Darier's disease is an uncommon inherited cutaneous disease which is diffic
ult to manage, especially in adolescence. The warty keratotic papules irrit
ate, smell and look unsightly. Histologically, the condition is characteriz
ed by the presence of focal acantholytic dyskeratosis, Treatment is unsatis
factory, particularly in patients with erosive flexural disease, but retino
ids reduce the hyperkeratosis. Now the mutation causing Darier's disease ha
s been identified, there is a real possibility of more effective treatment
in the future.