Nevoid Basal Cell Carcinoma Syndrome: Report of a case
Korean Journal of Pathology
; : 263-267, 1995.
Article
in Ko
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| ID: wpr-12414
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ABSTRACT
Nevoid basal cell carcinoma syndrome (NBCC syndrome) is a very rare autosomal dominant dermatopathy characterized by a primary triad with variable secondary anomalies. The chief features include nevoid basal cell carcinomas, one or more skeletal anomalies, and multiple odontogenic keratocysts of the jaw. We report a case of NBCC syndrome in a 43-year old male who had multiple nevoid basal cell carcinomas on the retroauricular area, face, chest wall, and back, which have been present since childhood. Skull x-rays revealed relatively well-circumscribed cystic, radiolucent lesions on bilateral rami of both upper and lower jaws, calcification of the falx cerebri, agenesis of the right coronoid process, and a bifid chin. The cystic lesions were histologically confirmed as keratocysts.
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Limits:
Child
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Female
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Humans
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Male
Language:
Ko
Journal:
Korean Journal of Pathology
Year:
1995
Type:
Article