Keratinocytes constitutively express high levels of NLRP1 and germline mutations of NLRP1 in humans lead to a number of skin-related inflammatory diseases, including multiple self-healing palmoplantar carcinoma (MSPC), familial keratosis lichenoides chronica (FKLC)13, vitiligo14,15, autoinflammation with arthritis and dyskeratosis (AIADK)16,17, and juvenile-onset recurrent respiratory papillomatosis (JRRP)18. Here, NLRP1 is linked to respiratory papillomatosis, juvenile recurrent, congenital.