Early Onset and Rapidly Progressive Periodontitis
by Dinh X. Bui, D.D.S., M.S.

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 progressive periodontitis.

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 not significant.
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 program.



1. Hormand J., Frandsen A. Juvenile periodontitis. Location of bone loss in relation to age, sex, and teeth. Journal of Clinical Periodontology, 6: 407, 1979.

2. Watanabe, K. Prepubertal periodontitis: a review of diagnostic criteria, pathogenesis, and differential diagnosis. Journal of Periodontal Research; 25: 31, 1990.

3. Bimpstein E., Lustmann J., Sela M., Neriah Z., Soskolne W. Periodontitis associated with Papillon Lefevre Syndrome. J. Periodontology, 61: 373, 1990.

4. Reuland-Bosma W., van Dijk. Periodontal disease in Down’s syndrome: a review. J Clin Periodontol, 13: 64, 1986.

5. Liakoni H., Barber P., Newman HN. Bacterial penetration of pocket soft tissues in chronic adult and juvenile periodontitis cases. J. Clin. Periodontol, 14: 22, 1987.

6. Wennstrom A., Lyndhe J. Healing following surgical and nonsurgical treatment of juvenile periodontitis. A five year longitudinal study. J. Clin. Periodontol, 13: 869, 1986.

7. Christersson, L., Slots J., Rosling B., Genco R. Microbiological and clinical effects of surgical treatment of localized juvenile periodontitis. J. Clin. Periodontol.,12: 465, 1985.

8. Moor, W., Holdeman L.V., Smibert R., Cato. E., Burmeister, J. Palcanis. Comparative bacteriology of juvenile periodontitis. Infection and immunity, 507, 1985.

9. Bial, J., Mellonig, J.T. Radiographic evaluation of juvenile periodontitis. Journal of Periodontology, 58:321, 1986.

10. Manson, J. Lehner, T. Clinical features of Juvenile periodontitis. Journal of periodontology, 636, 1994.

11. Clark, R. Page, R., Wilde, G. Defective Neutrophil Chemotaxis in Juvenile Periodontitis, 18:694, 1977.

12. Slots, J., Rosling, B. Suppression of periodontopathic microflora in localized juvenile periodontitis by systemic tetracycline. Journal of Clin. Periodontology, 10:465, 1983.

13. Tsai, C., McArthur, W., Baehni, P., Evian, C., Genco, R., Taichman, N. Serum neutralizing activity against actinobacillus actinomycetemcomitans leukotoxin in juvenile periodontitis. Journal of Clin. Periodontology, 8:338, 1981.

14. Carranza, Newman. Text book of Clinical Periodontology, eigth edition. W.B. Saunders, 1996.

15. Vrahopoulos, T. Liakoni, H., Barber P., Neuman HN. Ultrastructure of the periodontal lesion in a case of Papillon-Lefevre syndrome (PLS). J Clin. Periodontol, 15: 17, 1988.

16. Waldrop, T., Mackler, B., Schur P., Killoy, W. Immunologic study of human periodontosis (juvenile periodontitis). Journal of Periodontology, 52:8, 1981.

17. Saxen, L. Prevalence of juvenile periodontitis in Finland. Journal of Clinical Periodontology, 7: 177, 1980.

18. Page, R., Altman, L., Ebersole, J., Vandesteen, G., Dahlberg, W., Willams, B., Osterberg, S. Rapidly progressive periodontitis. A distinct clinical condition. Journal of periodontology, 54: 197, 1982.