Idiopathic gingival enlargement associated with generalized aggressive periodontitis in a 19-year-old female.

Gingival enlargement, one of the manifestations of gingival and periodontal disease, is also known as gingival overgrowth. Idiopathic gingival enlargement is a rare gingival overgrowth, which is of an undetermined cause. This unknown etiology has now been linked to specific genes and idiopathic gingival enlargement is at times referred to as hereditary gingival enlargement. This condition is a benign, slow growing proliferation of gingival tissues. Aggressive periodontitis is the rapid form of periodontal disease which is characterized by extensive periodontal tissue destruction, increased host-susceptibility toward periodontal disease progress and a genetic predilection toward disease occurrence. We present a rare case of idiopathic gingival fibromatosis associated with generalized aggressive periodontitis in a young female. The patient presented with classic clinical and radiographic presentation associated with gingival enlargement and aggressive periodontitis. The diagnosis was then confirmed by histopathological and neutrophil functions tests.

INTRODUCTION

Generalized gingival enlargement can have a variety of etiological factors, and it can at the same time also have an undetermined etiology. Since a few years increasing efforts have been made to understand the genetic, molecular and cellular basis of gingival enlargement. The generalized gingival enlargement with an unknown etiology is termed as idiopathic gingival enlargement (IGE). Idiopathic gingival enlargement is a rare occurrence and presents itself either as an isolated disorder or as a part of a syndrome. Several ongoing investigations to establish the exact genetic linkage and heterogeneity associated with the disease exist, however the exact etiology still eludes us.[1] IGF is now an established hereditary gingival enlargement (HGF) and the terms IGF and HGF are used interchangeably.[2345] Hereditary gingival enlargement displays both an autosomal dominant mode of inheritance in some patients and an autosomal recessive in other cases. Males and females are equally affected in this disease.

Clinically IGE is characterized by gingival overgrowth, pink colored gingiva which is firm in consistency, and non-hemorrhagic.[67] IGE can affect both deciduous as well as permanent dentition, however it has been shown to worsen during adolescence.[89101112] IGE is a slowly progressive benign overgrowth affecting all anatomic parts of the gingiva and causing esthetic and functional problems.[1131415161718]

Aggressive periodontitis is differentiated from chronic periodontitis on the basis of the age of onset of the disease, rapid rate of disease progression, nature and composition of associated subgingival microbiota, alterations in host immune response and a familial aggregation of diseased individuals.[192021] Aggressive periodontitis includes two distinct entities: Localized aggressive periodontitis and Generalized aggressive periodontitis. Generalized aggressive periodontitis appears clinically as interproximal attachment loss involving at least three permanent teeth other than the first molars and incisors. The disease is episodic in nature and is associated with a poor serum antibody response to infecting agents.[22] Various factors have been identified that increase the risk of developing aggressive periodontitis, including familial aggregation, single nucleotide polymorphisms, neutrophil functional defects, antibodies to specific bacteria, herpes virus infection, smoking, and stress.[21]

We report here a case of idiopathic gingival enlargement associated with generalized aggressive periodontitis.

CASE REPORT

A 19-year-old girl reported to our outpatient department with the complaint of swelling in her gums, inability to chew food and loosening of her teeth which had been progressively increasing over a period of time. The dental history revealed that the patient's gums had been enlarging since 6-7 years and a history of dental extraction due to severe mobility. Unfortunately, no records were present with the patient. Patient did not have any relevant medical history and was not under any anti-epileptic, anti-hypertensive, or immunosuppressive medications. There was no family history of similar presentation, with her case being the first in their family.

Intra-oral examination revealed generalized bleeding on probing with severe gingival overgrowth which was pink, firm, and fibrotic in consistency [Figures 1–4] and involving both arches. The patient had generalized Grade III tooth mobility. Generalized deep combined pockets with a mean-value of 10 mm, with clinical attachment loss with a mean value of 7 mm. Oral hygiene index was scored with a mean value of 2 and gingival index had a mean value of 1.6.

Figure 1

Pre-operative photograph showing the upper and lower anterior teeth

Figure 4

Pre-operative photograph showing the clinical probing depth

Pre-operative photograph showing left side posterior teeth

Pre-operative photograph showing the right side posterior teeth

Full mouth IOPA radiographs revealed generalized severe alveolar bone loss [Figures 5 and 6]. Multiple impacted teeth were also seen (13, 17, 18, 23, 28, 33, 37, 38, 43, 47, 48). The peripheral blood results were normal and correlated with an absence of any history of systemic disease. Hormonal tests revealed normal estrogen and progesterone levels.

Figure 5

Pre-operative IOPA X-rays

Figure 6

Pre-operative OPG

A portion of the excised gingiva was sent for histopathologic investigation [Figures 7–9]. H and E stained section shows parakeratinised stratified squamous epithelium, presence of a thickened acanthotic epithelium with elongated rete ridges, and with focal hyperplasia. Underlying connective tissue stroma was collagenous with numerous fibroblasts and showed mild inflammatory cell infiltrate. Few blood vessels were also evident. The phagocytic ability of the patient's polymorphonuclear leukocytic cells was assessed with a nitro-bluetetrazolium (NBT) reduction test and the results indicated the reduced phagocytic activity. Neutophil chemotaxis assay for assessing the chemotaxis of the PMN's was also within normal limits.

Figure 7

Surgical photograph showing the excised gingiva after extraction

Figure 9

Histopathological analysis of the gingival tissue

Surgical photograph taken immediately after suturing

On the basis of the history, examination and investigations diagnosis of generalized severe aggressive periodontitis associated with generalized idiopathic gingival enlargement was made, with unfavorable prognosis.[22]

Full mouth scaling and root planing along with systemic anti-microbial therapy was given. Anti-microbial therapy consisted of amoxicillin (500 mg thrice a day) and metronidazole (400 mg twice a day) for a period of 7 days.[23242526]

After 4 weeks, the patient was re-evaluted and her remaining teeth were extracted and/or dis-impacted in regular intervals except for the canines [Figures 10 and 11]. Complete healing of the site was followed by gingivectomy to remove the excess soft-tissue. We are in the midst of fabricating the implant supported over-denture for her.

Figure 10

Maxillary arch after 6 weeks of treatment

Figure 11

Mandibular arch after 6 weeks of treatment

DISCUSSION

The known causes of gingival overgrowth are inflammation, leukemia, drugs and inheritance. Systemic drugs known to cause gingival overgrowth are anti-seizure, immuno-suppresser, and anti-hypertensive drugs. The family, medical, and drug histories were noncontributory in our case; hence it was diagnosed as idiopathic gingival fibromatosis. IGF manifests due to congenital or hereditary causes which are not clearly understood. Hence, the terms idiopathic gingival fibromatosis and hereditary gingival fibromatosis are often used interchangeably.[27] It is usually associated with the eruption of the teeth, both primary and permanent dentition, and reduces or disappears with the loss of the teeth. The enlargement is firm, un-esthetic and usually covers the clinical crown of the teeth making the oral hygiene maintenance difficult. Hence, clinically the gingival enlargement is usually superimposed with gingival inflammation and in some susceptible hosts this may lead to periodontitis. However, our patient presented with minimum local deposits (OHIS-2) which was completely inconsistent with the extent of periodontal tissue destruction. Several impacted teeth seen in our case could be the delayed eruption of the permanent teeth, one of the common problems associated with gingival enlargement.[13] Our patient's case history shows that her gingival enlargement worsened during her adolescent growth-phase. It is well established that the sex-hormones influence the host response in the periodontal disease progression; however the exact mechanism of this influence on gingival overgrowth is still unknown.[272829]

IGF is also known to present a variable clinical picture with in the same family, and this could be the reason of a negative family history in our patient. However, since she is the eldest child, her siblings could present a similar finding with their permanent teeth, and a lack of dental awareness could be the reason for the false-negative history of HGF in her parents.[30] HGF shows both autosomal dominant as well as autosomal recessive modes of inheritance. It was only recently that the penetrance rate of HGF was calculated, thus quantifying the recurrence risk. The penetrance of HGF could vary from low with offspring recurrence rate of 0.078, and sibling recurrence rate of 0.085, to complete penetrance with half of the family being affected. The unaffected individuals are shown to transmit the disease in an autosomal dominant mode of inheritance.[313233] The HGF following the autosomal recessive mode of inheritance is usually accompanied with systemic alterations, hinting at a syndromic association. Hypertrichosis and/or mental retardation are the most common syndromic feature associated with gingival overgrowth.

Histological analysis of the biopsy sample revealed the presence of dense connective tissue fibrils and mild inflammatory cell infiltrate. Both these findings are consistent with those found in gingival fibromatosis.[34]

Aggressive periodontitis is a complex periodontal infection occurring in a susceptible host and caused by the biofilm. The host inflammatory response to the biofilm is primarily responsible for the loss of the periodontal attachment and the alveolar bone supporting the teeth. Localized and generalized aggressive periodontitis are two different and distinct diseases which differ in more than just the extent of the periodontal loss. Clinically, they differ in the number of teeth affected, and the pattern of damage. These diseases are associated with completely different subgingival microflora and they also have different genetic risk factors. Before the 1999 classification, age of onset was an important differentiating factor between the chronic and aggressive forms of the periodontal disease, and the 1999 classification deleted the age-dependent words such as adult and juvenile. However, the age is still an important factor that can be used to differentiate between the chronic and aggressive periodontitis. Given the same amount of attachment loss and supporting alveolar bone loss the patients with aggressive periodontitis are significantly younger than those with the chronic periodontitis, thus age can be used as a distinguishing feature in preliminary decision making. At 19 years of age our patient reported severe attachment loss and supporting alveolar bone loss. The local factors were inconsistent with the extent of the periodontal destruction, and there were no systemic contributing factor.[35] The patient's clinical presentation was unique with severe gingival overgrowth along with the loss of clinical attachment and supporting bone loss, leading to the diagnosis of idiopathic gingival enlargement in combination with generalized aggressive periodontitis.[36] The neutrophil function test performed for our patient demonstrated a defected neutrophil phagocytosis. Neutrophil defects play an important role in the development of aggressive periodontitis. The neutrophil function test consisted of a powerful in vitro assay to assess neutrophil function. In this assay, the precise contribution of phagocytes to the host defense is assessed by determining phagocytosis and killing of Candida spp. blastoconidia. This helps in monitoring and comparing host cell behavior upon challenge with periodontopathic bacteria. The value of this test for our patient was MPN-3 (mean particle number of ingested candida) which meant an abnormal phagocytic function and thus could be a possible factor in the occurrence of such widespread destruction of periodontal supporting tissues. Aggressive periodontitis shows genetic predisposition and has been linked to both autosomal as well as X-chromosomes.[37]

CONCLUSION

This case report presents a case of non-syndromic idiopathic gingival fibromatosis superimposed with generalized aggressive periodontitis and highlights the need for more research focusing on the etiology of such cases, and exploring the association between these two diseases.