Early Onset and Rapidly Progressive Periodontitis
Periodontitis has been classified according to the rate
of progression (slowly and rapidly progressive) and according
to the age at onset (adult periodontitis and early onset
peridontitis). When combined as in the case of early onset
rapidly progressive periodontitis which occurred in patient
with localized juvenile periodontitis and prepubertal periodontitis,
the lesions are advanced and very destructive in a short
period of time. This paper will reviews various articles
involved the prepubertal periodontitis and the localized
juvenile periodontitis as occured in patient with rapidly
Prepubertal periodontitis appears before the age of puberty
and rapidly destroyed the periodontium. It is associated
with immunologic or other systemic problems such as Papillon
Lefevre syndrome, hydrophosphatasia, agranulocytosis, Down
syndrome, and others. According to Caranza and Newman in
the text book of clinical periodontology, prepubertal periodontitis
has it onset before 11 years of age in the primary or mixed
dentition, and it frequently persists after puberty. The
disease may be localized, of which either PMNs or monocytes
but not both cell types affected, whereas in the generalized
type, both PMNs and monocytes involved and the defect appear
to be cell adherence. The localized type involved few teeth
and the gingival tissue exhibit only minor inflammation
and minimal plaque. Destruction is not as rapid as generalized
form. Recurrent ostitis media is not a frequent finding
and usually there is no history of frequent infections.
The disease is amendable to curettage and antibiotic therapy.
The classical diagnostic feature of generalized form of
prepubertal periontitis is the fiery red acute inflammation
pervading the marginal and attached gingiva around all the
teeth, gingival proliferation, cleft formation, and recession.
Onset is at the time of tooth eruption. Alveolar bone destruction
may couple with the destruction of the tooth root and occurred
at the alarming rate. Peripheral white blood cell count
is markedly elevated. Ostitis media and skin and upper respiratory
infections are frequent findings. Periodontitis are refractory
to therapy and all the primary teeth are affected. The permanent
dentition may or may not be affected. The systemic involvement
will be discussed more detail as followed.
Papillon Lefevre syndrome is the classical inherited and
autosomal recessive disease. Parents are not affected and
both must carry the autosomal genes for the syndrome to
appear in the offspring. The cause of the disease is still
unknown, but a number of local factors have been implicated.
The syndrome was first thought to be a combination of ecto-
and mesodermal malformation (Wannenmacher 1938). Smith and
Rosenzweig 1967 investigate the role of defect cementum.
Gorlin et al. 1964, Lyberg 1982, and Schroeder et al. 1983
reported the role of defect gingival epithelium and Shoshan
et al. 1970 suggested the functional imbalance of collagenolytic
activity in the periodontal ligament. Bacterial flora studies
of plaque in a case of PLS revealed the similarity to the
flora of adult periodontitis. Spirochete rich zone in the
apical portion of the pocket, as well as spirochete adherence
to the cementum and microcolony formation of mycoplasma.
Recent studies of subgingival plaque from the LPS patients
revealed a predominance of gram anaerobic rods, including
B. gingivalis, capnocytophaga, and spirochetes. (Newman
et al. 1977, Jung et al. 1981). It has no sexual predilection,
and can occur in siblings. The incidence of disease is one
to four cases per million (Gorlin et al. 1964). The classical
features of the Papillon Lefevre syndrome is the occurrence
of the triads of symptoms: hyperkeratotic skin lesions,
severe destruction of the periodontium, and may be the calcification
of the dura. Due to the location of the hyperkeratotic lesions,
the disease is also termed keratosis palmoplantaris, occurrence
along with the premature periodontal destruction of teeth.
Another name for the disease is the hyperkeratosis palmoplantaris
Unna Thost (Unna 1883) and Meleda’s disease (Hovorka
and Ehlers 1897). Gorlin et al. 1964 reported the main clinical
features of the disease as diffuse palmo-plantar hyperkeratosis,
and generalized, severe and rapidly progressive prepubertal
periodontitis, leading to early loss of both deciduous and
the permanent teeth. The cutaneous and the periodontal changes
usually appear together before the age of four. The skin
lesions consists of hyperkeratosis and ichthyosis of localized
areas on palms, soles, knees, and elbows. Periodontal involvement
consists of early inflammatory changes and lost of primary
teeth by five or six years of age. The permanent dentition
erupted normally, but within the few years the permanent
teeth are lost owing to the destructive periodontal disease.
By the age of 15, patients are usually edentulous except
for the third molars. These, too, are lost a few years after
they erupted. Vrahopoulos et al. 1987 reported the ultrastructure
of periodontal lesion in the case of PLS by the use of scanning
electron microscope and the transmission electron microscope
on the extracted permanent teeth along with their associated
soft tissue. Base on the result, they suggested that the
impaired deposition of cementum and/or a defective ligament
may have resulted in an abnormal periodontal attachment
highly susceptible to destruction. Alternatively, the rate
of the disease advance may have been so fast as to interfere
with ligament and cementum formation. They also found that
the plaque nearest to the advancing front of the lesion
in PLS was restricted to mainly the gram coccoid and rod
shaped bacteria, similar to those known to occur in other
aggressive forms of periodontitis. Finally, they suggested
that the scarcity of microorganism within the pocket of
tissue indicated that bacterial invasion was unlikely to
be responsible for the high level of tissue destruction.
Enrique Bimpstein et al. 1990 studied the immune system
and its association to the PLS disease. The patient had
high count of actinomycetemcomitan and surface translocating
bacteria even after antibiotic therapy. Antibodies to three
serotypes of A.A., capnocytophaga, and wollinella recta
significantly increased. Finally, PMNs release significant
amount of superoxide compared to the controls.
Down ‘s syndrome is the another disease which characterized
by the high occurence periodontal disease in young children.
The disease was first recognized by Langdon-Down. The prevalence
of periodontal disease is almost 00% in children with DS
under the age of 30 years. Oral hygiene usually poor but
not commensurate with the destruction of the periodontium.
ANUG is also found in this patient population. Endogeneous
factors which might play role in the rapid progression of
the periodontal breakdown is the PMN and monocyte functional
defect, abnormal colagen biosynthesis, and an abnormal capillary
morphology. Nonspecific and specific defense mechanism is
reduced. Reduced amount of T cell whereas the B cell and
immunoglobulins are almost normal.
Juvenile Periodontitis, today is termed Localized juvenile
periodontitis, is seen during puberty, in the circumpubertal
puberty and adolescent period. They also is referred previously
as periodontosis, precocious advanced alveolar atrophy,
juvenile atrophy, juvenile paradentosis, juvenile parodontopathia,
and localized juvenile periodontitis.
First described by Gottlieb 1923 as diffuse atrophy and
later by Orban and Weinmann in 1942 as periodontosis to
indicate the degeneration of the periodontal ligament. J.D.
Manson and T. Lehner described the clinical features of
juvenile periodontitis as diffuse, typical and atypical
localized forms of bone loss. The onset of disease is at
the age of puberty with greater prevalence of females than
males (Miller, Wolf, and Seidler in 1941, and Benjamin and
Baer in 1967). Immunological investigation of these patients
showed a dissociation between lymphocyte transformation,
macrophage migration inhibition, and serum immunoglobin
concentration. There is an impairment in DNA synthesis of
lymphocytes stimulated by Gram negative plaque organism,
in the absence of serum inhibitory factor, and a significant
increase in the serum immunoglobulin concentrations. Thomas
Waldrop et al. define the relative distribution of lymphocytes
and plasma cells and the nature of the cell associated immunoglobulins
IgG, IgA, IgM, IgD, IgE, and IgG. Plasma cell were the predominant
cells involved. The quantity and the distribution of inflammatory
cells increased with clinical severity. 76% of the plasma
cell in LJP lesions lacked demonstrable cytoplasmic heavy
chain Ig determinants typical of such cell. The predominant
Ig staining cells present were IgG followed by IgA and IgM.
Robert A.Clark, Roy C. Page, and Gregory Wilde examined
the neutrophil chemotaxis in these patients and found that
a reduction of chemotatic response of the patient PMNs to
preformed chemoattractants, coupled with the reduced level
of complement-derived serum chemotactic activity. The serum
from LJP patient contains the heat stable, non-dialyzable
factor that markedly inhibited the chemotaxis of normal
neutrophils. Other study demonstrated the role of Actinomycetemcomitans
in the disease destruction. PMNs dysfunctions increase the
likelihood of infection by microorganisms which do not readily
colonize the gingival crevice in otherwise normal individual.
Leukotoxin producing strains of A.A. is an example of such
organism. Statistical comparisons of the flora associated
with juvenile periodontitis, severe periodontitis, and the
moderate periodontitis indicated that difference in bacterial
composition of affected sites in these populations were
The prevalence of the disease of Juvenile periodontitis
is studied in Finland (represent majority caucasian population)
yield an estimate of 1%. In the study, the regions involved
lesion usually the first molars, mandibular incisors and
minor involvement of bicuspid. J. Hormand and A. Frandsen
in their study of juvenile periodontitis and the localization
of bone loss in relation to age, sex, and teeth reveals
the initial involvement of first molars, incisors and subsequent
involvement of other teeth. The majority of juvenile periodontitis
cases exhibit symmetrical involvement of fist molars and
incisors and a few additional teeth. The study also supported
the female predilection in the disease’s incidence.
Many of these patients has no dental caries and less microbial
plaque and calculus than would be expected. The bone loss
usually characterized by vertical legions as seen in the
radiograph. Radiographically, the lesion also reveals a
more dilection for the defect to be occur on the mesial
rather than distal. Although frequently arc-shaped and bilateral,
the bone loss was symmetrical only occasionally. More than
one first molar was always involved. Incisors were not always
involved. The first molars were involved more frequently
than the incisors. Only one proximal surface on the first
molars/incisors might demonstrated bone loss. Molar furcation
is also a frequent finding.
Treatment of LJP has been investigated thoroughly. The
use of systemic tetracycline coupled with scaling and root
planing perform initially to reduce the count of total subgingival
bacteria, especially the A.A. Periodontal A.A. infections
cannot be resolved by root surface debridement alone but
can be cured by systemic tetracycline (Jorgen Slot and Rosling
1983). The regimen of tetracycline has been suggested of
1g/day for 14 days.
Lars Christenson, Slots, Rossling, and Genco studied the
microbiological and clinical effects of surgical treatment
of localized juvenile periodontitis and showed that suppression
of subgingival A.A. can be accomplished with removal of
involving periodontal tissue using open flap debridement.
Finally, five year longitudinal study of healing following
surgical (open flap debridement) and non surgical treatment(scaling
and root planing) of juvenile periodontitis demonstrated
that the response of the periodontal tissue to therapy is
exactly the same as that found for the same kind of treatment
in other cases.
Prepubertal, Juvenile, and rapidly progressive periodontitis
are all associated with abnormalities in peripheral blood
neutrophil or monocyte function, or abnormalities in serum
complement, that these abnormalities may predispose the
patient to periodontitis. In families with high prevalence
of the early onset, there is indication of the genetic basis
in the leukocyte function defect. Patient with JP most likely
resulted from a X-link dominant trait. Prepubertal periodontitis
seems to be more common to male than female. Patient with
localized juvenile periodontitis have antibodies specific
to the antigenic determinants of the actinobacillus actinomycetemcomitan.
Patients with prepubertal or rapidly progressing periodontitis
have bacterial flora consist of bacteroides, eikenella,
Fusobacterium, and other gram negative rods. They also manifest
serum antibodies specific to many of these species. Treatment
for these patients are complex, difficult, and expensive.
The disease seems to be refractory to antibiotic therapy.
Treatment require aggressive measure with strict control
of oral hygiene and closely monitored maintenance program.
The disease nevertheless can be halted with curettage and
debridement, coupled with antibiotic therapy and improved
tooth brushing. Thus in conclusion, patient must be strictly
controlled in term of oral hygiene and proper maintainance
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