lichen sclerosus

Question 1

epidemiology

Answer 1

white females, ages 8-13 and 40-50

Question 2

common associations

Answer 2

thyroid disease; HLA-DQ7

Question 3

what is the targets AutoAb

Answer 3

ECM-1

**mutated in lipoid proteinosis

Question 4

skin findings

Answer 4

• can be genital>extra genital location
• sclerotic, ivory white atrophic, figure 8 +/- follicular plugging
• c/o constipation in kids
• c/o dysuria, dyspareunia

Question 5

complications

Answer 5

SCC (5% risk); phimosis in men; fusion of labia majora/minora, loss of clitoral hood, narrowing of vaginal opening

Question 6

Tx

Answer 6

topical steroids (ultra potent, clobetasol and mometasone)&raquo_space;> topical CI (may have increased risk of SCC or HPV reactivation)

In refractory cases, think PUVA/UVA or systemic immunosuppressants

Question 7

pathology

Answer 7

red, white and blue sign

red - orthohyperkeratotic stratum corneum
white - edematous papillary dermis
blue = band like lymph infiltrate

Question 8

may resolve in childhood, but may have relapsing course in adults

Answer 8

fact

Question 9

there is a hormonal component particularly in women

Answer 9

better in pregnancy, worse after menopause and with OCP use