SAVI is another type I interferonopathy with skin and lungs involvement, produced by monogenic STING gain—of—function mutation, with skin involvement ranging from mild chilblain vasculopathy to digital amputations, nasal septum perforation, livedo reticularis, facial rash, periungual erythema, onychodystrophy [52, 53]. This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.