16 - 90 - HIRSUTISM AND HYPERTRICHOSIS

Question 1

defined as terminal body hair growth in women in a male distribution

Answer 1

Hirsutism

Question 2

what score of Ferriman-Gallwey is considered hirsute?

Answer 2

8 or higher

Question 3

excess hair has a more generalized distribution and independent of androgens

Answer 3

hypertrichosis

Question 4

most potent androgen

Answer 4

dihydrotestosterone (DHT)

DHT is produced from the peripheral enzymatic conversion of testosterone by 5α-reductase

Question 5

cutaneous signs of hyperandrogenism

Answer 5

hirsutism, acne, acanthosis nigricans, androgenetic alopecia, seborrheic dermatitis, and signs of virilization

Signs of virilization include clitoromegaly, male pattern balding, deepening voice, or decreased breast size

Question 6

most commonly involved areas in hirsutism

Answer 6

**upper lip **> thighs > lower abdomen > upper back

Question 7

this scale is a systematic assessment tool for the degree of hirsutism of nine body locations, which when combined, allow for an overall diagnosis of hirsutism

Answer 7

modified Ferriman and Gallwey (mFG) scoring

• It assigns a score of 1 to 4 in nine body areas, with an increasing numeric score corresponding to greater hair density
• The score varies from 0 (no hair) to 4 (extensive hair growth) in each area

Question 8

Normal hair growth is defined by a modified Ferriman and Gallwey (mFG) score of?

Answer 8

8 or less

Question 9

what is the modified Ferriman and Gallwey (mFG) score of mild hirsutism

Answer 9

8 - 14

Question 10

modified Ferriman and Gallwey (mFG) score of greater than 15 indicates what severity of hirsutism?

Answer 10

moderate to severe hirsutism

Question 11

Question 12

most common associated cause of hyperandrogenism in women of reproductive age and is a common secondary cause of hirsutism

Answer 12

Polycystic ovarian syndrome, also known as SteinLeventhal syndrome

Question 13

SAHA syndrome

Answer 13

seborrhea, acne, hirsutism, and androgenetic alopecia

Question 14

HAIR-AN

Answer 14

hyperandrogenism (HA), insulin resistance (IR), and acanthosis nigricans (AN)

Question 15

biochemical hallmark of Adrenal hyperplasia

Answer 15

elevated 17-hydroxyprogesterone (17-OHP), substrate of 21-hydroxylase

Question 16

Question 17

the only one prescription medication that is approved for the treatment of unwanted facial hair

Answer 17

topical eflornithine

• shown to slow down the hair growth cycle and can be used in combination with other hair removal techniques.
• Eflornithine works through inhibiting ornithine decarboxylase, which results in a shortening of the anagen phase of the hair growth cycle.
• The major adverse effects of topical eflornithine are burning or tingling of the treated area.

Question 18

Question 18

First-line medical therapy of hirsutism

Answer 18

oral contraceptives (OCPs)

usually in combination with antiandrogens, such as spironolactone and flutamide

Question 18

the only hair removal methods that may provide permanent hair reduction or long-lasting results

Answer 18

Electrolysis and laser hair reduction

• Electrolysis uses a fine needle insertion into individual follicles to destroy the hair follicle via current (galvanic electrolysis), a high-frequency alternating current (thermolysis), or a blend of the two
• Electrolysis is the favored method of treatment for lightly pigmented hair because only anagen hair follicles with dark bulb areas can be destroyed by the light source of laser.

Question 19

defined as hair growth that is excessive in a localized or diffuse pattern not considered to be in a male pattern distribution

Answer 19

Hypertrichosis

Question 20

rare, inherited disorder in which an excess of lanugo hair remains over the entire body after birth or may develop in the first few months of life.

Answer 20

Congenital hypertrichosis lanuginosa, at times controversially referred to as Ambras syndrome

• Only the palms, soles, mucous membranes, and glans penis are spared.
• Some of these patients lose the hair during childhood, but others retain the excess body hair.

Question 21

Question 22

similar to CHL in presentation but with terminal hair growth on the body instead of lanugo hair.

Answer 22

Congenital generalized hypertrichosis

Question 23

autosomal dominant disorder characterized by newborns with thick scalp hair and excessive hair growth on the **forehead, face, back, and extremities**

Answer 23

Osteochondrodysplasia with hypertrichosis (Cantú syndrome) ## Footnote * The hypertrichosis usually persists over time. Associated findings include skeletal abnormalities and a variety of cardiac abnormalities. * The diagnosis is established based on clinical findings and confirmed by detection of a heterozygous pathogenic variant in ABCC9 or KCNJ8.

Question 24

genetic syndrome characterized by severe overgrowth of the **hair and gums**

Answer 24

Gingival fibromatosis with hypertrichosis

Question 25

most commonly reported syndromes in which **hypertrichosis** is a secondary characteristic

Answer 25

Cornelia de Lange syndrome, also known as Brachmann-de Lange syndrome ## Footnote * t is characterized by severe mental retardation, cutis marmorata, frequent limb abnormalities, and characteristic facial features (micrognathia, high palate, low frontal hair line) with the hypertrichosis most prominent as thickened convergent (synophrys) eyebrows with hypertrichotic eyelashes