Table 4.

Onset and general manifestations of the “pyogenic” autoinflammatory disorders in childhood.

	Onset	Clinical Signs Observed in Children at Onset
Pyogenic arthritis/pyoderma gangrenosum/acne (PAPA) syndrome	First infancy	Skin ulcerations, pyoderma gangrenosum often associated with cystic acne, sterile pyogenic oligoarthritides
Majeed syndrome (MS)	First 2 years of life	Diffuse neutrophilic dermatosis, chronic non-bacterial osteomyelitis, dyserythropoietic anemia
Deficiency of the interleukin-1 receptor antagonist (DIRA)	Neonatal period	Pustular rash, nail abnormalities, ichthyosis-like changes of the skin, multifocal osteomyelitis, multi-organ failure