Familial occurrences of malignant melanoma may be related to an inherited syndrome of precursor cutaneous lesions with distinct clinical and histologic features. Recognition of the syndrome in the relatives of melanoma patients identifies a subset of family members at high risk for melanoma, facilitating their early diagnosis and treatment. Further studies of these families may provide insight into the biology of malignant melanoma and the pathophysiology of malignant transformation in benign nevi.

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