Skin and visceral granulomas have been described in several other IEIs with predominance of DNA repair defects including ataxia telangiectasia [AT], Artemis deficiency, Nijmegen-breakage syndrome [NBS], PRKCD deficiency (36, 37) and ligase IV deficiency (38–41) (Table 1). This evidence concerns the gene PRKCD and ataxia telangiectasia.