What are the symptoms and treatment options for lichen sclerosis?

Lichen Sclerosus: Clinical Manifestations and Complications

Primary Symptoms

Lichen sclerosus most commonly causes intractable pruritus (itching) in the anogenital region, often severe enough to disturb sleep, along with pain from erosions and fissures. 1

In Adult Women

• Intense itching that worsens at night and disrupts sleep 1
• Dyspareunia (painful intercourse) resulting from erosions, fissures, or introital narrowing 1
• Dysuria from fissuring in the genital area 1
• Urinary symptoms, though these may be less common than in the general population 1
• Some patients remain asymptomatic with incidental findings on examination 1

In Children (Girls)

• Striking ecchymosis (bruising) that can be mistaken for sexual abuse, though the two are not mutually exclusive 1
• Constipation from painful perianal fissuring 1
• Dysuria from genital fissures 1
• Perianal involvement is frequent in young girls 1

In Adult Men

• Difficulty with sexual intercourse (male dyspareunia) 1
• Phimosis and paraphimosis from constrictive posthitis 1
• Painful erections due to foreskin tightening 1
• Poor urinary stream and dysuria from meatal involvement 1
• Pruritus is not a prominent symptom in men, unlike women 1
• Perianal disease is extremely rare in males 1, 2

Physical Findings and Scarring Complications

Characteristic Appearance

• Porcelain-white papules and plaques with areas of ecchymosis 1, 2
• Fragile, thinned, and atrophic skin prone to fissures and tears 1
• In men: grayish-white discoloration affecting glans, foreskin, and frenulum 1, 2

Progressive Scarring in Women

• Loss of labia minora 1
• Sealing of the clitoral hood and burying of the clitoris 1
• Introital stenosis (narrowing of vaginal opening), though severe cases are rare except in LS/LP overlap syndrome 1
• Labial fusion 3

Progressive Scarring in Men

• Severe phimosis with nonretractile foreskin 1, 2
• Meatal stenosis from perimeatal scarring 1, 2
• Urethral stricture disease along the pendulous urethra in 20% of cases 1, 2
• Erectile dysfunction from skin inelasticity 1, 2

Malignant Transformation Risk

Lichen sclerosus carries a 4-6% risk of progression to squamous cell carcinoma, predominantly in the anogenital region, making long-term surveillance essential. 1, 4, 5

• Malignancy risk is highest in female genital LS 1
• Less common in penile LS, though histological evidence of LS is found in approximately 40% of penile carcinoma specimens 2
• Not associated with extragenital LS 1
• Malignancy has not been reported in prepubertal girls 1

Surveillance Indicators

• Persistent areas of hyperkeratosis, erosion, or erythema 1
• New warty or papular lesions 1
• Any non-healing lesions within affected skin 3
• Disease that fails to respond to adequate treatment 1

Urological Complications (Males)

• Urinary obstruction from meatal stenosis 1, 2
• Microincontinence from dysfunctional naviculomeatal fossa 1
• Urethral involvement typically starts at the meatus and may extend proximally 1
• Rare complication of renal failure following meatal obstruction has been reported in children 1

Treatment Approach

Ultra-potent topical corticosteroids (clobetasol propionate 0.05%) applied twice daily for 2-3 months represent first-line therapy for all ages and both sexes. 3, 6, 7

Key Treatment Principles

• Asymptomatic patients with clinically active disease (ecchymosis, hyperkeratosis, progressing atrophy) should still be treated 1
• Treatment aims to: relieve symptoms, prevent anatomical changes, and theoretically reduce malignancy risk 4
• Topical steroids can be safely continued during pregnancy and postpartum 1
• After initial therapy, maintenance with twice-weekly application may be needed 4

Surgical Indications

• Surgery has no place in uncomplicated LS 4
• Reserved for: malignancy treatment, correction of scarring complications, and severe irreversible phimosis in males 1
• In men, circumcision is effective in the majority of cases, but recurrences are well-described 5
• Circumcision should not be performed without adequate trial of topical steroids first 1

Important Clinical Pitfalls

Misdiagnosis Concerns

• In children, striking ecchymosis may mimic sexual abuse, but LS can also be triggered by trauma through Koebnerization 1
• Suspicious features for abuse include: LS in older prepubertal girls, poor treatment response, associated STIs, or other abuse signs 1

Treatment Failures

• Consider secondary problems: contact allergy to medication, urinary incontinence, herpes simplex, intraepithelial neoplasia, malignancy, psoriasis, or mucous membrane pemphigoid 1
• Evaluate for secondary sensory problems (vulvodynia) that persist after successful LS treatment 1
• Assess for mechanical problems from scarring requiring surgical intervention 1

Follow-up Requirements

• Long-term follow-up is essential due to malignancy risk 4, 7
• Patients using <60g of topical corticosteroid in 12 months with well-controlled disease do not require specialized clinic follow-up 1
• Secondary care follow-up is reserved for complicated, unresponsive disease or history of prior squamous cell carcinoma 1