Background. Familial melanoma has been associated with ''clinically at
ypical moles'' or ''dysplastic nevi,'' (DN) which are markers for incr
eased melanoma risk. In addition, melanomas in these kindreds present
at a younger age, and tend to be multiple. Methods. Melanoma incidence
rates were determined for 710 members of 311 melanoma families, defin
ed as kindreds in which melanoma had occurred in two or more blood rel
atives. Patients were classified either clinically or histologically a
s expressing DN. Melanomas that occurred before the first examination
were recorded, and patients were followed prospectively for new melano
mas. Results. In prospective follow-up, the age-adjusted melanoma inci
dence rate was 1710/100,000 patient-years in family members with DN. I
n contrast, the rate was zero (no melanomas occurred) in family member
s without DN. For family members with DN, but without a history of mel
anoma, the age-adjusted incidence rate of melanoma was 413/100,000 pat
ient-years, whereas the rate was 2779/100,000 patient-years in family
members with DN and a history of melanoma. Conclusions. Dysplastic nev
i and a history of melanoma are strong risk factors for subsequent mel
anoma. Prognostic factors are greatly improved for patients with melan
omas diagnosed in follow-up compared with the first two melanomas in e
ach kindred. These findings warrant surveillance of individuals with D
N who are members of familial melanoma kindreds.