Introduction: Endocrine disorders during Von Recklinghausen’s Disease or neurofibromatosis type 1 (NF1) are rare and particularly observed in children. However, autoimmune diabetes mellitus (DM) remains exceptional and unusual during this phacomatosis. We report an original case of Latent Autoimmune Diabetes in Adults (LADA) associated with NF1.Case Report: A 32-year-old Tunisian male, known to have NF1 since childhood, was admitted for significant recent weight loss (10 kg in one month) with high blood glucose levels. The biological tests confirmed the diagnosis of DM with marked ketoacidosis: fast blood glucose at 16 mmol/l, postprandial glucose at 21 mmol/l, and HbA1c at 9.9%. Radiological and endoscopic investigations did not indicate pancreatic and/or duodenal tumors. Anti-GAD and anti-IA2 autoantibodies were positively confirming the diagnosis of LADA. The assessment of degenerative complications and screening for possible other autoimmune diseases were negative. The evolution was favorable under intensive insulinotherapy.Conclusion: The association of DM type 1 with NF1 remains exceptional and only four cases are found in the literature, all pediatrics. Our observation is, to our knowledge, the first reporting this association in adult (LADA with NF1)
