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Objective: To ascertain histology changes of hereditary gingival fibromatosis (HGF) and the location of HGF gene., Methods: A pedigree analyses of HGF; and the ultrastructure of gingival overgrowth tissue was observed with electron microscopy. The overgrowth of the HGF gene was defined with microsatellite markers., Results: The connective tissue of HGF consisted of coarse collagen bundles and several kinds of cells arranged abnormally, such as: epithelial cells, smooth muscle cells and so on; the HGF locus had been mapped to chromosome 5q13-q22., Conclusions: The gingival pathologic changes resemble "hamartoma"; the findings has implications for identification of the underlying genetic basis of HGF.

This report addresses the complex nature of oral diagnosis, treatment and long-term case management in the hereditary form of recurrent gingival fibromatosis. Case management is discussed in relation to a 13-year-old girl who presented with recurrent, progressive gingival enlargement requiring consecutive periodontal and orthodontic treatment. The initial course of treatment included 4-quadrant gingivectomy with reverse bevel incisions, followed by orthodontics. Microscopic examination of the gingivectomy specimens supported the clinical diagnosis. Three years later, recurrence of the condition was observed in all quadrants. To facilitate orthodontic tooth movement and to achieve optimal esthetics, another full-mouth gingivectomy was performed. There was no recurrence of the condition a year later. It is recommended that patients with this condition be monitored closely after gingivectomy, so that the treatment requirements of localized areas can be addressed as needed.

Gingival fibromatosis is usually seen as an isolated finding or occasionally in association with other features as part of a syndrome. The combination of gingival enlargement, hypertrichosis, epilepsy and mental retardation is also a commonly reported syndrome that features gingival fibromatosis. The following report is about a mentally retarded patient who has shown no sign of hypertrichosis, but has been taking phenobarbital as a long-term therapy drug for anti-convulsion. Long-term management of this patient has been carried out from the age of one-and-a-half years to 14 years old. The patient's clinical features, treatment received, histopathologic presentation of gingival fibromatosis and proper management of the condition are discussed.

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.

Objective: The purpose of this study was to analyze the ultrastructure of gingival connective tissue from patients in one family affected by hereditary gingival fibromatosis (HGF)., Study Design: Electron microscopic examination was performed with gingival tissue from 10 patients from a Brazilian family with 132 members. Fifty of 96 persons at risk for this disorder were affected, which is consistent with an autosomal dominant pattern of inheritance., Results: The extracellular matrix showed flocculent material and collagen fibrils with structural abnormalities and variation in diameter. Increased numbers of oxytalan fibers were identified; however, elastic fibers were rare in the analyzed areas., Conclusions: The structural alterations found in HGF appear similar to those described in certain other heritable collagen disorders, suggesting that HGF should be included in the group of hereditary diseases in which connective tissue alterations have a distinct pattern, in contrast to reactive fibrotic gingival enlargements with no genetic component.

Drug therapy and hereditary factors are two of the main causes of gingival overgrowth (GO). Both of these forms of GO are associated with increased extracellular matrix production by fibroblasts. Transforming growth factor beta (TGF-beta) is an important mediator of wound healing and tissue regeneration, which stimulates fibroblasts to produce extracellular matrix materials. The aim of this immunohistochemical study was to determine whether there is any altered expression of TGF-beta isoforms or its receptors in tissue from patients with drug-induced GO (DIGO; n=10) and hereditary gingival fibromatosis (n=10) when compared to non-overgrowth tissue (n=10). Compared to control tissues, significantly more fibroblasts expressed TGF-beta1 in both DIGO and hereditary gingival fibromatosis tissues (P<0.03). Cells expressing TGF-beta2 were present at control levels in DIGO but were significantly reduced in hereditary gingival fibromatosis (P<0.02). By contrast, the number of TGF-beta3-positive cells was the same in overgrowth tissues and controls. However, because of differences in total fibroblast densities between groups, there was a proportional increase in TGF-beta3 as well as TGF-beta1 expressing cells within both overgrowth populations (P<0.0001). Furthermore, representation of the TGF-beta2-positive phenotype was reduced in hereditary gingival fibromatosis (P<0.01) but increased in DIGO (P<0.005) compared to controls. Absorbance measurements of the positive cell populations showed that the level of expression was significantly higher for TGF-beta1 in hereditary gingival fibromatosis (P<0.002) and significantly lower for TGF-beta3 in DIGO (P<0.03). No significant differences in the numbers of TGF-betaRI- or RII-positive cells were detected between overgrowth tissues and controls. However, there were increases in the proportion of receptor-positive cells in the total cell population analysed in overgrowth tissues (P<0.0001). These results indicate qualitative and quantitative differences in TGF-beta isoform and receptor expression by fibroblasts in gingival overgrowth that may contribute to disease pathogenesis.

Hereditary gingival fibromatosis is a proliferative fibrous lesion of the gingival tissue that causes esthetic and functional problems. Both genetically and pharmacologically induced forms of gingival fibromatosis exist. This paper reports a case of severe generalized hereditary gingival fibromatosis in a 5-year-old girl. Treatment consisted of surgical removal of the hyperplastic fibrous tissue in a series of conventional gingivectomies. Postoperative examination 36 months after the final procedure revealed no signs of recurrence.

Five patients with small, exophytic, asymptomatic fibrous nodules, single or multiple, of the mandibular mucogingival zone of the anterior labial gingiva are presented. Such nodules are benign and resemble small fibromas. The clinical and histologic appearance distinguishes them from entities such as retrocuspid papilla, reticular mandibular gingival ridges, oral hamartomas associated with Cowden's syndrome, tuberous sclerosis oral lesions, and oral nodules secondary to epidermolysis bullosa. Gingival fibrous nodule, or gingival nodule, is the proposed name for this lesion, which is essentially a variation of normal oral structures that may be mistaken for disease.

Gingival fibromatosis is frequently an isolated condition, but rarely associated with some uncommon syndromes. This paper describes an 11-year-old patient with pronounced gingival enlargement, cherubic facial appearance, and psychomotor retardation and discusses the major aspects of the case. The most striking finding orally was the presence of grossly hyperplastic gingiva, which completely covered all teeth except the occlusal surfaces of some teeth. The swelling in the lower part of the face and the appearance of sclera beneath the iris suggest cherubism. The diagnosis was confirmed by the detection of giant cell regenerative granuloma and perivascular eosinophilic particles and osteoclasts after biopsy of the mandible. In this case, surgery was the only effective way to treat the patient. A full-mouth gingivectomy procedure was performed under general anesthesia in 2 stages. The case was followed for 12 months and no recurrence was seen. An appropriate oral hygiene regimen was established.

In this paper, we describe two siblings with Juvenile Hyaline Fibromatosis (JHF) who were diagnosed at the age of 34 and 29 years respectively. JHF is a very congenital disease, mainly diagnosed in the first few years of life, with less than 40 published cases in literature. All the main clinical features of this syndrome, which may be summarised as multiple subcutaneous tumours, marked gingival hypertrophy, flexion contractures and osteolytic lesions were present in both of these cases. Clinical, radiological and histological differential diagnosis of JHF were made. Recent information about histopathology, treatment and prognosis of JHF was also reviewed.

Background: Systemic hyalinoses are genetic generalized fibromatoses characterized by an accumulation of hyalin in the dermis. Two distinctive syndromes are recognized in the literature: infantile systemic hyalinosis (ISH) and juvenile hyaline fibromatosis (JHF). ISH and JHF are sometimes difficult to separate since they show significant overlap., Observations: We report on 3 children from two unrelated families suffering from JHF. The first child is severely handicapped by joint contracture, massive hyperplasia of the gingivae, diffuse skin papules and subcutaneous nodules occupying the scalp, face, perianal area, palms, soles and chest. At the same age, the second child only shows pearly skin papules on the face, groin and perianal area and gingival hyperplasia without joint stiffness or any other subjective complaint. The third patient, a brother of the second child, developed mild skin abnormalities by the end of the first year. The occurrence in siblings and consanguinity in the second family suggests autosomal recessive inheritance. Histological skin examination in the 3 cases showed hyaline deposition in the dermis and abnormal ultrastructure of fibroblasts. Biochemical findings showed mucopolysaccharide abnormalities in both families., Conclusion: Our patients do not only illustrate the different expressions of JHF but also show some overlap with ISH, suggesting a common cause for both disorders. Genetic studies will finally answer this question.

Hereditary gingival fibromatosis (HGF) is an oral condition characterized by the enlargement of the gingiva of both the maxilla and mandible. To study the cell proliferation index of fibroblasts from HGF and normal gingiva (NG), cell cultures from 4 members of the same family with HGF and from 4 healthy patients were established. Our results obtained from 6 different cell proliferation assays clearly showed that the cell proliferation rate was significantly higher in fibroblasts from HGF than from normal gingiva. HGF and control fibroblasts in subconfluent culture densities were typically spindle, but in saturation density HGF cells were shorter than control cells. These data suggest that the higher proliferative index of HGF fibroblasts possibly has a role in the pathogenesis of gingival outgrowth in HGF patients.

Opercula of teeth delayed in eruption were examined histopathologically and immunohistochemically to determine the possible causes for tooth eruption failure. Specimens were obtained from 58 patients with non-erupted teeth by surgical removal of their gingival opercula. Among the 61 specimens, 31 (50.8%) were diagnosed as pericoronal myxofibrous hyperplasia (PMH), 8 (13.1%) as infantile ameloblastic fibromatosis (IAF), and 19 (31.2%) as odontomas. Histopathologically, PMH is characterized by hyperplasia of odontogenic mesenchymal tissues with a myxoid appearance in which odontogenic epithelial islands and mesenchymal multinucleated giant cells are scattered randomly. Between the mucosal epithelium and the PMH, there is a layer of fibrosis, whose matrix is strongly immunopositive for tenascin. The PMH seems to induce its overlying gingival mucosa to remodel the connective tissue, which obstructs tooth eruption. IAF is usually located adjacent to the PMH and shows an ameloblastic fibroma-like histology with atrophic ameloblastic components and poor encapsulation. The findings suggest that IAF associated with PMH is not a true neoplasm and should be distinguished from ameloblastic fibromas by the name of IAF, and that both lesions are included in the range of hamartomas formed only in the pericoronal tissue of teeth in eruption. We propose to categorize these lesions into a new disease entity of pericoronal hamartomas of odontogenic origin.

A case is described in which a child appeared for evaluation with marked gingival overgrowth, facial dysmorphism, and abdominal defects consistent with prune-belly syndrome. The relationship between this case and other reports of gingival enlargement are discussed. Coincidence of the oral, facial, and abdominal abnormalities has not been previously reported.

Juvenile hyaline fibromatosis is a multisystemic disorder characterized by a triad of cephalic fibrous outgrowths, gingival hyperplasia, and flexion contractures. The aim of this study was to find new ultrastructural features that could be useful for differentiating this entity from other types of fibromatosis. Mucosal lesions processed for light and electron microscopy by routine techniques showed hyperactive-appearing spindle-shaped fibroblasts and dysplastic mesenchymal cells. Dilated rough endoplasmic reticulum, prominent Golgi complexes, and multivesicular bodies as well as single membrane vesicles filled with fibrillogranular material were seen within the cytoplasm of dysplastic mesenchymal cells. Many fibrillogranular vesicles contained smaller vesicles. There were also invaginations of the cell membrane that contained fibrillogranular material similar to that seen in the single membrane vesicles, suggesting engulfment of an extracellular substance. The stroma contained both normal and serrated collagen fibrils, microfibrils, and two types of fibrillogranular material, one of them with a characteristic banding pattern. Our clinical and histopathologic findings resemble those previously described in cases of infantile systemic hyalinosis and juvenile hyaline fibromatosis. So many features of these two conditions overlap that it is difficult not to consider them as parts of a spectrum of the same disorder.

Juvenile hyaline fibromatosis is a rare autosomal recessive interstitial disease characterized by nodes and tumours of skin and soft tissues as well as by gingival hyperplasia. The authors described a case of 28-year-old male based on histopathological diagnosis. The patient was admitted to the hospital thrice in his life with the diagnosis of arthrogryphosis. Last time he presented with extensive secondary impetigo in extremities and pachydermia, polymalformation syndrome, multiple subcutaneous tumours, gingival hypertrophy, contractures, osteolytic lesions and positive family history. In histology, tumoriform lesions showed a structureless hyaline matrix often with chondroosseous metaplasia and calcium salts. More or less numerous cells in the matrix had a fibroblastoid appearance with eosinophilic cytoplasm, oval nuclei and frequently pericytoplasmic halo. Electron microscopy revealed dilated cisterns of rough endoplasmic reticulum and a hypertrophied Golgi apparatus. Particles representing calcium salts according to their density were rare. Immunohistochemistry of tumour cells showed vimentin, alpha-1-antichymotrypsin and alpha-1-antitrypsin. The findings concurred with the literature in which, nevertheless, the immunohistochemical picture were not mentioned.

Hereditary gingival fibromatosis (HGF) is a fibrotic enlargement of the gingiva. HGF gingiva contains large amounts of collagen and other extracellular matrix (ECM) molecules. In vitro, HGF fibroblasts produce greater amounts of the ECM components fibronectin (FN) and type 1 collagen than normal human gingival (GN) fibroblasts. Transforming growth factor beta (TGF beta) is a cytokine important in regulating tissue repair and regeneration after injury, and stimulating fibroblast proliferation and the production of FN and collagens. The objective of this study was to determine whether HGF fibroblasts produce TGF beta and, with the use of neutralizing antibodies to TGF beta isoforms, if their increased expression of FN and type 1 collagen is under autocrine TGF beta control. The HGF strains produced greater amounts of TGF beta1 and TGF beta2 (P < or = 0.003) as well as FN (P < or = 0.04) and type 1 collagen (P < or = 0.03) (measured by specific ELISA) than the GN strains. Treatment of HGF fibroblasts with anti-TGF beta1, beta2, or beta3, as well as a combination of all 3 antibodies, decreased their FN production by up to 60% (P < or = 0.04), and was able to decrease FN production by HGF fibroblasts to the levels of the GN fibroblasts. When used alone, the neutralizing antibodies decreased type 1 collagen production by the HGF fibroblasts by up to 40% (P = 0.014), and treatment with all 3 antibodies caused decreases of up to 55% (P = 0.0005). The results suggest that autocrine stimulation by the increased amounts of TGF beta isoforms made by HGF fibroblasts contributes to their increased production of FN and type 1 collagen.

Human gingival fibroblasts derived from tissue explants of two patients with dilantin-induced gingival hyperplasia (DGH) and one patient with idiopathic gingival fibromatosis (GF) were studied with respect to the effect of epidermal growth factor (EGF) on the proliferative characteristics of these cells. Immunohistochemical staining showed that there were more EGF receptor-positive cells among DGH fibroblasts than among either normal gingival fibroblasts (NG) or GF cells. Furthermore, EGF binding studies showed that, in spite of there being no disparity in binding affinity among all these cells, DGH fibroblasts possessed approximately two-fold more EGF receptors than either NG or GF cells. In addition, the growth-promoting effect of exogenously added EGF was concentration-dependent in DGH fibroblasts but was not in either NG or GF cells. All of the above findings clearly demonstrate that DGH and GF fibroblasts exhibit distinct disparity in proliferative responsiveness to EGF and suggest that different mechanisms may be involved in the pathogenesis of these two forms of gingival hyperplasia. These observations also suggest a possible therapeutic approach for blocking EGF-induced cell proliferation in DGH.

HEREDITARY GINGIVAL FIBROMATOSIS (HGF) is a fibrotic enlargement of the gingiva. HGF gingiva contains large amounts of interstitial collagen and other extracellular matrix (ECM) molecules. Increased proliferation and elevated production of the ECM molecules type I collagen and fibronectin (FN) could contribute to the clinical increased bulk of HGF gingiva. Fibroblast strains from HGF gingiva and normal human gingival fibroblast strains (GN) were used in this in vitro study. Fibroblast proliferation was determined by ELISA which measured the incorporation of 5-bromo-2'-deoxyuridine into DNA. The results showed that HGF fibroblast strains proliferated more rapidly than GN fibroblasts (68% to 488% increase, depending on the strains) (P < or = 0.01), the only exception being one HGF strain versus one normal strain. All HGF strains produced greater amounts of FN (measured by ELISA) than all of the normal fibroblast strains (23% to 49% increase, depending on the strain) (P < or = 0.04). Similarly, all HGF strains made significantly greater (P < or = 0.3) amounts of type I collagen (also measured by ELISA) than all of the normal strains (55% to 235% increase, depending on the strain). The results show that, in vitro, HGF fibroblasts display several phenotypic characteristics of activated fibroblasts: increased proliferative rates as well as increased production of FN and type I collagen, consistent with in vitro studies of fibroblasts derived from other types of fibrotic tissue. These results suggest that the increased proliferation of HGF fibroblasts and their increased production of extracellular matrix molecules such as collagen and FN may contribute to the clinical gingival enlargement characteristics of HGF.

Gingival fibromatosis is an uncommon but benign oral disease which causes progressive and non-hemorrhagic enlargement of the gingiva. There are two types of gingival fibromatosis: idiopathic gingival fibromatosis (GF), which is of unknown cause and may be associated with hereditary factors, and drug-induced gingival fibromatosis which is caused primarily by phenytoin intake. In cases of gingival fibromatosis, either the teeth are delayed in emergence or most of the crowns are embedded in the soft tissue even after full eruption. The objective of this study was to examine the basis of excess collagen formation in the two types of gingival fibromatosis in four patients admitted to the dental clinic at Kaohsiung Medical College Hospital, Kaohsiung, Taiwan. There were two male patients, aged ten (Case 1) and sixteen (Case 2), with idiopathic gingival fibromatosis and hypertrichosis, and two female patients, aged sixteen (Case 3) and eleven (Case 4), with Dilantin-induced gingival hyperplasia (DGH). Cultures of gingival fibroblast were established either from clinically excised hyperplastic tissues or from pre-orthodontic surgical normal gingiva. The synthesis of collagen and levels of prolyl hydroxylase, a key enzyme in collagen synthesis, were examined in the healthy and affected fibroblasts. Our studies showed that after two days in culture, fibroblasts from all four patients multiplied faster than healthy gingival fibroblasts, though the amount of DNA and protein per cell remained unchanged. In addition, all cultures (except Case 1) had a 2- to 3- fold increase of prolyl hydroxylase activity over that of the controls. As in the cases of prolyl hydroxylase activity, Case 1 did not show any change in collagen synthesis when compared to the control. However, Cases 2, 3, and 4 showed appreciable collagen increases in the cell and medium: 61% and 60% for Case 2; 16% and 36% for Case 3; and 21% and 80.7% for Case 4 respectively.

Juvenile hyaline fibromatosis is an extremely rare inherited condition, probably resulting from an inborn error of metabolism. It is characterized by cutaneous nodules, gingival hypertrophy and joint contractions. It affects children but usually it is not present at birth, and is microscopically characterized by a conspicuous hyalinization of the connective tissue.

Hereditary gingival fibromatosis, or HGF, is characterized by varying degrees of attached gingival hyperplasia. The authors describe a case of generalized severe hereditary gingival fibromatosis involving the maxillary and mandibular arches. Removal of excess gingival tissue by conventional gingivectomy dramatically improved the patient's appearance.

Presented in this paper is a clinico-pathological report of 11 cases of gingival fibromatosis. Of the 11 patients in our series, 2 cases (18.2%) with family history began to have symptom from age of 3, which is manifested by the involvement of whole gum. 9 patients (81.8%), for which the etiology was unknown, had onset of the disease at the periods of deciduous dentition, mixed dentition or permanent dentition, with localized or overall involvement of gingiva. The disease is characterized by diffuse or localized proliferation of gum. Microscopically, the proliferating gum tissue revealed well-developed fibrotic structure with few cells; and the fibers were densely arranged without clear boundary. The fibrotic tissues showed a diminishing of blood vessels and the infiltration of inflammatory cells is not conspicuous.

Hereditary gingival fibromatosis is an uncommon congenital anomaly of undetermined etiology, the condition is manifested as a dense, diffuse, smooth or nodular overgrowth of the gingival tissue, usually begins with the eruption of the permanent incisors. However, it may happen as early as the eruption of the deciduous teeth. The case, a 30 years old woman with normal stature, visited our dental clinic because of "overgrown gum" which greatly affected mastication and appearance. A review of her past medical history revealed no relevant diseases. Oral examination showed gross fibrous enlargement of gingival without inflammation or swelling. Her father, two sisters and one daughter also suffered from this disease. Microscopically, there is a obvious increase in the amount of connective tissue that is relatively avascular and consists of densely arranged collagen bundles and numerous fibroblasts. The surface epithelium is thickened and acanthotic with elongated rete pegs. Gingivectomy with flap operation were performed to correct the gingival overgrowth. After periodontal treatment, two fixed partial dentures were constructed to replace the missing right upper and lower first molars. One year after operation, no obvious recurrence was noted clinically.

Juvenile hyaline fibromatosis (JHF) is a rare hereditary disorder named by Drescher et al. in 1969. As recently as 1985, only 30 cases had been reported worldwide. We report the case of a 9-year-old girl who was diagnosed with JHF at age 3 and has been closely followed since. She initially had slowly growing multiple soft tumors over her entire body as well as hypertrophic gingiva and mild bone deformities. She was originally misdiagnosed with infantile myofibromatosis at age 3. However, at age 6, because of the light and electron microscopic findings of the tumors, she was diagnosed as having JHF. Currently, at age 9, she has nodular lesions developing over her body as well as bone changes that are progressing with no evidence of regression.

Fibromatosis may be defined as diffuse poorly circumscribed overgrowth of the fibrous tissue that infiltrates adjacent normal tissues. They are difficult to eradicate surgically, and recur but not metastasize. Gingival fibromatosis is generally regarded as a disease that leads to an extensively diffuse and remarkable hyperplasia of the maxillo-mandibular gingiva. Occasionally, this lesion covers all teeth. The histogenesis of the fibromatosis remains unexplained. Trauma, endocrine, idiopathic factors and genetic factors have been implicated, but it is uncertain whether any of then play a major role in the development of the disease. Occasional cases with familial history have been reported. The treatment of choice would appear to been block resection of the tumor and surrounding normal structures. Although, this lesion has a high recurrent rate. For this reason, in many of the case reports, that it has been recommended that the follow up period is considerably less than three years.

This article describes the occurrence of numerous calcifications, amyloid deposits, and islands of odontogenic epithelium in the gingiva of 3 siblings with familial gingival fibromatosis. These microscopic features have not been reported previously in patients with this condition.

A case of hereditary gingival fibromatosis is presented. Treatment consisted of apically positioned flap surgery and CO2 laser evaporation. Diagnostic and treatment issues are discussed.

Hereditary gingival fibromatosis can occur as an isolated trait or as part of a syndrome. We report on three generations of one family featuring an autosomal dominant syndrome with variable expression of gingival fibromatosis with associated hearing deficiencies, hypertelorism, and supernumerary teeth. We propose that this represents a new syndrome within the spectrum of those including gingival enlargement.

Hereditary gingival fibromatosis (GF) is a special type of fibrous overgrowth classified as non-inflammatory gingival enlargement. Microscopically, the connective tissue consists of coarse collagen bundles and fibroblasts. The ultrastructural examination of fibrous gingival hyperplasia reveals that fibroblasts phagocyte the mast cell granules and mast cells stimulate collagen synthesis which results in hyperplasia. In the ultrastructural examination of phenytoin-induced hyperplasia, fibroblasts, phagocytosing mast cell granules were also found. Based on these findings, the purpose of this study is to establish whether there is a relationship between fibroblasts and mast cells in GF. The gingival tissues of 5 patients with GF were examined ultrastructurally. In the connective tissue, well-defined bundles of collagen fibres were found together with fibroblasts and capillaries. There were mast cells around these capillaries which had collapsed lumens. The proximity of the mast cells and fibroblasts may indicate that mast cells play some role on collagen synthesis of fibroblasts.

Twenty-six persons with Laband syndrome have been reported in the literature to date. We report two further unrelated cases and suggest that a skeletal anterior open bite and symmetric soft tissue swelling of the vault of the palate be added to the variable features associated with the syndrome. There is clear evidence in the literature of autosomal dominant inheritance but our analysis indicates that in some cases recessive transmission is likely.

Hereditary gingival fibromatosis is characterized by varying degrees of attached gingival hyperplasia and may in rare cases present as a feature of a generalized syndrome. It is usually inherited as an autosomal dominant condition though recessive forms are described. The dental and genetic features of an affected brother and sister with a probably unique autosomal recessive hereditary fibromatosis syndrome are presented.

This report describes two patients with familial gingival fibromatosis. Treatment for both patients involved gingivectomies to provide acceptable gingival function and appearance. The first patient has been monitored for only a short period, the second patient for 11 years. Neither patient has shown any recurrence of the condition.

Hypertrichosis universalis congenita is an extremely rare disorder characterized by generalized hypertrichosis. It is generally accepted as being inherited as an autosomal dominant trait with varying expression. Many aspects of this disease are still unknown. Several reports associating hypertrichosis and gingival fibromatosis raise the question of whether they are separate entities or the same disease with different expressions of the underlying process. Hypertrichosis universalis congenita occurred in a 6-year-old girl without known family history. Her facial features were simian-like and her gingiva was moderately hyperplastic. We pose the question of whether or not these phenomena are related.

Familial gingival fibromatosis affecting six members among three generations of an Islamic family is reported. Clinical findings are reported and the inheritance pattern is discussed. Histopathologic examination of the excised tissue revealed mainly fibroblasts with prominent, rough-surfaced endoplasmic reticulum. Cells with vesicular nuclei, resembling mast cells, were also observed.

Forty-nine cases of isolated familial and idiopathic gingival fibromatoses, consisting of 12 cases from six families and 37 cases of idiopathic gingival fibromatosis, were reviewed. Pedigrees of five families revealed various penetrances and genetic heterogeneity as suggested by the presence of both autosomal dominant and autosomal recessive inheritances. Ultrastructurally, the lesions were composed of fibroblast-like cells and myofibroblast-like cells, with the former being the predominant cell type. The 267 cases of familial and idiopathic gingival fibromatoses were analyzed, and they with or without hypertrichosis, mental retardation, and/or epilepsy. These included 49 cases seen by the authors, 50 cases from the Japanese literature, and 168 cases from non-Japanese literature. Isolated gingival fibromatosis occurred more frequently after age of 12 years (P less than 0.0074). There was no significant difference in age of onset between generalized and localized forms of the idiopathic gingival fibromatosis. Gingival fibromatosis with hypertrichosis and mental retardation and/or epilepsy occurred frequently before 12 years (P less than 0.069). It has been shown that heterogeneity of the gingival fibromatosis is a result of either histologic heterogeneity, genetic heterogeneity, or a combination with other systemic disorders.

Two unrelated children with a severe form of juvenile hyaline fibromatosis are described. In addition to painful flexion contractures of all of the large joints, oral and skin lesions, and typical radiologic appearance of osteolytic defects, both girls had marked growth retardation and recurrent infections. Both children died in early infancy of overwhelming infection.

It is presented a case of fibromatous gingival idiopathic hyperplasy of rare imponence. While describing the clinic characteristic the morphology of the lesion, the provoked functional troubles and the therapeutic program are evidenced. It is also confirmed its pathologic characteristic on hereditary base.

Five cases of familial and idiopathic gingival fibromatosis were reported. One case exhibited peculiar histologic features, which consisted of active growth of the fibroblastic cells. One hundred and ninety-six cases of gingival fibromatosis, whose clinical and/or pathologic findings were known, were collected from foreign literatures. The heterogeneity in gingival fibromatosis was discussed.

The elastic fibers system, as visualised by orcein after potassium peroxymonosulfate oxidation in fibromatous lesions of the oral mucosa, is generally sparse. Rich orcein + fibers network was observed in only 5 of our 20 cases of reactive fibromas. Orcein + fibers are absent in the inflammatory areas. These observations rise the problem of the elastogenesis by the fibroblast in healing and neoplasia, and confirm the role played by the proteolytic enzymes during inflammation.

A case report of gingival fibromatosis in association with growth hormone (GH) deficiency due to a lack of growth hormone releasing factor (GRF) is presented. The girl is the youngest member of a family of eight children, five of whom lack the same hormone and have or have had similar gingival enlargements. After the growth hormone deficiency had been diagnosed and hormone substitute administered the dental age of the girl presented came closer to that of her age and sex-matched controls but did not reach the corresponding values even though the teeth were exposed by excising the overgrown gingiva. Test fibroblasts cultured from the overgrown gingiva proliferated at a slower rate than those cultured from age-matched controls. Total RNA was extracted from the test and three control fibroblasts and examined by Northern hybridisation using cDNAs for pro alpha 1(I) and pro alpha 1(III) chains. The amount of type I and III procollagen mRNAs were lower in the test fibroblasts as compared to the controls.

The aetiopathogenesis, histology and clinical treatment of a rare case of familial gingival fibromatosis are analysed with particular emphasis on the relationship between the histological picture and the pathogenesis of the condition. In tackling the problem of treatment, the paper recommends the use of gingivectomy with an internal rather than the classic external chamfer.