Gingival fibromatosis-hypertrichosis syndrome

Gingival fibromatosis-hypertrichosis syndrome (also known as gingival hypertrophy with hypertrichosis, or fibromatosis gingivae with hypertrichosis) is a rare genetic disorder characterized by the combination of progressive gingival overgrowth (fibromatosis) and excessive hair growth (hypertrichosis). The gingival enlargement typically becomes apparent during childhood, often coinciding with the eruption of the primary or permanent teeth. The overgrown gum tissue can be firm and fibrous, potentially covering the teeth partially or completely, which may interfere with dental alignment, chewing, speech, and oral hygiene. Hypertrichosis in this syndrome is generalized, meaning excessive hair growth occurs over much of the body, and is usually present from birth or early infancy. The condition primarily affects the oral cavity and integumentary system (skin and hair). Some cases have been associated with additional features such as intellectual disability, epilepsy, or facial dysmorphism, though the core phenotype centers on gingival fibromatosis and hypertrichosis. The syndrome has been linked to mutations in various genes depending on the family studied, and genetic heterogeneity has been reported. Treatment is primarily symptomatic and supportive. Surgical reduction of the gingival tissue (gingivectomy) is the mainstay of management for the oral overgrowth, though recurrence after surgery is common and repeated procedures may be necessary. Hair removal methods such as laser therapy, electrolysis, or depilatory agents may be used to manage hypertrichosis. Regular dental follow-up is essential to monitor for recurrence of gingival overgrowth and to maintain oral health. Genetic counseling is recommended for affected families.

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