[2][3] PAPA syndrome usually begins with arthritis at a young age, with the skin changes more prominent from the time of puberty.

[citation needed] Pyoderma gangrenosum is variably expressed, which means that it is not always present in all individuals with the disease.

Pathergy is an important feature (this term refers to the tendency of ulcers to arise at points of injury).

There are reports of lesions developing at the site of a joint replacement wound, central venous line and intravenous drip insertion.

Treatments directed at tumor necrosis factor (TNF) (infliximab, etanercept) and interleukin-1 (anakinra) have shown a good response in resistant arthritis and pyoderma gangrenosum.