APLAID presents with recurrent blistering skin lesions, bronchiolitis, arthralgia, ocular inflammation, enterocolitis, absence of autoantibodies, and mild immunodeficiency, with a decrease in circulating IgM and IgA antibodies, decreased numbers of class‐switched memory B cells, and decreased numbers of Natural Killer T (NKT) cells.148 The phenotype in APLAID is thought to be the consequence of the GOF mutations that create an extra phosphorylation site which enhances activation PLCG2 by compromised (although not completely abrogated) autoinhibition of PLCG2 activity. The gene discussed is CD79A; the disease is autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation.