Oct 08, 2018 idiopathic gingival fibromatosis is a common, slowlyprogressing, benign condition of the gums. It is described variously as fibromatosis gingivae, gingivostomatitis, hereditary gingival fibromatosis, idiopathic fibromatosis, familial elephantiasis, and diffuse fibroma. Combining these studies with our findings, we believe that dental plaque. Clinically, it is characterized by benign gingival enlargement with normal color and firm consistency and nonhemorrhagic symptomatic illness 2. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth. In severe cases, the excess tissue may cover the crowns of the teeth, thus causing functional, esthetic, and periodontal problems, such as bone loss and bleeding, due to the presence of. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 2.
Kemoli summary hereditary gingival hyperplasia hgf is a rare condition characterised by hyperplastic, dense. Hereditary gingival fibromatosis hgf or idiopathic gingival fibromatosis is a rare, benign, asymptomatic, nonhemorrhagic and nonexudative proliferative fibrous lesion of gingival tissue occurring equally among men and women, in both arches with varying intensity in individuals within the same family 1. A difference in the healing patterns of the two patients is described, and a possible reason for this difference is offered. It causes teeth being partially or totally covered by enlarged gingiva, causing esthetic and functional problems.
Hereditary gingival fibromatosis is a rare benign oral condition which is characterized by slow and progressive enlargement of the maxillary and mandibular arches covering the teeth to various extents. Oral pathology general section hereditary gingival fibromatosis report of a family s. Hgf is characterized by a slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and. Hereditary gingival fibromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. Archived tissue specimens of hereditary gingival fibromatosis, gingival fibromatosis and dental abnormality syndrome and normal gingiva were subject to morphological analysis and immunohistochemical staining. Sep 26, 2018 hereditary gingival fibromatosis hgf is a common, slowlyprogressing, benign condition of the gums. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and rarely heredity. Hereditary gingival fibromatosis is associated with either autosomal dominant such as rutherford 22 and laband syn. Hereditary gingival fibromatosis and sensorineural healing loss in a 42year old man with jones syndrome.
Hereditary gingival fibromatosis hgf is a rare gingival lesion 1 in 7,50,000 that presents as localized or generalized fibrotic enlargement of the attached gingivae as well as the gingival margin and interdental papillae. Wegeners granulomatosis is a vasculitides in which the gingival enlargement takes a specific texture and colour of a strawberry and is thus known as strawberry like gingivitis. It may be either hereditary or idiopathic, with hereditary examples occurring as an isolated finding or as part of a syndrome e. Hereditary gingival fibromatosis hgf is the most common. Gingival fibromatosis, 1 genetic and rare diseases. Hereditary gingival fibromatosis in a family with the. Introduction hereditary gingival fibromatosis is a slowly progressive benign enlargement that affects the marginal gingiva, attached gingiva and interdental papilla 1. A family exhibiting the characteristics of hereditary gingival fibromatosis has been presented. Hereditary gingival fibromatosis pubmed central pmc. The most common genetic form, hereditary gingival fibromatosis, is usually transmitted as an autosomal dominant trait. The condition may occur as an isolated disorder or as a part of a syndrome. Gingival enlargement is defined as an overgrowth or increase in size of gingivae.
Hereditary gingival fibromatosis hgf is a rare gingival. Education and motivation of patient should be properly done and is essential for maintenance of proper oral hygiene and regular recall visits 15. In early stages, this gingival enlargement is usually located in the area of the interdental papilla. Hereditary gingival fibromatosis hgf is the most common genetic form of gingival fibromatosis. Generalized gingival enlargement can be caused by a variety of etiological factors. Hereditary gingival fibromatosis is characterized by a slow benign enlargement of gingival tissue. While the specific cause of this disorder remains a mystery, medical professionals have observed that hgf tends to repeatedly. Abstract gingival fibromatosis is characterized by gingival tissue overgrowth of a firm and fibrotic nature. The clinical presentation of hgf is variable, both in the distribution number of teeth involved and in the degree severity of expression. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and heredity, rarely. Two missense mutations in kcnq1 cause pituitary hormone.
Pdf hereditary gingival fibromatosis hgf is an uncommon condition. Although the gingival enlargement does not directly affect the alveolar bone, the gingival. Hereditary gingival fibromatosis specifically occurs in the absence of other identifiable medical syndromes or medications known to cause gum overgrowth. Hereditary gingival fibromatosis may present as an autosomaldominant or less commonly autosomalrecessive mode of inheritance. According to these data, a diagnosis of hereditary gingival fibromatosis was established. In the present case the method of treatment implied was surgery by scalpel method along with oral prophylaxis. Rest finalexontruncating mutations cause hereditary gingival. Gingival hyperplasia is a bizarre condition causing esthetic, functional, psychological, and masticatory disturbances of the oral cavity. Case report hereditary gingival fibromatosis a case report. Idiopathic gingival fibromatosis associated with generalized. The patient was recalled 6 weeks post surgery, gingival swelling was still evident in relation to maxillary left posterior region where the teeth had been extracted. It was previously called elephantiasis gingivae, hereditary gingival. The gingival enlargement in hgf can be so severe that it can cover the crowns of teeth completely, causing severe functional derangement and facial disfigurement.
Gingiva fibromatosis an overview sciencedirect topics. It is usually transmitted both as autosomal dominant trait and autosomal recessive inheritance although sporadic cases are commonly reported. Hereditary gingival bromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. It has also been called hereditary gingival overgrowth, idiopathic gingival overgrowth, elephantiasis gingiva, hypertrophied gingiva and hereditary gingival hyperplasia. Hereditary gingival fibromatosis, gingival enlargement, gingivectomy. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Gingival overgrowth go or gingival enlargement refers to important changes of gums. Hereditary gingival fibromatosis hgf is a rare gingival lesion 1 in 7,50,000 that presents as localized or generalized fibrotic enlargement of the attached gingivae as well as the gingival.
Hereditary gingival fibromatosis how is hereditary gingival. By haplotype construction and analysis in a 5generation chinese family segregating autosomal dominant hereditary gingival fibromatosis, ye et al. Gingival fibromatosis, 2 genetic and rare diseases. Hereditary gingival fibromatosis hgf is a rare disease in both autosomal dominant and recessive types and has a frequency of 1. Clinical science minichromosome maintenance 2 and 5. Gingival fibromatosis is a rare disease, which can be seen w. Gingival fibromatosis is a rare condition in which accumulation of extracellular matrix components causes slowly progressive enlargement of the gingiva. If a systemic disease or syndrome is suspected, the patient is directed to a geneticist for additional clinical examination and specialized diagnostic tests. Gingival fibromatosis can be caused by number of factors, e. Pdf hereditary gingival fibromatosis is a rare, genetically inherited overgrowth condition that is clinically characterized by a benign fibrous. A thorough physical examination and a complete medical history. Hereditary gingival fibromatosis med oral patol oral cir bucal. Effect of treatment of hereditary gingival fibromatosis on. Hereditary gingival fibromatosis is a rare, genetically inherited overgrowth condition that is clinically characterized by a benign fibrous enlargement of maxillary and mandibular keratinized gingiva.
Pdf hereditary gingival fibromatosis associated with. Hereditary gingival fibromatosis with distinct dental. Such features are absent in hereditary gingival fibromatosis. Shaban, elyan al machot, thomas hoffmann department of periodontology, tu dresden, germany introduction fig. We present case reports of two patients first cousinsan 8yearold girl and a yearold boywith an existing diagnosis of hgf, who were assessed. Hereditary or idiopathic hyperplasia of the gums, ele phantiasis gingivac, fibromatosis gingivae, symmetrical fibroma of the palate, and. Hereditary gingival fibromatosis hgf is a rare gingival lesion 1 in 7,50,000 that presents as localized or generalized fibrotic enlargement of the attached. Hereditary gingival fibromatosis hgf is an extremely rare oral health condition which affects gum tissue and can often lead to severe discomfort and leave those affected unable to eat. Gingival fibromatosis is a rare and heterogeneous group of disorders that develop as slowly progressive, local or diffuse enlargements within marginal and attached gingiva or interdental papilla. Hereditary gingival fibromatosis hgf is a rare disorder characterized by a benign, nonhemorrhagic, fibrous gingival overgrowth that can appear in isolation or as part of a syndrome. Clinically, a pink gingiva with marked stippling can be seen to cover almost all the tooth, in many cases preventing. Hereditary gingival fibromatosis also referred to as idiopathic gingival hyperplasia, elephantiasis gingivae etc. Hereditary gingival fibromatosis a case report and.
Epithelialmesenchymal transition a possible pathogenic. Unusual clinical and histologic findings in a child with mixed. Hereditary gingival fibromatosis autosomal dominant. Gingival enlargement, gingival fibromatosis, gingivectomy. On the cellular and molecular mechanisms of druginduced. Refinement of the gingf3 locus for hereditary gingival. Hereditary gingival fibromatosis is a rare oral disease with a wide variety of names. Both genetic and pharmacologically induced forms of gingival fibromatosis are known. The following are some diagnostic tools used to identify hereditary gingival fibromatosis. Hereditary gingival fibromatosis describes a group of rare genetic conditions in which the gums become enlarged, growing over the teeth. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 3. The condition may be related to hereditary factors and occurs as a nonsyndromic hereditary gingival fibromatosis hgf or as a part of a syn. Hereditary gingival fibromatosis hgf is a rare, genetically transmitted disorder that only affects approximately 1 in 750,000 people.
Gingival fibromatosis, 3 genetic and rare diseases. Hereditary gingival fibromatosis is frequently an isolated condition of little consequence apart from a cosmetic problem and occasional associations with hypertrichosis andor epilepsy. The treatment consisted of a rigorous oral hygiene program in addition to professional debridement and gingivectomy at both maxillary quadrants. Hereditary gingival fibromatosis briggs family dentistry.
A physical examination will allow the physician to determine, if there is a growth on the gums. Hereditary gingival fibromatosis hgf is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. It can cover teeth in various degrees, and can lead to aesthetic disfigurement. In severe cases functional, periodontal, esthetic and psychological problems may occur. The condition is most frequently reported to be transmitted as an autosomaldominant trait, but autosomalrecessive inheritance has also been reported. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management. Surgical procedures consisting of gingivectomy and gingivoplasty were performed on two members of the family. Here, an interesting case report of massive hgf has been presented. Hereditary gingival fibromatosis hgf is an uncommon condition characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of the gingiva. It may develop as an isolated disorder but can feature along with a syndrome. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival enlargement, elephantiasis gingival, hereditary gingival hyperplasia, idiopathic fibromatosis, and hypertrophied gingival is a rare 1 in 750,000 hereditary condition characterized by slow, progressive enlargement of the gingival.
An association of hgf with periodontitis requires a combined antiinfective and surgical treatment approach. Two missense mutations in kcnq1 cause pituitary hormone deficiency and maternally inherited gingival fibromatosis. Hereditary gingival fibromatosis hgf is characterized by an accumulation of. In case 2, the girl, a unilateral hereditary gingivo fibromatosis 63 breast hypertrophy has been noted since the age of i4 years, and she is due to be admitted for a mammaplasty, but the association of this with the bilateral gingival condition is speculative. Four patients 3males and 1 female, with age range between 2045years old and with no history of systemic diseases or medications intake were referred to.
Gingival fibromatosis is a rare overgrowth condition characterized by a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva summary by hart et al. Gingival fibromatosis gingival hyperplasia may result from systemic medication with calciumchannel blockers, cyclosporin, dilantin, and phenytoin, or it may be hereditary. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and esthetic concerns. Click on the link to view a sample search on this topic. The condition may occur isolated or as part of a syndrome. Disease definition hereditary gingival fibromatosis hgf is a rare benign, slowly progressive, noninflammatory fibrous hyperplasia of the maxillary and mandibular gingivae that generally occurs with the eruption of the permanent or more rarely the primary dentition or even at birth. Hereditary gingival fibromatosis, also known as elephantiasis gingivae, hereditary gingival hyperplasia, idiopathic fibromatosis and hypertrophied gingivae, is a rare 1 in 750,000 autosomal dominant hereditary condition characterized by slow, progressive enlargement of the gingivae, affecting both sexes equally. Generalized gingival enlargement is a rare condition of varying etiology, resulting in oral hygiene impairment and increased risk of periodontitis conclusion hereditary gingival fibromatosis a case report hala a. Hereditary gingival fibromatosis definition of hereditary. This condition has been noted to be present from birth it has a congenital presentation. Once the correlations between gene mutations, molecular changes, histology, and clinical situation are clear, they can be.
Gingival enlargements are quite common and may be either inflammatory, noninflammatory, or a combination of both. Kavvadia k, pepelassi e, alexandridis c, arkadopoulou a, polyzois g, tossios k. Cephalometric findings showed severe mandibular deficiency and vertical. This paper presents a case of 9yearold female patient suffering from hgf with chief complaint of mouth protrusion. Hereditary gingival fibromatosis hgf, is usually transmitted as an autosomal dominant trait material and methods. A clinical report of surgical and prosthetic management pierreluc michaud, dmd, msc, frcdca and aditya patel, hbsc, dds, mperio, frcdcb hereditary gingival. Combining these studies with our findings, we believe that dental plaque accumulation and. Hereditary gingival fibromatosis hgf, previously known as gingival.
Idiopathic gingival fibromatosis is a common, slowlyprogressing, benign condition of the gums. In amed gingival tissue due to plaque accumulation may become red. Evidence of genetic heterogeneity for hereditary gingival. Outcomesresolutions the prognosis of hereditary gingival fibromatosis is very good with suitable treatment a complete excision usually results in low tumor recurrence however, the prognosis of this condition also depends on the severity of any underlying. Therefore, the importance of postoperative followup and adequate hygiene should be emphasized to hgf patients. A mutation in the sos1 gene causes hereditary gingival fibromatosis type 1.
Clinically, it is characterized by benign gingival enlargement with normal color and firm consistency and nonhemorrhagic symptomatic illness. Jorgenson and cocker 1974 stated that there are both dominant and recessive forms of gingival fibromatosis, with generalized and focal types being variable expression of the same disorder. Hgf is principally transmitted as an autosomal dominant trait. Jones syndrome gingival fibromatosis with progressive deafness. Early onset of hereditary gingival fibromatosis in a 28monthold. Hereditary gingival fibromatosis with extreme ridge thickness. Hgf is characterized as a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of keratinized gingiva. Fibromatosis gingivae, gingival fibromatosis definition of. It usually develops as an isolated disorder but can be one feature of a syndrome. This condition negatively affects the quality of life. It can be inherited hereditary gingival fibromatosis hgf.
The gingiva is characterized as pink, firm and fibrous, with little tendency to bleed. Sep 05, 2011 hereditary gingival fibromatosis is a rare benign oral condition characterised by slow and progressive enlargement of both maxillary and mandibular attached gingiva. Gingival fibromatosis and significant tooth eruption delay in an 11yearold male. Hereditary gingival fibromatosis is characterized by various degrees of attached gingival overgrowth. There are, however, several uncommon or rare eponymous syndromes described in which gingival fibromatosis can be a feature. Causes of gingival enlargement can be due to plaque accumulation, due to poor oral hygiene, inadequate nutrition, or systemic hormonal. Hereditary gingival fibromatosis hgf is a rare condition that can occur as an isolated disease, chromosomal abnormality, or as part of a number of syndromes. Gingival fibromatosis gf is a rare condition characterized by pathological, diffuse or local growth of gingiva. Hereditary gingival fibromatosis first reported by goddard and gross in 18561, is a rare, benign, nonhaemorrhagic fibrous enlargement of gingival tissue.
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