Introduction

  • Description: Lichen sclerosus is a chronic condition of the vulvar skin that is characterized by thinning, distinctive skin changes, and inflammation. It is non-neoplastic and involves glabrous skin and the vulva. The term lichen sclerosus et atrophicus has been dropped because the epithelium is metabolically active and not atrophic. At one time, the condition was also referred to as kraurosis vulvae.

  • Prevalence: Common.

  • Predominant Age: Late reproductive to early menopausal (however, it may be seen as early as 6 months).

  • Genetics: No genetic pattern. Familial aggregations have been reported.

Etiology and Pathogenesis

  • Causes: Unknown. Proposed—immunologic (autoimmune), genetic, inactive or deficient androgen receptors, epidermal growth factor deficiency.

  • Risk Factors: None known.

Signs and Symptoms

  • Intense itching common (99%)

  • Dysuria

  • Anal discomfort

  • Dyspareunia

  • Thinned, atrophic-appearing skin, with linear scratch marks or fissures (the skin often has a “cigarette-paper” or parchment-like appearance). These changes frequently extend around the anus in a figure-eight configuration.

  • Perianal involvement can create the classic figure-eight or hourglass shape.

  • Atrophic changes result in thinning or even loss of the labia minora and significant narrowing of the introitus.

  • Fissures, scarring, and synechiae cause marked pain for some patients.

  • Extragenital lesions reported in up to 13% of women with vulvar disease.

Diagnostic Approach

Differential Diagnosis

  • Lichen simplex chronicus (hyperplastic vulvar dystrophy)

  • Scleroderma

  • Vitiligo

  • Paget’s disease

  • Vulvar candidiasis

  • Squamous cell hyperplasia or carcinoma (when thickening is present)

  • Associated Conditions: Vulvodynia, hypothyroidism, vulvar squamous cancer (5% lifetime risk). Vaginal involvement is uncommon.

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