An autoinflammatory syndrome characterized by the onset of systemic autoinflammation in the first months of life. Features include lymphadenopathy, hepatosplenomegaly, fever, panniculitis, and nodular skin rash. Additional manifestations may include inflammation of the optic nerve, intracranial hemorrhage, and lipodystrophy. The disease is autoinflammatory disease, X-linked (MONDO_0800129). Also known as: NEMO deleted exon 5 syndrome, autoinflammatory syndrome, X-linked.