Markedly elevated S100 levels are a hallmark of SJIA, Familial Mediterranean Fever (FMF) and PSTPIP1 associated inflammatory diseases (PAID) such as pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome or PSTPIP1-associated myeloid-related proteinaemia inflammatory (PAMI) syndrome (71). Here, PSTPIP1 is linked to pyoderma gangrenosum.