In an article on vitamin A therapy in cases of Darier's disease Carleton and Steven1 stated that it occurred to Peck that "since the chief pathologic change is a follicular dyskeratosis, the disease might possibly be due to vitamin A deficiency." They listed follicular hyperkeratosis as one of the diseases associated with vitamin A deficiency which is amenable to vitamin A therapy. Weidman2 has expressed the view that "Darier's disease . . . is fundamentally and predominantly keratotic, as witness the original name, dyskeratosis follicularis." Weidman3 wanted to stress the keratotic feature which may be follicular, but he could recall only 1 case in which the sheath of the follicle participated in the dyskeratosis.
These statements induced me to review the literature and to study the biopsy material from 9 cases of typical keratosis follicularis in an attempt to determine whether the primary lesion of Darier's disease is a