Fulminant type 1 diabetes in Caucasians: A report of three cases

Fulminant type 1 diabetes is a new clinical entity in which the process of beta-cell destruction, and the subsequent progression of hyperglycaemia and ketoacidosis, are extremely rapid. Until now, this subtype of type 1 diabetes has only been reported in the Asian population, especially Japanese and Koreans.

We report here on three cases of fulminant type 1 diabetes in Caucasian French women. Both the clinical and biological characteristics of these patients are similar to those reported in Japanese studies. Notably, all patients experienced severe ketoacidosis (pH<7.1) that occurred abruptly after the onset of hyperglycaemic symptoms (<6 days), with near-normal HbA(1c) values at diagnosis (5.6, 6.4 and 6.8%). Patients were treated in the intensive care unit with basal-bolus insulin therapy with no remission of their diabetes; pancreatic islet-related autoantibodies were all negative. Fasting C-peptide levels were undetectable, suggesting complete destruction of pancreatic beta-cells. HLA phenotyping of these Caucasian patients did not find the specific HLA haplotype (DRB1*0405-DQB1*0401) previously found to be linked to fulminant type 1 diabetes in Japanese patients.

These are the first cases of fulminant type 1 diabetes reported in Caucasians. These cases reveal new perspectives as regards the worldwide distribution of this intriguing clinical entity.