Here, we report the first case of a Chinese patient with APLAID caused by a novel PLCG2 gene mutation with rare manifestation of gangrenous pyoderma and concomitant high serum immunoglobulin (Ig) E level, which has not been reported before. Here, PLCG2 is linked to autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation.