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 often occurs in postmenopausal women. Although the condition is benign, it is associated with an increased risk of squamous cell carcinoma. Lichen sclerosus is diagnosed clinically and should be confirmed via punch biopsy in adults, which can concurrently screen for squamous cell carcinoma. Treatment primarily consists of superpotent topical steroids but may also include surgical excision in steroid-refractory disease.

• Prevalence: rare disease (occurs in < 2% of the female population) ^[1]
• Sex: most commonly affects (perimenopausal and postmenopausal) women ^[1]
• Mean age of onset: : 52 years and, less commonly, prepubertal girls (7–8 years of age) ^[1]

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]

• Early disease ^[2]
  • Papules and plaques that are white, polygonal, well-demarcated, and potentially surrounded by a red inflammatory halo
  • Dominant symptom: severe pruritus, possibly pain/soreness
  • Most commonly affects the anogenital area
    • Women
      • Figure-eight appearance involving the vulva and perianal area
      • May be associated with dyspareunia and dysuria
    • Men: lesions on the glans penis potentially associated with phimosis and dysuria
    • Both: anal lesions may be associated with anal fissure and/or painful defecation
  • Extragenital lesions: oral and areas of skin (e.g., back, shoulders, neck, wrists, thighs, and under the breast)
• Advanced disease: ulceration, hemorrhage, lichenification, skin thinning/fragility, and erosive scarring

• Diagnosis based on clinical presentation ^[3]
• Punch biopsy is recommended in adult patients
  • Confirms diagnosis and rules out squamous cell carcinoma
  • Shows epidermal atrophy with hyperkeratosis and/or dermal fibrosis and sclerosis ^[4]

• Vaginal infections
• Lichen planus
• Atrophic vaginitis
• Psoriasis
• Chronic cutaneous lupus erythematosus
• Lichen simplex chronicus
• Pityriasis rosea
• Lichenoid mycosis fungoides
• Tinea
• Candidiasis

The differential diagnoses listed here are not exhaustive.

• First-line: superpotent topical steroids (clobetasol, sometimes betamethasone)
• Second-line: topical calcineurin inhibitors (e.g., tacrolimus) ^[3]
• If necessary, surgical excision

• Benign condition
• Increased risk of squamous cell carcinomas, e.g., vulvar carcinoma ^[5]
• Destructive scarring (steroids may prevent) ^[6]

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