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

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 Ecadherin, 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.