Lichen sclerosus

Abstract

Lichen sclerosus is a progressive, inflammatory dermatosis characterized with porcelain white sclerotic plaques most commonly in the anogenital area of postmenopausal women. Children and men can also be affected. Extragenital involvement may occur. Even though the etiology is unknown; autoimmune, genetic, hormonal and infectious factors are suggested. Lichen sclerosus is associated with autoimmune diseases such as thyroid disease, alopecia areata, vitiligo, diabetes and pernicious anemia. An increased incidence of autoantibodies to the extracellular matrix protein 1 is shown in sera of patients with lichen sclerosus. Familial occurence and an association with HLA DQ7 supports the presence of genetic factors in its etiology. However, the role of estrogen and androgens could not be demonstrated as etiologic factors. Clinical similarity with acrodermatitis chronica atrophicans led researches to be held for the detection of Borrelia burgdorferi antibodies ended with conflicting results. Disease may be triggered with trauma in the presence of genetic predisposition. Pruritus is the main symptom. Pain, burning and dysesthesia may occur. Lichen sclerosus may be confused with sexual abuse in girls. Furthermore, it is more severe in the presence of sexual abuse. If not treated it leads to permanent anatomical disorders in the anogenital region. It is one of the most common cause of acquired phimosis in boys. Meatal stenosis and urinary obstruction may develop. Erectile dysfunction and dyspareunia due to introital stenosis may cause psychosexual problems in men and women. Perianal involvement causes constipation in women and girls. Long-standing anogenital lesions have the risk of developing squamous cell carcinoma. Verrucous carcinoma rarely occurs. Extragenital symptoms do not have similar risks. Ultra-potent topical corticosteroids are used as the first line therapy. In the case of unresponsiveness to corticosteroids the alternative approaches are topical immunmodulators,UVA1, calcipotriol, retinoids, and photodynamic therapy. In the presence of neoplasia and other complications surgical interventions are considered. Due to mainly preputial involvement, circumcision is an alternative procedure to topical corticosteroids in men. Often it may be curative. Lichen sclerosus is a disease with serious complications that needs life-long follow-up. Patients should be trained and encouraged for effective participation to the treatment and follow-up period.