Hereditary Gingival Fibromatosis - Clinical Features, Etiology, and Management

What Is Hereditary Gingival Fibromatosis?

Hereditary gingival fibromatosis (HGF) is a rare disorder that might manifest as a single illness, a chromosomal defect, or one of several syndromes. Gingival growth in HGF patients can be so extreme that it entirely covers tooth crowns, severely impairing function and deforming the face. Palpably stiff, the hyperplastic gingiva has a normal hue and a lot of stippling on the neighboring gingiva. Individual differences can be seen in the gingival enlargement degrees.

There are two forms of HGF classifications: symmetric type and nodular type. The symmetric type is defined by uniform gingival expansion, whereas the nodular type is characterized by the presence of numerous tumors in dental papillae. The most prevalent kind of gingival expansion is symmetrical, though both types can coexist. The symmetric type might have a generalized or localized shape and is often unilateral. The maxillary molar and tuberosity regions are typically affected by the localized form, especially on the palatal surface. Teeth are nearly entirely covered when there is extensive involvement.

Does Hereditary Gingival Fibromatosis Have a Familial Link?

• In periodontal diseases and conditions classification developed by Armitage in 1999. Hereditary Gingival Fibromatosis is listed in the categories of gingival lesions that have genetic origins. In addition, research indicates that various cases may occur within the same family of individuals who suffer from HGF.

• HGF can also occur due to association with other genetic or systemic syndromes. It usually presents the autosomal dominant inheritance pattern (a way in which a genetic trait or condition is passed from parents to children). However, this condition has also been found among individuals with no family history or familial linkage (20 percent of cases).

Who Develops Hereditary Gingival Fibromatosis?

The incidence of hereditary gingival fibromatosis is thought to be 1 in 750,000. Both sexes are equally impacted. Patients who are toothless (edentulous) have not reported experiencing it.

The most common mode of inheritance for hereditary gingival fibromatosis is autosomal dominant, which means that each offspring of an affected parent has a 50 percent chance of getting the disorder. Nonetheless, there have also been reports of families with apparent maternal inheritance and autosomal recessive inheritance.

What Is the Etiology of Hereditary Gingival Fibromatosis?

The primary etiologic factor of this condition is genetic. Along with that, the various systemic conditions that can be linked with the occurrence of hereditary gingival fibromatosis include -

• Cowden's syndrome (a genetic disorder characterized by various noncancerous tumor-like growths called hamartomas).

• Murray-Puretic syndrome (a rare condition characterized by skin lesions, joint contractures, gingival hypertrophy, and bone lesions).

• Epilepsy (a nervous system disorder causing abnormal brain activity).

• Zimmermann-Laband syndrome (a rare genetic disorder characterized by head, face, hands, and feet abnormalities), and most children with this disorder have abnormally large gums (gingival fibromatosis).

• In individuals with hypertrichosis (excessive hair growth on the body).

However, the healthcare professional should always rule out the systemic conditions before suspecting the origin of gingival enlargement as a hereditary form. A family history of the disease is also an important factor in studying the predisposition risk and probable linkage in individuals suspected of this condition. However, the clinical examination by the dentist without any underlying systemic diseases can help in the confirmative diagnosis and the progress of this condition.

What Are the Clinical Features of Hereditary Gingival Fibromatosis?

Hereditary gingival fibromatosis may appear during the eruption of permanent teeth and is not always linked to primary teeth eruption. However, there have been some cases where it has been found in the primary dentition phase. The gingival enlargement in hereditary gingival fibromatosis may vary in severity, and in some cases, they may be completely covered. The most commonly affected areas are in the tuberosity area or around the buccal gingiva if it is on the mandibular molars.

In severe cases of hereditary gingival fibromatosis, fibrous gingival tissues may be present over both the maxillary and mandibular gingiva. In severe cases, the enlargement can be sufficient to cover the primary or permanent tooth crown completely.

The clinical features of hereditary gingival fibromatosis include -

• A rosy-colored appearance of the gingiva.

• Fibrous consistency of the gingiva.

• The stippling effect is seen on the gingiva without any signs of inflammation that differentiate it from most other gingival diseases or enlargement conditions.

• The affected areas usually surround the teeth partially or completely and can be localized or generalized.

• The patient may experience discomfort in the affected region with a diverse degree of severity but does not involve the underlying alveolar bone.

• Other common symptoms include interference with speech, trouble in lip closure, and difficulty chewing.

Also, this condition would be a psychological burden that affects self-esteem and self-confidence because it may be a hereditary condition that can be present since childhood.

What Is the Differential Diagnosis of Hereditary Gingival Fibromatosis?

The hereditary gingival fibromatosis, on clinical examination, appears as a nodular and hyperplastic form of the gingiva, which may feel firm or even bony hard on examination.

The differential diagnosis conditions for this condition include drug-induced gingival hyperplasias from drugs like Phenytoin, Cyclosporine, and Nifedipine.

What Is the Management of Hereditary Gingival Fibromatosis?

Hereditary gingival fibromatosis does not resolve spontaneously, and the preferred treatment of choice is a surgical procedure called a gingivectomy. This surgery can be done using either the internal or external bevel incision technique.

• The external bevel incision is more conservative and useful in the mixed dentition phase than the internal bevel incision.

• Internal bevel incision should be ideally performed only after the eruption sequence is complete. However, the drawback is that it can only be performed when a sufficient part of the permanent tooth is formed. Implementing this surgical technique post-permanent tooth eruption reduces the chance of clinical recurrence of symptoms and the prognosis.

Parents should also be informed and educated about the potential recurrence rates of HGF lesions and the correct age group for the gingivectomy procedure after the eruption of permanent teeth. They should also be motivated socially and morally to encourage the child or young adult.

Conclusion:

Hereditary Gingival Fibromatosis is a challenging condition that requires time management to counteract negative psychological experiences. Parents should be aware of the symptoms of this condition and should consult a dentist as soon as possible to avoid complications. Surgery is the best approach to treating this condition, reducing its recurrence rate.