Hereditary gingival fibromatosis a case report and. 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. Fibromatosis gingivae definition of fibromatosis gingivae. Sep 26, 2018 hereditary gingival fibromatosis hgf is a common, slowlyprogressing, benign condition of the gums. Gingival fibromatosis with hypertrichosis syndrome is an extremely rare genetic condition characterized by profound overgrowth of hair and gums, as well as other variable features.
Hereditary gingival fibromatosis international journal of oral. Hereditary gingival fibromatosis hgf is a rare disorder characterized by a benign, nonhemorrhagic, fibrous gingival overgrowth that can. Hereditary gingival fibromatosis is characterized by a slow benign enlargement of gingival tissue. Idiopathic gingival fibromatosis associated with generalized aggressive periodontitis. The gingiva is characterized as pink, firm and fibrous, with little tendency to bleed. Gingival fibromatosis, 2 genetic and rare diseases. It can be inherited hereditary gingival fibromatosis hgf. This paper presents a case of 9yearold female patient suffering from hgf with chief complaint of mouth protrusion.
Hereditary gingival fibromatosis is most commonly inherited in an autosomal dominant manner, meaning that each child of an affected parent has a 50% chance of developing the condition. By haplotype construction and analysis in a 5generation chinese family segregating autosomal dominant hereditary gingival fibromatosis, ye et al. Genetic heterogeneity of hereditary gingival fibromatosis. A physical examination will allow the physician to determine, if there is a growth on the gums. This condition has been noted to be present from birth it has a congenital presentation. It is usually transmitted both as autosomal dominant trait and autosomal recessive inheritance although sporadic cases are commonly reported. Free and attached gingival tissues are both involved, but extension beyond the mucogingival junction is not observed. Superficial fibromatoses such as palmar, plantar and penile fibromatosis have sometimes been linked to certain diseases such as diabetes, liver. The patient presented with generalized diffuse gingival enlargement involving the maxillary and mandibular.
Hereditary or idiopathic hyperplasia of the gums, ele phantiasis gingivac, fibromatosis gingivae, symmetrical. 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. Idiopathic gingival fibromatosis associated with generalized. Gingival fibromatosis is characterized by a large increase in the gingival dimension which extends above the dental crowns, covering them partially or completely. Hereditary gingival fibromatosis with distinct dental. In some types of fibromatosis such as desmoid tumours, it is thought that the condition may be related to trauma, hormonal factors, or have a genetic association. A case report rashi chaturvedi, mds, dnb abstract idiopathic gingival fibromatosis, a benign, slowgrowing proliferation of the gingival tissues, is genetically heterogeneous. The most common genetic form, hereditary gingival fibromatosis, is usually transmitted as an autosomal. Causes of gingival enlargement can be due to plaque accumulation, due to poor oral hygiene, inadequate nutrition, or systemic. The following are some diagnostic tools used to identify idiopathic 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. 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. A heightened gingival response to plaque during pregnancy results in pregnancyassociated gingivitis 14. Gingival fibromatosis with hypertrichosis is a cutaneous condition characterized by dark terminal hairs on the peripheral face, central back, and extremities. Hereditary gingival fibromatosis hgf is the most common genetic form of gingival fibromatosis. Hereditary gingival fibromatosis is rare, affecting only one in 750,000 people. In the localised form, the overgrowth occurs mainly on the lip. Localized gingival enlargement in a 27year old pregnant patient 15. Gingival fibromatosis is classified as two types according to its form.
Microscopic view of gingival enlargement in pregnant patient showing abundance of blood vessels and interspersed inflammatory cells 16. Gingival hyperplasia is a bizarre condition causing esthetic, functional, psychological, and masticatory disturbances of the oral cavity. Generalized gingival enlargement can be caused by a variety of etiological factors. Hereditary gingival fibromatosis hgf, also known as idiopathic gingival hyperplasia, is a rare condition of gingival overgrowth. 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. Introduction hereditary gingival fibromatosis is a slowly progressive benign enlargement that affects the marginal gingiva, attached gingiva and interdental papilla 1. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. If you have problems viewing pdf files, download the latest version of adobe reader. In severe cases functional, periodontal, esthetic and psychological problems may occur. It is usually inherited as an autosomal dominant trait, but some cases are idiopathic and others are produced by drugs. Oral pathology general section hereditary gingival fibromatosis report of a family s. Gingival fibromatosis gf is a rare condition characterized by pathological, diffuse or local growth of gingiva. Hereditary gingival fibromatosis pubmed central pmc. The purpose of this study was to analyze a case of idiopathic gingival fibromatosis, using histochemical and immunohistochemical staining and transmission electron microscopy.
Introduction hereditary gingival fibromatosis hgf is a rare condition characterized by progressive enlargement of the gingivae, with a reported incidence of 1 in 7 50,000 1. The objective of this work is to present a rarely occurring type of gingival fibromatosis. 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. If mild, it involves only the interdental papillae gum between the teeth, but usually, it affects the marginal gum gum over the teeth also. This condition is usually part of a syndrome or, rarely, an isolated disorder. Histochemical, immunohistochemical, and ultrastructural. Many forms of gingival hyperplasia are of unknown etiology and termed as idiopathic gingival fibromatosis. Hereditary gingival fibromatosis med oral patol oral cir bucal. For language access assistance, contact the ncats public information officer. 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. Aug 12, 2014 a heightened gingival response to plaque during pregnancy results in pregnancyassociated gingivitis 14. Hereditary gingival fibromatosis hgf is a rare gingival.
Gingival enlargements are quite common and may be either inflammatory, noninflammatory, or a combination of both. A thorough physical examination and a complete medical history. A case of hereditary gingival fibromatosis associated with generalized aggressive periodontitis reported earlier indicated the possible emergence of a. Hereditary gingival fibromatosis hgf is a rare gingival lesion 1 in 7,50,000 that presents as localized or generalized fibrotic enlargement of. Hereditary or idiopathic hyperplasia of the gums, ele phantiasis gingivac, fibromatosis gingivae, symmetrical fibroma of the palate, and. Cephalometric findings showed severe mandibular deficiency and vertical. The examined group consisted of 12 patients 8 males and 4 females aged 18 to 59. Oct 08, 2018 idiopathic gingival fibromatosis is a common, slowlyprogressing, benign condition of the gums. In chapter 5 of this thesis the goal was to combine the. Hereditary gingival fibromatosis hgf is a rare disease in both autosomal dominant and recessive types and has a frequency of 1. Gingival fibromatosis is a hyperplasia of the connective tissue of jaw periosteum, with no symptoms of inflammation or pain. 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 gingiva is characterized as pink, firm and fibrous, with little tendency to. However, families with autosomal recessive inheritance and apparent maternal inheritance have also been reported. Idiopathic gingival fibromatosis case report science. After treatment regular recall visits are necessary in order to evaluate oral hygiene and stability of periodontal treatment. 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. Management of idiopathic gingival fibromatosis surgery. It can cover teeth in various degrees, and can lead to aesthetic disfigurement. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and rarely heredity. Hereditary gingival fibromatosis hgf is a rare condition which manifests itself by an enlarged gingival tissue covering teeth to various extents. Hereditary gingival fibromatosis hgf is a benign, proliferative gingival overgrowth affecting humans.
Hereditary gingival fibromatosis also referred to as idiopathic gingival hyperplasia, elephantiasis gingivae etc. Introduction prevalence and aetiology gingival fibromatosis is a rare disease with a prevalence of 1 in 175,000 individuals and an equal distribution between males and females, characterised by a generalised or localised thickening of the gingival. Gingival fibromatosis is a rare disease characterized by enlargement of the gingiva. Hereditary gingival fibromatosis hgf is a rare condition which manifests itself by gingival overgrowth covering teeth to variable degree i. In amed gingival tissue due to plaque accumulation may become red. Education and motivation of patient should be properly done and is essential for maintenance of proper oral hygiene and regular recall visits 15. Hereditary gingival fibromatosis hgf is a rare, benign disorder with slowly progressive enlargement of maxillary and mandibular gingiva. Hereditary gingival fibromatosis hgf is an uncommon condition characterized by an accumulation of extracellular matrix resulting in a fibrotic enlargement of the gingiva. Hereditary gingival fibromatosis presents as a localised or generalised overgrowth of the attached keratinised gums of variable severity. Hgf is characterized by a slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and. Case report hereditary gingival fibromatosis a case report.
Hereditary gingival fibromatosis is a rare oral disease with a wide variety of names. Clinically, it is characterized by benign gingival enlargement with normal color and firm consistency and nonhemorrhagic symptomatic illness. Pubmed is a searchable database of medical literature and lists journal articles that discuss gingival fibromatosis, 2. Gingival enlargement is defined as an overgrowth or increase in size of gingivae. Clinically, a pink gingiva with marked stippling can be seen to cover almost all the tooth, in many cases preventing. Gingival fibromatosis with hypertrichosis wikipedia. Gingival fibromatosis with hypertrichosis syndrome.
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. Both genetic and pharmacologically induced forms of gingival fibromatosis are known. The condition may occur isolated or as part of a 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. Gingival enlargement is associated with multiple factors including inflammation, medications, neoplasia, hormonal disturbances and heredity, rarely. The examined group consisted of 12 patients 8 males and 4. The condition may be related to hereditary factors and occurs as a nonsyndromic hereditary gingival fibromatosis hgf or as a part of a syn. After two gingivectomies, the condition appears to be recurring.
Cephalometric findings showed severe mandibular deficiency. This case report presented the clinical features of a typical idiopathic gingival enlargement which was treated with external bevel gingivactomy. Hereditary gingival fibromatosis hgf is a rare condition that can occur. Although the gingival enlargement does not directly affect the alveolar bone, the gingival. The following are some diagnostic tools used to identify hereditary gingival fibromatosis. Hereditary gingival fibromatosis can occur as an isolated condition or as part of a genetic syndrome. In the present case the method of treatment implied was surgery by scalpel method along with oral prophylaxis.
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. 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. Causes of gingival enlargement can be due to plaque accumulation, due to poor oral hygiene, inadequate nutrition, or systemic hormonal. The patient was a 39yearold japanese man, in whom the gingiva was enlarged throughout the. Click on the link to view a sample search on this topic. Idiopathic gingival enlargement is a type of gingival fibromatosis characterized by progressive overgrowth of the gingival tissues. 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. Kemoli summary hereditary gingival hyperplasia hgf is a rare condition characterised by hyperplastic, dense.
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