PSMB4 and lipodystrophy: (24), 10 patients with PRAAS (previously referred to as CANDLE-Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature), harboring monogenic mutations in PSMB8 (n = 6) and PSMB4 (n = 1), or digenic mutations in PSMB4/PSMB9 (n = 2) and PSMA3/PSMB8 (n = 1), were treated, with a major clinical improvement as compared to SAVI.