Darier disease: candy-cane nails and hyperkeratotic papules

Case

A 43-year-old woman presented to our clinic with nail changes and a pruritic malodourous rash on the neck and trunk that began 27 years earlier. Her mother had similar cutaneous and nail findings. Physical examination of her fingernails revealed alternating red and white longitudinal bands, with V-shaped notches at the distal nail edges (figure 1). Clinical evaluation of the skin showed numerous hyperkeratotic papules and plaques on her neck, chest, submammary region and abdomen (figure 2). After treatment with compounded triamcinolone, Silvadene ointment and oral glycopyrrolate, her rash and symptoms were ameliorated. Genetic testing of the patient and her two children (ages 5 and 8) revealed mutations in the ATP2A2 gene, but to date, her children do not have any cutaneous findings.

Discussion

Darier disease, also known as follicular dyskeratosis and Darier–White disease, is an autosomal dominant inherited condition which was first described independently by Drs Darier...

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