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Fibromatosis, gingival, 4(HGF4; GINGF4)

MedGen UID:
370209
•Concept ID:
C1970245
•
Disease or Syndrome
Synonyms: GINGF4; Hereditary gingival fibromatosis, 4
 
Monarch Initiative: MONDO:0012598
OMIM®: 611010

Definition

Hereditary gingival fibromatosis is a benign disorder manifested by a slowly progressive overgrowth of the oral gingival tissues, which results in the teeth being partially or totally engulfed by keratinized gingiva (summary by Zhu et al., 2007). For general phenotypic information and a discussion of genetic heterogeneity of hereditary gingival fibromatosis, see GINGF1 (135300). [from OMIM]

Professional guidelines

PubMed

Four novel mutations of FAM20A in amelogenesis imperfecta type IG and review of literature for its genotype and phenotype spectra.
Nitayavardhana I, Theerapanon T, Srichomthong C, Piwluang S, Wichadakul D, Porntaveetus T, Shotelersuk V
Mol Genet Genomics 2020 Jul;295(4):923-931. Epub 2020 Apr 3 doi: 10.1007/s00438-020-01668-8. PMID: 32246227
Hyaline fibromatosis syndrome: Clinical update and phenotype-genotype correlations.
Casas-Alba D, Martínez-Monseny A, Pino-Ramírez RM, Alsina L, Castejón E, Navarro-Vilarrubí S, Pérez-Dueñas B, Serrano M, Palau F, García-Alix A
Hum Mutat 2018 Dec;39(12):1752-1763. Epub 2018 Sep 17 doi: 10.1002/humu.23638. PMID: 30176098

Recent clinical studies

Etiology

Use of calcium channel blockers in dermatology: a narrative review.
Lo Y, Lin LY, Tsai TF
Expert Rev Clin Pharmacol 2021 Apr;14(4):481-489. Epub 2021 Feb 26 doi: 10.1080/17512433.2021.1894128. PMID: 33612036
Syndromes with gingival fibromatosis: A systematic review.
Costa CRR, Braz SV, de Toledo IP, Martelli-Júnior H, Mazzeu JF, Guerra ENS, Coletta RD, Acevedo AC
Oral Dis 2021 May;27(4):881-893. Epub 2020 May 25 doi: 10.1111/odi.13369. PMID: 32335995
Gain-of-Function Mutations in KCNN3 Encoding the Small-Conductance Ca(2+)-Activated K(+) Channel SK3 Cause Zimmermann-Laband Syndrome.
Bauer CK, Schneeberger PE, Kortüm F, Altmüller J, Santos-Simarro F, Baker L, Keller-Ramey J, White SM, Campeau PM, Gripp KW, Kutsche K
Am J Hum Genet 2019 Jun 6;104(6):1139-1157. Epub 2019 May 30 doi: 10.1016/j.ajhg.2019.04.012. PMID: 31155282Free PMC Article
In vitro testing the potential of a novel chimeric IgG variant for inhibiting collagen fibrils formation in recurrent hereditary gingival fibromatosis: chimeric antibody in a gingival model.
Gawron K, Lazarz-Bartyzel K, Lazarz M, Steplewska K, Pyrc K, Potempa J, Chomyszyn-Gajewska M
J Physiol Pharmacol 2014 Aug;65(4):585-91. PMID: 25179091
Infantile systemic hyalinosis.
Giri PP, Raushan R, Ghosh A, Pal P
Indian Pediatr 2012 Jan;49(1):62-4. PMID: 22318104

Diagnosis

Hyaline fibromatosis syndrome with a novel 4.41-kb deletion in ANTXR2 gene: A case report and literature review.
Zhu Y, Du X, Sun L, Wang H, Wang D, Wu B
Mol Genet Genomic Med 2022 Aug;10(8):e1993. Epub 2022 Jun 20 doi: 10.1002/mgg3.1993. PMID: 35726349Free PMC Article
Epilepsy in KCNH1-related syndromes.
Mastrangelo M, Scheffer IE, Bramswig NC, Nair LD, Myers CT, Dentici ML, Korenke GC, Schoch K, Campeau PM, White SM, Shashi V, Kansagra S, Van Essen AJ, Leuzzi V
Epileptic Disord 2016 Jun 1;18(2):123-36. doi: 10.1684/epd.2016.0830. PMID: 27267311
Infantile systemic hyalinosis.
Giri PP, Raushan R, Ghosh A, Pal P
Indian Pediatr 2012 Jan;49(1):62-4. PMID: 22318104
Gingival fibrous nodule.
Giunta JL
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999 Oct;88(4):451-4. doi: 10.1016/s1079-2104(99)70060-0. PMID: 10519753
Periodontal and soft-tissue abnormalities.
Delaney JE
Dent Clin North Am 1995 Oct;39(4):837-50. PMID: 8522045

Therapy

Use of calcium channel blockers in dermatology: a narrative review.
Lo Y, Lin LY, Tsai TF
Expert Rev Clin Pharmacol 2021 Apr;14(4):481-489. Epub 2021 Feb 26 doi: 10.1080/17512433.2021.1894128. PMID: 33612036
Epilepsy in KCNH1-related syndromes.
Mastrangelo M, Scheffer IE, Bramswig NC, Nair LD, Myers CT, Dentici ML, Korenke GC, Schoch K, Campeau PM, White SM, Shashi V, Kansagra S, Van Essen AJ, Leuzzi V
Epileptic Disord 2016 Jun 1;18(2):123-36. doi: 10.1684/epd.2016.0830. PMID: 27267311
Collagen synthesis in idiopathic and dilantin-induced gingival fibromatosis.
Huang JS, Ho KY, Chen CC, Wu YM, Wang CC, Ho YP, Liu CS
Kaohsiung J Med Sci 1997 Mar;13(3):141-8. PMID: 9109300
Enhanced cytokine production and collagen synthesis of gingival fibroblasts from patients with denture fibromatosis.
Nakao K, Yoneda K, Osaki T
J Dent Res 1995 Apr;74(4):1072-8. doi: 10.1177/00220345950740040701. PMID: 7782537
Collagenolysis by human gingival fibroblast cell lines.
Rose GG, Robertson PB
J Dent Res 1977 Apr;56(4):416-24. doi: 10.1177/00220345770560040901. PMID: 265971

Prognosis

Idiopathic gingival fibromatosis and primary analysis of dominant bacteria in subgingival biofilm: a case report.
Zhu J, Fan X, Ding L, Song T
J Int Med Res 2024 Apr;52(4):3000605241245302. doi: 10.1177/03000605241245302. PMID: 38663906Free PMC Article
Hub Genes, Possible Pathways and Predicted Drugs in Hereditary Gingival Fibromatosis by Bioinformatics Analysis.
Yang RX, Shi F, Du SN, Luo XY, Wang WQ, Yuan ZL, Chen D
Chin J Dent Res 2024 Mar 28;27(1):101-109. doi: 10.3290/j.cjdr.b5128671. PMID: 38546525
Hyaline fibromatosis syndrome: Clinical update and phenotype-genotype correlations.
Casas-Alba D, Martínez-Monseny A, Pino-Ramírez RM, Alsina L, Castejón E, Navarro-Vilarrubí S, Pérez-Dueñas B, Serrano M, Palau F, García-Alix A
Hum Mutat 2018 Dec;39(12):1752-1763. Epub 2018 Sep 17 doi: 10.1002/humu.23638. PMID: 30176098
In vitro testing the potential of a novel chimeric IgG variant for inhibiting collagen fibrils formation in recurrent hereditary gingival fibromatosis: chimeric antibody in a gingival model.
Gawron K, Lazarz-Bartyzel K, Lazarz M, Steplewska K, Pyrc K, Potempa J, Chomyszyn-Gajewska M
J Physiol Pharmacol 2014 Aug;65(4):585-91. PMID: 25179091
Infantile systemic hyalinosis.
Giri PP, Raushan R, Ghosh A, Pal P
Indian Pediatr 2012 Jan;49(1):62-4. PMID: 22318104

Clinical prediction guides

Hub Genes, Possible Pathways and Predicted Drugs in Hereditary Gingival Fibromatosis by Bioinformatics Analysis.
Yang RX, Shi F, Du SN, Luo XY, Wang WQ, Yuan ZL, Chen D
Chin J Dent Res 2024 Mar 28;27(1):101-109. doi: 10.3290/j.cjdr.b5128671. PMID: 38546525
Clinics and genetic background of hereditary gingival fibromatosis.
Strzelec K, Dziedzic A, Łazarz-Bartyzel K, Grabiec AM, Gutmajster E, Kaczmarzyk T, Plakwicz P, Gawron K
Orphanet J Rare Dis 2021 Nov 24;16(1):492. doi: 10.1186/s13023-021-02104-9. PMID: 34819125Free PMC Article
Epilepsy in KCNH1-related syndromes.
Mastrangelo M, Scheffer IE, Bramswig NC, Nair LD, Myers CT, Dentici ML, Korenke GC, Schoch K, Campeau PM, White SM, Shashi V, Kansagra S, Van Essen AJ, Leuzzi V
Epileptic Disord 2016 Jun 1;18(2):123-36. doi: 10.1684/epd.2016.0830. PMID: 27267311
In vitro testing the potential of a novel chimeric IgG variant for inhibiting collagen fibrils formation in recurrent hereditary gingival fibromatosis: chimeric antibody in a gingival model.
Gawron K, Lazarz-Bartyzel K, Lazarz M, Steplewska K, Pyrc K, Potempa J, Chomyszyn-Gajewska M
J Physiol Pharmacol 2014 Aug;65(4):585-91. PMID: 25179091
Testosterone stimulates proliferation and inhibits interleukin-6 production of normal and hereditary gingival fibromatosis fibroblasts.
Coletta RD, Reynolds MA, Martelli-Junior H, Graner E, Almeida OP, Sauk JJ
Oral Microbiol Immunol 2002 Jun;17(3):186-92. doi: 10.1034/j.1399-302x.2002.170309.x. PMID: 12030972

Recent systematic reviews

Syndromes with gingival fibromatosis: A systematic review.
Costa CRR, Braz SV, de Toledo IP, Martelli-Júnior H, Mazzeu JF, Guerra ENS, Coletta RD, Acevedo AC
Oral Dis 2021 May;27(4):881-893. Epub 2020 May 25 doi: 10.1111/odi.13369. PMID: 32335995

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