Unilateral segmental Darier disease following Blaschko lines: a case report and review of the literature.

Abstract

Darier disease (DD) is an autosomal-dominant skin disorder that is characterized by multiple keratotic papules, loss of epithelial adhesion, and abnormal keratinization. We describe an unusual case of late-onset unilateral segmental DD that follows the lines of Blaschko. Our patient did not exhibit other classic findings of DD. Our case and review of the literature suggest that lesions previously classified as acantholytic dyskeratotic epidermal nevi (ADEN) are actually unilateral segmental presentations of DD.