Unusual clinical and histologic findings in a child with. A father and a daughter of a family showing gingival hereditary fibromatosis were treated, and gingival biopsies were obtained. A mutation in the sos1 gene causes hereditary gingival. 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 a rare condition which is marked by enlargement of gingival tissue that covers teeth to various extents leading to aesthetic disfigurement. The onset of disease is concurrent with the eruption of permanent teeth. 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. Squamous cell carcinoma arising in familial gingival. The most common genetic form, hereditary gingival fibromatosis, is usually transmitted as an autosomal dominant trait. Gingival fibromatosis is a rare condition in which accumulation of extracellular matrix components causes slowly progressive enlargement of the gingiva. Evidence of genetic heterogeneity for hereditary gingival. Hereditary gingival fibromatosis pubmed central pmc. 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. 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. Hereditary gingival fibromatosis hgf is a rare, autosomal dominant form of gingival overgrowth. Gingival enlargement is defined as an overgrowth or increase in size of gingivae. Towards the targeted management of hereditary gingival.
Gingival fibromatosis, 3 genetic and rare diseases. This condition leads to esthetic, functional, psychological and. Hereditary gingival fibromatosis hgf is a rare gingival lesion 1 in 7,50,000 that presents as localized or. 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. Hereditary gingival fibromatosis hgf is a rare condition that can occur as an isolated disease, chromosomal abnormality, or as part of a. The condition may occur isolated or as part of a syndrome. Hereditary gingival fibromatosis with distinct dental. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and esthetic concerns. 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. Hgf is a genetically heterogeneous disorder and can be transmitted either as an autosomaldominant or autosomalrecessive trait or appear sporadically. 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 hgf is a common, slowlyprogressing, benign condition of the gums.
Refinement of the gingf3 locus for hereditary gingival. We aimed to evaluate the fibroblasts and histological alterations to give new clues. Hereditary gingival fibromatosis is a rare disease that could appear isolated or 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. 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 hgf is the most common genetic form of gingival fibromatosis. 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. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and heredity, rarely.
Hereditary gingival fibromatosis hgf is a rare condition with the prevalence of one per 175000 population and equal distribution in sexes. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare. It may be either hereditary or idiopathic, with hereditary examples occurring as an isolated finding or as part of a syndrome e. Gingiva fibromatosis an overview sciencedirect topics.
It may develop as an isolated disorder but can feature along with a syndrome. This paper presents a case of 9yearold female patient suffering from hgf with chief complaint of mouth protrusion. Genetic linkage of hereditary gingival fibromatosis to. 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. Both genetic and pharmacologically induced forms of gingival fibromatosis are known. Hereditary gingival fibromatosis hgf is a rare disorder characterized by a benign, nonhemorrhagic, fibrous gingival overgrowth that can. Generalized gingival enlargement can be caused by a variety of etiological factors. Hereditary gingival fibromatosis is characterized by various degrees of attached gingival overgrowth. The autosomal recessive inheritance of hereditary gingival. It usually develops as an isolated disorder but can be one feature of a syndrome.
Hereditary gingival fibromatosis also referred to as idiopathic gingival hyperplasia, elephantiasis gingivae etc. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for gingival fibromatosis, 3. This study presents a case of a 28yearold female patient and 18yearold male who belong to the same family suffering from hgf with chief complaint of overgrowing swelling gingiva. Hereditary gingival fibromatosis in a family with the. Nonsyndromic hereditary gingival fibromatosis bmj case. There are, however, several uncommon or rare eponymous syndromes described in which gingival fibromatosis can be a feature. It is an autosonal recessive condition displaying male sexbiased penetrance hhg demonstrates a pleiotropic association with superior fur quality. Hereditary gingival fibromatosis hgf is a rare benign disorder characterized by progressive overgrowth of gingiva.
Gingival biopsies were collected from the mandibular gingiva and used for histological evaluation as well as to. Emergence of hereditary hyperplastic gingivitis in. Hereditary gingival fibromatosis can occur as an isolated condition or as part of a genetic syndrome. Associated findings that have been reported in some cases, include hypertricho. Hereditary gingival fibromatosis hgf is a rare, genetically transmitted disorder that only affects approximately 1 in 750,000 people. This case report gives an overview of gingival fibromatosis in a. Hereditary gingival fibromatosis is rare, affecting only one in 750,000 people. 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 be inherited hereditary gingival fibromatosis hgf.
In the present case the method of treatment implied was surgery by scalpel method along with oral prophylaxis. Hereditary gingival fibromatosis, journal of intellectual. Hgf is principally transmitted as an autosomal dominant trait. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth. Hereditary gingival fibromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. Clinically, it is characterized by benign gingival enlargement with normal color and firm consistency and nonhemorrhagic symptomatic illness. Although the clinical and histopathologic characteristics of hgf are explicit, the pathogenic mechanism remains unclear. Hereditary gingival fibromatosis hgf is characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of keratinized gingiva. Hereditary gingival fibromatosis how is hereditary. The most common genetic form, hereditary gingival fibromatosis hgf, is usually transmitted as an autosomal. Affected individuals have a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of the oral masticatory mucosa. Igg variant on collagen fibril formation in the cell culture of gingival fibroblasts taken from a patient with hereditary gingival fibromatosis with a high propensity for recurrence. Hereditary gingival fibromatosis a case report archana.
Clinically, a pink gingiva with marked stippling can be seen to cover almost all the tooth, in many cases preventing. Gingival fibromatosis gingival hyperplasia may result from systemic medication with calciumchannel blockers, cyclosporin, dilantin, and phenytoin, or it may be hereditary. Corliii and pindlrorg, 1964 and epilepsy ramon, berman and bubis, 1967. The excessive growth of gingival tissue may cause displacement of teeth, overretention of primary teeth, and spacing. Hereditary gingival fibromatosis is a rare benign oral condition characterised by slow and progressive enlargement of both maxillary and mandibular attached gingiva. 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. The clinical presentation of hgf is variable, both in the distribution number of teeth involved and in the degree severity of expression. A histological study revealed dense fibrous tissue, basal lamina disruption, and epithelial cell. Hereditary gingival fibromatosis is associated with either autosomal dominant such as rutherford 22 and laband syn. What is hereditary gingival fibromatosis hereditary gingival fibromatosis describes a group of rare genetic conditions in which the gums become enlarged, growing over the teeth. 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. Education and motivation of patient should be properly done and is essential for maintenance of proper oral hygiene and regular recall visits 15. This condition has been noted to be present from birth it has a congenital presentation.
Such features are absent in hereditary gingival fibromatosis. After treatment regular recall visits are necessary in order to evaluate oral hygiene and stability of periodontal treatment. Hereditary gingival fibromatosis how is hereditary gingival fibromatosis abbreviated. It was found that the hereditary form usually detected in younger patients affecting either primary andor permanent dentition. Hereditary gingival fibromatosis hgf is a rare condition that can occur. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe aesthetic, emotional and functional impairment. Hereditary gingival fibromatosis hgf is a rare oral disease. Hereditary gingival fibromatosis is a rare disorder characterised by gradual enlargement of the gingival mucosa. Hgf is characterized as a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of keratinized gingiva. Affected individuals have a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of the.
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. Hereditary gingival fibromatosis is frequently an isolated condition of little consequence apart from a cosmetic problem and occasional associations with hypertrichosis andor epilepsy. Kemoli summary hereditary gingival hyperplasia hgf is a rare condition characterised by hyperplastic, dense. Genetic loci for autosomal dominant forms of hgf have been localized to chromosome 2p21p22 hgf1 and chromosome 5qq22 hgf2. Hereditary gingival fibromatosis hgf is a rare disease in both autosomal dominant and recessive types and has a frequency of 1. Hereditary gingival fibromatosis is a disorder for which the etiology remains unknown. Cephalometric findings showed severe mandibular deficiency and vertical. A mutation in the sos1 gene causes hereditary gingival fibromatosis type 1 thomas c. Hereditary gingival fibromatosis specifically occurs in the absence of other identifiable medical syndromes or medications known to cause gum overgrowth. Hereditary gingival fibromatosis is a rare condition characterised by severe gingival hyperplasia that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality.
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