Resulting syndromes manifested in these patients have been named PLAID (PLCγ2-associated antibody deficiency and immune dysregulation), with key symptoms being cold-induced urticaria, antibody deficiency, and susceptibility to infection and autoimmunity13, and APLAID (autoinflammatory PLAID), which is characterized by skin lesions, bronchiolitis, arthralgia, ocular inflammation, enterocolitis, absence of autoantibodies and mild immunodeficiency14. Here, PLCG2 is linked to agammaglobulinemia.