Additionally, this review describes the manifestations of interferonopathies with monogenic SLE presentations as well as the pathogenic mechanism of related genes, highlighting the importance of identifying those diseases, especially for patients with early-onset clinical IFN signatures, skin vasculopathy with chilblains, livedo reticularis and panniculitis, involvement of the central nervous system, and interstitial lung disease. This evidence concerns the gene IFNA1 and panniculitis.