Abstract

Darier disease (Darier-White disease, keratosis follicularis) is a rare autosomal dominantly inherited keratinization disorder caused by mutations in the ATP2A2 gene. The disease is characterized by yellow to brown, scaly keratotic papules and plaques with a predilection for the seborrheic regions of the anterior chest, mid back, hair margins, and the flexures1 . Other associated clinical manifestations, including palmoplantar papules, mucosa and nail changes, have also been founded. Localized Darier disease is rare in the literature2 . Here we report a Darier disease with localized lesions, which presented with oily keratotic papules restricted to the buttocks.

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