구강과 측인두강 및 전신에 발생한 신경섬유종증

MULTIPLE NEUROFIBROMATOSIS WITH ORAL, PARAPHARYNGEAL SPACE AND WHOLE BODY LESIONS

Neurofibromatosis(Von Recklinghausen s disease) is an autosomal dominant disease that affects one in 2,500-3,000 births. The clinical presentation is characterized by multiple pigmented lesions of the skin, known as cafe-au-lait spots, and neurofibromas of the skin. The criteria for diagnosis are at least six cafe-au-lait spots, each greater than 1.5㎝ in diameter and positive family history and biopsy of a neurofibroma. Management of benign neurofibromatosis must be individualized for each patient and ranges from incisional biopsy to wide local excision. Genetic counseling is advised for all patients with this disease, since no cure is available. We report the case of multiple neurofibromatosis with oral, parapharyngeal space and whole body lesions that was treated with surgical excision.