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).
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