Premature Pubarche: An Analysis of 102 Patients

Abstract

Premature pubarche is the clinical appearance of coarse sexual pubic hair before 8 years of age in the female and 10 years in the male, in the absence of signs of the gonadarche (thelarche in girls, testicular enlargement in boys).¹ Other signs of androgen excess in these children include the development of axillary hair or adult axillary odor, acne, and accelerated growth. In relationship to chronologic age, premature pubarche is associated with increased serum concentrations of dehydroepiandrosterone (DHEA) and/or its sulfate (DHAS) as well as androstenedione, testosterone, and dihydrotestosterone in many subjects.²

Normal adrenarche is accompanied by increased adrenocortical activity of 3β hydroxysteroid dehydrogenase and 17–20 lyase (P450-cl7) enzymes, possibly due to an autoregulatory mechanism associated with the increased mass of the growing adrenal.^3,4 Height age and skeletal maturation are modestly advanced for chronologic age, and some but not all children with premature pubarche are overweight for height. This developmental pattern has been considered a benign pubertal variant, with subsequent normal linear growth, sexual maturation, and fertility.^5,6 However, this concept has been challenged by reports of children with variant or mild forms of congenital adrenal hyperplasia (CAH) or with the carrier status for this disorder.^7–9