| | PAPA syndrome | | Pyogenic Arthritis (sterile), Pyoderma gangrenosum, Acne
– Inherited (AD), CD2 binding protein 1 (CD2BP1) mutation; CD2BP1 is a pyrininteracting protein, which is part of inflammatory pathway associated with familial Mediterranean fever, Muckle-Wells syndrome, and familial cold urticaria
– Skin changes typically present near or at puberty |
| | SAPHO
(Chronic recurrent multifocalosteomyelitis) | | Synovitis, Acne (conglobata), Pustulosis (palmoplantar), Hyperostosis, Osteitis
– Inflammatory bone changes (commonly involving sternoclavicular joint, spine {spondyloarthropathy} and long bones); peripheral arthritis also common
– 1 st line treatment: bisphosphonates suggested in many case reports and series; other treatments include mainstay therapies for psoriatic arthritis (methotrexate, anti-TNF a agents, etc.) |
