PSMB8 and lipodystrophy: Nakajo-Nishimura syndrome (NNS) is a very rare disorder starting in infancy with pernio-like rashes during the first cold months, gradually evolving into lipodystrophy mainly in the face and upper extremities and remittent fevers, firstly described in 1939 in Japan [99, 100, 108]; a distinct homozygous PSMB8 mutation encoding the proteasome β5i subunit has been identified as its genetic cause, leading to reduced proteolytic activity and accumulation of ubiquitin proteins.