J Korean Cleft Palate-Craniofac Assoc.  2002 Apr;3(1):89-92.

A Case of Basal Cell Nevus Syndrome with Symmetric Basal Cell Carcinomas on Neck

• Kim SH
• Kim HK
• Kim SH
• Kim SH
• Lee TJ

Abstract

Basal cell nevus syndrome is an autosomal dominant disorder characterized by developmental and skeletal anomalies, palmo-plantar pits, odontogenic keratocysts, ectopic calcification, and occurrence of various types of tumors including basal cell carcinoma. Within our country, 9 cases at dentistry and 3 cases at dermatology were reported. The patient was a 66-year-old woman. Chief complaint was multiple dark brownish colored papules which were symmetrically distributed around the neck. Excision and biopsy were done under general anesthesia and the wound was healed without complication. Histological examination of the lesion revealed 'basal cell carcinoma'. During the evaluation of metastasis of basal cell carcinoma, at PNS CT we found odontogenic keratocyst on the left alveolar process of maxilla by accident. Physical examination revealed pits of the palm and sole. These are consistent with the major diagnostic criteria for basal cell nevus syndrome. Hereby we report one case of basal cell nevus syndrome.

Keyword

Basal cell nevus syndrome; Basal cell carcinoma; Odontogenic keratocyst; Pits of palms and soles

MeSH Terms

Aged
Alveolar Process
Anesthesia, General
Basal Cell Nevus Syndrome*
Biopsy
Carcinoma, Basal Cell*
Dentistry
Dermatology
Female
Humans
Maxilla
Neck*
Neoplasm Metastasis
Odontogenic Cysts
Physical Examination
Wounds and Injuries