Abstract
We report two sisters with basal cell nevus syndrome (BCNS).
Case 1 : 26-year-old woman (elder sister). Case 2 : 24-year-old woman (younger sister). They had family histories with their mother and grandfather. Both sisters had multiple jaw cysts, palmar pits, calcification of falx cerebri and syndactyly (bilateralII-IIItoes). Elder sister had multiple basal cell carcinomas on her upper back and head, but younger sister had only melanocytic naevi. According to the diagnostic criteria of Evans, both their diseases were diagnosed as BCNS.
We compared the Japanese literatures with the foreign literatures about major manifestations and frequencies of their findings in BCNS.
