Table 4.

Causes of isolated premature pubarche

• Premature adrenarche

• Idiopathic premature pubarche

• Hypertrichosis

• Central precocious puberty

• Exogenous androgenic and anabolic steroids

• Virilizing disorders

1. Dexamethasone-suppressible disorders

a. Virilizing congenital adrenal hyperplasia

(1) 21-hydroxylase deficiency, classic and nonclassic

(2) 11ß-hydroxylase deficiency, classic and nonclassic

(3) 3ß-hydroxysteroid dehydrogenase deficiency, classic and nonclassic

b. Rare congenital disorders of adrenal steroid metabolism

(1) Cortisone reductase deficiency (and apparent CRD)

(2) Apparent sulfotransferase deficiency

c. Peripheral androgen metabolic disorders

(1) Portosystemic shunting

2. Dexamethasone-resistant androgen and glucocorticoid excess

a. Endogenous Cushing’s syndrome

b. Glucocorticoid resistance

3. Dexamethasone-resistant androgen excess without glucocorticoid excess

a. Virilizing tumor

b. Gonadal hyperandrogenism

(1) Gonadotropin-independent precocious puberty

(2) Human chorionic gonadotropin-secreting germ cell tumors

(3) Familial isolated luteinizing hormone excess