Abstract：
Darier disease and Hailey-Hailey disease are severe, monogenetic dermatological disorders with mutations affecting all cells, making them liable to exhibit extra-dermal symptoms. The aim of this study is to assess broad cognitive function in individuals with these diseases, using an experimental, case-control set-up comparing cognition in patients with that in healthy controls matched for age, sex and level of education. Cognition was assessed with the Cambridge Neuropsycho-logical Test Automated Battery. Patients with Darier disease (n = 29) performed significantly poorer on 5 of the 10 key cognitive measurements, while patients with Hailey-Hailey disease (n = 25) did not perform differently from controls. The main conclusion is that patients with Darier disease exhibit significant impairment in cognitive function, which reinforces the view that Darier disease should be regarded as a disorder affecting multiple organs, and should therefore be given medical consideration, and possibly treat-ment, as such.
摘要：
达里尔病和海利-海利病很严重，具有影响所有细胞的突变的单基因皮肤病,使他们容易表现出皮肤外症状。这项研究的目的是评估患有这些疾病的个体的广泛认知功能，使用一个实验性的，病例对照设置，将患者的认知能力与年龄相匹配的健康对照进行比较，性别和教育水平。使用剑桥神经心理测试自动电池评估认知。Darier病患者（n=29）在10项关键认知测量中的5项表现明显较差，而Hailey-Hailey病患者（n=25）的表现与对照组没有差异。主要结论是Darier病患者表现出明显的认知功能损害,这强化了这样的观点，即戴里尔病应被视为一种影响多个器官的疾病，因此，应该给予医学考虑，可能还有治疗，因此。
