Table 6.

Onset and general manifestations of interferon-related autoinflammatory disorders in childhood.

	Onset	Clinical Signs Observed in Children at Onset
Proteasome-associated autoinflammatory syndromes (PRAAS)	Early childhood	Recurrent fevers, nodular skin rashes and annular violaceous plaques evolving to panniculitis-induced lipodystrophy (loss of adipose tissue), large nose, lips and ears, eyelid swelling, muscle weakness and atrophy, joint contractures, disproportionately long and thick fingers, hepatosplenomegaly, basal ganglia calcifications
STING-associated vasculopathy with infancy onset (SAVI)	Neonatal period	Skin vasculitis with violaceous scaling or pernio-like lesions on fingers, toes or nose, usually exacerbated by cold exposure, that progress to ulcerations of extremities or to autoamputation phenomena, chronic interstitial lung disease
Aicardi-Goutières syndrome (AGS)	Neonatal or prenatal period	Subacute encephalopathy, cerebral and basal ganglia calcifications, spasticity, dystonia, microcephaly, eye abnormalities, livedo reticularis, digital vasculitis with chilblain lesions on hands and feet, hepatosplenomegaly, silent positivity of autoantibodies