The susceptibility genes for CM-related SJS/TEN with SOC, PTGER3, and IKZF1 also have functional interactions with TLR3; EP3 (PTGER3) negatively regulated TLR3-dependent ocular surface inflammation (Ueta et al., 2011b; Ueta et al., 2012a; Ueta et al., 2012c); and IKZF1 mRNA was upregulated by TLR3 in human epidermal keratinocytes and conjunctival epithelial cells (Ueta et al., 2018). Here, TLR3 is linked to Schwartz-Jampel syndrome.