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Darier Disease: A Case Report, Jalil A. A., Rosnah Binti Zain, Van Der Waal, I.

Prof. Dr. Rosnah Binti Zain

Darier disease, also known as keratosis follicularis, dyskeratosis follicularis, and benign dyskeratosis, is a rare disorder of keratinisation that primarily affects the skin and, to a lesser extent, the oral mucosa. It was described independently by both Darier and White in 1889. It has a prevalence of 1:100,000 of the population and is inherited as an autosomal dominant trait.1 The age of onset is childhood or adolescence. Patients usually present with multiple small firm reddish-brown papules on the forehead, scalp, neck, shoulders, chest, and limbs. Other cutaneous signs include punctate keratotic pits of the palms and soles and dystrophy of …