AN ELECTRON-MICROSCOPE STUDY OF DYSKERATOSIS AND ACANTHOLYSIS IN DARIER'S DISEASE.

Abstract

(parier's disease is an uncommon hereditary disease which primarily involves the epidermis. Grossly, it is characterized by the formation of keratotic papules which are frequently located in the so-called seborrheic areas. The histological picture of this disorder has several typical features: in the dermis there is a mild, non-specific, perivascular infiltrate; dermal villi protrude into the epidermis; supra-basal detachment of the spinal layer leads to the formation of lacunae which contain acantholytic cells; higher up in the epidermis there are dyskeratotic, round, epidermal cells (corps ronds) which constitute the most distinctive histological feature of Darier's disease; and “grains, which resemble parakeratotic cells, are embedded in a hyper-keratotic, horny layer (1).> The hereditary background of Darier's disease seems to be widely accepted (2). Because of the close resemblance between the morphologic criteria of Hailey-Hailey's disease and of Darier's disease, some authors feel that these two disorders are related, the former being a bullous variant of the latter (3, 4). Genetic studies by other investigators indicate, however, that Hailey-Hailey's disease and Darier's disease occur independently in the involved families and are not known to be associated genealogically or to overlap (2, 5).