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Fulminant type 1 diabetes is characterized by diabetes with an abrupt onset, severe metabolic acidosis at diagnosis, a low HbA1c level and negativity for islet cell-related autoantibodies, and this illness has been classified as type 1B diabetes by the WHO. The prevalence of this disease is higher in Japan than any other country and recently, there have been an increasing number of such case reports in Korea. Genetic factors and environmental factors such as virus infection and an immune mechanism have been suggested as the mechanism of the pathophysiology, but this remains to be clarified.