2025 Volume 68 Issue 12 Pages 440-446
The patient was an 84-year-old woman who had been treated with linagliptin for type 2 diabetes since 83 years of age. Two months after developing papuloerythroderma of Ofuji associated with sigmoid colon cancer, her HbA1c level rapidly increased to 16.7 % and her blood glucose level reached 811 mg/dL, leading to diabetic ketoacidosis. The presence of multiple islet-related autoantibodies initially suggests acute onset type 1 diabetes. However, insulin secretion remained preserved for 22 months after the onset, leading to the diagnosis of slowly progressive type 1 diabetes (probable). The patient carried the class II HLA haplotypes associated with disease susceptibility (DRB1*09:01-DQB1*03:03) and disease resistance (DRB1*15:02-DQB1*06:01), which may have influenced the disease phenotype and preservation of insulin secretion. To our knowledge, this is the first reported case of coexisting papuloerythroderma of Ofuji, a combined Th2/Th22 immune disorder and slowly progressive type 1 diabetes (probable). We herein discuss this case, including its pathology.