RESUMEN
Insulin autoimmune syndrome or Hirata's disease is a rare condition characterized by hypoglycemia associated with endogenous autoimmune hyperinsulinism. This report concerns the case of a 28-year-old Latin American woman with Graves' disease who developed insulin autoimmune syndrome and then subsequently became pregnant. She displayed symptoms related to severe hypoglycemia due to hyperinsulinemia, elevated C-peptide, and anti-insulin antibodies. Prior to pregnancy she was treated with corticosteroids and had ablative treatment with iodine-131. During follow-up of both conditions, the patient became pregnant, and clinically and biochemically hyperthyroid, for which total thyroidectomy was performed during the second trimester of pregnancy. Anti-insulin antibodies, blood glucose, and C-peptide remained normal throughout pregnancy. At 40 weeks of gestation she gave birth to a healthy female newborn with normal blood glucose values. Molecular genetic analysis determined the following genotypes: HLA-DRB1*03:01 / HLA-DRB1*04:01 in the mother; and HLA-DRB1*04:01 / HLA-DRB1*08:02 in the daughter. Because some HLA-DRB1*04 alleles are associated with susceptibility to insulin autoimmune syndrome induced by environmental factors, the patient was advised regarding the future use of drugs with a sulfhydryl group and possible triggering factors for insulin autoimmune syndrome. At 6-month follow-up the daughter presented normal growth and development, as well as normal plasma glucose values, and this remained the case at five-year follow-up.
RESUMEN
Background: Clitoromegaly is often a sign of androgen excess; however, non-hormonal causes must be ruled out. We report the case of an adolescent with isolated clitoromegaly without clinical or biochemical evidence of hyperandrogenism. Case: A 16-year-old female was referred due to a clitoromegaly of 12 months of evolution. Examination of the pubic region revealed normal female genitalia with an enlarged clitoris, 4 cm long and 2.5 cm wide. The clitoris was painless, soft on palpation, and mobile over deeper layers. There were no signs of virilization, and the patient did not report dysuria or difficulties with sexual intercourse. Her medical record was also unremarkable, with no female circumcision, family history of birth defects, or genital abnormalities. Hormone profile blood tests were normal. Pelvic ultrasound examination was normal, but a high-resolution scan with a linear transducer confirmed the presence of a cyst, lying anterior to the clitoral body and glans. The cyst was surgically removed with special care to preserve the clitoral neurovasculature. The pathological report disclosed an epidermoid clitoral cyst. The patient described emotional well-being, satisfactory sexual function, and no discomfort after a year of follow-up. Conclusion: Epidermal clitoral cysts represent an unusual cause of clitoromegaly. These cysts should be ruled out as a differential diagnosis after an exhaustive semiological and endocrinological examination.