GNLY and Schwartz-Jampel syndrome: However, it is important to note that HMGB1 is associated with different physiological functions related to the innate immune response and dependent on its redox state.26, 27 Consequently, HMGB1 may be important in the pathogenesis of the keratinocyte damage seen in SJS/TEN, exacerbated by the infiltration of activated innate and adaptive immune cells, and the subsequent localized release of cytotoxic proteins, including granulysin, to augment keratinocyte injury.