Lichen Sclerosus

Background

• Lichen sclerosus is a chronic, inflammatory, mucocutaneous condition of unknown etiology that most commonly presents in postmenopausal women as atrophy and whitening of the skin of the vulva.
• Much less commonly, it can occur in men, prepubertal girls, or boys. In males it is almost always associated with an uncircumcised penis.
• It is associated with autoimmune disorders, particularly in women, and may be triggered by localized trauma, such as a skin irritation or surgical instrumentation to the area.
• The condition is typically progressive and may lead to scarring, pain, and dyspareunia, as well as phimosis in men. Long-standing disease is also associated with an increased risk of vulvar intraepithelial neoplasia (VIN) or vulvar squamous cell carcinoma (VSCC) in women or squamous cell carcinoma of the penis in men.

Evaluation

• Females most commonly present with vulvar itching, burning, or pain, while males are more likely to present with difficulty retracting the foreskin, erectile dysfunction due to tightened foreskin, or dysuria. Consider lichen sclerosus in children presenting with dysuria, constipation, or pain and difficulty with defecation.
• A physical exam in women or girls typically shows atrophic, shiny, white to gray or red patches, papules, or plaques circumferentially located around vagina or anus, often described as in a "figure of eight pattern."
• A physical exam in boys and men typically shows thinning or atrophic skin, white-grey plaques, or fissuring of the skin of the prepuce or glans, or phimosis of the foreskin.
• Consider a biopsy to confirm the clinical diagnosis if the diagnosis is uncertain, if there is any suspicion of malignancy, or if the area is resistant to the initial treatment.

Management

• Early diagnosis and treatment even for asymptomatic patients is advised to reduce scarring complications of lichen sclerosus.
• Advise skin care with emollients for soap substitution and moisturization in addition to treatment with topical steroids (Strong recommendation ).
• For initial treatment of symptoms and lesions, advise ultrapotent topical corticosteroids with a tapering course over about 3 months (Strong recommendation).
  • for female patients, use topical clobetasol propionate 0.05% ointment applied topically nightly for 4 weeks, then alternate nights for 4 weeks, then twice weekly for 4 weeks
  • for male patients, use topical clobetasol propionate 0.05% ointment applied topically nightly for 1-3 months
• In male adults or children with phimosis, refer for surgical treatment if a trial of topical corticosteroids fails to clear symptoms (Strong recommendation).
• In female adults and children who respond to topical corticosteroids, use long-term maintenance with ultrapotent corticosteroid twice weekly, or as needed for symptoms (Strong recommendation ). Maintenance treatment appears to reduce risk of scarring complications and squamous cell carcinoma.
• For patients who are unresponsive to 12 weeks of topical corticosteroids, or who are unable to tolerate corticosteroids, consider other diagnoses, including malignancy or infection, and assess for potential local irritants.
• For second-line treatment in female patients unresponsive to topical corticosteroids, consider intralesional steroids to hyperkeratotic areas (Weak recommendation ).
• Systemic medications are usually reserved for refractory cases.
• Consider surgical treatment for complications such as labial fusion, or stenosis due to scarring or adhesions that inhibit urinary or sexual functioning, or for treatment of a coexisting carcinoma (Weak recommendation ).
• There is very limited evidence for the efficacy of phototherapies or plasma-rich platelet injections for symptoms, or for fat grafting and adipose-derived stem cells for vaginal atrophy.
• Lichen sclerosus may follow a chronic, waxing and waning course, and patients should be followed closely to monitor disease activity and to screen for malignant changes.