Darier disease (keratosis follicularis) is a rare genetic disorder, which is greatly influenced by the environmental factors. Apart from the skin involvement the disease could affect other organs and it can be also accompanied by a range of certain complications and comorbidities.
The author presents a comprehensive review of this disease. The text summarizes history, epidemiology, clinical presentation, segmental and atypical forms, dermoscopy, etiopathogenesis, differential diagnostics and current therapeutic options of Darier disease.
To the best of the author's knowledge, the topic of Darier disease has not been reviewed in this extent to date. Causal treatment of this genodermatosis is not known, nevertheless the deteriorated quality of patient's life could be highly improved by lifestyle changes and by setting of the accessible treatment.