|Year : 2015 | Volume
| Issue : 4 | Page : 175-178
Idiopathic elephantiasis gingivae with generalized aggressive periodontitis: A rare case report and literature review
Shahabe Saquib1, Varsha Jadhav2, Joel Koshi2, Mahesh Ahire3
1 Department of Periodontics and Community Dental Sciences, College of Dentistry, King Khalid University, Abha, Saudi Arabia
2 Department of Periodontology, Yogita Dental College and Hospital, Ratnagiri, Maharashtra, India
3 Department of Periodontology, S.M.B.T Dental College and Hospital, Sangamner, Maharashtra, India
|Date of Web Publication||17-Feb-2016|
Department of Periodontics and Community Dental Sciences, College of Dentistry, King Khalid University, Abha
Source of Support: None, Conflict of Interest: None
Idiopathic gingival enlargement is a rare entity with unknown etiology. Diagnosis of the case is of utmost important for the comprehensive treatment planning. In the present case, the clinical presentation and intervention of the patient reported with diffuse firm and nonedematous enlargement, disfigurement of the face, difficulty in speech, and mastication. Periodontal status showed severe attachment loss with minimal local factors, which is typical for generalized aggressive periodontitis. Timely detection of the disease with the critical planning of treatment and routine follow-up with good oral hygiene practices are good enough to combat the morbidity of this disease.
Keywords: Esthetic problem, generalized aggressive periodontitis, idiopathic gingival enlargement, speech and masticatory difficulty
|How to cite this article:|
Saquib S, Jadhav V, Koshi J, Ahire M. Idiopathic elephantiasis gingivae with generalized aggressive periodontitis: A rare case report and literature review. J Dent Res Rev 2015;2:175-8
|How to cite this URL:|
Saquib S, Jadhav V, Koshi J, Ahire M. Idiopathic elephantiasis gingivae with generalized aggressive periodontitis: A rare case report and literature review. J Dent Res Rev [serial online] 2015 [cited 2021 Mar 1];2:175-8. Available from: https://www.jdrr.org/text.asp?2015/2/4/175/176684
| Introduction|| |
Goddard and Gross (1856) first reported the case of idiopathic gingival enlargement (IGE).  Other proposed terminologies for the same disease are hereditary gingival fibromatosis, congenital familial fibromatosis, elephantiasis gingivae, diffuse fibroma, and familial elephantiasis. A study of several families found the mode of inheritance to be autosomal recessive in some cases and autosomal dominant in others  and has an incidence of 1:3,50,000.  Genetic loci for autosomal dominant forms of IGE have been localized to chromosome 2p21-p22 (IGE1) and chromosome 5q13-q22 (IGE2). According to the recent finding by Hart et al., a defect is reported in the Son of sevenless-1 gene on chromosome 2p21-p22 (IGE1) as a possible cause of this clinical presentation. 
The lesion can present as a localized or generalized enlargement of attached gingiva depending on the severity and extent of the disease. The enlargement usually begins with the eruption of the primary or secondary dentition and may regress after extraction, suggesting that the teeth (or the plaque attached to them) may be triggering factors. Cases of IGE show the pale pink color of the gingiva with quite firm and fibrous consistency and low tendency for bleeding. In most of the cases, enlargement is generalized and symmetric in form. In some rare cases, nodular asymmetrical and multiple enlargements are also seen. 
IGE can occur as a sole entity affecting only gingiva, or it may be associated with certain syndromes [Table 1]. The differential diagnosis for IGE is important because a number of diseases mimic the clinical features of IGE though clinically IGE can be diagnosed by its peculiar gingival features and positive family history, histological features may be of diagnostic help in addition to clinical and family history.
The generalized aggressive periodontitis is characterized by rapid and severe attachment loss. The disease is broadly classified into localized and generalized form. It affects the patients under the age of 30, but older ones may be affected. It had poor serum antibody response to infecting agents and pronounced episodic destruction of the attachment and alveolar bone. Hence, it affects at least three permanent teeth other than first molars and incisors.  The generalized aggressive periodontitis can be distinguished from chronic periodontitis by age of onset, rapid rate of progression of attachment loss, apparent lack of local factors, nature and composition of sub-gingival microflora, alterations in host immune response toward the bacterial challenge, and familial aggregation of the disease in individuals. ,,
| Case Report|| |
An 18-year-old female patient reported to the outpatient department of periodontology, with complaining of gingival swelling which compromised her speech, mastication, and esthetics and led to the considerable disfigurement of her facial profile. The patient reported that the gingival enlargement started with the eruption of permanent dentition, but progressed slowly to cover the parts of permanent dentition [Figure 1]. The patient's medical history seemed to be noncontributory to the development of gingival enlargement. The patient had no history of use of drugs, i.e., phenytoin, nifedipine, or cyclosporine, which are known to cause gingival enlargement. Family history revealed no such dental and periodontal abnormality in the family. Complete and differential blood counts were performed to detect any abnormality in the white blood cell, but blood smear showed a normal reading.
|Figure 1: Front view of the oral cavity showing diffuse gingival enlargement|
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The intraoral examination revealed generalized gingival overgrowth including both maxillary and mandibular arches extending into vestibular areas. The pink, leathery consistency tissue was firm on palpation and partly covered crowns of entire dentition. Gingival enlargement enclosed major surfaces of teeth of the left side of both arches illustrating grade III gingival enlargement [Figure 2] and [Figure 3]. Because of the severe enlargement, oral hygiene maintenance was a problem with the patient, which may act as a stimulus for further aggravation of disease. Severe gingival enlargement in the anterior region of the jaw resulted in anterior open bite, with incompetent lip seal and mouth breathing. As a known fact, mouth breathing leads to aggravate gingival and periodontal disease. The intra oral examination confirmed that the patient used to masticate unilaterally from the right side [Figure 2] and [Figure 3]. Gingival and periodontal examination revealed generalized deep periodontal pockets up to or more than 10 mm in depth with mean attachment loss of 7 mm. Generalized grade I mobility with severe malalignment of teeth. A scanty amount of plaque, calculus, and food debris was entrapped into the posterior teeth of the left side, which were partially visible with no significant inflammation present around them. Orthopentamogram revealed generalized bone loss up to middle third of the roots and malaligned teeth [Figure 4]. Incisional biopsy was taken for histopathological examination. Histopathology revealed hyperplastic epithelium with the underlying connective tissue showing the presence of dense thick collagen bundles with abundant fibroblasts [Figure 5]. Based on above findings, the diagnosis of "IGE with generalized aggressive periodontitis" was made.
|Figure 5: Histopathological section showing hyperplastic epithelium and dense collagen in the connective tissue|
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| Discussion|| |
The cause is unknown, and thus the condition is designated as "idiopathic." Some cases have a hereditary basis and genetic origin. IGE is one of the most common forms of gingival disease of genetic origin. IGE is characterized by fibrous enlargement of gingiva caused by an increase in submucosal connective tissue elements.  In cases of IGE, there is diffuse enlargement of gingiva affecting all three parts [Table 1]. Males and females are equally affected with no gender predilection.  Enlargement becomes evident with the eruption of teeth, either permanent or deciduous, suggestive of trauma-induced tissue reaction as the stimulating factor for the enlargement. ,,
IGE is mainly of two forms, i.e., nodular and symmetric. A combination of both the forms may also be seen. Most common type observed is "symmetric" type of enlargement. 
Generalized aggressive periodontitis is a complex periodontal infection generally affects healthy individual <30 years of age and characterized by rapid attachment loss. The main etiological factors responsible for the disease are susceptible host response and pathogenic biofilm. Typical clinical feature for the disease is lack of clinical inflammation in spite of deep periodontal pockets and minimal plaque deposits, which is inconsistent with the attachment loss, poor serum antibody response to pathogens, and elevated level of pathogens such as Porphyromonas gingivalis and Actinobacillus actinomycetemcomitans. The generalized aggressive periodontitis shows genetic predisposition and has been linked to both autosomal as well as X-chromosomes. 
In the present case, the patient reported with diffuse firm and nonedematous enlargement, disfigurement of the face, difficulty in speech, and mastication. The enlargement was predominant on the left side of both the jaws. The lesions showed minimal inflammatory component and no bleeding tendency. These findings were confirmed on histology, which showed dense collagen bundles with minimum inflammatory and vascular component. At 18 years of age, the patient reported severe attachment loss and supporting alveolar bone loss. The local factors were inconsistent with the extent of the periodontal destruction, and there was no systemic contributing factor. Clinical and histological examination of the patient leads to the diagnosis of IGE with generalized aggressive periodontitis.
Many authors agree to the same modality of treatment, but there are controversies among them regarding the exact time period during which the condition should be treated. According to majority of the authors, the best time period for treatment is when full permanent dentition is present in the oral cavity; since the recurrence rate is higher.  Treatment depends on the severity of the condition. In mild cases thorough scaling and home care is sufficient to maintain gingival health. In cases of severe overgrowth surgical removal by gingivectomy or undisplaced flap is the treatment of choice. Recurrence rate is unpredictable, and it is supposed to be dependent on oral hygiene maintenance by the patient.
| Conclusion|| |
IGE is not an uncommon type of gingival enlargement. The diagnosis of this disease is often achieved by excluding all probable etiologies of gingival enlargement. Timely detection of the disease with the critical planning of treatment and routine follow-up with good oral hygiene practices are good enough to combat the morbidity of this disease.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]