Recurrent idiopathic gingival fibromatosis with generalized aggressive periodontitis: A rare case report.

Gingival fibromatosis is an enlargement of the gingival tissue, which may be localized or generalized, characterized by hypertrophy and proliferation of the connective tissue, predominantly Type I collagen, with occasional presence of an increased number of cells, supposedly fibroblasts. Gingival fibromatosis occurs more commonly as a side- effect of systemic drugs, such as phenytoin, cyclosporine, nifedipine, or due to hereditary factors. However, in some cases, it is idiopathic. We report one such case in 30 -year- old - female who presented with a complaint of swelling of gums since 2 months. This case appears to be of special interest because of the recurrent nature of gingival enlargement along with excessive osseous destruction.

INTRODUCTION

Gingival fibromatosis is a bizarre condition causing aesthetic, functional, psychological, and masticatory disturbance. It is characterized by progressive enlargement of the gingiva caused by an increase in the sub-epithelial connective tissue elements.[123] Causes of this enlargement are varied such as plaque accumulation, poor oral hygiene, inadequate nutrition, or hormonal stimulation. It may be related to long term intake of certain drugs such as nifedipine, phenytoin. It is also seen in several blood dyscrasias, e.g. leukemia, thrombocytopenia, or thrombocytopathy.[4]

Idiopathic gingival fibromatosis is a rare hereditary condition (1 in 750,000 people) characterized by a slow, progressive increase in the gingiva. It is also known as gingival elephantiasis, hereditary gingival hyperplasia, and gingival hypertrophy. This condition is generally diagnosed alone or occasionally in association with a number of syndromes, such as Zimmerman–Laband, Rutherfold and Ramon syndrome;[5] juvenile hyaline fibromatosis; or juvenile hyalinosis.[6] Isolated cases of gingival fibromatosis with amelogenesis imperfecta and aggressive periodontitis have also been reported.[78]

We report a case of idiopathic gingival fibromatosis with aggressive periodontitis and without any associated systemic findings, along with its management.

CASE REPORT

A 30-year-old female patient reported to the outpatient department of oral diagnosis with a complaint of recurrent swelling all over the gums since 2 years, which was gradual in onset and increased in size since 2 months. Along with this, there was difficulty in mastication, bleeding gums while brushing the teeth and bad breadth since 2 months. Patient reported similar swelling 2 years ago, for which surgical management in form of gingivectomy was carried out. The medical history was not contributory. Family history was also void of any genetic disorders.

On extra-oral examination, the patient had incompetent lips and convex profile. There was mild asymmetry of the face with fullness over both right and left side of face. On palpation, the swelling was soft to touch. Single, left submandibular lymph node was palpable, which was tender, soft and movable.

Intra-oral examination revealed massive generalized, diffuse, nodular enlargement of the gingiva involving both maxillary and mandibular arch [Figure 1]. It was firm and leathery in consistency. The gingiva appeared pink in color with generalized loss of stippling. Grade III mobility was seen with 17, 25, 26, 31, 32, 35, 37, 38, 41, 42 and Grade II mobility was observed with 34 and 43. Bleeding on probing and pus discharge was seen in association with majority of teeth. Posterior teeth were barely visible as the enlarged gingiva covered occlusal 1/3rd of crown [Figures 2 and 3]. Based upon clinical features, a provisional diagnosis of chronic inflammatory generalized gingivitis with advanced periodontitis was made.

Figure 1

Intra-oral photograph showing upper and lower arch

Figure 2

Intra-oral photograph showing upper right and left posterior teeth

Figure 3

Intra-oral photograph showing lower right and left posterior teeth

Radiographic examination revealed presence of all permanent teeth except 16 and 36 and severe generalized vertical and horizontal bone loss in maxillary and mandibular posterior teeth. The remaining teeth showed migration with spacing. Few carious teeth were noted [Figure 4].

Figure 4

Panoramic radiograph showing generalized bone loss

Blood chemistry was nonsignificant except for mild lymphocytosis and low hemoglobin level (9 g/dL). Red blood cell indices were within normal limits. Differential count showed mild lymphocytosis. No abnormality in morphology of cells was reported.

Excisional biopsy of gingiva was performed and subjected to histopathological examination. Histological features showed parakeratinized stratified squamous epithelium with elongated rete ridges at places. Connective tissue showed collagen fibers arranged in bundles and coalescing pattern. Marked chronic inflammatory cells were present [Figure 5].

Figure 5

Photomicrograph showing elongated rete ridges, collagen fibers arranged in bundles and chronic inflammatory infiltrate (H and E, ×10)

On the basis of medical, family, drug history, clinical, radiographic and histopathological findings, a final diagnosis of recurrent idiopathic fibromatosis with aggressive periodontitis was given.

Treatment plan included oral prophylaxis followed tetracycline irrigation and administration of chlorhexidine mouth wash and extraction of Grade III mobile teeth. Gingivectomy with flap surgery was planned subsequently for remaining teeth.

DISCUSSION

Gingival fibromatosis has been associated with a variety of factors, including medications, inflammation and systemic disorders. Gingival fibromatosis may occur as an inherited condition – hereditary gingival fibromatosis, as an iatrogenic gingival hyperplasia associated with medications such as nifedipine, phenytoin, verapamil and cyclosporine; or may be idiopathic. It can also be caused by inflammation and leukemic infiltration.[5] Gingival proliferation is caused by one or more causes. These includes an increase in proliferation of resident tissue fibroblasts, a reduced level of metalloproteinases synthesis (matrix metalloproteinases-1 and matrix metalloproteinase-2), resulting in low levels of extracellular matrix-degrading, an increase in collagen Type I production, heat-shock protein 47 production, and other extracellular matrix components.[4]

Idiopathic gingival fibromatosis is a rare hereditary condition that has no specific cause.[9] It is usually noticed during or after eruption of permanent dentition and occasionally during the eruption of the deciduous dentition. The enlargement is usually prominent on the labial and buccal surface of the gingiva, occasionally palatal and lingual surfaces are also involved. Severity varies from minimal enlargement of free gingiva to complete submersion of crown of the teeth. Pain is not the common symptom and bleeding is rare to occur unless underlying inflammation is present. At first, the gingiva is smooth and finely stippled, but with age they become coarser and may throw up papillary projection. The lesion more commonly involves the molar segments, especially upper molars. Labial enlargement leads to an open lip posture and mouth breathing, which in turn leads to further enlargement. The enlargement creates conditions favorable for the accumulation of plaque and material alba by accentuating the depth of the gingival sulci. In such cases, secondary inflammatory changes may obscure the preexisting noninflammatory enlargement. Idiopathic gingival enlargement is differentiated from chronic inflammatory hyperplasia by the absence of reddening of papillae, no loss of stippling and absence of pitting on pressure. The clinical appraisal of the patient and history of drug intake will aid in differential diagnosis.[10]

Histological features of gingival fibromatosis have been focused on the connective tissue alterations, which shows an increased amount of collagen fiber bundles running in all direction associated with few fibroblast and the epithelium shows elongated rete pegs.[1112]

The treatment of choice in this condition is gingivectomy/gingivoplasty to satisfy patient's aesthetic demands. However, considering the recurrence of the lesion, radical treatment with extraction of Grade III mobile teeth and gingivectomy was planned in our patient. Because the lesion has tendency to recur following repeated gingivectomy procedure, patient and family members tend to have increased psychological stress. Therefore, psychological counseling is required for both patients and close relatives.

CONCLUSION

This case highlights the unusual coexistence of nonsyndromic idiopathic gingival fibromatosis with generalized aggressive periodontitis. Diagnosis was based on clinical, radiographic, histopathologic, and hematological assessment. However, further research is needed to establish a syndromic association between the two conditions based on genetic evaluation and linkage studies, to improve prognosis if possible.