University of Kirkuk

College of Dentistry

Periodontology

Hereditary Gingival fibromatosis

Name:-‫محمد عبدالهادي إسماعيل‬

Stage:-4th
Group:- E
Date:- June,1,2020
 Hereditary gingival fibromatosis(HGF)

Introduction:-
Hereditary gingival fibromatosis (HGF). It’s one of gingival Gingival
diseases of genetic origin and are uncommon conditions. It may occur
singly or in association with other inherited syndromes. This condition is
also known as, elephantiasis gingivae, diffuse fibroma, familial
elephantiasis, idiopathic fibromatosis; hereditary gingival
fibromatosis, congenital familial fibromatosis .It is inherited as
autosomal dominant trait. The condition does not manifest itself until after
eruption of permanent teeth and is most commonly seen associated with
the permanent teeth.

HGF is characterized as a benign, slowly progressive, non

hemorrhagic, fibrous enlargement of keratinized gingiva. It can cover
teeth in various degrees, and can lead to aesthetic disfigurement. Fibrous
enlargement is most common in areas of maxillary and mandibular tissues
of both arches in the mouth. Phenotype and genotype frequency of HGF
is 1:175,000 where males and females are equally affected but the cause is
not entirely known. It mainly exists as an isolated abnormality but can also
be associated with a multi-system syndrome.

Hereditary Gingival Fibromatosis may be familial or idiopathic .The

familial variation may occur with a number of other inherited syndromes
when it could be associated with some of the following; for example
Zimmerman Laband syndrome, Murray Puretic drescher (juvenile
hyaline fibromatosis), Rutherfurd, Cross, Cowden syndrome, multiple
hamartomas, tuberous sclerosis.
Clinical Features:-
HGF present either localized or generalized overgrowth of the attached
keratinized gum of variable severity . if mild will affect interdental papilla
but usually it affect marginal gingiva , in localized form the over growth will
be in the lip/check side of gum of the lower molar teeth and the
maxillary tuberosity. The generalized form will affect all 4 quadrant of
the mouth and may extend to the palate.

Gingival overgrowth usually begin at 10year age, Rarely it start with

primary deciduous teeth

The gum swelling is normal pink color but it’s firm to touch with the little
tendency to bleed , it affect both upper and lower jaw and both labial and
palatal side of the gum
Symptoms:-

 The patient complain from discomfort during eating

 Difficulty when speaking
 Swelling of the gum
Signs:-

 Most obvious sign is gingival overgrowth (overgrowth of the

gums)
 Increasing mobility of teeth
 Abnormally shaped teeth and abnormal movement of teeth
 Inflammation of the gingiva
 Not necessarily any signs of pain but experiencing pain is
possible
 In some cases, Hereditary Gingival Fibromatosis may cause
bleeding from the gums, or gum ulcerations.
Causes:-
Genetic Causes:-
mutation in SOS1, son-of-sevenless gene, is responsible for this
disease. SOS1 is a guanine nucleotide-exchange factor that functions in
the transduction of signals that control cell growth and differentiation. A
mutation in the SOS1 gene results in a
single nucleotide insertion. Specific linkage studies have localized
the mutation for isolated, nonsyndromic autosomal dominant forms of
gingival fibromatosis to chromosomes 2 and 5, more specifically 2p21-
p22 and 5q13-q22

 HGF1 - Caused by a mutation in the SOS1 gene localized on

chromosome 2p21-p22
 HGF2 - Caused by a mutation in the SOS1 gene localized on
chromosome 5q13-q22

Non Genetic Causes:-

 Inflammation
 Hormonal Imbalance
 Neoplasia
 More commonly associated with an autosomal dominant
gene inheritance
 Multi-system syndromes: Zimmerman-Laband
syndrome, Jones syndrome, Ramon syndrome, Rutherford
syndrome, juvenile hyaline fibromatosis, systemic infantile
hyalinosis, and mannosidosis
 Some unknown causes
Histopathological Description:-
The typical histopathology of the lesion involves hyperplasia of the
epithelium with elongated rete ridges extending into the underlying
connective tissue The connective tissue consists of excess collagen, but
has relatively few fibroblasts and blood vessels. Enlarged fibroblasts
appear to alternate with thin and thick collagen fibrils. Elastic and
oxytalan fibers are also present in HGF lesions. Unlike in normal gingiva,
coarse and fine dense collagen fiber bundles are oriented in all
directions. Small osseous calcifications and abundant neurovascular
bundles may also be present. The excess of gingival tissue may provide
new niches for the growth of microorganisms, plaque accumulation and
pseudopockets formation resulting in inflammatory infiltration of the
gingival connective tissue.
Diagnosis:-
There are very few ways to test a patient for HGF. Currently, the most
common way to diagnose a patient is by means of a physical evaluation.
The physician can make a physical evaluation of the patient and send them
to a dentist or better yet a specialist like a periodontist to evaluate signs of
gingival overgrowth, quality of gingiva, inflammation, mechanical
difficulties of the mouth, tooth conditions, and any sort of discomfort.
Aside from obvious physical symptoms seen in a physical evaluation,
molecular tests can be run to check if there is a mutation in
the SOS1 gene to confirm the diagnosis. If there is indeed a mutation in
this gene coupled with the typical physical symptoms, then it is quite
probable that a patient suffers from this disease. Also, looking at family
history is also becoming more prominent in aiding to diagnose the patient.
Otherwise, researchers are working to find new and better ways to test for
the presence of HGF

Prevention:-
Since this condition is generally agreed upon to be hereditary, nothing
can be done to prevent HGF. However, in some cases where it can
develop as a result of rare multi-system syndromes, such as: Zimmerman-
Laband, Jones, Ramon Syndrome, Rutherford Syndrome, Juvenile Hyaline
Fibromatosis, Systemic Infantile Hyalinosis, and Mannosidosis, it is best
for one to simply monitors the possible progression for HGF with
regular dental check-ups.
If the patient's disease is treated by means of surgery, it is recommended
that the patient undergoes post-surgical therapies for maintenance and
periodic monitoring of gums for the sake of the possibility of re-
occurrence of HGF
Treatment:-
Good oral hygiene is important and may be the only treatment required
when gum enlargement is minimal.

The excessive gum tissue can be removed by:

 Surgical gingivectomy (cutting away the excessive gum tissue)

 Carbon dioxide laser (vaporising it).

If Untreated:-

It will cause the followings

 Too much gingiva exposure
 Oral morbidity
 Chronic infection of areas between the gums and teeth, or at the gum
line
 Systemic every-day troubles including functional and aesthetic problems
of the mouth
 Malocclusion

Prognosis:-
. Hereditary Gingival Fibromatosis as a form of periodontal tissue reaction
may impose a challenge to periodontists as well.. Recurrence of this
conditions can occur several months to several years after surgery

Conclusions:-
HGF is a rare disorder characterised by varying degrees of attached
gingival overgrowth. Aesthetic and functional compromises often demand
surgical intervention; although recurrence cannot be predicted. Good
aesthetic result was achieved without the recurrence of gingival
overgrowth. After treatment regular recalls are necessary in order to
evaluate oral hygiene and stability of periodontal treatment.
References:-

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