Hirsutism & Virilism

Question 1

what are the 2 types of hair that adults have?

Answer 1

vellus - non-pig’d, soft, covers entire body

terminal = pig’d, covers scalp, axilla, pubic area. Androgens stim th econversion of vellous to terminal hair.

Question 2

what is an abnormal increase in terminal hair due to?

Answer 2

androgen excess, or increased 5alpha-reductase activity

Question 3

what does 5alpha reductase do?

Answer 3

converts TS to DHT (more potent)

Question 4

what is hirsutism?

Answer 4

increase in terminal hair on face, chest, back, lower abdomen, inner thighs in a woman

Question 5

what is escutcheon?

Answer 5

shape of patch of pubic hair - diamond in a male, triangle in female

Question 6

what is virilization?

Answer 6

dvpt of male features - voice deepening, frontal balding, increased m. mass, clitoromegaly, breast atrophy, male body habitus

Question 7

what enzyme is present in z. glomerulosa but lacking in z. reticularis and z. fasciculata?

Answer 7

aldosterone synthase

Question 8

what enzyme does the z. glomerulosa lack?

Answer 8

17-alpha-hydroxylase (can’t make cort & androgen)

Question 9

elevations and def’ies of which hrms can cause excess androgens?

Answer 9

elevated ACTH

enzymatic blockade of either cort or aldo syn (stuff gets shunted towards DHEAS syn pathway)

Question 10

what are the 2 categories of adrenal disorders that can cause virilization?

Answer 10

neoplastic (more often cause virilization)

non-neoplastic (rarely causes virilization)

Question 11

what are the adrenal disorders that cause virilization?

Answer 11

cushing

CAH

Question 12

how to make dx of Cushings?

Answer 12

overnight DXM suppression test - give DXM at night, then measure cort levels in a.m. Should be low in a normal pt. But if > 10microg/dL, dx of Cushings is made.

Check 24-h urine free cortisol

Question 13

what is most common form of CAH?

Answer 13

21-alpha hydroxylase def’y

Question 14

CP of CAH?

Answer 14

salt wasting (due to lack of aldo)
adrenal insuff’y
ambiguous genitalia at birth (due to excess androgens)

Question 15

CP of “adult-onset” CAH?

Answer 15

mild virilization, menstrual irregularities

Question 16

what labs should be checked when you suspect CAH?

Answer 16

17-alpha-hydroxyprogesterone (if elevated, suggests 21-alpha hydroxylase def’y)

Question 17

what are the non-neoplastic ovarian disorders that can cause virilization?

Answer 17

PCOS
Theca Lutein cysts
stromal hyperplasia & hyperthecosis

Question 18

why does androgen excess occur in PCOS?

Answer 18

excess LH => many ovarian cysts, increased ovarian androgen production

Question 19

what is a theca lutein cyst?

Answer 19

theca cells that produce excess androgens that are sec’d into circl’n instead of shunting them to the granulosa cells for aromatization to estrone & estradiol

Question 20

what is stromal hyperthecosis?

Answer 20

hyperplasia of stroma with foci of androgen production

Question 21

what’s the diff b/w functional and non-functional ovarian tumors?

Answer 21

fct’l tumors produce androgens, nonfct’l ones do not.

Question 22

what are the functional ovarian tumors?

Answer 22

Sertoli-Leydig cell tumors
granulosa-theca cell tumors
hilar (Leydig) cell tumors
germ cell tumors (gonadoblastomas)
luteoma (during pg'y only)

Question 23

how do non-fct’l tumors cause virilization?

Answer 23

the stimulate the surrounding stroma => increased androgens

Question 24

what labs should be ordered in w/u of hirsutism/virilization?

Answer 24

free TS
17-OHP
DHEAS

Question 25

what does rapid-onset virilization & TS > 200 ng/dL suggest?

Answer 25

ovarian ca

Question 26

tx of ovarian causes of hirsutism?

Answer 26

prednisone (suppresses androgen production)
finasteride
spironolactone
surgery if tumor
OCPs, minipill