A Case Report Of Basal Cell Nevus Syndrome.
- Author:
Dong Mok RYU
1
;
Byung Jun CHOI
;
Sang Chull LEE
;
Yeo Gab KIM
;
Baek Soo LEE
Author Information
1. Department. of Oral & Maxillofacial Surgery, Dental Collage, Kyung-Hee University, Korea.
- Publication Type:Case Report
- Keywords:
Basal Cell Nevus Syndrome;
Odontogenic Cysts
- MeSH:
Amputation;
Basal Cell Nevus Syndrome*;
Biopsy;
Blindness;
Carcinoma, Basal Cell;
Central Nervous System;
Eye Abnormalities;
Humans;
Intellectual Disability;
Jaw Cysts;
Odontogenic Cysts;
Ribs;
Skin;
Spine;
Toes
- From:Journal of the Korean Association of Oral and Maxillofacial Surgeons
2002;28(2):136-140
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Basal cell nevus syndrome is inherited as an autosomal dominant trait with variable expressivity. This syndrome comprises a number of abnormalities such as multiple nevoid basal cell carcinomas of the skin, skeletal abnormalities as bifid rib and fusion of vertebrae, central nervous system abnormalities as mental retardation, eye abnormalities, and multiple odontogenic kerato cysts. In 1960, Gorlin and Goltz first described the features of this disease as constituting a true syndrome; since then, it has been realized that it is much more complex and encompassing than initially thought. This patient has many symtoms of basal cell nevus syndrome. we has known multiple jaw cysts through panorama and facial computed tomography. He has hyperchromatism on basal cell through skin biopsy. In ophthalmologic consult, he has blindness on right. On his past medical history, amputation was done on his toes for polyductalism. - So we report with literature reviews
