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Abstract

Darier’s Disease

Synonyms

Synonyms for Darier’s disease are keratosis follicularis, Darier’s disease, and dyskeratosis follicularis.

Definition

Darier’s disease is an autosomal−dominant abnormality of keratinization that shows keratotic papules originating from both follicles and interfollicular epidermis. Later, papules appear crusted and verrucous.

Surface Microscopy

After removing dirty brownish keratotic plaques (acantholysis), areas of regularly arranged punctiform papillary capillaries (“naked papillae”) emerge. The papillary dermis has not changed.

Histopathology

Focal suprabasal acantholytic dyskeratosis and lacunae in the slightly acanthotic epidermis as well as hyperkeratosis and patchy parakeratosis are typical signs. Eosinophilic dyskeratotic cells with premature cornification (corps ronds and grains) issue in the epidermis.