Marzano et al. described a missense variant of uncertain significance (VUS) with pathogenic evidence in NLRP3 gene (NM_001243133.2:c.2107 C > A; p.Gln703Lys) in a PASH patient, reinforcing the potential causative role of dysregulated innate immunity and IL-1β-dependent autoinflammation in this spectrum of diseases [22]. The gene discussed is IL1B; the disease is Pyoderma gangrenosum-acne-suppurative hidradenitis syndrome.