Hereditary gingival fibromatosis: Clinical and ultrastructural features of a new family
نویسندگان
چکیده
OBJECTIVE This article describes the diagnosis, clinical and microscopic (histopathology and ultrastructural) features and treatment of a new family with hereditary gingival fibromatosis (HGF) and highlights the importance of this genetic condition. STUDY DESIGN To characterize the pattern of inheritance and the clinical features, members of a new family with HGF were examined. The pedigree was reliably constructed including the four latest generations of family. Hematoxylin and eosin staining and ultrastructural analysis were performed with the gingival tissue. RESULTS Examination of the family pedigree revealed that the patient III-2 represent the index patient of this family (initial patient with a mutation), which was transmitted to her daughter through an autosomal dominant mode of inheritance. The affected patients showed a generalized gingival overgrowth. The patient was treated with surgical procedures of gingivectomy and gingivoplasty. The diagnosis was confirmed by histopathology examination that showed a well-structured epithelium with elongated and thin papillae inserted in fibrous connective tissue with increased amount of collagen. The ultrastructural aspects of the tissue show collagen fibrils exhibiting their typically repeating banding pattern with some fibrils displaying loops at their end. Moreover, it was possible to seen in some regions fibrillar component presenting tortuous aspects and loss of the alignment among them. CONCLUSIONS This HGF frequently resulted in both esthetic and functional problems. The genetic pattern of this Brazilian family suggested a new mutation, which was later transmitted by an autosomal dominant trait.
منابع مشابه
Hereditary gingival fibromatosis with distinct dental, skeletal and developmental abnormalities.
A case of a 9-year-old child with hereditary gingival fibromatosis, supernumerary tooth, chest deformities, auricular cartilage deformation, joint laxity and undescended testes is described. The exact mode of inheritance is unclear; a new mutation pattern is possible. These features resemble but differ from the previously reported Laband syndrome. The dental treatment consisted of surgical remo...
متن کاملSurgical Management of a Severe Case of Gingival Fibromatosis
Gingival fibromatosis (GF) is a fibrous overgrowth of gingiva that maybe idiopathic or may have an inheritance pattern wherein it is known as hereditary gingival fibromatosis (HGF). This case report describes a 16-year old female patient who presented generalized extensive gingival overgrowth, involving the maxillary and mandibular arches. Diagnosis of GF was substantiated by the patient’s clin...
متن کاملHereditary Gingival Fibromatosis and its management: A Rare Case of Homozygous Twins
Hereditary gingival fibromatosis (HGF) is a rare condition which manifests itself by gingival overgrowth covering teeth to variable degree i.e. either isolated or as part of a syndrome. This paper presented two cases of generalized and severe HGF in siblings without any systemic illness. HGF was confirmed based on family history, clinical and histological examination. Management of both the cas...
متن کاملHereditary Gingival Fibromatosis Diagnosis and Treatment
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 fashion. Occurring as an isolated trait (HGF) and/or as a component of a syndrome, the affected gingiva is characterized by firm, asymptomatic, nonhemorrhagic ...
متن کاملHereditary gingival fibromatosis with extreme ridge thickness and insufficient interarch distance: A clinical report of surgical and prosthetic management.
Hereditary gingival fibromatosis is a rare genetic disorder resulting in gingival overgrowth that can be found in both dental arches. As a result of the gingival overgrowth and associated dental displacement, affected patients occasionally present with increased occlusal vertical dimension and/or inadequate lip closure. Depending on the disorder's severity, these patients can be challenging to ...
متن کامل