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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2002 October;137(5):307-14

language: Italian

Darier’s disease. Report of 14 cases and review of the literature

Aste N., Pau M., Sollai G., Biggio P.

Clinica Dermatologica, Università degli Studi di Cagliari, Cagliari


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Darier’s disease is a disorder of keratinisation which usually shows autosomal dominant inherance, although frequent sporadic cases also occur. Onset is usually between the ages of 5 and 10 years, with a peak at puberty. The disease is characterized by papular hyperkeratotic lesions distributed prevalently in seborrhoeic areas of the scalp, face, chest and back. Other features my be seen, including nail involvement, palmar pits, hypertrophic intertriginous plaques, verrucous papules on the dorsal hands. Involvement of the mucous membranes is less frequent. Histology is characterised by dyskeratosis with round bodies and granules and by suprabasal acantholysis with lacunae or clefts in which the cells of basal layer, disposed in a single row, grow in an irregular manner. Fourteen cases of Darier’s disease, 7 males and 7 females, observed from 1980 to 2000 at the Department of Dermatology of the University of Cagliari are described. The age range was 12 to 62 years. The disease began between the ages of 6 and 34 years, with a peak onset between 11 and 15 years. Five patients had no family history of Darier’s disease. Some epidemiological, clinical and therapeutical observations are presented as a contribution to the knowledge of such an uncommon disease.

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