Current concepts on gingival fibromatosis‐related syndromes

Abstract

Gingival fibromatosis is a rare, benign, slowly-growing fibrous overgrowth of the gingiva, with great genetic and clinical heterogeneity. Gingival fibromatosis/overgrowth can be inherited as an isolated trait (hereditary gingival fibromatosis) and/or as a component of a syndrome, or it can be drug induced. As a clinical manifestation of a syndrome, gingival fibromatosis is usually associated with generalized hypertrichosis, mental retardation, or epilepsy. Gingival fibromatosis and its related syndromes are mainly inherited in an autosomal-dominant manner, but autosomal-recessive inheritance has also been reported. Clinical syndromic presentation includes Zimmermann-Laband syndrome, Ramon syndrome, Rutherford syndrome, Cowden syndrome, Cross syndrome, Göhlich-Ratmann syndrome, Avani syndrome, and I-cell disease. However, a phenotypic overlap has been suggested, as many combinations of their systemic manifestations have been reported. Treatment of choice is usually gingivectomy with gingivoplasty. Before any therapy, clinical practitioners must take into consideration the clinical course of a particular syndrome and every possible functional and esthetic disorder.

Changing spectrum of Zimmerman-Laband syndrome: a six years follow up case report of a family

“…11 The present report supports that ZLS has a wide clinical spectrum because of the presence of massive idiopathic gingival enlargement, hypertrichosis, hyperextensibility of joints and some additional clinical features. With the notice of additional clinical features of bimaxillary protrusion with disproportionately long arms, forearms and fingers, phenotyping characters of ZLS could be stretched and resumed to be variable.…”

Generalized gingival enlargement associated with aggressive periodontitis in a 20-year-old patient

“…Idiopathic cases are less frequent reported than the ones associated with hereditary factors, including syndromes and non-syndromic conditions, inflammatory diseases of gingiva, non-genetic diseases and medications 2,10 . On the other hand, aggressive periodontitis (AP) is a specific type of periodontal disease in which the amounts of biofilm are inconsistent with the extensive periodontal destruction.…”

“…We may be facing a new syndrome or simply an occasional finding 2 . The real meaning and importance of the association between GE and AP remains unknown.…”

The Autosomal Recessive Inheritance of Hereditary Gingival Fibromatosis

“…According to various studies the pedigree analyses of HGF families confirm the autosomal dominant inheritance of HGF trait, although autosomal recessive or even as X-linked inherited cases have also been reported in some literature [7, 8]. Mutation in SOS-1 or son-of-sevenless gene is responsible for this disease; this has been reported by several authors [5, 9–11]. It is a guanine nucleotide-exchange factor that functions in the transduction of signals that control cell growth and differentiation [11].…”