The screening of more patients to confirm this and to specify the phenotype of this monogenic trait caused by ectopic ASIP expression is pertinent, particularly as MCR agonists, such as setmelanotide as recently approved for deficiencies in components of the leptin–melanocortin signaling pathway7, provide a promising treatment option for these patients; however, concomitant stimulation of MC1R with setmelanotide causes skin pigmentation and may change melanoma risk38. Here, MC1R is linked to melanoma.