning eiectron microscopy: subgingival plaque (original
1/3 of the root (original magnification x 10,200). Abundant spiro- magnification x 9,600), Round cells with short parietal appendices
chaetes in the apical border plaque. (B-!ymphocytes),
 Prepubertal periodontitis 217

non-specific pathogenic flora, with

BACTEROIDES lillii STREPTOCOCCUS Bacteroides and Fusobaeterium associ-
ated with numerous spirochaetes (New-
ico - man et al. 1977, Jung et al. 1981, Pre-
FUSOBACTERIUM L J EUBACTERIUM
us & Gjermo 1987, Preus 1988).
We therefore chose to use repeated
local chemical treatment for short
periods instead of wide-sprectrum sys-
temic antibiotic therapy (Khoo et a!.
1983, Lander et al. 1986, Hasket et al.
50 - 1986, Stanley et al, 1989, Vignarajah
et al, 1989). The use of local chemical
treatment also enabled us to observe
that the initial chemotaxis deficiency
was linked to the presence of the micro-
organisms (Preus & Gjermo 1987),
whereas there had been no change in
the myeioperoxidasc deficiency. The pa-
tient's skin, eye and respiratory tract
troubles are therefore almost certainly
Fig. 7. Prevalence of cultivable microorganisms in subgingival plaque. (A) Pretreatment. (B) linked to this latter deficiency, which
60 days after the beginning of the treatment. (C) Up-to-date. might also in part induce the perio-
dontal pathology, which wouid then be
perpetuaded by the presence of the
factors together with systemic antibiotic should be done before the appearance pathogenic flora and the linked chemo-
treatment has not proved to be efficient of the remaining permanent teeth and taxis deficiency.
for preventing the progression of the be combined with administration of Hyperkeratosis palmoplantaris in
periodontal pathology in PLS and t"e- antibiotics. PLS would seem to be associated with
sulting involvement of the permanent This protocol was followed in the an ectodermal anomaly. It has therefore
teeth (Rateitschak-Piuss & Schroeder present case, with extraction of the pri- been suggested that the periodontitis
1984). In recent years, Tinanoff et al. mary teeth several months before the may be triggered by a change in the
(1986) and Preus & Gjermo (1987) have appearance of the permanent teeth, bui function of the junctiona! epithelium,
proposed a treatment protocol consist- no antibiotic therapy was administered; which would no longer act as a defence
ing of not only local control of the irri- sub-gingival irrigation with chlorhex- barrier against micro-organisms (Vrah-
tant factors, but also of extraction of idine was perfomied to control the opoulos et al. 1988). Sloan et al. (1984)
teeth, either primary or pemianent. that periodonta! pathogenic micro-organ- did not find this structural deficiency,
are involved in the pathology. This isms. Our microbioiosical data show and our own clinical data are also in

d e
Fig. S. Periodontal conditions around maxillary and mandibular first molais and incisor, up-to-date.
 218 D'Angelo et al.
confiict with this theory; the structural
alteration of the epithehum should, in
fact, induce a periodontal lesion not
only of the primary, but aiso of the per-
manent dentition, whatever type of
therapeutic management is followed.
Our results therefore lead us to conclude
that PP in PLS involves non-specific
pathogenic fiora. probably linked to a
deficiency in the host defense mechan-
isms, which may be in part connected
with substances produced by the micro-
organisms.
We therefore conciude that the elim-
ination of the pathogenic microfiora
during primary dentition by early ex-
traction of the teeth involved plus daily
local mechanical and chemical control
of the irritant factors, may prevent
consequent damage to the permanent
teeth in PP associated with PLS.
Acknowledgement
We would like to thank Professor A.
Carrassi for his technical expertise and
assistance in evaluation of the Scanning
Electron Microscopy.
Zusammenfassung
Die Behandlung der prdpubertdren Parodonti-
tis. Ein Fallbericht mit Diskussion
In dieser Veroffentlichung wird die Behand-
lung der prapubertaren Parodontitis bei
einem 3-jahrigen Madchen mit dem Papillon-
Lefevre Syndrom beschrieben. Urspriinglich
wurde bei dem Patienten ein Myeloperoxyda-
semangel konstaliert. Mikrobioiogische Tests
stellten fest, dass Bacteroides und Fusobacte-
rium 60% bzw. 25% der gesamten kultivierten
Mikroflora ausmachten. Als Initialtherapie
wurden alle Milchzahne mit dem Locke-
rungsgrad 3 extrahiert, gefolgt von Scaling.
Wurzelglattung und taglicher subgingivaler
Irrigation mit 0,2% Chlorhexidinlosung. Die
Fig. 9. Ortopantomograph at the end of the observation period.
noch vorhandenen Milchzahne wurden einige
Monate vor dem Durchbruch des ersten blei-
benden Molaren extrahiert. Die Behandlung
wurde dann mit taglichen subgingivaien
Chlorhexidinspiilungen und wochentlicher
professioneller Mundhygiene vervollstandigt.
Im Alter von 6 Jahrcn erscheint die Gingiva
der bieibenden Ziihoe normal, mit normalen
Sulkustiefen. Die mikrobioiogische Untersu-
chung zeigt ein Vorherrschen kokkoider For-
raen und auf den Rontgenaufnahmen werden
keine Zeichen pathologischer Veranderungen
des Parodont gefunden.
Resume
Traitement de la parodontite prepubertaire.
Presentation ct discussion d'un cas
La paliente est une filletle de trois ans ayant
la maladie de Papillon-Lefevre, El!e avait une
carence de myeloperoxidase et les tests micro-
biologjques avaient mis en evidence des Bac-
teroides et des Fusobacterium representant
respectivement 60 et 25% du nombre total de
la flore cultivee. Le traitement initial a
consiste en I'avulsion de toutes les dents tem-
poraires ayant une mobilite du 3'™ degre
detartrage, lissage radiculaire et irrigation
sous-gingivale quotidienne avec une solution
de chlorhexidine 0,2%. Quelques mois avant
i'eruption des premieres molaires permanen-
tes les demieres dents temporaires ont ete
avulsees. La patiente a ete traitee par irriga-
tion sous-gingivale quotidienne avec de la
chlorhexidine et un nettoyage dentaire pro-
fessionnel hebdomadaire. A 6Vj ans, les dents
permanentes sont entourees d'une gencive
saine avec sillon normal, les tests microbiolo-
giques ont mis en evidence une forte propor-
tion de formes cocoi'des et ies radiographies
n'indiquent auciine pathologie parodontale.
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Address:
M. D'Angeto
via Leonardo da Vinci, 94
90145 Palermo
Italv