Many congenital syndromes are diagnosed with the aid of cutaneous findings. Certain medical conditions are detected early based on the appearance of premature, numerous, or abnormal skin lesions. The association of melanocytic nevi with specific syndromes is prominent with certain conditions and less consistent with others. Melanocytic nevi are fairly prevalent in the general population and often exhibit familial clustering; therefore, close attention must be paid to all associated findings in order to recognize the pattern of various syndromes and facilitate counseling and timely intervention. This chapter describes syndromes associated with melanocytic nevi.
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