Longitudinal, subungual, red or white streaks, or both, associated with distal wedge-shaped subungual keratoses are the nail signs diagnostic for Darier-White disease.
Histopathologic changes typical of Darier-White disease, with suprabasilar clefts containing acantholytic cells, are present in the dorsal portion of the proximal nail fold, the nail matrix, and the epidermis of the volar skin. In addition, multinucleate epithelial giant cells can be seen in the cornified cells of the nail bed and the nail plate. The cornified layer is thickened from the proximal nail fold to the volar skin.
Keratotic papules on the dorsal portion of the nail fold clinically may resemble acrokeratosis verruciformis but histologically have features of Darier-White disease.
Rarely, Darier-White disease can be limited to the nails.
Zaias N, Ackerman AB. The Nail in Darier-White Disease. Arch Dermatol. 1973;107(2):193–199. doi:10.1001/archderm.1973.01620170005001
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: