CLINICAL DENTISTRY AND RESEARCH 2012; 36(3): 54-58

Correspondence

Banu Özveri Koyuncu, DDS, PhDDepartment of Oral and Maxillofacial Surgery,

Faculty of Dentistry, Ege University,

Bornova, 35100 İzmir, Turkey

Phone: +90 232 3881108

Fax: +90 232 3398289

E-mail: banuozverikoyuncu@yahoo.com

Banu Özveri Koyuncu, DDS, PhD Assistant Professor, Department of Oral and Maxillofacial Surgery,

Faculty of Dentistry, Ege University,

İzmir, Turkey

Erdoğan Çetingül, DDS, PhDProfessor, Department of Oral and Maxillofacial Surgery,

Faculty of Dentistry, Ege University,

İzmir, Turkey.

Şükrü Kandemir, DDS, PhD Professor, Department of Periodontology,

Faculty of Dentistry, Ege University,

İzmir, Turkey

Taha Ünal, DDS, PhDProfessor, Department of Pathology,

Faculty of Dentistry, Ege University,

İzmir, Turkey

HEREDITARY GINGIVAL FIBROMATOSIS: A CASE REPORT

ABSTRACT

Hereditary gingival fibromatosis (HGF) is characterized by

the slowly progressive fibrous enlargement of gingival tissue

that causes aesthetic and functional problems. It is usually

transmitted as an autosomal dominant trait and develops as an

isolated disorder but can also be one of the features of various

syndromes. The present case report describes a Turkish family

with individuals having HGF for the last three generations.

Treatment consisted of surgical removal of the hyperplastic

fibrous tissue in a series of gingivectomies for 54-year-old

male patient who had generalized severe gingival overgrowth

covering almost all maxillary and mandibular teeth.

Key words: Gingival Enlargement, Gingivectomy, Hereditary Gingival Fibromatosis

Submitted for Publication: 09.21.2011

Accepted for Publication : 06.25.2012

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Hereditary GinGival Fibromatosis

INTRODUCTION

Hereditary gingival fibromatosis (HGF) is characterized

by a slowly progressive, non-hemorrhagic, fibrous benign

enlargement of maxillary and mandibular keratinized

gingiva, the main feature is mature collagenous connective

tissue growing. The hyperplastic gingiva may be localized

or generalized, usually having normal color and firm

consistency with abundant stippling, either unilateral

or bilateral.1,2 Histologically, gingival tissue from HGF is

characterized by a mucosa in which the epithelium shows

rete ridges extending into the underlying connective tissue,

which shows an increased density of collagen fibrils and a

decrease in fibroblasts.3

HGF may occur as an isolated finding or in association

with other systemic features as part of a syndrome such

as Zimmerman-Laband,4 Jones,5 Ramon,6 and Rutherford7

syndromes, Juvenile hyaline fibromatosis,8 systemic infantile

hyalinosis,9 and mannosidosis. 10

The condition is usually identified as an autosomal

dominant condition though recessive forms are also

described in the literature.11 Linkage studies have localized

for isolated, nonsyndromic autosomal dominant forms of

gingival fibromatosis to chromosome 2p21-p2212 and to

chromosome 5q13-q22 .13

The enlargement usually begins at the time of the eruption

of the permanent dentition, although it may also occur

during the eruption of deciduous teeth or even at birth.12

The enlarged gingivae may cover a portion or all the crowns

of teeth, thus potentially interfering with speech, closure of

the lips, and mastication.14 It may delay eruption of teeth

and make cleaning of the teeth virtually impossible. When

the gingiva is the only tissue involved, gingival fibromatosis

might be in association with hypertrichosis, and/or mental

retardation, and/or epilepsy.15

This case report presents HGF in a Turkish family for three-

generation.

CASE REPORT

A 54-year-old male presented with a complaint of excessive

gingival hyperplasia involving both the mandibular and

maxillary arches. On examination, a severe and generalized

diffuse gingival hyperplasia involving the marginal,

interdental and attached gingiva was observed covering

almost all the surfaces of crowns causing severe eating,

speaking and aesthetic problems (Figure 1). Gingiva

was pink with a firm and dense consistency. Poor Oral

hygiene was observed in response to tissue overgnowth.

Radiographic evaluation demonstrated severe alveolar bone

loss (Figure 2). The patient’s medical history has revealed no

signs of hypertrichosis or mental retardation, and no history

of epilepsy or intake of medication known to cause gingival

overgrowth. He referred to our clinic to have a better

esthetic appearance. The patient stated that he had such a

psychological and emotional stress until he reaches to this

age with a fair of surgery.

There was a family history of hereditary gingival overgrowth

as his mother and son had gingival enlargement, involving

the maxilla as well as the mandible. The patient revealed

that his mother and son had several gingival excisional

surgeries because of slowly progressing hyperplasia. None

of the individuals in the family had epilepsy or any type

of seizure disorder, and had not taken any medications

associated with gingival hyperplasia. The mother’s age is 79

and was operated three times about 40 years ago. The son

stated that he had several gingival excisional surgeries 12

years ago. The recurrence was seen after the surgeries in

both of them, the son and mother (Figures 3 and 4).

After giving detailed information to the patient about

the management and taking an informed consent, the

patient decided to initiate the treatment. Multiple surgical

procedures including maxillary and mandibular vestibular

and palatal/lingual gingivectomies, with reverse bevel

incisions, were performed under local anesthesia, to obtain

Figure 1. Patient with severe gingival hyperplasia in maxilla and mandible.

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CLINICAL DENTISTRY AND RESEARCH

Figure 3. Son’s intraoral photograph showing recurrence.

Figure 2. Orthopantomograph prior to operation.

Figure 4. Mother’s intraoral photograph showing recurrence in the mandibular cuspid-bicuspid area.

tissue showing bundles of collagen fibers. The patient

was diagnosed with gingival fibromatosis based on the

microscopic findings.

A 0.12% chlorhexidine gluconate rinse was prescribed for

administration twice a day for 2 weeks. The patient was

seen at 1, 3 and 4 weeks postoperatively. There were no

postoperative complications. After 30 months of follow-up,

poor oral hygiene was observed (Figure 6).

To achieve optimal esthetic appearance, we recommended

orthodontic and prosthetic treatment but the patient

refused these treatments. Also patient’s son refused any

treatment because of overgrowth of the gingival tissue

following the operations.

DISCUSSION

Hereditary gingival fibromatosis is rare, affecting only one in

750,000 people.16 It usually develops as an isolated disorder

but can be one feature of a syndrome.17 In this case, the

patient did not exhibit any signs or symptoms suggesting

that the condition was syndromic.

HGF can be inherited as an autosomal dominant though

recessive forms are described in the literature.13 The mode

of genetic transmission in our case, points to an autosomal

dominant gene, because family members (the patient’s

mother and son) of both sexes were affected.

Reports of the histologic, morphologic, and cellular

characteristics of gingival tissues from HGF patients

compared with controls are not always consistent, although

an increase in the amount of collagen is generally reported.18

In recent years, studies aimed to clarify the pathogenic

mechanisms of collagen accumulation in HGF gingivae

but there has been controversy about this mechanism:

gingival fibrosis is a result of increased collagen synthesis

and/or decreased degradation, or alteration in fibroblast

proliferation.19-21

The proliferation and expression of growth factors of

HGF keratinocytes are abnormal. However, the exact role

of keratinocytes in HGF pathogenesis is still unknown.

According to Meng et al.22 HGF keratinocytes have an

important role in HGF pathogenesis by inducing extracellular

matrix (ECM) accumulation by fibroblasts. These authors

reported that results strongly suggest that keratinocyte–

fibroblast interactions contribute to the pathogenesis of

HGF.

The hyperplastic gingiva has usually got a normal color

and firm consistency.2 The clinical presentation of HGF is

a smoother gingival contour (Figure 5). A biopsy sample of

the gingival tissues was submitted for histologic evaluation.

Microscopic examination of the specimens confirmed

for hereditary gingival hyperplasia. Sections showed a

hyperparakeratinized hyperplastic stratified squamous

epithelium with the underlying fibrous connective

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Hereditary GinGival Fibromatosis

variable in both the distribution (number of teeth involved)

and in the degree (severity) of expression. Buccal and

lingual tissue may be involved in both the mandible and

maxilla, and the severity of hyperplasia may range from

slight enlargement to total coverage of the dentition and

may vary between individuals within the same family.11 In

our case, the hyperplastic gingiva was of normal color and

had a firm consistency. The patient was not able to chew

especially at the anterior region, as the gingiva was almost

covering the maxillary teeth.

Gingival tissue enlargement usually begins with the

eruption of the permanent dentition. There are also cases

which have been reported to occur during the eruption of

the deciduous dentition; however it rarely appears at birth.16

Since recurrence could be expected within a few months

after surgery and may return to the original condition

within few years, the patient may undergo to repeated

gingivectomy procedures. Reports about recurrence rates

are conflicting,23-25 so the long-term benefit of periodontal

reduction surgery cannot be predicted. In severe cases of

hereditary gingival fibromatosis, full-mouth tooth clearance

has been advocated, as some evidence24,25 suggests that the

condition does not recur if the teeth have been extracted.

It was stated that there is less chance of recurrence if the

gingivectomy is delayed until the permanent dentition is in

place.26 However, one report recorded that, deciduous teeth

were retained and permanent teeth were entrapped in the

overgrown gingival tissue. Thus, delaying the gingivectomy

with the goal of avoiding recurrence was contraindicated.

These authors have also stated that as long as patient

is informed about the likelihood of recurrence, repeated

gingivectomies is generally well accepted and well tolerated.

On the other hand, Shekar27 reported that repeated

gingivectomy procedures often causes further increase

in the patients and parents’ psychological and emotional

stress.

Kelekis-Cholakis et al.28 stated that orthodontic treatment

might stimulate recurrence in some patients, especially

if periodontal hygiene is impeded by the orthodontic

appliance but they did not observe significant recurrence

during the first 3 years.

Poor oral hygiene is one of the factors that may aggravate

gingival enlargement as it is seen in the members of this

family. Therefore, the importance of postoperative follow-

up and adequate hygiene should be emphasized to HGF

patients.29,30

CONCLUSION

Hereditary gingival fibromatosis is a rare disease characterized by the proliferative fibrous overgrowth of the gingival tissue. Enlargements in the gingiva may cover the dental crowns resulting in both aesthetic and functional problems. Multiple surgical procedures, monthly periodontal examinations and prophylaxis are an essential part of the treatment protocol. However, additional effort by the patient is needed to cope with HGF.

REFERENCES

1. Gorlin RJ, Cohen MM, Levi LS, editors. Syndromes of the head and neck. 3rd ed. New York: Oxford Press; 1990.

2. Rushton MA. Hereditary or idiopathic hyperplasia of the gums. Dent Pract Dent Rec 1957; 7: 136-146.

3. Araújo CS, Graner E, Almeida OP, Sauk JJ, Coletta RD. Histomorphometric characteristics and expression of epidermal growth factor and its receptor by epithelial cells of normal gingiva and hereditary gingival fibromatosis. J Periodontal Res 2003; 38: 237-241.

Figure 5. Photograph following gingivectomy in the maxilla.

Figure 6. Clinical view showing poor oral hygiene after 30 months.

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4. Shah N, Gupta YK, Ghose S. Zimmermann-Laband syndrome with bilateral developmental cataract-a new association? Int J Paediatr Dent 2004; 14: 78-85.

5. Hartsfield JK Jr, Bixler D, Hazen RH. Gingival fibromatosis with sensorineural hearing loss: an autosomal dominant trait. Am J Med Genet 1985; 22: 623-627.

6. Pina-Neto JM, Moreno AF, Silva LR, Velludo MA, Petean EB, Ribeiro MV et al. Cherubism, gingival fibromatosis, epilepsy, and mental deficiency (Ramon syndrome) with juvenile rheumatoid arthritis. Am J Med Genet 1986; 25: 433-441.

7. Witkop CJ Jr. Heterogeneity in gingival fibromatosis. Birth Defects Orig Artic Ser 1971; 7: 210-221.

8. Bedford CD, Sills JA, Sommelet-Olive D, Boman F, Beltramo F, Cornu G. Juvenile hyaline fibromatosis: a report of two severe cases. J Pediatr 1991; 119: 404-410.

9. Landing BH, Nadorra R. Infantile systemic hyalinosis: report of four cases of a disease, fatal in infancy, apparently different from juvenile systemic hyalinosis. Pediatr Pathol 1986; 6: 55-79.

10. Berg T, Riise HM, Hansen GM, Malm D, Tranebjaerg L, Tollersrud OK et al. Spectrum of mutations in alpha-mannosidosis. Am J Hum Genet 1999; 64: 77-88.

11. Singer SL, Goldblatt J, Hallam A, Winters JC. Hereditary gingival fibromatosis with a recessive ode of inheritance: case reports. Aust Dent J 1993; 38: 427-432.

12. Hart TC, Pallos D, Bowden DW, Bolyard J, Pettenati MJ, Cortelli JR. Genetic linkage of hereditary gingival fibromatosis to chromosome 2p21. Am J Hum Genet 1998; 62: 876-883.

13. Xiao S, Bu L, Zhu L, Zheng G, Yang M, Qian M et al. A new locus for hereditary gingival fibromatosis (GINGF2) maps to 5q13-q22. Genomics 2001; 74: 180-185.

14. Clark D. Gingival fibromatosis. J Can Dent Assoc 1987; 80: 137-140.

15. Aldred MJ, Bartold PM. Genetic disorders of the gingivae and periodontium. Periodontol 2000; 18: 7-20.

16. Fletcher JP. Gingival abnormalities of genetic origin: preliminary communication with special reference to hereditary gingival fibromatosis. J Dent Res 1966; 45: 597-612.

17. Haytaca MC, Ozcelik O. The phenotypic overlap of syndromes associated with hereditary gingival fibromatosis: follow-up of a family for five years. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007; 103: 521-527.

18. Almeida JP, Coletta RD, Silva SD, Agostini M, Vargas PA, Bozzo L et al. Proliferation of fibroblasts cultured from normal gingiva

and hereditary gingival fibromatosis is dependent on fatty acid synthase activity. J Periodontol 2005; 76: 272-278.

19. Johnson BD, el-Guindy M, Ammons WF, Narayanan AS, Page RC. A defect in fibroblasts from an unidentified syndrome with gingival hyperplasia as the predominant feature. J Periodontal Res 1986; 21: 403-413.

20. Shirasuna K, Okura M, Watatami I, Hayashido Y, Saka M, Matsuya T. Abnormal cellular property of fibroblasts from congenital gingival fibromatosis. J Oral Pathol 1988; 7: 381-385.

21. Coletta RD, Almeida OP, Graner E, Page RC, Bozzo L. Differential proliferation of fibroblasts cultured from hereditary gingival fibromatosis and normal gingiva. J Periodontal Res 1998; 33: 469-475.

22. Meng L, Ye X, Fan M, Xiong X, Von den Hoff JW et al. Keratinocytes modify fibroblast metabolism in hereditary gingival fibromatosis. Archives of Oral biology 2008; 53: 1050-1057.

23. Cuestas-Carnero R, Bornancini CA. Hereditary generalized gingival fibromatosis associated with hypetrichosis: report of five cases in one family. J Oral Maxillofac Surg 1988; 46: 415-420.

24. Kharbanda OP, Sidhu SS, Panda SK, Deshmuck R. Gingival fibromatosis: study of three generations with consanguinity. Quintessence Int 1993; 24: 161-164.

25. Danesh-Meyer MJ, Holbrow DW. Familial gingival fibromatosis: a report of two patients. N Z Dent J 1993; 89: 119-122.

26. James PL, Prasad SV. Gingival fibromatosis: report of a case. J Oral Surg 1971; 29: 55-59.

27. Shekar I. Idiopathic gingival fibromatosis. Saudi Dental Journal 2002; 14: 143-145.

28. Kelekis-Cholakis A, Wiltshire WA, Birek C. Treatment and Long-term Follow-up of a Patient with Hereditary Gingival Fibromatosis: A Case Report. J Can Dent Assoc 2002; 68: 290-294.

29. Şahin S, Saygun NI. Hereditary gingival fibromatosis. GÜ Diş Hek Fak Derg 2008; 25: 45-50.

30. Bayar GR, Özkan A, Şençimen M, Altuğ HA, Gülses A, Günhan Ö. Idiopathic gingival fibromatosis: a case report. Gülhane Tıp Derg 2011; 53: 294-296.