Darier disease, also known as keratosis follicularis or Darier-White disease, is an autosomal dominantly inherited disease caused by mutations in the ATP2A2 gene. Darier disease presents in early adolescence to mid-adult life with greasy hyperkeratotic papules in a seborrheic distribution, along with palmoplantar pits, acrokeratosis verruciformis-like papules, and characteristic nail findings ("candy-cane" nails with V-shaped notch at the free edge). Patients are at an increased risk of bacterial or viral skin infections.

Mucosal involvement, primarily of the oral mucosa, is well known. Anogenital involvement, with papular intertriginous and anogenital lesions, is an uncommon phenomenon. Anogenital lesions could present as an isolated finding or along with the classic findings of Darier disease. A rare variant presentation is that of vegetating plaques in the groin folds in particular.