Lichen sclerosus

Lichen sclerosus is a chronic inflammatory disease of unknown cause that is characterized by white, atrophic plaques with intense pruritus affecting the skin, nails, hair, and/or mucous membranes. It most commonly affects the anogenital area and women. Lichen sclerosus is diagnosed clinically. Biopsy is recommended in adult patients and in children with atypical lesions. First-line treatment is potent or superpotent topical steroids. Alternative treatments include intralesional corticosteroids, other topical pharmacotherapies (e.g., calcineurin inhibitors, retinoids), phototherapy, laser therapy, and surgery. Although the condition is benign, it is associated with an increased risk of squamous cell carcinoma (SCC) and requires lifelong monitoring.

• Prevalence: rare disease (occurs in < 2% of the female population) ^[1]
• Sex
  • Females > males
  • Most commonly affects (perimenopausal and postmenopausal) women ^[1]
• Mean age of onset: bimodal age distribution (possibly due to lower estrogen levels)
  • Prior to puberty (mean of 5 years)
  • During perimenopause/menopause (mean of 52 years)

Epidemiological data refers to the US, unless otherwise specified.

• Unknown
• Factors such as genetic predisposition, autoimmunity, hormonal changes are thought to play a role. ^[1]

General features

• Description: well-demarcated, white papules and plaques potentially with a surrounding red inflammatory halo
• Locations
  • Anogenital (most common); in children may be confused with child sexual abuse ^[3][4][5]
  • Extragenital, e.g., oral, back, shoulders, neck, wrists, thighs, and under the breast (rare in children) ^[4]
• Associated symptoms: : severe pruritus, possibly pain/soreness
• Advanced disease: ulceration, hemorrhage, lichenification, skin thinning/fragility, and erosive scarring

Anogenital involvement ^[2]

• Female genitalia
  • Figure-eight appearance involving the vulva and perianal area
  • Atrophic, wrinkled appearance ^[6][7][8]
  • May be associated with dyspareunia, dysuria, labial adhesions, clitoral phimosis, and narrowed introitus ^[6]
• Male genitalia
  • Lesions on the glans penis
  • May be associated with phimosis and dysuria (see “Balanitis xerotica obliterans”)
• Anal lesions may be associated with
  • Anal fissures
  • Painful defecation with secondary constipation (especially in children)

• Diagnosis is usually clinical. ^[6]
• Skin biopsy (e.g., punch biopsy, shave biopsy)
  • Indications
    • All adults: to evaluate for SCC (e.g., vulvar carcinoma) and exclude differential diagnoses ^[6]
    • Diagnostic uncertainty: e.g., children with atypical lesions
  • Findings: epidermal atrophy, hyperkeratosis, dermal fibrosis, and sclerosis ^[9]

Biopsy is not usually recommended for pediatric patients with classic genital lesions. ^[3][4][6]

• Vaginal infections, e.g., vaginal candidiasis
• Lichen planus
• Atrophic vaginitis
• Psoriasis
• Chronic cutaneous lupus erythematosus
• Lichen simplex chronicus
• Pityriasis rosea
• Lichenoid mycosis fungoides
• Tinea
• Child sexual abuse ^[3]

The differential diagnoses listed here are not exhaustive.

Approach ^[4][6][10]

• Initiate induction therapy.
• Reassess in 1–3 months and titrate medications. ^[4][4][6]
• Following remission (typically achieved in 3–6 months), start maintenance therapy. ^[4][6]
• For persistent or worsening disease, refer to specialists (e.g., dermatology, gynecology, urology) for
  • Biopsy of new or changing lesions to rule out malignancy ^[4][6]
  • Consideration of advanced treatments ^[6]

Induction therapy ^[4][6][10]

There is no consensus regarding the induction of lichen sclerosus. Reevaluate every 1–3 months and titrate medications based on clinical response. ^[4][6][10]

• First-line: Initiate a high-potency or ultra-high potency topical steroid ointment. ; ^[3][4][6]
  • Clobetasol propionate 0.05% ^[4][6][10]
  • Betamethasone dipropionate 0.05% ^[4]
  • Mometasone furoate 0.1% ^[4]
• Second-line options ^[6]
  • Intralesional corticosteroid injections
  • Topical calcineurin inhibitors (with caution): tacrolimus, pimecrolimus

Ointments are preferred for their tolerance, efficacy, and barrier effects. ^[4]

Maintenance therapy ^[4][6][10]

There is no consensus regarding the maintenance of lichen sclerosus. Titrate based on clinical response. ^[4]

• Continue topical medications (e.g., steroids) at the lowest effective dose to maintain remission. ^[4][6]
• Add emollients. ^[7][8]
• Monitor for the development of adverse effects (e.g., atrophy) and complications. ^[4][6][10]

Although lichen sclerosus is a benign condition, continue lifelong maintenance therapy to maintain remission and monitor for SCC, e.g., vulvar carcinoma. ^[4][6][10]

Advanced treatments ^[6][10]

• Topical or oral retinoids
• Oral immunomodulators: cyclosporine, methotrexate
• Laser therapy or phototherapy
• Surgery in patients with adhesions, phimosis (penile or clitoral), or malignancy ^[4][5]

Vulvectomy is not indicated for the treatment of lichen sclerosus. ^[4]

• Increased risk of SCC, e.g., vulvar carcinoma ^[6]
• Destructive scarring (may be prevented by steroids) ^[6]

We list the most important complications. The selection is not exhaustive.