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. The gene discussed is NLRP1; the disease is autoinflammation with arthritis and dyskeratosis.