Familial Gingival Fibromatosis With Unusual Histologic Findings

Abstract

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.

Hereditary Gingival Fibromatosis Associated With Generalized Aggressive Periodontitis: A Case Report

“…2,34, 35 We have not observed any calcified structures associated with odontogenic epithelial remnants in the gingiva. 36 An electron microscopic analysis of the ultrastructure of gingival connective tissue from one family of HGF patients who has been extensively studied 9,35 revealed collagen fibrils with structural abnormalities including diameter variations, as well as the presence of increased oxytalan and very few elastic fibers. 37 Previous reports examining the biological pathways that may influence HGF showed that the expression and production of MMP-1 and MMP-2 and their inhibitors, TIMP-1 and TIMP-2 were not altered, 16 while TGF-β production was two times higher in HGF fibroblasts compared to control fibroblasts.…”

Effect of Transforming Growth Factor‐β1, Interleukin‐6, and Interferon‐γ on the Expression of Type I Collagen, Heat Shock Protein 47, Matrix Metalloproteinase (MMP)‐1 and MMP‐2 by Fibroblasts from Normal Gingiva and Hereditary Gingival Fibromatosis

“…3 The most prominent pathologic manifestation of this disease is an excessive accumulation of extracellular matrix, predominantly type I collagen. 4,5 Many studies have shown increased transcriptional and translational levels of type I collagen in both tissue and fibroblast cultures derived from gingiva of HGF patients. [6][7][8] During the collagen biosynthesis, nascent single procollagen polypeptides, immediately after translation, undergo modification in the endoplasmatic reticulum, form triple helical chains, and are secreted as procollagen into the extracellular space via the Golgi apparatus.…”

Calcified fibromatosis of the neck in 4-year old girl: Rapid growth, rapid therapy

“…Stippled calcium may be present rather in juvenile aponeurotic fibroma. Calcified fibromatosis was also reported in gingiva and breast [14,15].…”