STING-associated vasculopathy, infantile-onset (SAVI) is an autoinflammatory disease starting in early childhood with a vasculopathy causing erythematous, pustular, or blistering infiltrates with scaling on acral locations such as the cheeks, ears, nose, and digits that may even lead to necrotizing skin lesions resulting in mutilation and scarring (43). The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.