Darier-White syndrome

(redirected from Darier-White Disease)
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Darier-White syndrome

A rare autosomal dominant disorder of keratinisation characterised by multiple focal keratotic papules with acantholytic dyskeratosis. It first appears in childhood or adolescence with the formation of numerous flesh-colored papules in sebaceous areas of the skin glands—chest, scalp, forehead, back, ears, groin, nasolabial folds—which, with time, become encrusted. The nails are dystrophic, palms possibly pitted; variable retinitis pigmentosa and cataracts.

Molecular pathology
Defect in the desmosome-keratin filament complex due to ATP2A2 mutations.
References in periodicals archive ?
The nail in Darier-White disease. Arch Dermatol 1973; 107: 193-9.
Darier disease (DD) or Darier-White disease, also known as keratosis follicularis, is an autosomal dominantly inherited genodermatosis characterized by greasy hyperkeratotic papules in seborrheic regions, nail abnormalities, and mucous membrane changes.1,2 The disease was first reported independently by Darier and White in 1889.
Darier's disease, also called Darier-White disease or keratosis follicularis, is a genodermatosis that affects 1 in 55,000 to 100,000 persons.