[A mandibular swelling].
Nevoid basal cell carcinoma syndrome (NBCCS) is characterised by skeletal anomalies, cutaneous basal cell carcinomas and multiple keratocysts. NBCCS is an autosomal dominant disorder, but can have a variable phenotypic penetration. NBCCS can also arise spontaneously. The prevalence is 1:60.000 and 50-65% of patients with NBCCS have affected family members. A recently diagnosed patient is presented and the manifestations of the syndrome are discussed.
de Lange
J
J
afdeling Mondziekten en Kaakchirurgie, Isala Klinieken in Zwolle. lotte.jan@wxs.nl
Norbruis
O F
OF
D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery*; D002648:Child; D006801:Humans; D008297:Male; D008336:Mandibular Diseases / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D009807:Odontogenic Cysts / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery