Gingival fibromatosis with hemi-osseous hyperplasia of jaws, focal maxillary viral papillomatosis of gingiva, fissured tongue and congenitally missing anterior teeth: A case report and surgical management of a new syndrome.

Gingival fibromatosis is characterized by fibrotic enlargement of the gingiva that can occur as inherited or sporadic form. Inherited form can be an isolated trait or as a component of a syndrome. This article reports a 35 year old male patient affected by gingival fibromatosis associated with hemiosseous hyperplasia of mandible, maxilla, and zygoma on the right side, viral papillomatosis of maxillary anterior gingiva, fissured tongue and congenitally missing anterior teeth. The patient was subjected to phase I and phase II periodontal therapy. There was no evidence of recurrence of the enlargement after one year but the papillomatosis recurred. Gingival fibromatosis has been reported to be associated with various other abnormalities but not with those described in our case. This observation raises the possibility that the coexistence of these entities in our case may represent a new syndrome.

Caso para diagnóstico / Case for diagnosis

Fibromatose hialina juvenil e hialinose sistêmica infantil são desordens autossômicas recessivas, raras da infância. À histologia, há depósito de material hialino na derme e subcutâneo. As características clínicas principais são: lesões pápulo-nodulares, hipertrofia gengival, contratura articular, lesões ósseas osteolíticas e retardo no crescimento. Mutações no mesmo gene foram identificadas nas duas condições, sugerindo que sejam espectros da mesma doença.

Juvenile hyaline fibromatosis and infantile systemic hyalinosis are rare autossomal recessive disorders with onset in infancy or early childhood. Histological examination shows deposit of hyaline material in the dermis and subcutaneous tissue. Clinical features include papulonodular skin lesions, gingival hypertrophy, flexion contractures of joints, osteolytic bone lesions and stunted growth. Mutations in the same gene were detected in both conditions, suggesting that they may be variants of the same disorder.

Tratamiento de la hiperplasia gingival en una escuela odontológica de Brasil: conceptos generales, diagnóstico y tratamiento / Treatment of gingival hyperplasia in a dental school of Brazil: general concept, diagnosis and treatment

La hiperplasia gingival es una condición patológica benigna en la que aumenta de volumen de tejido gingival de forma lenta y gradual, causando importantes molestias estéticas y funcionales en los pacientes. Se clasifica como idiopática, inflamatoria, hereditaria y asociada a drogas, según su etiología, donde cada una tiene sus peculiaridades que deben ser bien comprendidas por el cirujano-dentista, a fin de lograr el tratamiento adecuado para cada caso. Este artículo revisa la literatura reciente sobre la hiperplasia gingival, abordando su etiología y tratamiento, presentando un caso clínico de fibromatosis gingival hereditaria y su tratamiento quirúrgico.

The gingival hyperplasia is a condition in which increase in volume in benign, slow and gradual development of the gingival tissue, causing significant discomfort and aesthetic function in patients. It is classified as idiopathic, inflammatory, and hereditary drug, according to its etiology, and that each variant has its peculiarities that must be well understood by the Clinical-Dentist, in order to achieve the appropriate treatment for each case. This paper reviews the recent literature on gingival hyperplasia, addressing its etiology and treatment given, and clinical case report of hereditary gingival fibromatosis in the surgical therapy was performed successfully.

Gingival fibromatosis and growth hormone deficiency syndrome--report of a rare case and review of literature.

Oikarinen et al in 1989 reported a syndrome associated with generalized gingival fibromatosis and growth hormone deficiency. This is a case report of a 15-year-old female patient who presented to the Government Dental College, Chennai with generalized gingival fibromatosis and growth hormone deficiency. Interestingly, the histopathology of the excised gingival overgrowth showed dense collagenous connective tissue in which were strewn calcified structures that resembled cementum. This syndrome is being reported for the second time after its first case report in 1989 by Oikarinen et al. We are herewith reporting this case for its rarity with a brief review of literature of syndromes associated with generalized gingival fibromatosis.

Efeitos da ciclosporina A sobre a síntese de proteínas de matriz extracelular de fibroblastos de gengiva normal e de portadores de fibromatose gengival hereditária / The effect of cyclosporin on the synthesis of extracelular matrix proteins in fibroblasts of patients with hereditary gingival fibromatosis

A fibromatose gengival pode ser hereditária ou induzida por drogas. O padräo da doença sugere que os fibroblastos gengivais podem produzir grandes volumes de substância extracelular. Um dos efeitos colaterais da ciclosporina A (CsA) é a induçäo de fibromatose gengival. Os efeitos de 3 diferentes concentraçöes de CsA sobre a morfologia, síntese de colagenase, de fibronectina e atividade colagenolítica, foram estudados "in vitro" em 6 linhagens de fibroblastos, 3 de gengivas normais e 3 de fibromatose gengival hereditária. Os fibroblastos foram cultivados com meio contendo concentraçöes de 250, 500 e 1.000 ng/ml de CsA, por 24, 48 e 78h. O meio foi coletado e submetido a ensaios enzimográficos e de atividade colagenolítica. A síntese de fibronectina foi avaliada através de "dot blot" e de proteína total pela análise eletroforética do sobrenadante.