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  • Title: [A mandibular swelling].
    Author: de Lange J, Norbruis OF.
    Journal: Ned Tijdschr Tandheelkd; 2005 Aug; 112(8):298-301. PubMed ID: 16128217.
    Abstract:
    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.
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