Athetosis

Gingival fibromatosis represents the fibrous hyperplasia of the gingival tissue. Clinical examination reveals enlargement of buccal and palatal gingival tissue. Many forms of gingival fibromatosis are of unknown etiology and termed as idiopathic gingival fibromatosis. However, several authors use various terms such as gingivomatosis and elephantiasis to describe these lesions. Our aim in this case report is to present five patients (one female, four males) with unusual clinical forms of gingival hyperplasia and to discuss the histopathological and clinical features in comparison to similar enlargements. Clinical examinations did not reveal increased periodontal pocket depths, besides plaque and gingival index scores were found to be in normal range. All of the patients were systemically healthy and were not subject to medications, which could lead to gingival hyperplasia. Additionally, clinical appearance of the lesions did not show any signs of trauma. Excisional biopsies were performed in all cases. The pathological examinations of the specimens demonstrated fibroconnective tissue characteristics, which were in accordance with the clinical appearance of all patients. Lesions healed successfully without sequelae or infection, and no recurrence was observed after 1-year follow-up.

Introduction

Gingival fibromatosis frequently presents itself as a generalized and irregular enlargement of the attached and marginal gingiva both in the facial and lingual aspects. Various sorts of drugs (immunosuppressants, anticonvulsants, and calcium channel blockers)12 or genetic disorders3-5 can lead to gingival enlargement. However, numerous nonfamilial examples of this condition are recognized and classified as idiopathic.6-9 Gingival fibromatosis, also known as elephantiasis gingiva, hereditary gingival hyperplasia, idiopathic fibromatosis, and hypertrophied gingiva, is a rare condition characterized by slow, progressive enlargement of the gingival.10-12 The hyperplastic gingiva usually presents normal color and firm consistency, with abundant stippling.

This entity is transmitted in an autosomal dominant trait and has been described as predominantly hereditary in nature.13 However, rare autosomal recessive inheritance can also be coincided.14-17 The lesions were described as occurring only in the molar regions of one or both jaws, with a predilection for the gingiva, extending from the molars to the tuberosity or retromolar region. This anomaly is classified as two types according to its form. The localized nodular form is characterized by the presence of multiple enlargements in the gingiva. The most common symmetric form results in uniform enlargement of the gingiva, although its etiology is unknown. Both forms may vary in shape and volume and cover the dental crowns.14 When located in the palate, the gingival masses are generally pear-shaped, with a pedunculated attachment to the underlying gingiva.

Histologically, the fibrous connective tissue presents bundles of coarse collagenous fibers, a high degree of differentiation in premature fibroblasts, and scarce blood vessels. Moreover, dense epithelium with elongated papillae and hyperkeratosis is observed.18-20 Small calcified particles, islands of osseous metaplasia, ulceration of the overlying mucosa, deposition of amyloid, and islands of odontogenic epithelium have also been reported in previous cases.15

Treatment consists of surgical excision of the hyperplastic tissue to restore gingival contours. The most commonly used method of removing large quantities of gingival tissue is the conventional external bevel gingivectomy. However, carbon dioxide laser has also been used in some studies.21,22

In this report, we aimed to present five patients with gingival enlargements in palatal and mandibular molar regions and to discuss the histopathological features.

Case Reports

Five patients (one 36-year-old woman and four men 20, 21, 25, 27 years old) were referred to the Department of Periodontology at different times complaining about esthetic deformities in their maxillary palatal region. No prolonged medications, anorexia, weight loss, seizures, or hearing loss following the anamnesis of the patients were noted. Further questioning revealed that none of their family members was affected with any form of gingival enlargement, nor was there any familial history of hypertrichosis, mental retardation, or epilepsy.

Microbial dental plaque accumulation was not evident in any of the patients at clinical examination, and gingival index score averages were approximately lower than 1. Additionally, probing pocket depth measurement scores ranged between 2 and 3 mm. Interproximal bone loss was unremarkable in panoramic radiographs.

The lesions were observed unilaterally or bilaterally only in the premolar-molar region of the maxillae. They were firm, fibrous, and covered with smooth, relatively pale oral mucosa. An undercut area was present below the lesions in which a dental floss could be passed (Figs. 1-5). The patients declared that the swellings had begun to appear when they were between 16 and 18 years old. Additionally, the patients had no specific clinical complaints despite the presence of the gingival masses. However, the decision for total excision of the lesions was made due to speech and esthetic problems the patients had. The lesions of all patients were almost kidney-shaped and 3 to 5 cm in horizontal dimension (Fig. 6).

The patients had no complaints after the excision of lesions (Fig. 7). After 1 week, surface epithelialization of surgical sites was almost completed (Fig. 8). Clinical examination revealed complete healing of the surgical sites and at the end of the first year no evidence of recurrence was observed (Fig. 9). Furthermore, the phonation and the chewing functions of the patients were improved.

Pathological Findings

Histological examination of all samples revealed a fibroconnective tissue with extensive collagen bundles and rete ridges, which was consistent with the clinical appearance. Additionally, numerous multinucleated stellate and fusiform fibroblasts were observed. No ulceration and atypical cells in the oral epithelium were observed. The overlying surface of the epithelium exhibited hyperkeratosis, acanthosis, and elongation of rete ridges and mononuclear infiltration of plasma cells and lymphocytes. Normal vascular structure was present in the subepithelial area. Mixoid degeneration and hyalinization of the fibrous tissue were found to be correlated with the long-term appearance of the lesions. The histological features were found to be consistent with the clinical features of the lesions and the diagnosis of symmetrical gingival fibromatosis (Fig. 10-13).

Discussion

This article reports five cases of gingival fibromatosis. Two cases presented bilateral palatal gingival enlargement and the other three were in unilateral form. These distinct types of enlargements observed resembled other types of gingival enlargements due to leukemic infiltration12 and to the use of drugs such as phenytoin, cyclosporine, and calcium channel blockers.1 Furthermore, the overgrowth of the gingiva could have been a feature of several multisystem syndromes, such as Laband syndrome (ear, nose, bone, and nail defects with hepatosplenomegaly),23 Rutherfurd syndrome (corneal dystrophy, mental retardation, and impairment of dental eruption by radicular resorption), or Cross syndrome (microphthalmia, mental retardation, athetosis, and hypopigmentation).24 Additionally, the similar clinical appearance of these lesions to hereditary gingival flbromatosis (HGF) might have led to misdiagnosis. These complications in the clinical diagnosis necessitates a detailed anamnesis of the patients. Past medical history can eliminate the probability of drug-induced enlargements. Histopathological investigation may also help in clarifying the existence of neoplastic leukocytes, which are abundant in leukomic gingival hyperplasia. As to our consideration, the most difficult part in the differential diagnosis could be the presence of HGF, which is frequently associated with hypertrichosis, mental retardation, epilepsy, and familial disturbance.24,25 The reported lesions were located not only in the palatal molar region but also in the buccal gingiva of both jaws. Moreover, the association with hypertelorisim might also have been present.15 The patients reported in this article were systemically healthy and had no familial history gingival enlargement. Furthermore, no signs of the syndromes listed above and the characteristics of HGF were noted. These syndromes and HGF usually affect the primary dentition or less frequently begins at the time of permanent dentition. This was not the case in our patients when their past medical history was scrutinized. Idiopathic gingival fibromatosis seemed to be the most suitable diagnosis for our cases with regard to clinical findings.

Rushton8 evaluated several gingival flbromatosis cases and first used the term "symmetrical fibroma" to describe this condition. According to this author, these lesions occurred only in the molar region of a single jaw or both jaws, extending from the molars to the tuberosity. The lesions were pear-shaped, firm, fibrous, and covered with smooth healthy mucosa, which tended to protrude in their natural course of development and when fully formed an undercut space occurred above. These were observed in localized and generalized forms. In contrast to the generalized form, the majority of the patients with localized lesions exhibited relatively late clinical onset in the second decade of their life span (from the age of 10 years to late adolescence) and those forms were not generally hereditary in nature.

In clinical examination of our patients, the localization and the shape of the lesions were similar to those described by Rushton.8 However, three patients had unilateral palatal enlargements. Jorgenson and Cocker26 noted that the localized and generalized forms of gingival flbromatosis could be of genetic origin and the localized form was not always symmetrical. They suggested that the term of focal gingival flbromatosis in lieu of the previously proposed designation would be suitable. The description of these authors seemed to be more accurate to our cases. Rushton8 described the presence of multinucleated giant cells in samples of generalized and localized gingival flbromatosis and considered these cells as fibroblasts. The cells were observed in the areas of mucoid degeneration. These histopathological results were also observed the patients in this case report. However, Giles and Agnew27 and Gould and Escobar13 described three cases of bilateral gingival flbromatosis lacking multinucleated giant cells. Stellate and multinucleated cells have been reported in several other oral mucosal and skin lesions, including giant cell fibroma,28 retrocuspid papilla,29 and irritation fibroma.29 Gould and Escobar13 stated that the identification of these cells could not be helpful for differential diagnosis and accepted as a unique finding. Nevertheless, the clinical features and histopathological appearance of the lesions reported in this article do not correspond to those of idiopathic gingival flbromatosis. Additionally, a dense collagen accumulation in fibrous tissue, hyalinization, the presence of premature granulation tissue, and the absence of acute inflammatory cells may indicate that our cases are histologically different from the irritation fibroma. In our opinion, stellate and multinucleated giant cells may play a definitive role in the histopathological diagnosis.

Conclusions

Idiopathic gingival flbromatosis is characterized by the proliferative fibrous overgrowth of the gingival tissue with varying degrees of involvement. Basically, the idiopathic enlargements do not cause any complaint in patients, except for the impaired appearance and speech and functional disturbances. The problems often necessitate surgical intervention and recurrence is not predicted. However, the etiology of these lesions still remains indefinite, which makes further studies necessary in understanding the underlying etiology.

References

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Guarantor: Lt Com Ahmet Eralp Akca

Contributors: Lt Com Ahmet Eralp Akca*[dagger]; Lt Col Kerim Ortakoglu[double dagger]; Maj Levent Pikdöken§; Lt Col Salih Deveci¶

* Department of Periodontology, Center of Dental Sciences, Gulhane Military Medical Academy, 06018 Etlik. Ankara. Turkey.

[dagger] Reprints: Dr A. Eralp Akca, Gulhane Askeri Tip Akademisi Dishekimligi Bilimleri Merkezi Periodontoloji AD 06018 Etlik, Ankara, Turkey; e-mail: akcaeralp@hotmail.com or akcaeralpy@yahoo.com.

[double dagger] Department of Oral Surgery. Center of Dental Sciences, Gulhane Military Medical Academy, 06018 Etlik, Ankara, Turkey.

§ Department of Periodontology. Haydarpasa Hospital, Gulhane Military Medical Academy, 06018 Etlik, Ankara, Turkey.

¶ Department of Pathology, Gulhane Military Medical Academy, 06018 Etlik, Ankara, Turkey.

This manuscript was received for review in January 2004. The revised manuscript was accepted for publication in September 2004.

Reprint & Copyright © by Association of Military Surgeons of U.S., 2005.

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