What is Dysplastic nevus syndrome?

Dysplastic nevus syndrome, also known as familial atypical multiple mole–melanoma syndrome, is an inherited cutaneous condition described in certain families, and characterized by unusual nevi and multiple inherited melanomas. First described in 1820, the condition is inherited in an autosomal dominant pattern, and caused by mutations in the CDKN2A gene. In addition to melanoma, individuals with the condition are at increased risk for pancreatic cancer.

The diagnosis of dysplastic nevus syndrome is based on clinical presentation and family history. Treatment consists of resection of malignant skin lesions. Screening for pancreatic cancer may be considered, particularly if there is a family history.