Dysplastic nevi. Occurrence in first- and second-degree relatives of patients with 'sporadic' dysplastic nevus syndrome

Differences and Similarities in Epidemiology and Risk Factors for Cutaneous and Uveal Melanoma

“…Some studies suggested that individuals with atypical nevus syndromes are more predisposed to developing both cutaneous and ocular melanomas [ 10 , 39 , 81 ]. The atypical mole syndrome (AMS) [ 81 , 82 ], the dysplastic nevus syndrome (DNS) [ 83 ], the B-K mole syndrome [ 84 ], and the familial atypical mole and malignant melanoma syndrome (FAMMMS) [ 85 ] describe the presence of clinically atypical nevi with a family history of melanoma. A dominant genetic predisposition is suspected when UM occurs more than once in a single family, or when bilateral UM occurs in a single individual or with CM and multiple atypical nevi [ 86 , 87 ].…”

Mycosis fungoides associated with malignant melanoma and dysplastic nevus syndrome

“…Young age (case 5), positive family history (cases 1 and 2), presence of dysplastic nevus syndrome 20,21 (case 6), and presence of multiple primary melanomas 22 (case 2) point to a genetic predisposition to melanoma in these patients. Although the patient in case 6 with dysplastic nevus syndrome reported no family history of dysplastic nevi, as many as 60% of “sporadic” dysplastic nevus syndrome patients have affected family members 23 on further investigation, suggesting that, like familial melanoma, the dysplastic nevus phenotype is under genetic control.…”

The dysplastic nevus: From historical perspective to management in the modern era

“…6 An autosomal-dominant mode of inheritance of the nevus phenotype has been described in melanoma-prone families. 22 It has been reported that up to 30–40% of these families harbor a mutation in the CDKN2A locus, which encodes the p16 and ARF tumor suppressor proteins. 23 Rare mutations have also been described in a several families in the proto-oncogene CDK4.…”