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Premature Pubarche (PP), or the development of pubic hair before the age of 8 years in girls or 9 years in boys, is most commonly caused by Idiopathic Premature Adrenarche (IPA). There is an increased production of Dehydroepiandrosterone (DHEA) and DHEA Sulfate (DHEAS) by the adrenal zona reticularis without a concomitant rise in Cortisol. IPA is a diagnosis of exclusion and differential diagnosis must include milder and nonclassic variants of Congenital Adrenal Hyperplasia (CAH), that can account for 0 to 40% of cases of PP. Early morning basal 17Hydroxyprogesterone (17OHP) above 200 ng/dL is 100% sensitive and 99% specific for CAH. INTRODUCTION

Premature pubarche: distinguishing between nonclassic congenital adrenal hyperplasia and idiopathic premature adrenarche