MULTIPLE NEUROFIBROMATOSIS WITH ORAL, PARAPHARYNGEAL SPACE AND WHOLE BODY LESIONS.
- Author:
Seong Seob OH
1
;
Hong Jun HWANG
;
IL Kyu KIM
;
Jin Ho CHO
;
Hyung Don KIM
;
Nam Sik ON
;
Eun Jin PARK
Author Information
1. Department of Dentistry, College of Medicine, Inha University.
- Publication Type:Original Article
- MeSH:
Biopsy;
Cafe-au-Lait Spots;
Diagnosis;
Genetic Counseling;
Humans;
Neurofibroma;
Neurofibromatoses*;
Parturition;
Skin
- From:Journal of the Korean Association of Oral and Maxillofacial Surgeons
1999;25(2):178-181
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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 1.5cm 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.
