Genetic variants were discovered in MEVF, PSTPIP1, NLRC4, WDR1, NOD2, OTULIN, NCSTN, and GJB2, which may be attributed to identification by WES, the presence of additional inflammatory disorders, or the polygenic nature of HS.27 The variety of mutations seen in cases of syndromic HS is indicative of the complex, multifactorial nature of the disease pathogenesis. The gene discussed is NLRC4; the disease is histiocytic sarcoma.