Darier disease (also known as keratosis follicularis or Darier-White disease) is a rare, autosomal dominant keratinization disorder characterized by greasy, hyperkeratotic papules and plaques involving seborrheic regions, including the face, chest, back, and flexural regions [1, 2]. Almost all patients have nail involvement [2]. The pathognomonic nail finding is alternating red and white longitudinal streaks in a “candy-cane” pattern with distal V-shaped notching [3]. There is oral involvement in 15–50% of cases [3, 4], including white papules of the gingiva (cobblestoning), gingival hypertrophy, and obstructive sialadenitis [5]. Darier disease is due to a mutation in ATP2A2, a gene encoding sarcoplasmic/endoplasmic reticulum ATPase type 2 (SERCA2), an endoplasmic reticulum calcium pump. It is hypothesized that the dysfunctional SERCA2 pump causes accumulation of calcium in the cytosol, leading to reduced calcium in the keratinocyte endoplasmic reticulum [6]. Deficiency of calcium in the keratinocyte endoplasmic reticulum interferes with protein trafficking and causes acantholysis [6].