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- Lichen sclerosus (LS) is an uncommon chronic inflammatory mucocutaneous condition. LS shows a predilection for the anogenital area, most commonly affecting the vulva in women and the glans and foreskin in men.
- Extragenital lesions constitute about 15–20% of all cases of LS and usually involve the neck, shoulders, arms, and upper trunk. Rarely, other sites may be affected such as the oral mucosa, plantar surface, and nails.
LS is a rare entity. Occurs at all ages and in both genders, but a higher incidence is present in postmenopausal women. The male-to-female ratio varies between 1:3 and 1:10.
The exact prevalence is unknown but is estimated to be between 0.1% and 0.3% among patients referred to a community-based dermatology department.
Etiology and Pathophysiology
- The etiology of LS is unknown. Some studies have shown a link between autoimmune diseases and LS in women, whereas this association in men has not been analyzed to the same extent.
- A recent study analyzed serum autoantibody profiles in patients with LS and compared them to healthy controls. Autoantibodies directed against specific skin protein and molecules such as extracellular matrix protein 1 (ECM1) were demonstrated in most patients with LS, making ECM1 a tentative target for autoimmune mechanisms in LS.
- Additionally, an autoimmune phenotype involving immunologic changes on the level of T and B cells and increased levels of TH1-specific cytokines with resultant dense T-cell infiltration and enhanced BIC/miR-155 expression has been observed.
About 10% of patients with LS have relatives with the same condition. Nevertheless, the pathogenetic relevance of these findings is uncertain. Oxidative changes in the DNA and TP53 mutations (tumor suppressor gene) have also been described, and the chromosome involved is the 17p13.1.
Menopause, trauma (due to chronic friction and scratching), urogenital surgical procedures or sexual abuse during childhood, and infections (vulvitis/urethritis) are considered risk factors for the development of LS.
No clear preventive methods are reported in the current guideline; however, early aggressive treatment may prevent disease progression.
Commonly Associated Conditions
20–30% of women with LS also have autoimmune diseases. Thyroid dysfunction (12–16%); inflammatory bowel diseases, pernicious anemia, alopecia areata, rheumatoid arthritis, morphea, and scleroderma have been associated with LS in women, whereas in men, conditions such as diabetes mellitus and atopic dermatitis commonly coexist with LS.