Pruritic Linear Papules on a 75-Year-Old Woman: A Case of Localized Darier-White Disease

Darier-White disease (DWD), commonly called Darier disease or keratosis follicularis, is a genodermatosis seen in clinical practice. It exists more commonly in the generalized form but can present as a localized condition. Localized DWD is a rare entity characterized by epidermal changes that are like those seen in the generalized form but that are confined to a small area of skin. This entity is postulated to result from a postzygotic mutation and has the potential to be transmitted to offspring, which may result in the severe generalized form. We report a case of localized DWD diagnosed after biopsy of a recalcitrant linear dermatitis and discuss the salient features of this condition.