Review patients with anogenital lichen sclerosus (LS), with or without extragenital LS, initially every 1 to 6 months until the condition has stabilized. Once stable, review every 6 to 12 months to ensure disease control and to evaluate for malignant transformation.[56]Kirtschig G, Kinberger M, Kreuter A, et al; European Dermatology Forum. EuroGuiDerm guideline on lichen sclerosus. Jun 2023 [internet publication].
https://www.guidelines.edf.one//uploads/attachments/clmub3q4l0an5uhjrluc4r0yq-lichen-sclerosus-gl.pdf
[84]Lee A, Bradford J, Fischer G. Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women. JAMA Dermatol. 2015 Oct;151(10):1061-7.
https://www.doi.org/10.1001/jamadermatol.2015.0643
http://www.ncbi.nlm.nih.gov/pubmed/26070005?tool=bestpractice.com
When symptom control has been achieved in patients with only extragenital disease, give advice to self-monitor for the development of anogenital disease.
Closely inspect the skin for texture or color change; any evidence of this should prompt consideration of flares of LS or signs of development of differentiated vulvar intraepithelial neoplasia (dVIN) - a precursor for squamous cell carcinoma (SCC). Particularly look for any nonhealing hyperkeratotic areas, erosions, or firm papules/nodules, which should be considered suspicious for dVIN. If lesions persist despite therapy or progress, biopsy is indicated.
Bear in mind that there is a risk of neoplastic change (occurs in approximately 5% of women with anogenital LS).[84]Lee A, Bradford J, Fischer G. Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women. JAMA Dermatol. 2015 Oct;151(10):1061-7.
https://www.doi.org/10.1001/jamadermatol.2015.0643
http://www.ncbi.nlm.nih.gov/pubmed/26070005?tool=bestpractice.com
Risk can be minimized by good disease control. One longitudinal cohort study of 507 women with vulvar LS found a significant difference in the number of cases of SCC in the 357 treatment-compliant patients who attended regular follow-up (0 cases) versus 7 cases of SCC in the non-compliant/partially compliant patients.[84]Lee A, Bradford J, Fischer G. Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women. JAMA Dermatol. 2015 Oct;151(10):1061-7.
https://www.doi.org/10.1001/jamadermatol.2015.0643
http://www.ncbi.nlm.nih.gov/pubmed/26070005?tool=bestpractice.com
As LS is known to be associated with autoimmune diseases, some experts recommend screening for associated autoimmune diseases: for example, with an autoantibody screen and assessment of thyroid status.[56]Kirtschig G, Kinberger M, Kreuter A, et al; European Dermatology Forum. EuroGuiDerm guideline on lichen sclerosus. Jun 2023 [internet publication].
https://www.guidelines.edf.one//uploads/attachments/clmub3q4l0an5uhjrluc4r0yq-lichen-sclerosus-gl.pdf
Studies have shown that LS in children may have a more chronic course than previously thought.[90]Boero V, Cavalli R, Caia C, et al. Pediatric vulvar lichen sclerosus: does it resolve or does it persist after menarche? Pediatr Dermatol. 2023 May-Jun;40(3):472-5.
https://www.doi.org/10.1111/pde.15308
http://www.ncbi.nlm.nih.gov/pubmed/36998082?tool=bestpractice.com
[106]Morrel B, van der Avoort IAM, Ewing-Graham PC, et al. Long-term consequences of juvenile vulvar lichen sclerosus: a cohort study of adults with a histologically confirmed diagnosis in childhood or adolescence. Acta Obstet Gynecol Scand. 2023 Nov;102(11):1469-78.
https://www.doi.org/10.1111/aogs.14668
http://www.ncbi.nlm.nih.gov/pubmed/37632250?tool=bestpractice.com
One observational retrospective study of 31 premenarchal girls diagnosed with LS who were followed up for clinical evaluation following menarche found that 58% still had symptomatic disease and a further 26% had persistent clinical signs of LS despite being asymptomatic.[90]Boero V, Cavalli R, Caia C, et al. Pediatric vulvar lichen sclerosus: does it resolve or does it persist after menarche? Pediatr Dermatol. 2023 May-Jun;40(3):472-5.
https://www.doi.org/10.1111/pde.15308
http://www.ncbi.nlm.nih.gov/pubmed/36998082?tool=bestpractice.com
These findings suggest the importance of long-term follow-up, even among patients who report resolution of symptoms following menarche; therefore, pediatric patients should also be monitored and maintenance therapy considered in select populations depending on disease control and severity.[90]Boero V, Cavalli R, Caia C, et al. Pediatric vulvar lichen sclerosus: does it resolve or does it persist after menarche? Pediatr Dermatol. 2023 May-Jun;40(3):472-5.
https://www.doi.org/10.1111/pde.15308
http://www.ncbi.nlm.nih.gov/pubmed/36998082?tool=bestpractice.com