A 60-year-old woman presented with a generalized pruritic rash since adolescence. She reported her son had a similar rash. Physical examination revealed greasy brown and keratotic papules and plaques in a seborrheic distribution, which included her chest, back, forehead, scalp margins, and ears (Figures 1 and 2). Histopathology revealed acantholytic dyskeratosis. The clinicopathological features along with the patient’s family history were consistent with Darier disease. The patient’s skin disease markedly improved with acitretin.