KMID : 0363220080460050703
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Korean Journal of Dermatology 2008 Volume.46 No. 5 p.703 ~ p.706
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A Case of Unilateral Darier`s Disease; Acantholytic Dyskeratotic Epidermal Nevus?
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Song Margaret
Seo Sang-Hee Ko Hyun-Chang Kim Moon-Bum Oh Chang-Keun Kwon Kyung-Sool
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Abstract
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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.
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KEYWORD
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Acantholytic dyskeratotic epidermal nevus, Darier`s disease, Nail change
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