A Case of Unilateral Darier's Disease; Acantholytic Dyskeratotic Epidermal Nevus? / 대한피부과학회지

ABSTRACT

Darier's disease is an uncommon genodermatosis, characterized by the symmetric eruption of keratotic papules, focal loss of epithelial adhesion and abnormal keratinization. A unilateral, or localized variant has been identified as a mosaic form, involving linear or 'zosteriform' epidermal lesions with clinical and histological features of Darier's disease. Some suggest that unilateral lesions should be classified as acantholytic dyskeratotic epidermal nevi, rather than localized Darier disease. A 17-year-old male presented with unilateral zosteriform distribution of brownish hyperkeratotic papules with ipsilateral v-notching of the thumb nail. A biopsy specimen revealed acantholysis, suprabasal cleft and multiple dyskeratotic cells. We report a case of unilateral Darier's disease which further supports unilateral dermatologic manifestation as a variant of Darier's disease.