Hereditary gingival fibromatosis

Classification according to ICD-10
K06.1	Gingival hyperplasia - gingival fibromatosis
ICD-10 online (WHO version 2019)

The Hereditary gingival fibromatosis (synonyms: Autosomal dominant gingival hyperplasia, autosomal dominant gingival fibromatosis) refers to a group of rare congenital diseases with the main feature of a fibromatosis of the gingiva .

distribution

The frequency is unknown; the inheritance is autosomal dominant .

Cause and classification

Depending on the underlying genetic change, different forms can be distinguished:

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

In the context of syndromes

• Fibromatosis, gingival - progressive hearing loss ( Jones syndrome )
• Gingival fibromatosis - facial dysmorphism
• Gingival fibromatosis-hypertrichosis syndrome (CGHT; hirsutism - congenital gingival hyperplasia; hypertrichosis with or without gingival hyperplasia; congenital generalized hypertrichosis terminalis)
• Jones Syndrome
• Juvenile hyaline fibromatosis
• Mannosidosis
• Ramon syndrome
• Rutherfurd Syndrome
• Infantile systemic hyalinosis
• Zimmermann-Laband Syndrome

Individual evidence

1. ↑ ^{a b} Hereditary gingival fibromatosis. In: Orphanet (Rare Disease Database).
2. ↑ GINGF1.  In: Online Mendelian Inheritance in Man . (English)
3. ↑ GINGF2.  In: Online Mendelian Inheritance in Man . (English)
4. ↑ GINGF3.  In: Online Mendelian Inheritance in Man . (English)
5. ↑ GINGF4.  In: Online Mendelian Inheritance in Man . (English)
6. ↑ Gingival fibromatosis - facial dysmorphism. In: Orphanet (Rare Disease Database).
7. ↑ Gingival fibromatosis-hypertrichosis syndrome. In: Orphanet (Rare Disease Database).
8. ↑ T. Ramakrishnan, Manmeet Kaur: Multispeciality Approach in the Management of Patient with Hereditary Gingival Fibromatosis: 1-Year Followup: A Case Report. In: International Journal of Dentistry. 2010, vol. 2010, p. 1, doi: 10.1155 / 2010/575979 .

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !