Clinicopathologic findings of guttate leukoderma in Darier disease: A helpful diagnostic feature

Darier disease (DD), also known as keratosis follicularis or Darier-White disease, is a rare autosomal dominant genodermatosis attributed to a mutation in the ATP2A2 gene. DD affects both males and females worldwide, with high penetrance (95%) and variable expressivity, allowing for varied clinical manifestations and disease severity among affected families and individuals.1 The characteristic feature of DD is the presence of firm, greasy, skin-colored to yellow-brown, hyperkeratotic papules distributed in seborrheic areas, such as the scalp, face, and trunk.