INSR and hyperinsulinism: Less severe disease is usually caused by an autosomal dominant gene mutation in the INSR gene, in which patients present in the peri-pubertal stage or beyond with acanthosis nigricans, dysglycemia (either fasting hypoglycemia and postprandial hyperglycaemia or frank diabetes) in the presence of severe hyperinsulinemia, oligomenorrhea, and hyperandrogenism in women.