J Korean Assoc Oral Maxillofac Surg.  2002 Apr;28(2):136-140.

A Case Report Of Basal Cell Nevus Syndrome

Affiliations
  • 1Department. of Oral & Maxillofacial Surgery, Dental Collage, Kyung-Hee University, Korea.

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

Keyword

Basal Cell Nevus Syndrome; Odontogenic Cysts

MeSH Terms

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
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