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Journal of Korean Cleft Palate-Craniofacial Association 2002;3(1):89-92.
A Case of Basal Cell Nevus Syndrome with Symmetric Basal Cell Carcinomas on Neck.
Sae Hwan Kim, Han Koo Kim, Seung Han Kim, Seung Hong Kim, Tae Jin Lee
1Department of Plastic and Reconstructive Surgery, College of Medicine, Chung-Ang University, Seoul, Korea. hkkiim@orgio.net
2Department of Pathology, College of Medicine, Chung-Ang University, Seoul, Korea.
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.
Keywords: Basal cell nevus syndrome; Basal cell carcinoma; Odontogenic keratocyst; Pits of palms and soles
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