Zosteriform Darier's disease versus acantholytic dyskeratotic epidermal nevus

Patients with keratotic lesions distributed in a unilateral, linear, zosteriform or localized pattern and revealing histologic features of dyskeratotic acantholysis have been reported. There is still some controversy regarding the appropriate nosologic placement of this entity. Some believe it represents a localized form of Darier s disease, while others argue it is a variant of epidermal nevus. We report a case of a 42-year-old physician who presented with a 15-year history of an asymptomatic eruption that had been diagnosed as "chronic zoster." Physical exam revealed hyperkeratotic papules and plaques in a dermatomal distribution. The controversy regarding the correct nosologic placement of such a patient is discussed.