KS is an autosomal recessive genodermatosis resulting from loss-of-function mutations in FERMT1, the gene that encodes Kindlin-1 leading to skin atrophy, blistering, photosensitivity, hyper or hypo-pigmentation, increased light sensitivity as well as a high incidence of cSCC [15]. Here, FERMT1 is linked to skin squamous cell carcinoma.