RNF31 and lymphangiectasis: Moreover, the L72P missense mutation in the PUB domain of HOIP in a patient with multiorgan autoinflammation, immunodeficiency, amylopectinosis, and systemic lymphangiectasia [135], and another case of HOIP deficiency with early-onset immunodeficiency and autoinflammation, but not amylopectinosis and lymphangiectasia [136], were recently identified.