An acantholytic dyskeratotic epidermal naevus with other features of Darier's disease on the same side of the body.

Many epidermal naevi with the histology of Darier's disease have been reported. In the absence of associated features of Darier's disease, they cannot be assumed to have a common pathogenesis with it, and it has been suggested that they are better classified as acantholytic dyskeratotic epidermal naevi rather than naevoid Darier's disease. We describe a patient with such a naevus who had typical nail and palmar changes of Darier's disease on the same side of the body. We suggest that in at least some cases the naevus has the same genetic defect(s) as generalized Darier's disease, and discuss the possibility that a patient with such a naevus could occasionally transmit Darier's disease to an offspring.