Naslov
Pachyonychia congenita - major and minor symptoms

Autori
Basta-Juzbašić, Aleksandra ; Videnić, Nada ; Stanimirović, Andrija ; Skerlev, MIhael ; Pašić, Aida

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Abstracts of the 10th Congress of the European Academy of Dermatology & Venereology štampano u Journal of the European Academy of Dermatology and Venereology, vol 15 (supp 2) / Lotti, Torello - Brisel : Wiley-Blackwell, 2001

Skup
10th Congress of the European Academy of Dermatology & Venereology

Mjesto i datum
München, Njemačka, 10.10.2001. - 14.10.2001

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
pachyonychia congenita; symptoms; treatment

Sažetak
Pachyonychia congenita (Jadassohn-Lewandowsky syndrome) characterized by nail hypertrophy and dyskeratosis of skin and mucous membranes was studied in the population of Croatia. Altogether 25 cases were detected, so incidence of this condition in the Croatian population according to the presented data is 0.53 case per 100.000 inhabitants. The patients belonged to 7 families; 16 were manifest cases and 9 were abortive with some of the symptome of pachyonychia congenita syndrome. A majority of the families showed a dominant trransmission of the trait, but even in the same family there was considerable clinical variability of disease expression. In our patients we classified the most frequently observed symptoms such as palmoplantar keratoderma, pachyonychia, follicular hyperkeratosis nad hyperhidrosis into the major ones, and leukokeratosis, blisters, hoarsenes and cicatricial alopecia into minor ones. Patients were treated with various topical keratolytics, locally applied retinoic acid and in some periods with oral retinoids, etretinat or acitretin. Certain success was achieved in palmoplantar lesions, follicular hyperkeratosis and leukokeratosis, but nails remained almost te same.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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