Acrokeratosis Verruciformis of Hopf: A Localized Variant

Acrokeratosis Verruciformis of Hopf (AKV) is a rare autosomal dominant genodermatosis. It usually develops during early childhood affecting both sexes equally. Typically, the lesions are warty to convex, brownish to skin-colored papules on the dorsa of the hands and feet, forearms and legs. A possible relationship with Darier disease has long been postulated on a clinical basis mainly. But recently, evidence has suggested that AKV and Darier disease are allelic disorders. I am reporting these two unrelated cases as a unilateral localized variant of AKV. 687 Figure 1. Multiple pink colored convex papules on the dorsa of the right forearm. Figure 2. Hyperkeratosis, acanthosis, hypergranulosis, and church spires appearance of the epidermis. © 2004,