This report presents a rare case of pure classical congenital adrenal hyperplasia
(CAH) due to 21-hydroxylase deficiency in a 22-year-old woman with a 46 XX
genotype. The patient exhibited virilism, excessive hair growth, and primary
amenorrhea with absent secondary sexual characteristics. The diagnosis was
confirmed by 17-hydroxyprogesterone testing and the Synacthen test. Treatment
with hydrocortisone and spironolactone was followed by feminization surgery,
leading to the development of secondary sexual characteristics, including breast
development, a reduction in hirsutism, and the onset of regular menstruation.
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