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
Functional tumors - Sertoli-Leydig (arrhenoblastoma)
Make androgens, usually in young women
If rapid onset over months, be concerned for Sertoli-Leydig cell tumors (commonly diagnosed in women between the ages of 20-40, and are most often unilateral).
T elevated, LH/FSH suppressed
Granulosa-theca cell tumors
Generally make estrogens but may make androgens too
Luteomas
Benign tumor growing in response to b-HCG → virilization of pt, fetus!
Seen in pregnancy
Drugs that can cause virilization
Drugs: steroids, minoxidil, phenytoin, diazoxinde, cyclosporin
Idiopathic hirsutism
Can try finasteride (inhibits 5-alpha reductase) or sprionolactone (antiandrogen)
These in addition to OCPs
Lupron / Depo-provera are also reasonable 2nd line if not on OCPs
Hair loss postpartum
High estrogen levels in pregnancy cause increased synchronicity of hair growth, so that there can be significant alopecia afterwards (all hair in same phase, falls out at same time) - nothing to worry about.