Naslov
Dysplastic nevus syndrome associated with neurofibromatosis type I

Autori
Paštar, Zrinjka ; Lipozenčić, Jasna

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

Izvornik
Journal of the European Academy of Dermatology and Venereology / - Pariz, 2008

Skup
17th Congress of the European Academy of Dermatology and Venereology,

Mjesto i datum
Pariz, Francuska, 17.09.2008. - 21.09.2008

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Dysplastic naevus syndrome; Neurofibromatosis type I; case report

Sažetak
Dysplastic nevus syndrome (DNS) represents multiple atypical nevi associated with polygenetic inheritance pattern and may rarely occur together with neurofibromatosis type 1 (NF-1). NF-1 is an autosomal dominant disorder that primarily affects the development and growth of neural cell tissues. It causes tumors to grow on nerves, and produce other abnormalities such as skin changes and bone deformities. DNS is a marker of increased malignant melanoma (MM) risk while MM has been reported in up to 5% of patients with NF-1. We describe a case of DNS type I with NF-1 type I presenting with multiple neural tumors, cafe-au-lait spots, hamartoms in globus palidum and pigmented iris hamartomas (Lisch nodules). The importance of close follow-up of nevi of such patients for the development of MM is highlighted.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA