Naslov
Pachyonychia congenita in the croatian population

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

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

Izvornik
Book of Abstracts of Second Congress of Croatian Dermatovenerologists with international participation štampan u časopisu Acta Dermatovenerologica Croatica Vol 10 (2) / Lipozenčić, Jasna - Zagreb : Croatian Dermatovenerological Society, Grafoplast, 2002, 114-115

Skup
Second Congress of Croatian Dermatovenerologists with International Participation

Mjesto i datum
Opatija, Hrvatska, 16.05.2002. - 19.05.2002

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Pachyonychia congenita; Croatia

Sažetak
Pachyonychia congenita (Jadassohn-Lewandowsky syndrome) is characterized by nail hypertrophy and dyskeratosis of the skin and mucous membranes. On examinations for palmopalntar keratodermia, 25 cases of pachyonychia congenita were detected. The data collected revealed the incidence of this condition in the Croatian population to be 0.53 per 100.000 inhabitants. The patients belonged to 7 families. There were 16 cases of manifest and 9 aborttive cases with some of the pachyonychia congenita syndrome symptoms. A majority of the families showed a dominant mode of the trait transmission, however, considerable clinical variability of the disease expression was recorded even within the same families. In all patients, the most common symptoms such as palmoplantar keratoderma, pachyonychia, follicular hyperkeratosis and hyperhidrosis were classified as major ones, and leukokeratosis, blisters, hoarseness and cicatricial alopecia as minor ones. Patients were treated with various topical keratolytics, locally applied retinoic acid, and in some periods with oral retinoids, etretinate or acitretin. Some success was achieved in palmopalntar lesions, follicular hyperkeratosis and leukokeratosis, whereas nail lesions remained almost unchanged.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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