Dental treatment in the operating room in a patient with hyaline fibromatosis: clinical case report

Juvenile hyaline fibromatosis (JHF) is a rare hereditary disease with unknown etiology, characterized by the onset of clinical manifestations typically occurring at three to four months of age. It primarily affects the skin, causing pearl-like papules on the face, neck, retroauricular and perinasal regions, as well as large tumors on the scalp, trunk, limbs, and perianal nodules. Gingival hypertrophy is common and can affect feeding. Bone manifestations include osteolytic lesions, cortical thinning, and generalized osteopenia. The FHJ gene has been mapped to chromosome 4q21 and is associated with a deficiency in capillary morphogenesis protein 2 (CMG2). Treatment involves surgical excision of lesions, but recurrences are frequent. Gingivectomy and supportive measures like physiotherapy, adequate nutrition, and analgesia are also employed to improve quality of life. The prognosis varies and the average life expectancy is 20-30 years. This case report aims to describe a clinical case of FHJ and evaluate the patient's quality of life and functionality after dental treatment in a surgical center.