This condition has been noted to be present from birth it. The gingiva is characterized as pink, firm and fibrous, with little tendency to bleed. Hereditary gingival fibromatosis hgf is the most common genetic form of gingival fibromatosis that develops as a slowly progressive, benign, localized or generalized enlargement of keratinized gingiva. Hgf is characterized by a slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and. Autosomaldominant forms of gingival fibromatosis, which are usually nonsyndromic, have been genetically linked to the chromosomes 2p21p22 and 5qq22 3 5. Hereditary or idiopathic hyperplasia of the gums, ele phantiasis gingivac, fibromatosis gingivae, symmetrical fibroma of the palate, and. Hereditary gingival fibromatosis hgf is an uncommon condition characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of the gingiva.
The goal of this article is to describe one kindred affected with hgf and discuss the diagnosis, treatment, recurrence risk and histopathology features of the proband with mixed dentition. It causes teeth being partially or totally covered by enlarged gingiva, causing esthetic and functional problems. 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. It usually develops as an isolated disorder but can be one feature of a syndrome. Hereditary gingival fibromatosis pubmed central pmc. The 12 individuals examined all had typical findings, but gingival involvement was variable, ranging from mild to severe. Inflamed gingival tissue due to plaque accumulation may become red. For language access assistance, contact the ncats public information officer. Click on the link to view a sample search on this topic. If mild, it involves only the interdental papillae gum between the teeth, but usually, it affects the marginal gum gum over the teeth also. 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, gingival enlargement, gingivectomy. Idiopathic gingival fibromatosis associated with generalized. The condition results in the teeth being partially or.
This condition negatively affects the quality of life. Hereditary gingival fibromatosis is characterized by a slow benign enlargement of gingival tissue. Gingival enlargement is defined as an overgrowth or increase in size of gingivae. This condition leads to esthetic, functional, psychological and masticatory disturbance of the oral cavity. Gingival fibromatosis can be caused by number of factors including inflammation, leukemic infiltration, and use of medications such as phenytoin, cyclosporine or nifedipine, 14 and vigabatrin. Effect of treatment of hereditary gingival fibromatosis on. Hereditary gingival fibromatosis hgf is a rare disease in both autosomal dominant and recessive types and has a frequency of 1. Hereditary gingival fibromatosis gingival enlargement, commonly known as hyperplasia or hypertrophy, is pathologic overgrowth of the gingiva. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth.
Get a printable copy pdf file of the complete article 924k, or click on a page image below to browse page by page. Hgf is characterized as a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of keratinized gingiva. Hereditary gingival fibromatosis also referred to as idiopathic gingival hyperplasia, elephantiasis gingivae etc. Gingival fibromatosis is a rare condition in which accumulation of extracellular matrix components causes slowly progressive enlargement of the gingiva. Hereditary gingival fibromatosis is rare, affecting only one in 750,000 people. Fibrous enlargement is most common in areas of maxillary and mandibular. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
Case management is discussed in relation to a yearold girl who presented with recurrent, progressive gingival enlargement requiring consecutive periodontal and orthodontic treatment. Links to pubmed are also available for selected references. Mutation in sos1, sonofsevenless gene, is thought to be responsible for hereditary gingival fibromatosis. Hereditary gingival fibromatosis hgf is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. Early onset of hereditary gingival fibromatosis in a 28monthold. A mutation in the sos1 gene causes hereditary gingival fibromatosis type 1. Fibromatosis definition of fibromatosis by the free dictionary. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 2. Hereditary gingival fibromatosis hgf is a rare gingival. Introduction 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. Clinical science minichromosome maintenance 2 and 5.
This enlargement may be caused by overreaction to plaquebiofilm, infections or side effects of systemic conditions or medications. Hereditary gingival fibromatosis associated with growth. As a result of the gingival overgrowth and associated dental displacement, affected patients occasionally present with increased occlusal vertical dimension andor. It was previously called elephantiasis gingivae, hereditary gingival. These analyses refined the gingf locus in the family to an 8.
Hereditary gingival fibromatosis international journal of oral. Although the genetic mechanism is not fully understood, majority of research workers have attributed this condition related with hereditary factors. 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. This paper presents a case of 9yearold female patient suffering from hgf with chief complaint of mouth protrusion. Jul 23, 2019 hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth.
Hereditary gingival fibromatosis is characterized by a slow benign enlargement of gingival. A syndromic association between gingival fibromatosis and a wide variety of other genetically inherited disorders has been described. Clinically, it is characterized by benign gingival enlargement with normal color and firm consistency and nonhemorrhagic symptomatic illness. Hereditary gingival fibromatosis hgf is the most common genetic form of gingival fibromatosis. Most cases of hereditary gingival fibromatosis appear to be inherited in an autosomaldominant manner, although autosomal recessive inheritance has also been reported. Hereditary gingival fibromatosis can occur as an isolated. Hereditary gingival fibromatosis definition of hereditary. Hereditary gingival fibromatosis hgf is characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of keratinized gingiva. Hereditary gingival fibromatosis is a rare disorder characterised by gradual enlargement of the gingival mucosa.
Gingiva fibromatosis an overview sciencedirect topics. Get a printable copy pdf file of the complete article. 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. Most cases follow autosomal dominant genetics, with a reported incidence of 1 in 750,000. A thickened epithelium with long rete pegs is seen. Laser versus conventional surgery in management of hereditary gingival fibromatosis case series. Hereditary gingival fibromatosis hgf is a rare condition characterized by progressive enlargement of the gingiva. Hereditary, druginduced, and idiopathic gingival overgrowth have been reported. 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. Hereditary gingival bromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. Genetic linkage of hereditary gingival fibromatosis to. It is usually transmitted both as autosomal dominant trait and autosomal recessive inheritance although sporadic cases are commonly reported. Gingival fibromatosis, 3 genetic and rare diseases. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and rarely heredity.
In severe cases functional, periodontal, esthetic and psychological problems may occur. In the present case the method of treatment implied was surgery by scalpel method along with oral prophylaxis. The discovery of hypothalamic regulation of the hereditary gingival fibromatosis 337 fig. Hereditary gingival fibromatosis first reported by goddard and gross in 18561, is a rare, benign, nonhaemorrhagic fibrous enlargement of gingival tissue. 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 pseudopockets. The condition may occur isolated or as part of a syndrome. The condition may be related to hereditary factors and occurs as a nonsyndromic hereditary gingival fibromatosis hgf or as a part of a syn. Gingival fibromatosis may be familial or idiopathic 2. 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. Genetic disorders associated with gingival enlargement.
Clinical and radiographic characteristics of hereditary gingival. Oral pathology general section hereditary gingival fibromatosis report of a family s. This article reports a symmetric case of hereditary gingival fibromatosis of severe variety. Gingival fibromatosis is a rare and heterogeneous group of disorders that develop as.
Hereditary gingival fibromatosis presents as a localised or generalised overgrowth of the attached keratinised gums of variable severity. Sep 26, 2018 hereditary gingival fibromatosis hgf is a common, slowlyprogressing, benign condition of the gums. Familial gingival fibromatosis is a rare hereditary condition due to chromosomal abnormality which can occur as an isolated disease or as part of a syndrome and has an incidence of 1. Case report hereditary gingival fibromatosis a case report. Hereditary gingival fibromatosis is a rare oral disease with a wide variety of names. Gingival fibromatosis, genetic disease, pedigree, ultrastructure.
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. What are the clinical features of hereditary gingival fibromatosis. It can cover teeth in various degrees, and can lead to aesthetic disfigurement. It can be inherited hereditary gingival fibromatosis hgf. Hereditary gingival fibromatosis is characterized by various degrees of attached gingival overgrowth. Hereditary gingival fibromatosis can occur as an isolated condition or as part of a. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 3.
Hereditary gingival fibromatosis hgf is a common, slowlyprogressing, benign condition of the gums. The most common clinical abnormalities associated with gingival fibromatosis. Unusual clinical and histologic findings in a child with. If you have problems viewing pdf files, download the latest version of adobe reader. Associated findings that have been reported in some cases, include hypertricho. 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.
Hereditary gingival fibromatosis with extreme ridge. 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. Rest finalexontruncating mutations cause hereditary. Squamous cell carcinoma arising in familial gingival. Richa, tutor, unit of pedodontics and preventive dentistry, esic dental college and. Hgf is a genetically heterogeneous disorder and can be transmitted either as an autosomaldominant or autosomalrecessive trait or appear sporadically. The familial variation may occur with a number of other inherited. 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. Cephalometric findings showed severe mandibular deficiency and vertical. Overgrowth of keratinized gingival tissues is a common condition and is described under variety of names. Clinically, a pink gingiva with marked stippling can be seen to cover almost all the tooth, in many cases preventing. Kemoli summary hereditary gingival hyperplasia hgf is a rare condition characterised by hyperplastic, dense. In amed gingival tissue due to plaque accumulation may become red.
Gingival fibromatosis, 2 genetic and rare diseases. Such features are absent in hereditary gingival fibromatosis. Hereditary gingival fibromatosis a case report and. Although the gingival enlargement does not directly affect the alveolar bone, the gingival. This report addresses the complex nature of oral diagnosis, treatment and longterm case management in the hereditary form of recurrent gingival fibromatosis. Hereditary gingival fibromatosis how is hereditary gingival. Introduction a number of genetic disorders present with gingival manifestations which may be in the. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and esthetic concerns. 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. Gingival fibromatosis is characterized by a slowly progressive benign enlargement of the oral gingival tissues. Therefore, the importance of postoperative followup and adequate hygiene should be emphasized to hgf patients.
Generalized gingival enlargement can be caused by a variety of etiological factors. The condition may occur as an isolated disorder or as a part of a syndrome. Gingival fibromatosis gf is a rare condition characterized by pathological, diffuse or local growth of gingiva. 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. Corliii and pindlrorg, 1964 and epilepsy ramon, berman and bubis, 1967.
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