Pyrin-associated autoinflammation with neutrophilic dermatosis related to heterozygous MEFV gene variants in cis: Two case reports in a family

• We reported a Chinese pedigree of PAAND with two affected members, with MEFV P369S and R408Q variants in cis . • The patients were successfully treated with IL-1 inhibitor canakinumab. • Our clinical and functional data confirmed the heterogeneity of MEFV P369S/R408Q variants, and we speculated they might be associated with PAAND.