Darier disease is an autosomal dominant genodermatosis with incomplete penetrance and variable expressivity. Estimated incidence is 4 per million per 10 years. Men and women are equally affected. Characteristic mucocutaneous findings such as hyperkeratotic papules preferentially in seborrheic distribution areas and longitudinal erythronychia with V shaped notches at distal edge of nail and whitish oral mucosal papules in some people. Histological features includes acantholysis and dyskeratosis (corp ronds and grains)