着色性干皮病痴呆综合征又称De Sanctis—Cacchione氏综合征,呈常染色体隐性遗传。国内报告的着色性干皮病病例中,仅见2例合并神经精神症状,但非家族性。现将我院所见一个家系姐妹2例报告如下。例1,女17岁。于出生后40天发病,日 Coloring dry-skin disease Dementia syndrome, also known as De Sanctis-Cacchione’s syndrome, was autosomal recessive. In the domestic report of the case of tinctorial dry skin disease, only 2 cases with neuropsychiatric symptoms, but not familial. Now I see a family hospital sisters 2 cases are reported as follows. Example 1, female 17 years old. 40 days after birth morbidity, day