Abstract
Darier disease is a rare genetic disorder, which is greatly influenced by the environmental factors. Apart from the skin involvement the disease could be accompanied by a range of certain complications and comorbidities.
The authors present a comprehensive review of this disease. First part summarizes history, epidemiology, course, prognosis, clinical presentation including its extracutaneous involvement, segmental Darier disease, atypical variants, dermoscopy, histopathology, complications and comorbidities.
Second part thoroughly covers etiopathogenesis, differential diagnostics and current therapeutic options. The review also deals with an al-lelic disease called acrokeratosis verruciformis Hopf.
Causal treatment of this genodermatosis is not known to date, nevertheless the deteriorated qualilty of patient's life could be highly improved by lifestyle changes and by setting of the accessible treatment.