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Juvenile hyaline fibromatosis (Murray-Puretic-Drescher syndrome): Oral and systemic findings in siblings

1986; Elsevier BV; Volume: 62; Issue: 4 Linguagem: Inglês

10.1016/0030-4220(86)90289-6

1878-2175

James J. Sciubba, Theodore Niebloom,

Soft tissue tumor case studies

G ingival fibromatosis is a relatively uncommon benign oral condition, clinically characterized by a slowly progressive, nonhemorrhagic, nonpainful local or generalized enlargement of maxillary and mandibular gingiva. The gingival tissues are firm to palpation, with a keratinized, stippled surface and pink color. There are several conditions under which gingival fibromatosis is seen as the oral finding and can be correlated with systemic or extraoral findings, many of which have a genetic basis. In most cases the condition of gingival fibromatosis develops before the age of 20, and it most frequently occurs at the time of eruption of either the primary or the secondary dentition. A thorough review of the literature reveals that hereditary gingival fibromatosis, in addition to being found as an isolated entity, may also be a component in a variety of genetic syndromes, as noted in Table I.