Fibromatosis gingival hypertrichosis

Fibromatosis gingival hypertrichosis is a rare condition characterized by the excessive growth of both the gums (gingiva) and body hair (hypertrichosis). This condition is part of a group of diseases known as gingival fibromatosis, which involves the proliferation of gingival tissue leading to a thickening and overgrowth of the gums. The addition of hypertrichosis, or abnormal hair growth on the body, distinguishes fibromatosis gingival hypertrichosis as a unique clinical entity.

Etiology

The exact cause of fibromatosis gingival hypertrichosis is not fully understood, but it is believed to have a genetic component. It may be inherited in an autosomal dominant manner, suggesting that a single copy of the altered gene in each cell is sufficient to cause the disorder. Mutations in specific genes, such as the Sonic hedgehog (SHH) gene, have been implicated in some cases, though not all instances of the condition can be traced to a known genetic mutation.

Clinical Presentation

Individuals with fibromatosis gingival hypertrichosis exhibit pronounced gingival overgrowth, which can begin in childhood or adolescence. This overgrowth can cover the teeth entirely and may lead to difficulties in speaking, chewing, and maintaining oral hygiene. The hypertrichosis component involves excessive hair growth on various parts of the body, which can vary in severity among affected individuals.

Diagnosis

Diagnosis of fibromatosis gingival hypertrichosis is primarily clinical, based on the observation of the characteristic gingival overgrowth and body hair excess. Genetic testing may be helpful in confirming a diagnosis, especially in cases where a specific mutation is suspected. Dental examinations and imaging studies, such as dental X-rays, can assess the extent of gingival involvement and any impact on the teeth and underlying bone.

Treatment

Treatment for fibromatosis gingival hypertrichosis is symptomatic and may involve multiple specialties. Management of gingival overgrowth typically includes regular dental care, professional cleaning, and in some cases, surgical removal of excess tissue. Orthodontic treatment may be necessary to address any dental malocclusions resulting from the gingival overgrowth. Hair removal techniques, such as shaving, waxing, or laser hair removal, can manage hypertrichosis, though results may vary and require ongoing treatment.

Prognosis

The prognosis for individuals with fibromatosis gingival hypertrichosis varies. While the condition itself is not life-threatening, it can significantly impact quality of life and self-esteem. Regular dental care and management of hair growth can help mitigate some of the condition's effects, but ongoing treatment may be necessary to manage symptoms effectively.

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