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" Guttate leukoderma and acrokeratosis verruciformis of Hopf: a rare combination in Darier disease "


Document Type : AL
Record Number : 923114
Doc. No : LA5938q4rj
Language of Document : English
Main Entry : Sun, Christina W; Grossman, Shoshana K; Valdes-Rodriguez, Rodrigo; Lee, Jason B; Hsu, Sylvia
Title & Author : Guttate leukoderma and acrokeratosis verruciformis of Hopf: a rare combination in Darier disease [Article]\ Sun, Christina W; Grossman, Shoshana K; Valdes-Rodriguez, Rodrigo; Lee, Jason B; Hsu, Sylvia
Title of Periodical : Dermatology Online Journal
Volume/ Issue Number : 26/1
Date : 2020
Abstract : A distinct Darier phenotype presenting with confetti-like hypopigmented macules was first described in 1965. Designated as ''guttate leukoderma,'' this skin finding is a rarely-reported presentation of Darier disease. It has been theorized that the mutation in ATP2A2 causes defective E-cadherin, which in turn disrupts the adhesion of melanocytes to keratinocytes, thus leading to impaired dendrite formation, hindered melanin transfer, and ultimately to melanocyte apoptosis. Herein, we contribute a case of a 56-year old woman who presented with the rarely-described guttate leukoderma of Darier disease and acrokeratosis verruciformis of Hopf.
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