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Multiple benign juvenile melanoma is a rare entity, seen primarily in children. Two 3-year-old patients who developed multiple juvenile melanomas in areas of congenital hyperpigmentation are presented. The distinct clinical presentations of this lesion are discussed, and its ultrastructure is described.

It has recently been shown that both dysplastic and congenital nevi are precursors to malignant melanoma. These findings are based upon mathematical models that show an increased risk of the nevi evolving into melanoma over random choice. However, problems exist with these models that may invalidate their results. The recommendation to remove dysplastic and congenital nevi prophylactically based upon models such as these is premature.

The dysplastic nevus has not only been considered to be a "marker," but also a formal "precursor" of malignant melanoma. Therefore, these lesions are important to recognize clinically. This article presents a classification of the dysplastic nevus based upon its variable clinical presentations. It is hoped that this classification will assist the physician to recognize many of the clinical variants of this unusual melanocytic nevus and, thus, to identify patients at greater risk for the development of malignant melanoma.

In conclusion, although there are data, some quite convincingly implicating dysplastic nevi and congenital nevi (particularly "giant") as "precursors" of malignant melanomas, our ability to predict the magnitude of these associations is lacking. Thus, until additional basic and clinical research data are forthcoming, any recommendation to prophylactically remove all congenital nevi or all dysplastic nevi in order to decrease the incidence of malignant melanoma is premature. In regard to congenital nevi, evidence exists that giant (larger than 20 cm in diameter) congenital nevi may have a significant risk factor so as to warrant, when feasible, prophylactic excision of such lesions. In our opinion, no uniform recommendation can be made at this time for the management of small and medium-sized congenital nevi. Patients with familial dysplastic nevus syndrome should be followed carefully and educated concerning the early detection of malignant melanoma. Patients with sporadic dysplastic nevus syndrome deserve further study to enable us to accurately determine their risk of developing malignant melanoma.