Hereditary gingival fibromatosis

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Hereditary gingival fibromatosis

ORPHA:2024Malformation syndrome

Also called Autosomal dominant gingival fibromatosis · Autosomal dominant gingival hyperplasia · Hereditary gingival hyperplasia

What it is

Hereditary gingival fibromatosis (HGF) is a rare benign, slowly progressive, non-inflammatory fibrous hyperplasia of the maxillary and mandibular gingivae that generally occurs with the eruption of the permanent (or more rarely the primary) dentition or even at birth. It presents as a localized or generalized, smooth or nodular overgrowth of the gingival tissues of varying severity. It can be isolated, with autosomal dominant inheritance, or as part of a syndrome.

Key facts

Age of onset
All ages
Inheritance
Autosomal dominant
Classified as
Malformation syndrome

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

RESTDisease-causing germline mutation(s)
SOS1Disease-causing germline mutation(s)
GINGF2Candidate gene tested

ICD-10 codes

K06.1filed under a broader ICD-10 category

Cross-references

MEDDRA 10088210MESH C562884MONDO 0016070OMIM 135300OMIM 605544OMIM 609955OMIM 611010OMIM 617626UMLS C0399440

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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