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 purpose of this study is to assess broad cognitive function in individuals with both diseases using an experimental, case-control setup comparing cognition in patients compared with healthy controls matched for age, sex and level of education. Cognition was assessed with the Cambridge Neuropsychological Test Automated Battery. Results show that Darier disease patients (n=29) performed significantly poorer on five of the ten key cognitive measurements, while Hailey-Hailey disease patients (n=25) did not perform differently than controls. There was also a clear, albeit non-significant, trend for Darier disease patients to perform worse on the remaining five key measurements. The main conclusion is that Darier disease patients exhibits a significant impairment in cognitive function. This enforces the view that Darier disease should be seen as a disorder affecting multiple organs, and therefore acquire medical consideration and possibly treatment as such.

ESDR090 - Darier disease patients exhibit cognitive impairment while Hailey-Hailey disease patients do not: an experimental, matched case-control study

50th Annual Meeting of the European Society for Dermatological Research. VIRTUAL MEETING