KS is a rare genodermatosis characterized by skin fragility, skin atrophy, premature aging and poikiloderma. Several reports in the literature have also shown the presence of SCC´s arising in KS patients suggesting a predisposition of these patients to this neoplasia. However, no systematic study has been carried out investigating the frequency of SCC in KS and the outcome of the disease in these patients.Here, we studied the incidence of SCCs in a cohort of 44 well-characterized patients with KS by clinical and molecular techniques.A total of 10 patients developed SCC at different ages. The youngest patient with SCC was 33 years old. The cumulative risk increases up to around 50% at age 50 and plateau afterwards. This pattern is somewhat smaller than the cumulative risk of Recessive Dystrophic Epidermolysis Bullosa (RDEB) and remarkably, patients with KS appear to develop SCC 10-20 years later than patients with RDEB.Our study also showed that SCC developed in SK patients have the potential of becoming metastatic:  In our cohort, SCC lead to death in one case and to amputation in the other. An additional patient (alive) presented multiple metastasis. 
This study suggests that approximately 50% of patients with KS may develop SCC during their lifetime and 30% metastatic disease with the possibility of a lethal outcome.

A study of non-melanoma skin cancer in a series of well-characterized patients with Kindler Syndrome (KS)