engleski

Dysplastic nevus syndrome associated with neurofibromatosis type I

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.

Dysplastic naevus syndrome; Neurofibromatosis type I; case report

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