We reported a case of basal cell nevus syndrome (BCNS) . A 12-year old boy visited us for the examination of numerous skin pits on the palms. He showed hypertelorism, mandibular cyst, calcification of the falx, and mental retardation. Clinical diagnosis of BCNS was made by these findings. Examination of other family members did not reveal palmar pits or any other signs of BCNS. Skin biopsy from the palmar pits showed reduced thickness of the stratum corneum and the presence of basal cell carcinoma in the dermis.
Chromosome-analysis of peripheral leukocytes revealed the deletion of 9q21.3-22.1. His parents did not show any abnormalities in chromosome-analysis. Very few cases of BCNS have been reported that have basal cell carcinoma on the palms. [Skin Cancer (Japan) 2000; 15: 27-30]
