Histopathology confirmed pustular psoriasis, and clinical exome sequencing identified a heterozygous likely pathogenic variant in RAD21, associated with CdLS type 4 (CdLS4), and a heterozygous variant of uncertain significance in TNFAIP3, which is linked to familial autoinflammatory syndrome. Here, TNFAIP3 is linked to pustular psoriasis.