It exists on the same spectrum as SJS, but is characterized by more than 30% body surface area detachment.16 It is believed that drugs or metabolites, acting as receptors, bind to surface of keratinocytes and cause them to become antigenic and stimulate cytotoxic CD8+T cell response.17 In our case, the immune reaction was considered to be triggered by the use of phenobarbital. The gene discussed is CD8A; the disease is Schwartz-Jampel syndrome.