Lichen sclerosus (LS, LS&A, balanitis xerotica obliterans)

Question 1

Male: female predominance of lichen sclerosus? Whites vs non-white predominance?

Answer 1

• F>>>>M - Whites> non-whites

Question 2

Age of onset of LS?

Answer 2

• bimodal - Majority between 40-50 y/o post menopausal females - other peak is prepubertal girls (8-13 y/o)

Question 3

What other diseases is LS associated with?

Answer 3

• autoimmune conditions (Especially thryroid!!!)

Question 4

Most common region affected in LS?

Answer 4

• male and female anogenital region (85%)

Question 5

LS on the penis is called _____ and is a common cause of. _____

Answer 5

• balanitis xerotica obliterans
• common cause of phimosis (when foreskin cant be retracted)

Question 6

Pathogenesis of LS?

Answer 6

• idiopathic, but thought to be genetically predisposed if HLA-DQ7 positive
• majority of patients have IgG autoantibodies against ECM-1
• hormonal factors (predominance in postmenopausal women, resolves in pregnancy and with OCP use. )

Question 7

Genetic predisposition in LS?

Answer 7

• HLA- DQ7
• also 80% of patients have circulating IgG autoantibodies against ECM-1 (regulates basement membrane zone integrity)

Question 8

Clinical findings and symptoms of lichen sclerosus:

Answer 8

• sclerotic, ivory-white, atrophic, and flat-topped papules coalescing into plaques
• can also see follicular plugging (more common in extra genital LS)
• genital LS is usually symptomatic (itchy, painful, burning), but extragenital is usually asymptomatic

Question 9

Kids with LS who have pain, may experience difficulty with _____ and ____

Answer 9

• urination and defecation (painful when pooping because of sclerosed skin)

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Question 10

What is the clinical evolution of LS that occurs with time?

Answer 10

• starts as well demarcated, thin erythematous plaques–>epidermal atrophy, dermal scarring, hypopigmentation, dermal hemorrhage/bruising and fissures occur–>fusion of labia minora to majora/obliteration of clitoral hood can occur–>narrowing of vaginal introitus

Question 11

Why is LS commonly misdiagnosed as sexual abuse?

Answer 11

• the lesions can have hemorrhage/purpuric/ecchymotic components

Question 12

Patients w/ genital LS are at increased risk for ____

Answer 12

• SCC (5% risk)

Question 13

Histopathology of LS?

Answer 13

• compact orthokeratosis,
• follicular plugging
• epidermal atrophy with mild vacuolar interface changes
• papillary dermal edema or homogenization with underlying lichenoid lymphocytic infiltrate

- “red white and blue sign”- red= compact orthokeratosis

• white = hyalinized/edematous papillary dermis
• blue =band-like lymphocytic infiltrate

Question 14

Treatment of LS?

Answer 14

• be aggressive with high potency topical corticosteroids!! (clobetasol)
• this is safe even if used for long term (1-2 months straight to get it under control)
• second line is TCIs (can use along with TCS)

Question 15

Tx for phimosis caused by LS in males?

Answer 15

• circumcision if uncircumcised

Question 16

Prognosis/clinical course of LS in younger girls? older patients?

Answer 16

• in younger patients good chance to resolve with aggressive treatment within a few years
• older patients tends to be more chronic/relapsing