More recently, other autoinflammatory genes such as NCSTN (nicastrin) and NLRC4 (NLR family CARD domain containing 4) have been identified in PAPASH syndrome whereas causative variants in OTULIN and GJB2 (gap junction protein beta 2), - the latter involved in keratinization pathway -, have been reported in PASH patients [57]. The gene discussed is GJB2; the disease is Pyoderma gangrenosum-acne-suppurative hidradenitis syndrome.