Naslov
Mal de Meleda still present on the island of Adriatic sea

Autori
Basta-Juzbašić, Aleksandra ; Bakija-Konsuo, Ana ; Šitum, Mirna ; Nardelli-Kovačić M, Lončarić, Davor ; Seiwert, Sven.

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

Izvornik
Abstract Book of 3rd Congress of the Mediterranean Association of Dermatology / Amed - Kemer : Amed, 2001

Skup
3rd Congress of the Mediterranean Association of Dermatology

Mjesto i datum
Antalya, Turska; Kemer, Turska, 23.05.2001. - 27.05.2001

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Mal de Meleda; Adriatic sea

Sažetak
Mal de Meleda is a hereditary palmoplantar keratoderma which was named after Mljet Island (previously called Meleda) in Adriatic sea, Croatia. First cases originated on this island between 1397 and 1908, during which time the island was used for quaranting people from town of Dubrovnik suffering from plague and leprosy. Such a setting, characterized by reproductive isolation and high inbreding even nowadays, favor homozygosity and thus increased incidence of conditions (such as Mal de Meleda) which show an autosomal recessive inheritance pattern. The aim of this study was to fully characterise all 12 living cases originating from the island. Six field investigations (1998-1999) identified 12 patients and 37 family members who were interviewed and examined. The eight reconstructed genealogies implied a common ancestry of all cases but one. Previously reported obligate and facultative symptoms were confirmed in the majority of cases. The symptoms in these cases were milder when retinoids (etretinat or acitretin) were applied together with continuous symptomatic topical treatment.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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