Even though DNA repair deficiency is known to lead to immunodeficiency (19, 24), it is notable that in the newest update of the International Union of Immunological Societies (IUIS) Expert Committee in 2022, most of the listed DNA repair genes are involved in DSB repair (25) while defects in NER-associated genes such as ERCC2 are not included and relatively few proteins involved in NER are yet associated with immunodeficiency (3, 24). The gene discussed is ERCC2; the disease is immune system disorder.