American Journal of Clinical Dermatology

, Volume 19, Issue 5, pp 695–706 | Cite as

Diagnosis and Treatment of Vulvar Lichen Sclerosus: An Update for Dermatologists

  • Andrew Lee
  • Gayle Fischer
Review Article


Vulvar lichen sclerosus is an important skin disease that is common in women in their 50 s and beyond; however, it can also affect females of any age, including children. If not treated, it has the potential to cause significant and permanent scarring and deformity of the vulvar structure. In addition, if untreated, it is associated with a 2–6% lifetime risk of malignant squamous neoplasia of the vulva. Lichen sclerosus has been considered a difficult to manage condition; however, both serious complications can potentially be prevented with early intervention with topical corticosteroid, suggesting that the course of the disease can be treatment modified.


Compliance with Ethical Standards


No sources of funding were used to prepare this manuscript.

Conflict of interest

Andrew Lee and Gayle Fischer have no conflicts of interest that are directly relevant to the content of this study.


  1. 1.
    Fischer GO. The commonest causes of symptomatic vulvar disease: a dermatologist’s perspective. Australas J Dermatol. 1996;37:12–8.CrossRefPubMedGoogle Scholar
  2. 2.
    Goldstein AT, Marinoff SC, Christopher K, Srodon M. Prevalence of vulvar lichen sclerosus in a general gynecology practice. J Reprod Med. 2005;50:477–80.PubMedGoogle Scholar
  3. 3.
    Bleeker MC, Visser PJ, Overbeek LI, van Beurden M, Berkhof J. Lichen sclerosus: incidence and risk of vulvar squamous cell carcinoma. Cancer Epidemiol Biomarkers Prev. 2016;25:1224–30.CrossRefPubMedGoogle Scholar
  4. 4.
    Tong LX, Sun GS, Teng JM. Pediatric lichen sclerosus: a review of the epidemiology and treatment options. Pediatr Dermatol. 2015;32:593–9.CrossRefPubMedGoogle Scholar
  5. 5.
    Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus: an increasingly common problem. J Am Acad Dermatol. 2001;44:803–6.CrossRefPubMedGoogle Scholar
  6. 6.
    Smith SD, Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol. 2009;26:725–9.CrossRefPubMedGoogle Scholar
  7. 7.
    Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus. The course after puberty. J Reprod Med. 2002;47:706–9.PubMedGoogle Scholar
  8. 8.
    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;20:180–3.CrossRefPubMedGoogle Scholar
  9. 9.
    Lee A, Bradford J, Fischer G. Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women. JAMA Dermatol. 2015;151:1061–7.CrossRefPubMedGoogle Scholar
  10. 10.
    Cooper SM, Ali I, Baldo M, Wojnarowska F. The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease: a case-control study. Arch Dermatol. 2008;144:1432–5.PubMedGoogle Scholar
  11. 11.
    Harrington CI, Dunsmore IR. An investigation into the incidence of auto-immune disorders in patients with lichen sclerosus and atrophicus. Br J Dermatol. 1981;104:563–6.CrossRefPubMedGoogle Scholar
  12. 12.
    Kyriakis KP, Emmanuelides S, Terzoudi S, et al. Gender and age prevalence distributions of morphea en plaque and anogenital lichen sclerosus. J Eur Acad Dermatol Venereol. 2007;21:825–6.CrossRefPubMedGoogle Scholar
  13. 13.
    Hofer MD, Meeks JJ, Mehdiratta N, et al. Lichen sclerosus in men is associated with elevated body mass index, diabetes mellitus, coronary artery disease and smoking. World J Urol. 2014;32:105–8.CrossRefPubMedGoogle Scholar
  14. 14.
    Simpkin S, Oakley A. Clinical review of 202 patients with vulval lichen sclerosus: a possible association with psoriasis. Australas J Dermatol. 2007;48:28–31.CrossRefPubMedGoogle Scholar
  15. 15.
    Jacobs L, Gilliam A, Khavari N, Bass D. Association between lichen sclerosus and celiac disease: a report of three pediatric cases. Pediatr Dermatol. 2014;31:e128–31.CrossRefPubMedGoogle Scholar
  16. 16.
    Chakhtoura Z, Vigoureux S, Courtillot C, et al. Vulvar lichen sclerosus is very frequent in women with Turner syndrome. J Clin Endocrinol Metab. 2014;99:1103–4.CrossRefPubMedGoogle Scholar
  17. 17.
    Oyama N, Chan I, Neill SM, et al. Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet. 2003;362:118–23.CrossRefPubMedGoogle Scholar
  18. 18.
    Birenbaum DL, Young RC. High prevalence of thyroid disease in patients with lichen sclerosus. J Reprod Med. 2007;52:28–30.PubMedGoogle Scholar
  19. 19.
    Sherman V, McPherson T, Baldo M, et al. The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study. J Eur Acad Dermatol Venereol. 2010;24:1031–4.PubMedGoogle Scholar
  20. 20.
    Gao XH, Barnardo MC, Winsey S, et al. The association between HLA DR, DQ antigens, and vulval lichen sclerosus in the UK: HLA DRB112 and its associated DRB112/DQB10301/04/09/010 haplotype confers susceptibility to vulval lichen sclerosus, and HLA DRB10301/04 and its associated DRB10301/04/DQB10201/02/03 haplotype protects from vulval lichen sclerosus. J Invest Dermatol. 2005;125:895–9.CrossRefPubMedGoogle Scholar
  21. 21.
    Gambichler T, Terras S, Kreuter A, Skrygan M. Altered global methylation and hydroxymethylation status in vulvar lichen sclerosus: further support for epigenetic mechanisms. Br J Dermatol. 2014;170:687–93.CrossRefPubMedGoogle Scholar
  22. 22.
    Van de Nieuwenhof HP, Meeuwis KA, Nieboer TE, et al. The effect of vulvar lichen sclerosus on quality of life and sexual functioning. J Psychosom Obstet Gynaecol. 2010;31:279–84.CrossRefPubMedGoogle Scholar
  23. 23.
    Byren I, Venning V, Edwards A. Carcinoma of the vulva and asymptomatic lichen sclerosus. Genitourin Med. 1993;69:323–4.PubMedPubMedCentralGoogle Scholar
  24. 24.
    Zendell K, Edwards L. Lichen sclerosus with vaginal involvement: report of 2 cases and review of the literature. JAMA Dermatol. 2013;149:1199–202.CrossRefPubMedGoogle Scholar
  25. 25.
    Yahiro C, Oka M, Fukunaga A, et al. Mucosal lichen sclerosus/lichen planus overlap syndrome with cutaneous lesions of lichen sclerosus. Eur J Dermatol. 2016;26:204–5.PubMedGoogle Scholar
  26. 26.
    Owen CM, Yell JA. Genital lichen sclerosus associated with incontinence. J Obstet Gynaecol. 2002;22:209–10.CrossRefPubMedGoogle Scholar
  27. 27.
    Borghi A, Corazza M, Minghetti S, et al. Dermoscopic features of vulvar lichen sclerosus in the setting of a prospective cohort of patients: new observations. Dermatology. 2016;232:71–7.CrossRefPubMedGoogle Scholar
  28. 28.
    Dell EA, Miest RYN, Lohse CM, Torgerson RR. Vulvar neoplasms in 275 women with genital lichen sclerosus and impact of treatment: a retrospective chart review. J Eur Acad Dermatol Venereol. 2018. (Epub ahead of print).PubMedCrossRefGoogle Scholar
  29. 29.
    Te Grootenhuis NC, Pouwer AW, de Bock GH, et al. Prognostic factors for local recurrence of squamous cell carcinoma of the vulva: a systematic review. Gynecol Oncol. 2018;148:622–31.CrossRefGoogle Scholar
  30. 30.
    Jones RW, Sadler L, Grant S, et al. Clinically identifying women with vulvar lichen sclerosus at increased risk of squamous cell carcinoma: a case-control study. J Reprod Med. 2004;49:808–11.PubMedGoogle Scholar
  31. 31.
    van der Meijden WI, Boffa MJ, Ter Harmsel WA, et al. 2016 European guideline for the management of vulval conditions. J Eur Acad Dermatol Venereol. 2017;31:925–41.CrossRefPubMedGoogle Scholar
  32. 32.
    Nguyen Y, Bradford J, Fischer G. Lichen sclerosus in pregnancy: a review of 33 cases. J Invest Dermatol. 2017;137:S197.CrossRefGoogle Scholar
  33. 33.
    Dalziel KL, Millard PR, Wojnarowska F. The treatment of vulval lichen sclerosus with a very potent topical steroid (clobetasol propionate 0.05%) cream. Br J Dermatol. 1991;124:461–4.CrossRefPubMedGoogle Scholar
  34. 34.
    Lee A, Bradford J, Fischer G. Evidence-based (S3) guideline on (anogenital) lichen sclerosus. JEADV. 2015;29(10):e1–43 (J Eur Acad Dermatol Venereol. 2017;31:e57–e58).Google Scholar
  35. 35.
    La Spina M, Meli MC, De Pasquale R, et al. Vulvar melanoma associated with lichen sclerosus in a child: case report and literature review. Pediatr Dermatol. 2016;33:e190–4.CrossRefPubMedGoogle Scholar
  36. 36.
    Ellis E, Fischer G. Prepubertal-onset vulvar lichen sclerosus: the importance of maintenance therapy in long-term outcomes. Pediatr Dermatol. 2015;32:461–7.CrossRefPubMedGoogle Scholar
  37. 37.
    Smith SD, Fischer G. Paediatric vulval lichen sclerosus. Australas J Dermatol. 2009;50:243–8.CrossRefPubMedGoogle Scholar
  38. 38.
    Wood PL, Bevan T. Lesson of the week child sexual abuse enquiries and unrecognised vulval lichen sclerosus et atrophicus. BMJ. 1999;319:899–900.CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    Powell J, Strauss S, Gray J, Wojnarowska F. Genital carriage of human papilloma virus (HPV) DNA in prepubertal girls with and without vulval disease. Pediatr Dermatol. 2003;20:191–4.CrossRefPubMedGoogle Scholar
  40. 40.
    Day T, Burston K, Dennerstein G, et al. Vestibulovaginal sclerosis versus lichen sclerosus. Int J Gynecol Pathol. 2017. (Epub ahead of print).PubMedCentralCrossRefGoogle Scholar
  41. 41.
    Carlson JA, Mu XC, Slominski A, et al. Melanocytic proliferations associated with lichen sclerosus. Arch Dermatol. 2002;138:77–87.CrossRefPubMedGoogle Scholar
  42. 42.
    Sideri M, Jones RW, Wilkinson EJ, et al. Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD vulvar oncology subcommittee. J Reprod Med. 2005;50:807–10.PubMedGoogle Scholar
  43. 43.
    Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet. 1999;353:1777–83.CrossRefPubMedGoogle Scholar
  44. 44.
    Lewis FM, Tatnall FM, Velangi SS, et al. British Association of Dermatologists guidelines for the management of lichen sclerosus, 2018. Br J Dermatol. 2018;178:839–53.CrossRefPubMedGoogle Scholar
  45. 45.
    Cooper SM, Gao XH, Powell JJ, Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol. 2004;140:702–6.CrossRefPubMedGoogle Scholar
  46. 46.
    Renaud-Vilmer C, Cavelier-Balloy B, Porcher R, Dubertret L. Vulvar lichen sclerosus: effect of long-term topical application of a potent steroid on the course of the disease. Arch Dermatol. 2004;140:709–12.CrossRefPubMedGoogle Scholar
  47. 47.
    Bradford J, Fischer G. Long-term management of vulval lichen sclerosus in adult women. Aust N Z J Obstet Gynaecol. 2010;50:148–52.CrossRefPubMedGoogle Scholar
  48. 48.
    Corazza M, Borghi A, Minghetti S, et al. Clobetasol propionate vs. mometasone furoate in 1-year proactive maintenance therapy of vulvar lichen sclerosus: results from a comparative trial. J Eur Acad Dermatol Venereol. 2016;30:956–61.CrossRefPubMedGoogle Scholar
  49. 49.
    Chi CC, Kirtschig G, Baldo M, et al. Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev. 2011. Scholar
  50. 50.
    Lee A, Lim A, Fischer G. Fractional carbon dioxide laser in recalcitrant vulval lichen sclerosus. Australas J Dermatol. 2016;57:39–43.CrossRefPubMedGoogle Scholar
  51. 51.
    Sinha P, Sorinola O, Luesley DM. Lichen sclerosus of the vulva. Long-term steroid maintenance therapy. J Reprod Med. 1999;44:621–4.PubMedGoogle Scholar
  52. 52.
    Corazza M, Virgili A, Toni G, Borghi A. Mometasone furoate in the treatment of vulvar lichen sclerosus: could its formulation influence efficacy, tolerability and adherence to treatment? J Dermatolog Treat. 2018;29(3):305–9.CrossRefPubMedGoogle Scholar
  53. 53.
    Funaro D. Lichen sclerosus: a review and practical approach. Dermatol Ther. 2004;17:28–37.CrossRefPubMedGoogle Scholar
  54. 54.
    Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol. 2003;28:128–33.CrossRefPubMedGoogle Scholar
  55. 55.
    Val I, Almeida G. An overview of lichen sclerosus. Clin Obstet Gynecol. 2005;48:808–17.CrossRefPubMedGoogle Scholar
  56. 56.
    Kirtschig G, Cooper S, Aberer W, et al. Evidence-based (S3) guideline on (anogenital) lichen sclerosus. J Eur Acad Dermatol Venereol. 2017;31(2):e81–3.CrossRefPubMedGoogle Scholar
  57. 57.
    Kai A, Lewis F. Long-term use of an ultrapotent topical steroid for the treatment of vulval lichen sclerosus is safe. J Obstet Gynaecol. 2016;36:276–7.CrossRefPubMedGoogle Scholar
  58. 58.
    Mazdisnian F, Degregorio F, Mazdisnian F, Palmieri A. Intralesional injection of triamcinolone in the treatment of lichen sclerosus. J Reprod Med. 1999;44:332–4.PubMedGoogle Scholar
  59. 59.
    Jones RW, Scurry J, Neill S, MacLean AB. Guidelines for the follow-up of women with vulvar lichen sclerosus in specialist clinics. Am J Obstet Gynecol. 2008;198:496.e1–3.Google Scholar
  60. 60.
    Balasubramaniam P, Lewis FM. Long-term follow-up of patients with lichen sclerosus: does it really happen? J Obstet Gynaecol. 2007;27:282.CrossRefPubMedGoogle Scholar
  61. 61.
    Funaro D, Lovett A, Leroux N, Powell J. A double-blind, randomized prospective study evaluating topical clobetasol propionate 0.05% versus topical tacrolimus 0.1% in patients with vulvar lichen sclerosus. J Am Acad Dermatol. 2014;71:84–91.CrossRefPubMedGoogle Scholar
  62. 62.
    Hengge UR, Krause W, Hofmann H, et al. Multicentre, phase II trial on the safety and efficacy of topical tacrolimus ointment for the treatment of lichen sclerosus. Br J Dermatol. 2006;155:1021–8.CrossRefPubMedGoogle Scholar
  63. 63.
    Fischer G, Bradford J. Topical immunosuppressants, genital lichen sclerosus and the risk of squamous cell carcinoma: a case report. J Reprod Med. 2007;52:329–31.PubMedGoogle Scholar
  64. 64.
    Borghi A, Corazza M, Minghetti S, Virgili A. Topical tretinoin in the treatment of vulvar lichen sclerosus: an advisable option? Eur J Dermatol. 2015;25:404–9.PubMedGoogle Scholar
  65. 65.
    Sideri M, Origoni M, Spinaci L, Ferrari A. Topical testosterone in the treatment of vulvar lichen sclerosus. Int J Gynaecol Obstet. 1994;46:53–6.CrossRefPubMedGoogle Scholar
  66. 66.
    Bradford J, Fischer G. Surgical division of labial adhesions in vulvar lichen sclerosus and lichen planus. J Low Genit Tract Dis. 2013;17:48–50.CrossRefPubMedGoogle Scholar
  67. 67.
    Goldstein AT, King M, Runels C, et al. Intradermal injection of autologous platelet-rich plasma for the treatment of vulvar lichen sclerosus. J Am Acad Dermatol. 2017;76:158–60.CrossRefPubMedGoogle Scholar
  68. 68.
    Boero V, Brambilla M, Sipio E, et al. Vulvar lichen sclerosus: a new regenerative approach through fat grafting. Gynecol Oncol. 2015;139:471–5.CrossRefPubMedGoogle Scholar
  69. 69.
    Criscuolo AA, Schipani C, Cannizzaro MV, et al. New therapeutic approaches in the treatment of anogenital lichen sclerosus: does photodynamic therapy represent a novel option? G Ital Dermatol Venereol. 2017;152:117–21.PubMedGoogle Scholar
  70. 70.
    Shi L, Miao F, Zhang LL, et al. Comparison of 5-aminolevulinic acid photodynamic therapy and clobetasol propionate in treatment of vulvar lichen sclerosus. Acta Derm Venereol. 2016;96:684–8.CrossRefPubMedGoogle Scholar
  71. 71.
    Colbert RL, Chiang MP, Carlin CS, Fleming M. Progressive extragenital lichen sclerosus successfully treated with narrowband UV-B phototherapy. Arch Dermatol. 2007;143:19–20.CrossRefPubMedGoogle Scholar
  72. 72.
    Kreuter A, Tigges C, Gaifullina R, et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate treatment in patients with refractory generalized extragenital lichen sclerosus. Arch Dermatol. 2009;145:1303–8.PubMedGoogle Scholar
  73. 73.
    Haefner HK, Aldrich NZ, Dalton VK, et al. The impact of vulvar lichen sclerosus on sexual dysfunction. J Womens Health (Larchmt). 2014;23:765–70.CrossRefPubMedCentralGoogle Scholar
  74. 74.
    Harris V, Fischer G, Bradford JA. The aetiology of chronic vulval pain and entry dyspareunia: a retrospective review of 525 cases. Aust N Z J Obstet Gynaecol. 2017;57:446–51.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of DermatologyRoyal North Shore HospitalSt LeonardsAustralia
  2. 2.Sydney Medical School NorthernThe University of SydneySt LeonardsAustralia
  3. 3.Department of DermatologyKolling InstituteSt LeonardsAustralia

Personalised recommendations