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

  • Author(s): Sun, Christina W
  • Grossman, Shoshana K
  • Valdes-Rodriguez, Rodrigo
  • Lee, Jason B
  • Hsu, Sylvia
  • et al.
Creative Commons 'BY-NC-ND' version 4.0 license

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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