Hirsutism & Virilization Flashcards
Hirsuitism
More terminal hair; pubic hair changes to male diamond
Virilization
Male features develop (voice deepens, balding, muscle mass increases, clitoromegaly, breast atrophy)
Sources of androgens:
Can be adrenal (DHEAS elevated) or ovarian; both result in increased free T levels.
Adrenal disorders
Cushing syndrome
Congenital adrenal hyperplasia
Cushing syndrome
Cushing disease if from ACTH-secreting pituitary adenoma; also can be paraneoplastic or 2/2 adrenal tumor (which would suppress ACTH).
Work up for Cushing syndrome
Get overnight dexamethasone suppression test (should decrease endogenous production if normal negative feedback); or 24h urine for cortisol
Congenital adrenal hyperplasia
Usually 21-alpha-hydroxylase deficiency, causing 17-hydroxyprogesterone to build up (gets shunted down androgen pathway). Also don’t make cortisol or mineralocorticoids (adrenal insufficiency - hypotension, etc, and salt wasting)
If female, can present with ambiguous genitalia at birth or have late onset virilization
Other variations of CAH
Can also be 11-beta hydroxylase (precursor builds up with mineralocorticoid activity, so hypertensive) or 3B-HSD deficiency too.
Work up for congenital adrenal hyperplasia
Always check 17-OHP level
Can confirm with ACTH stim → check 17OHP 1h later (big rise =CAH)
Lower elevations can be c/w late-onset CAH or heterozygotes
Treatment for congenital adrenal hyperplasia
Can suppress adrenal production with prednisone 5mg every night at bedtime
Ovarian disorders: nonneoplastic
PCOS
Theca lutein cysts
Stromal hyperplasia/hyperthecosis
Theca lutein cysts
LH → theca cells → androgens → granulosa cells → estrogens normally
These cysts make too many androgens!
A/w molar pregnancy.
Dx with Bx
Stromal hyperplasia / hyperthecosis
Pts age 50-70, uniformly enlarged ovaries, large & fleshy
Areas of high utilization inside hyperplastic stroma
Can generally treat nonneoplastic ovarian disorders with
OCPs, which suppress LH/FSH and increase SHBG
GnRH agonists + add-back estrogen are another option
Ovarian disorders: neoplastic
Functional tumors - Sertoli-Leydig (arrhenoblastoma)
Granulosa-theca cell tumors
Luteomas