Nishimura EK, Jordan SA, Oshima H, Yoshida H, Osawa M, Moriyama M, et al
Most are located on the trunk but occasionally they are noted on the proximal limbs and genitalia
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group III: 3 points (RR: 85-1269) (Figure 3).3,15,30 Thus, the presence of dysplastic nevi and AMS can be considered as a spectrum of phenotypic expressions, which confers to the carriers different risks of developing melanoma.3,21 The risk spectrum varies from individuals with sporadic dysplastic nevi, with no personal or family history of melanoma, to the opposite extreme with AMS individuals belonging to families in which two or more members had melanoma, forming the so-called familial atypical multiple-mole melanoma syndrome (FAMMM).3,4,15,16,20,31 Besides presenting an increased risk of developing cutaneous melanoma, AMS patients tend to present neoplasia in unusual sites (such as the scalp) at an earlier age than the non-carriers of the syndrome.1 There is also increased risk for multiple melanomas and non-cutaneous melanoma in those patients, such as ocular melanoma.8 Prospective studies show that the risk of melanoma in members of families affected by AMS and FAMMM is significant, with an estimated cumulative risk of 49% in individuals 10 to 50 years of age and 82% in individuals 72 years of age.32 Histopathology Although the histopathologic exam is considered to be the gold standard for the diagnosis of melanocytic tumors, there are limitations in the histologic distinction between early melanomas and dysplastic nevi.12 In several studies, the reproducibility in grading the atypia remains poor to moderate, due to a lack of uniform criteria among dermatopathologists.12,18 Based on the World Health Organization (WHO) and NIH consensus, the histopathologic diagnosis of dysplastic nevi (Figure 4) is based on the major criteria (mandatory) and minor criteria (at least two must be present)23, detailed in Table 2
The provider confirms the diagnosis of HSDD, reviews medical history, and screens for cardiovascular risk factors
