Abstract
Contrary to the unitarian concept of acrokeratosis verruciformis and Darier''s disease, a comparative familial, clinical and histopathological analysis of 6 cases each of these 2 diseases has suggested that they are separate entities. Though clinically similar, acrokeratosis remained non-dyskeratotic throughout life, whereas the acral lesions of Darier''s disease showed, on careful scrutiny and follow-up, various gradations of benign acantholytic dyskeratosis. Malignant transformation indicated that dyskeratosis in Hopf''s disease would be very rare and of a different nature. The probability of a genetic linkage between the 2 genodermatoses is reviewed and discussed.