Nevoid basal cell carcinoma syndrome is inherited as an autosomal dominant trait with a high degree of penetrance and variable expressivity. The main features are basal cell carcinoma. Jaw cysts, skeletal anomalies, ectopic calcifications and
palmoplantar pits. We report varous clinical manifestations in brother and sister, which are jaw cyst, bifid rib, shortened fourth metacarpals, ectopic calcification, palmoplantar pits and basal cell carcinoma, Microscopically, histologic
patterns
of
basal cell carcinomas are superficial, nodulocystic, keratotic, pigmented, adenoid and follicullar nevoid. Lange numbers of basal cell carcinomas were electrodessicated and curetted under general anesthesia.
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