Lichen sclerosus

​Lichen sclerosus (LS, also known as balanitis xerotica obliterans in men) is an inflammatory skin condition with a chronic relapsing and remitting course.

Characteristic findings on examination of the cutaneous lesions are atrophic white plaques, typically found in the anogenital area, which may lead to scarring and anatomical changes.

There is a small but significant risk (approximately 5%) of vulvar squamous cell carcinoma in women with anogenital LS, especially if left untreated.

If extragenital disease is found, genital examination is recommended, as extragenital disease in isolation is rare.

The condition is often associated with autoimmune conditions (such as thyroid disease and vitiligo) in women.

Diagnosis is predominantly clinical, but confirmation by biopsy is recommended if there is diagnostic uncertainty or suspicion of malignancy.

First-line treatment for all patients with anogenital LS is a very-high potency corticosteroid. Initial management comprises daily therapy until the disease is controlled. In men and boys with genital LS, early circumcision is often curative.

Treatment of extragenital LS should be overseen by a specialist who will individualise the approach according to the site involved, typically only initiating therapy if symptoms are bothersome to the patient.

Once disease control is achieved, maintenance therapy (usually with a topical corticosteroid) may be required for the prevention of anogenital disease flares in particular.

​Lichen sclerosus (LS) is a chronic inflammatory skin condition. LS typically affects the genitalia but can also manifest as extragenital lesions.[1]Wallace HJ. Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc. 1971;57(1):9-30. http://www.ncbi.nlm.nih.gov/pubmed/5570266?tool=bestpractice.com [2]Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet. 1999 May 22;353(9166):1777-83. http://www.ncbi.nlm.nih.gov/pubmed/10348006?tool=bestpractice.com ​​ In women, it predominantly affects the vulva and anus and is associated with pruritus, pain, and dyspareunia; it can also lead to scarring and increases the risk of malignancy.[3]Sheinis M, Selk A. Development of the Adult Vulvar Lichen Sclerosus Severity Scale - a Delphi consensus exercise for item generation. J Low Genit Tract Dis. 2018 Jan;22(1):66-73. https://www.doi.org/10.1097/LGT.0000000000000361 http://www.ncbi.nlm.nih.gov/pubmed/29095746?tool=bestpractice.com [4]Micheletti L, Preti M, Radici G, et al. Vulvar lichen sclerosus and neoplastic transformation: a retrospective study of 976 cases. J Low Genit Tract Dis. 2016 Apr;20(2):180-3. http://www.ncbi.nlm.nih.gov/pubmed/26882123?tool=bestpractice.com [5]Vieira-Baptista P, Pérez-López FR, López-Baena MT, et al. Risk of development of vulvar cancer in women with lichen sclerosus or lichen planus: a systematic review. J Low Genit Tract Dis. 2022 Jul 1;26(3):250-7. http://www.ncbi.nlm.nih.gov/pubmed/35285455?tool=bestpractice.com ​ In men, genital disease almost exclusively occurs in those who are uncircumcised and may cause pain, dyspareunia, and scarring-related signs and symptoms such as changes in urinary stream and phimosis.[6]Shah M, van Bodegraven B. Male genital lichen sclerosus and associated symptoms range and severity: results of a questionnaire study. Skin Health Dis. 2023 Oct;3(5):e246. https://www.doi.org/10.1002/ski2.246 http://www.ncbi.nlm.nih.gov/pubmed/37799358?tool=bestpractice.com ​ Girls may present with similar symptoms as women; however, boys with the condition are usually asymptomatic and often present with phimosis.[7]Simms-Cendan J, Hoover K, Marathe K, et al. NASPAG clinical opinion: diagnosis and management of lichen sclerosis in pediatric and adolescent patients. J Pediatr Adolesc Gynecol. 2022 Apr;35(2):112-20. http://www.ncbi.nlm.nih.gov/pubmed/34610442?tool=bestpractice.com [8]Celis S, Reed F, Murphy F, et al. Balanitis xerotica obliterans in children and adolescents: a literature review and clinical series. J Pediatr Urol. 2014 Feb;10(1):34-9. https://www.doi.org/10.1016/j.jpurol.2013.09.027 http://www.ncbi.nlm.nih.gov/pubmed/24295833?tool=bestpractice.com [9]Chalmers RJ, Burton PA, Bennett RF, et al. Lichen sclerosus et atrophicus. A common and distinctive cause of phimosis in boys. Arch Dermatol. 1984 Aug;120(8):1025-7. http://www.ncbi.nlm.nih.gov/pubmed/6465907?tool=bestpractice.com