Background: Patients with the atypical mole syndrome are prone to cutaneous melanoma, but their risk of ocular melanoma has not been established. We studied the skin of 207 consecutive patients with eye melanoma referred to Moorfields Hospital in London, England, in an attempt to determine what percentage of these patients had the atypical mole syndrome phenotype.
Observations: Five patients were seen who had primary melanomas of both the eye and the skin. In three of these patients, the cutaneous melanomas were discovered only as a result of this study. The number of cutaneous melanomas expected in this cohort of patients with eye melanomas was no more than 0.4 on the basis of the United Kingdom incidence of both tumors. This difference was highly significant.
Conclusions: The occurrence of primary cutaneous melanoma in five patients from a cohort of 207 patients with eye melanoma (or the premalignant melanocytic lesion of the conjunctiva called "primary acquired melanosis") provides strong evidence of an association between cutaneous and ocular melanoma. Three of the five patients also had the atypical mole syndrome phenotype, suggesting that the atypical mole syndrome predisposes to both types of melanoma.