title: [Familial Darier disease and mental retardation in mother and her two sons].
abstract: Darier disease (DD) is an uncommon genetic skin disorder, which begins in adolescence or early adult life. This disease is observed more often among men and where the disease course is more severe. Many dermatologists observe in patients with DD neuropsychiatric disorders: psychosis, depression and rare mental retardation. We present familial DD in a mother and her two sons. Men demonstrated a typical onset and course of the disease. The onset of the disease in the 52nd year of age and the skin lesions (more characteristic for the Lyell disease) which occurred suddenly in women, were atypical. Besides skin lesions, mental retardation with a various degree of intensity was observed in all three patients.
authors.affiliation: Katedra i Klinika Dermatologii CM UJ 31-501 Kraków, ul. Kopernika 19.
authors.forename: Anna
authors.initials: A
authors.lastname: Wojas-Pelc
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Setkowicz
authors.forename: Jacek
authors.initials: J
authors.lastname: Pelc
mesh_terms: D000328:Adult; D007644:Darier Disease / Q000150:complications* / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000150:complications*; D007645:Keratoderma, Palmoplantar / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged
vernacular_title: Rodzinna choroba Dariera i upośledzenie umysłowe u matki i jej dwóch synów.