Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE), stimulator of interferon genes (STING)-associated vasculopathy with onset during infancy (SAVI), and Aicardi-Goutières syndrome (AGS) are Mendelian autoinflammatory interferonopathies characterized by early-onset systemic and organ-specific inflammation and a prominent interferon (IFN) response gene signature (IGS) [1]. This evidence concerns the gene STING1 and Aicardi-Goutières syndrome.