Fibromatosis gingivae Q38.6

Term for a heterogeneous group of clinical pictures which have in common an idiopathic, diffuse, non-neoplastic, connective tissue proliferation of the gingiva ( gingival hyperplasia) and hard palate. Some of the diseases are exclusively intraoral (see below gingivitis hyperplastica), some cases are combined with malformations of other organ systems (see below fibromatoses).

On the one hand, the disease occurs spontaneously; furthermore, various hereditary, autosomal-dominant (less frequently recessive) inherited forms are known:

• GINGF1 with mutations in the SOS1 gene at gene locus 2p22.1
• GINGF2 with mutations at the gene locus 5q13-q22
• GINGF3 with mutations at gene locus 2p23.3-p22.3
• GINGF4 with mutations at gene locus 11p15

Gingival fibromatosis is a partial symptom of various types of fibromatosis. syndromes (Balaji P et al. 2017):

• Gingival hyperplasia/hypertrichosis syndrome (OMIM 1354400)
• Ramon syndrome (OMIM 2266270)
• Jones syndrome (Gingival fibromatosis - progressive hearing loss)
• Gingival fibromatosis - Facial dysmorphia
• Juvenile hyaline fibromatosis
• Rutherfurd Syndrome
• Infantile systemic hyalinosis
• Zimmermann-Laband Syndrome

Discussed are idiopathic genesis as well as autosomal dominant inheritance, combined with local irritant factors. A mutation in the Son-of-Sevenless-1 gene is assumed to be a possible etiological cause of non-syndromal hereditary gingival fibromatosis
(Gawron K et al. 2016).

Start with eruption of the milk teeth, continuation after the 2nd dentition. In rare cases gingival hyperplasia can be combined with hypertrichosis ( Hypertrichosis lanuginosa) (so-called hair people).

The upper jaw is affected more than the lower jaw. The frontal area is more excessively affected than the lateral area.

Mostly symmetrically appearing gum growths of both jaws with smooth or papillomatous surface and blurred transition to the surroundings. Starting point are interdental papillae. Fibromatosis can completely overgrow the local teeth (pseudo-anodontics). Malocclusion, articulation and chewing difficulties are the consequences of gingival hyperplasia.

Fibre-rich tissue, fuzzy boundary, myxoid degeneration of connective tissue; multinucleated giant cells.

Reactive gingival hyperplasia due to ingestion of various Drugs(calcium channel blocker, phenytoin, Ciclosporin).

The therapy of choice is excision of fibromatous tissue (gingivectomy), if necessary extraction of teeth in places. The excision must be performed by skilled speialists (Almiñana Pastor PJ et al. 2017).

1. Almiñana Pastor PJ et al (2017) Hereditary gingival fibromatosis: Characteristics and treatment approach. J Clin Exp Dent 9:e599-e602.
2. Balaji P et al (2017) Gingival fibromatosis with hypertrichosis syndrome: Case series of rare syndrome. Indian J Dent Res 28:457-460.
3. Gawron K et al (2017) Analysis of mutations in the SOS-1 gene in two Polish families with hereditary gingival fibromatosis. Oral Dis 23:983-989.
4. Gawron K et al (2016) Gingival fibromatosis: clinical, molecular and therapeutic issues. Orphanet J Rare Dis. 11:9.
5. Ko YC et al (2016) Idiopathic Gingival Fibromatosis: Case Report and Review of the Literature. At J Dermatopathol 38:e68-71.