Gingival fibromatosis, 2 genetic and rare diseases. The documents contained in this web site are presented for information purposes only. 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. Normal anatomy pterygoid hamulus parotid papillae lingual papillae developmentalunerupted teeth odontogenic cysts eruption cysts developmental cysts eg thyroglossal, dermoid haemangioma lymphangioma maxillary and mandibular tori hereditary gingival fibromatosis lingual thyroid. Hereditary gingival fibromatosis is a rare benign oral condition characterised by slow and progressive enlargement of both maxillary and mandibular attached gingiva. 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. Gingival fibromatosisprogressive deafness syndrome jones. Complete oral rehabilitation of a patient with hereditary. Generalized gingival overgrowth can basically occur to be inherited as an isolated trait, associated with other diseases representing a syndrome, or can be induced as a side effect of systemic drugs. Occurring as an isolated trait hgf andor as a component of a syndrome, the affected gingiva is. Therefore, the importance of postoperative followup and adequate hygiene should be emphasized to hgf patients.
Fibromatosis gingivae, gingival fibromatosis definition. The gingival hyperplasia extended from the anterior to retromolar right mandible, surpassing the occlusal plane, which caused difficulty with lip closure and the imprint of her upper teeth on surface of the lesion. 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. Squamous cell carcinoma arising in familial gingival. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and rarely heredity. Original article decreased alpha 2 integrin gene expression in non. In case 2, the girl, a unilateral hereditary gingivofibromatosis 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. It is described variously as fibromatosis gingivae, gingivostomatitis, hereditary gingival fibromatosis, idiopathic fibromatosis, familial elephantiasis, and diffuse fibroma. According to these factors, it can be defined as hereditary gingival fibro matosis, nonfamilial gingival fibromatosis or druginduced gingival.
As a result of the gingival overgrowth and associated dental displacement, affected patients occasionally present with increased occlusal vertical dimension andor. Click on the link to view a sample search on this topic. Rest finalexontruncating mutations cause hereditary gingival. Such features are absent in hereditary gingival fibromatosis. 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. Generalized gingival enlargement can be caused by a variety of etiological factors. Hereditary gingival fibromatosis specifically occurs in the absence of other identifiable medical syndromes or medications known to cause gum overgrowth. This condition has been noted to be present from birth it has a congenital presentation. This condition leads to esthetic, functional, psychological and. A clinical report of surgical and prosthetic management pierreluc michaud, dmd, msc, frcdca and aditya patel, hbsc, dds, mperio, frcdcb hereditary gingival. The most common genetic form, hereditary gingival fibromatosis hgf. Refinement of the gingf3 locus for hereditary gingival. This paper presents a case of 9yearold female patient suffering from hgf with chief complaint of mouth protrusion.
Gingival fibromatosis gf is characterized by fibrotic enlargement of the gingiva that can be inherited as an isolated trait named hereditary gingival fibromatosis or as a component of a syndrome. Genetic linkage of hereditary gingival fibromatosis to. Sep 26, 2018 hereditary gingival fibromatosis hgf is a common, slowlyprogressing, benign condition of the gums. 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. Complete oral rehabilitation of a patient with hereditary gingival. Gingival enlargement is defined as an overgrowth or increase in size of gingivae. Oct 08, 2018 idiopathic gingival fibromatosis is a common, slowlyprogressing, benign condition of the gums. 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 leads to esthetic, functional, psychological and masticatory disturbance of the oral cavity. 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.
Hereditary gingival fibromatosis a case report archana. 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. Hgf is a genetically heterogeneous disorder and can be transmitted either as an autosomaldominant or autosomalrecessive trait or appear sporadically. Hereditary gingival fibromatosis is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality. Hereditary or idiopathic hyperplasia of the gums, ele phantiasis gingivac, fibromatosis gingivae, symmetrical fibroma of the palate, and. It can be inherited hereditary gingival fibromatosis hgf. The gingiva is characterized as pink, firm and fibrous, with little tendency to bleed.
Hereditary gingival fibromatosis is rare, affecting only one in 750,000 people. Leonhart, dds, md,d and dean morton, bds, mse hereditary gingival. Links to pubmed are also available for selected references. In the present case the method of treatment implied was surgery by scalpel method along with oral prophylaxis. Hereditary gingival fibromatosis with extreme ridge thickness. 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. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 2.
Idiopathic gingival fibromatosis associated with generalized. Gingival fibromatosis gingival hyperplasia may result from systemic medication with calciumchannel blockers, cyclosporin, dilantin, and phenytoin, or it may be hereditary. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 3. Hereditary gingival fibromatosis hgf is a rare benign, slowly progressive. Gingival fibromatosis, 3 genetic and rare diseases. Clinically, a pink gingiva with marked stippling can be seen to cover almost all the tooth, in many cases preventing. Hereditary gingival fibromatosis type 1 characterized by benign gingival outgrowth cause.
It can be isolated, with autosomal dominant inheritance, or as part of a syndrome. Hereditary gingival fibromatosis describes a group of rare genetic conditions in which the gums become enlarged, growing over the teeth. Get a printable copy pdf file of the complete article 924k, or click on a page image below to browse page by page. A clinical report amirali zandinejad, dds, msc,a merila atarodi, dds,b tamer abdelazim, dds,c ryan m. Oral pathology general section hereditary gingival fibromatosis report of a family s. Hereditary gingival fibromatosis is a rare oral disease with a wide variety of names. Hereditary gingival fibromatosis hgf is a rare disorder characterized by a benign, nonhemorrhagic, fibrous gingival overgrowth that can. 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. 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. Alleles were scored, and genotype data were entered into the pedigree file of the. In amed gingival tissue due to plaque accumulation may become red.
It is usually inherited as an autosomal dominant trait, but some cases are idiopathic and others are produced by drugs. 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. Hereditary gingival fibromatosis hgf is a rare condition that can occur. Archived tissue specimens of hereditary gingival fibromatosis, gingival fibromatosis and dental abnormality syndrome and normal gingiva were subject to morphological analysis and immunohistochemical staining.
Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and heredity, rarely. Hereditary gingival fibromatosis pubmed central pmc. Hereditary gingival fibromatosis hgf is characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of keratinized gingiva. Hereditary gingival fibromatosis with distinct dental. Hereditary gingival fibromatosis how is hereditary gingival fibromatosis abbreviated.
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 also referred to as idiopathic gingival hyperplasia, elephantiasis gingivae etc. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and aesthetic concerns. 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.
By haplotype construction and analysis in a 5generation chinese family segregating autosomal dominant hereditary gingival fibromatosis, ye et al. Fibromatosis gingivae, gingival fibromatosis definition of. Kemoli summary hereditary gingival hyperplasia hgf is a rare condition characterised by hyperplastic, dense. Hereditary gingival bromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. This paper presents a case of 9yearold female patient suffering from hgf with chief complaint of. Hereditary gingival fibromatosis and growth retardation. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management.
Hereditary gingival fibromatosis hgf is a rare gingival. Hereditary gingival fibromatosis hgf also referred to as idiopathic gingival hyperplasia is a rare type of slow growing gingival enlargement with great clinical and genetic heterogenity inherited usually as mendelian autosomal dominant trait. Hereditary gingival fibromatosis hgf is an uncommon condition characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of the gingiva. The growth is slow and progressive and is druginduced, idiopathic, or hereditary in etiology. Hereditary gingival fibromatosis how is hereditary. Idiopathic gingival fibromatosis is a common, slowlyprogressing, benign condition of the gums. Hereditary gingival fibromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. Hereditary gingival fibromatosis definition of hereditary. It can cover teeth in various degrees, and can lead to aesthetic disfigurement. Cephalometric findings showed severe mandibular deficiency and vertical.
Gingival fibromatosis, genetic disease, pedigree, ultrastructure. As a result of the gingival overgrowth and associated dental displacement, affected patients occasionally present with increased occlusal vertical dimension andor inadequate lip closure. Hereditary gingival fibromatosis with extreme ridge. The condition may occur isolated or as part of a syndrome. In severe cases, the gingival enlargement may cover the crowns of teeth and cause severe functional and esthetic concerns. Hgf is characterized as a benign, slowly progressive, nonhemorrhagic, fibrous enlargement of keratinized gingiva. 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 fibromatosis is associated with either autosomal dominant such as rutherford 22 and laband syn.
Full text full text is available as a scanned copy of the original print version. Case report hereditary gingival fibromatosis a case report. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth. Unusual clinical and histologic findings in a child with mixed. 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. 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. 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. Early onset of hereditary gingival fibromatosis in a 28monthold. Hereditary gingival fibromatosis hgf is a common, slowlyprogressing, benign condition of the gums.
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. 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. Unusual clinical and histologic findings in a child with. It occurs isolated or frequently as a component of various syndromes. 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.