“…Papillon-Lefèvre syndrome (PLS, OMIM 245000) is a rare autosomal recessive disorder caused by mutations in the CTSC gene, which is located at 11q14.2 and encodes the enzyme cathepsin C [1]. Cathepsin C activates proinflammatory proteases (eg, NE, PR3, CatG, NSP4, chymases, tryptases, and granzymes), regulates the function of immune cells [2][3][4], and controls the formation of the corneocyte envelope.…”