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| | PAPA syndrome | Deficiency of interleukin-1 receptor antagonist | Blau syndrome | CANDLE syndrome (Nakajo-Nishimura syndrome) |
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| Gene | PSTPIP1 (15q24-q25.1) | IL1RN (2q) | CARD15/NOD2 (16q2) | PSMB8 (6p21.3) |
| Inheritance | Autosomal dominant | Autosomal recessive | Autosomal dominant | Autosomal recessive |
| Protein encoded | CD2 antigen-binding protein 1 | Interleukin-1 receptor antagonist | NOD2 | 5i subunit of the immunoproteasome |
| OMIM | 604416 | 612852 | 186580 | 256040 |
| Onset | Infancy | Neonatal period | Before 3-4 years | Infancy |
| Fever | — | Absent | Inconstant | Recurrent |
| Rash features | Pyoderma gangrenosum, cystic acne | Pustular with ichthyosis-like changes | Brown-coloured and flaky, multiple subcutaneous granulomatous nodules | Cold-induced pernio-like or purpuric/vasculitic lesions, progressive lipodystrophic changes in the upper body |
| Articular signs | Sterile pyogenic oligoarthritis | Sterile multifocal osteomyelitis | Recurrent granulomatous symmetric polyarthritis with progressive trend, tenosynovial cysts, and “boutonnière” finger deformities | Joint contractures, clubbed fingers and toes |
| Ocular signs | — | — | Recurrent granulomatous panuveitis, chorioretinitis | — |
| Neurologic signs | — | — | — | Basal ganglia calcification |
| Treatment | Corticosteroids, tumor necrosis factor-inhibitors, and interleukin-1 antagonists | Interleukin-1 antagonists | Corticosteroids, immunosuppressive agents, tumor necrosis factor-inhibitors, and interleukin-1 antagonists | Corticosteroids, dapson, interferon-gamma modulators, and interleukin-6 antagonists |
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