한 家族에서 發生한 Nevoid Basal-Cell Carcinoma Syndrome

The nevoid basal-cell carcinoma syndrome in a Korean family

Three cases of nevoid basal cell carcinoma syndrome in a Korean family site presented. In our cases, nevoid basal cell carcinomas as a component of syndrome are not observed, whereas other aspects, such as palmar and plantar pitting, multiple jaw cysts, cleft lip and palate, bifid rib, bridging of sella, frontal and temporoparietal bossing, hypertelorism, and dystopia canthrom are observed. These findings support other author s finding of a high degree of penetrance but a variable expressivity. We will follow up them for high potentiality of occurrence of basal cell carcinoma with aging and high recurrence rate of jaw cyst.