Idiopathic Gingival Fibromatosis Associated with Generalized Aggressive Periodontitis: a Case Report
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Pratique CLINIQUE Idiopathic Gingival Fibromatosis Associated with Generalized Aggressive Periodontitis: A Case Report Auteure-ressource Rashi Chaturvedi, MDS, DNB Dre Chaturvedi Courriel : rashichaturvedi@ yahoo.co.in SOMMAIRE La fibromatose gingivale idiopathique est un état génétiquement hétérogène, qui se caractérise par une prolifération lente et bénigne des tissus gingivaux. Cet état fait habi- tuellement partie d’un syndrome et est rarement isolé. La parodontite agressive est un autre trouble du parodonte d’origine génétique, qui entraîne généralement une des- truction rapide et marquée des tissus de soutien des dents. La sensibilité accrue de la population hôte atteinte de parodontite agressive pourrait être due à l’effet combiné de plusieurs gènes et à leurs interactions avec divers facteurs environnementaux. Des anomalies fonctionnelles des neutrophiles ont aussi été mises en cause dans l’étiopatho- génie de la parodontite agressive. Nous présentons un rare cas de fibromatose gingivale idiopathique non syndromique associée à une parodontite agressive généralisée. Notre diagnostic repose sur une évaluation clinique et radiologique, des observations histopa- thologiques et une analyse immunologique de la fonction des neutrophiles par un test de réduction au nitro bleu de tétrazolium. Nous décrivons une démarche interdisciplinaire pour le traitement du patient. Pour les citations, la version définitive de cet article est la version électronique : www.cda-adc.ca/jcda/vol-75/issue-4/291.html diopathic gingival fibromatosis is a rare her- ally nonsyndromic, have been genetically linked editary condition that has no specific cause.1 to the chromosome 2p21-p222 and 5q13-q22.4 IInvestigations are ongoing to establish the Recently, a mutation in the son of sevenless-1 genetic linkage and heterogeneity associated (SOS-1) gene has been suggested as a possible with it.2–5 This condition may manifest as an cause of isolated (nonsyndromic) gingival fi- autosomal-dominant6–9 or, less commonly, an bromatosis, but no definite linkage has been autosomal-recessive mode of inheritance,10–12 established.5 either as an isolated disorder or as part of a Idiopathic gingival fibromatosis is a slowly syndrome.6 Syndromic gingival fibromatosis progressive benign enlargement that affects the has been associated with ancillary features such marginal gingiva, attached gingiva and inter- as hypertrichosis, mental retardation, epilepsy, dental papilla. The fibromatosis may potentially progressive sensorineural hearing loss and ab- cover the exposed tooth surfaces, causing es- normalities of the extremities, particularly of thetic and functional problems, and in extreme the fingers and toes.6,13 Autosomal-dominant cases may distort the jaws. The gingival tissues forms of gingival fibromatosis, which are usu- are usually pink and nonhemorrhagic, and have JADC • www.cda-adc.ca/jadc • Mai 2009, Vol. 75, No 4 • 291 ––– Chaturvedi ––– Figure 1: Preoperative intraoral frontal view Figure 2: Preoperative intraoral right lat- Figure 3: Preoperative intraoral left of the maxillary and mandibular arches. eral view of the maxillary and mandibular lateral view of the maxillary and man- arches. dibular arches. a firm, fibrotic consistency.1,13–17 Histopathologically, the Case History bulbous increased connective tissue is relatively avascular Diagnosis and has densely arranged collagen-fibre bundles, num- A 23-year-old man reported to the outpatient depart- erous fibroblasts and mild chronic inflammatory cells. The ment of our institute complaining of pain and swelling in overlying epithelium is thickened and acanthotic, and has the gums and an inability to chew food. The patient was also elongated rete ridges.1,13–18 Unusual findings include the pres- concerned about the progressively increased spacing be- ence of small calcified particles, amyloid deposits, islands of tween his upper front teeth and their movement away from odontogenic epithelium and osseous metaplasia in the con- their original positions. nective tissue, and ulcerations of the overlying mucosa.19 The medical history revealed that the patient’s gums Aggressive periodontitis comprises another group of had progressively enlarged over the last 6 to 7 years and genetically inherited diseases that represent a severe and rapidly progressive form of periodontitis. Characteristically, that the patient had pain during mastication and in- these diseases tend to exhibit familial aggregation and there creased tooth mobility, especially of all 4 first molars, are no factors in the medical history that seem associated 3 of which had to be extracted about 2 years before. Nothing with the condition.20 in the patient’s medical and family history seemed related Aggressive forms of periodontitis are currently consid- to the condition, and no associated endocrinological ab- ered to be multifactorial diseases that develop as a result normality could be detected. The patient was not receiving of complex interactions between specific host genes and any anti-epileptic, antihypertensive or immunosuppressive the environment. Various factors have been identified that medications that could contribute to the gingival enlarge- increase the risk of developing aggressive periodontitis, in- ment. However, the patient had smoked up to 20 cigarettes cluding familial aggregation, single nucleotide polymorph- a day for the past 4 to 5 years and drank alcohol about 2 or isms, neutrophil functional defects, antibodies to specific 3 times a week. bacteria, herpes virus infection, smoking and stress.21 Intraoral examination revealed moderate-to-severe gin- Generalized aggressive periodontitis usually affects gival overgrowth of a firm, dense and fibrotic consist- people under 30 years of age. They have a poor serum-anti- ency that involved both the maxillary and mandibular body response to infecting agents and pronounced episodic arches (Figs. 1 to 3). The patient had generalized tooth destruction of the attachment and alveolar bone. The loss of mobility with a severe pathologic migration, especially attachment affects at least 3 permanent teeth other than first of the upper anterior teeth. Full-mouth periodontal charting, molars and incisors.22 including assessment of probing depth and clinical attach- We report here an unusual case of a nonsyndromic, ment level, revealed deep pockets throughout the mouth, idiopathic gingival fibromatosis associated with general- and scanty plaque and calculus deposits. ized aggressive periodontitis. Only one other case with The radiographic findings, which corroborated those such an association has been reported to date.18 We of the clinical examination, revealed severe generalized al- discuss our patient’s clinical findings, histopathologic veolar bone loss (Fig. 4). The peripheral blood results were evaluation, analysis of neutrophil function and treatment normal and correlated with an absence of any history of plan. systemic disease. 292 JADC • www.cda-adc.ca/jadc • Mai 2009, Vol. 75, No 4 • ––– Idiopathic Gingival Fibromatosis ––– Figure 4: Preoperative panoramic radiograph of the maxillary and Figure 5: Gingival tissues excised during mandibular arches showing severe generalized alveolar bone loss. periodontal surgery were sent for histo- pathologic assessment. Figure 6: Photomicrograph of the Figure 7: Photomicrograph of the con- Figure 8: Photomicrograph illustrating the specimen illustrating the presence nective tissue illustrating the presence of presence of dense collagen bundles in the of a thickened acanthotic epithelium densely arranged collagen bundles with connective tissue with multiple areas of with elongated rete ridges and a numerous fibroblasts and few mixed inflam- neovascularization (original magnification densely fibrous connective tissue with matory cells, mainly around blood vessels 100×). numerous fibroblasts (original magni- (original magnification 400×). fication 100×). Based on all these findings, a provisional diagnosis of Analysis of Neutrophil Function idiopathic gingival enlargement with generalized aggressive The phagocytic ability of the patient’s polymorphonu- periodontitis was made. clear leukocytic cells was assessed with a nitro-blue- Histopathologic Findings tetrazolium (NBT) reduction test. During this test, The attached gingiva excised from the buccal and inter- polymorphonuclear leucocytes (PMNLs) were stimulated dental areas during surgery (Fig. 5) was immediately fixed with heat-killed spores of Candida albicans to evaluate in 10% buffered formaldehyde solution and sent for histo- the oxidative pathway. Normal granulocytes metabolically pathologic examination. The specimen was processed and activated by phagocytosis reduce NBT to blue formazan. then embedded in paraffin wax. Multiple 5-mm serial sec- Eighty percent of the patient’s cells contained reduced blue tions were prepared, stained with hematoxylin and eosin, formazan, which was the percentage of PMNLs producing a and viewed under a trinocular light microscope with flat- respiratory burst, thereby indicating the normal functioning field objective lenses at 100× and 400× magnifications. of the oxygen-dependent leukocytic bactericidal system. The microscopic evaluation of these sections revealed parakeratinized stratified squamous acanthotic epithelia Treatment with thin long rete ridges extending into the connective The patient initially underwent phase 1 periodontal tissue. The underlying connective tissue showed dense therapy that comprised scaling and root planing, oral wavy bundles of