P0741 / #1916: MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN (MIS-C) ASSOCIATED WITH COVID-19 INFECTION PRESENTING WITH STEVEN JOHSON SYNDROME

AIMS & OBJECTIVES: Multisystem inflammatory syndrome in children (MIS-C) is related to the SARS-CoV-2 Steven Johnson Syndrome (SJS) can also cause multisystem involvement as a result of serious inflammatory reaction We reported two pediatric cases of COVID-19 associated MIS-C syndrome presenting with SJS METHODS: We reported two pediatric cases of COVID-19 associated MIS-C syndrome presenting with SJS RESULTS: Both patient presented with fewer, prominent skin and respiratory findings Skin punch biopsy showed SJS Triggering infection agents were H influenza and COVID-19 Laboratory data showed a leukocytopenia with lymphopenia and eleveated acute phase reactants and impaired liver and kidney function Broad-spectrum antibiotic therapy and variable teratments including immunomodulators, plasma exchange were given Despite all treatments, severe hypoxia continued, they were put on the veno-venous extracorporeal membrane oxygenation (VV-ECMO) The first patient died surgical cannula complicaiton on the 17thday of VV-ECMO Despite effective ECMO support tissue hypoxia became prominent and second patient died on the 40rdday CONCLUSIONS: It is noteworthy that there are similar immunopathological mechanisms between SJS with COVID 19 infection This is the first cases of SJS associated with COVID-19 While the cytokine storm of the first patient were benign and under control, our second case had severe multiorgan failure and hyperinflammatory status of the patient could not be controlled with intensive immunmodulatory treatments The most noticeable difference 2 patients was that the first patient received tocilizumab treatment in the early period This suggests that tocilizumab therapy should be given early in both MIS-C and SJS-related cytokine storm management