Alternate terms for atypical nevus syndromes include B-K mole syndrome, familial atypical multiple mole melanoma syndrome, dysplastic nevus syndrome, and sporadic dysplastic nevus syndrome.

This concept was first introduced into the literature in 1978 with independent and simultaneous reports by two different investigators. The multiple designations are a result of a disease concept that is in evolution. A National Institutes of Health (NIH) conference has settled on the clinical term "atypical" rather than the histologic term "dysplastic," which was felt to be confusing and poorly defined. Whether these syndromes will eventually be defined as a group of distinct entities with the common feature of an atypical melanocytic nevus, or a continuum of disease with a variable risk for malignant melanoma, remains to be seen.

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