Hereditary gingival fibromatosis

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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

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 .