Thus, variants in the MEFV gene cause familial mediterranean fever (FMF, OMIM #249100); in NLRP3, cryopyrin-associated periodic syndrome (CAPS, OMIM #120100); in TNFRSF1A, TNF receptor-associated periodic syndrome (TRAPS, OMIM#142680); in MVK, Hyper-IgD syndrome (HIDS, OMIM#249100) and in PSTPIP1, pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPAS, OMIM#604416). Here, PSTPIP1 is linked to hyperimmunoglobulinemia D with periodic fever.