The Atypical Nevus Syndrome is defined by the following criteria:
- A first-degree (e.g., parent, sibling or child) or second-degree (e.g. grandparent, aunt, uncle) relative with malignant melanoma.
- A large number of nevi, often more than 50, some of which are atypical nevi.
- Nevi that demonstrate certain microscopic features.
Individuals with benign melanocytic lesions and a positive family history for this syndrome must be closely monitored.
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