Lichen sclerosus is a chronic inflammatory skin disease affecting children and adults, mostly women, leading to atrophy of the epidermis, fibrosis and scarring, often accompanied by pruritus. It most often affects the anogenital area, where it causes functional impairment and is associated with an increased risk of vulvar skin cancer, however it can also occur in other localizations.
The pathogenesis of the disease is unclear. Various factors are considered, including genetic predisposition and local irritation.
The autoimmune nature of the disease, mediated by Th1 cell cytokines, accompanied by the increased expression of microRNA-155 and the production of antibodies to extracellular matrix glycoprotein 1, is most commonly proposed. The diagnosis is usually clinical, biopsy is mostly not necessary.
The treatment of choice are strong topical corticosteroids (clobetasol), circumcision is often curative in men. For more extensive skin involvement, application of phototherapy is possible (especially UVA1, less UVB311), rarely systemic therapy with methotrexate and corticosteroids is used.
Due to the chronic nature of the disease and the possible development of malignancy, long-term fol-low-up of patients is recommended.