A Case of Unilateral Darier's Disease; Acantholytic Dyskeratotic Epidermal Nevus?.
- Author:
Margaret SONG
1
;
Sang Hee SEO
;
Hyun Chang KO
;
Moon Bum KIM
;
Chang Keun OH
;
Kyung Sool KWON
Author Information
1. Department of Dermatology, School of Medicine, Pusan National University, Busan, Korea. drkmp@hanmail.net
- Publication Type:Case Report
- Keywords:
Acantholytic dyskeratotic epidermal nevus;
Darier's disease;
Nail change
- MeSH:
Acantholysis;
Adolescent;
Biopsy;
Darier Disease;
Humans;
Keratins;
Male;
Nails;
Thumb
- From:Korean Journal of Dermatology
2008;46(5):703-706
- CountryRepublic of Korea
- Language:Korean
-
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.