Vulvar Lichen Sclerosus et Atrophicus in girls

The review is devoted to diagnosing and treating lichen sclerosus (LS), a chronic inflammatory dermatosis with a predisposition to lesions of the anogenital region in girls. An essential role in the aetiology of this form of pathology is attributed to genetic mutations, injuries, infections and autoimmune diseases, and hormonal and local factors. The peak incidence is observed in 4–6 year girls, accounting for 7–15% of all cases of vulvar LS. The main goal of pharmacotherapy for vulvar LS is to relieve clinical symptoms and prevent scarring and adhesions.

The authors analyzed international and domestic publications from 2017 to 2021. PubMed and Google Academy databases were used for the search, keywords: lichen sclerosus, girls. The retrospective and prospective cohort, randomized clinical trials, case series and literature reviews, and clinical guidelines were considered. Our review presents modern data on the diagnosis and treatment of SL in girls, which will be useful for both pediatric gynecologists and doctors of related specialties.

The first symptoms of LS are usually nonspecific and are misdiagnosed by non-profile specialists. Some symptoms of LS may disappear spontaneously after menarche, and the course of the disease may be latent. This is why it is generally accepted that the epidemiology of LS is underestimated. Despite this, it can be assumed that the etiology and pathogenesis of LS is probably multifactorial. This review describes several leading etiological factors regarding the potential etiopathogenesis of vulvar LS in girls.

Contribution:
Adamyan L.V., Sharkov S.M., Sibirskaya E.V. — the concept and design of the study;
Pivazyan L.G. — collection and processing of material;
Avetisyan J.S. — writing text;
Sibirskaya E.V. — editing.
All co-authors — approval of the final version of the article, responsibility for the integrity of all its parts.

Acknowledgment. The study had no sponsorship.

Conflict of interest. The authors declare no conflict of interest.

Received: September 21, 2021
Accepted: October 28, 2021
Published: November 15, 2021

1. Fistarol S., Itin P. Diagnosis and treatment of lichen sclerosus. Am. J. Clin. Dermatol. 2012; 14(1): 27-47. https://doi.org/10.1007/s40257-012-0006-4

2. Powell J., Wojnarowska F. Lichen sclerosus. The Lancet. 1999; 353(9166): 1777-83. https://doi.org/10.1016/s0140-6736(98)08228-2

3. Virgili A., Borghi A., Toni G., Minghetti S., Corazza M. Prospective clinical and epidemiologic study of vulvar lichen sclerosus: analysis of prevalence and severity of clinical features, together with historical and demographic associations. Dermatology. 2014; 228(2): 145-51. https://doi.org/10.1159/000356163

4. Kreuter A., Kryvosheyeva Y., Terras S., Moritz R., Möllenhoff K., Altmeyer P., et al. Association of autoimmune diseases with lichen sclerosus in 532 male and female patients. Acta. Dermato. Venereologica. 2013; 93(2): 238-41. https://doi.org/10.2340/00015555-1512

5. Ismail D., Owen C. Paediatric vulval lichen sclerosus: a retrospective study. Clin. Exp. Dermatol. 2019; 44(7): 753-8. https://doi.org/10.1111/ced.13894

6. Friedland R., Ben-Amitai D., Didkovsky E., Feinmesser M., Zvulunov A. Vascular lesions in genital lichen sclerosus in pediatric patients. Pediatr Dermatol. 2020; 37(5): 849-52. https://doi.org/10.1111/pde.14277

7. Kirtschig G. Lichen sclerosus - presentation, diagnosis and management. Dtsch Arztebl. Int. 2016; 113(19): 337-43. https://doi.org/10.3238/arztebl.2016.0337

8. Nerantzoulis I., Grigoriadis T., Michala L. Genital lichen sclerosus in childhood and adolescence - a retrospective case series of 15 patients: early diagnosis is crucial to avoid long-term sequelae. Eur. J. Pediatr. 2017; 176(10): 1429-32. https://doi.org/10.1007/s00431-017-3004-y

9. Powell J., Wojnarowska F. Childhood vulvar lichen sclerosus: An increasingly common problem. J. Am. Acad. Dermatol. 2001; 44(5): 803-6. https://doi.org/10.1067/mjd.2001.113474

10. Sokolova A., Grebenyuk V., Dolya O. Clinical features of genital lichen sclerosus et atrophica in males and females. Klinicheskaya dermatologiya i venerologiya. 2016; 15(1): 54. https://doi.org/10.17116/klinderma2016151554-59

11. Orszulak D., Dulska A., Niziński K. Pediatric vulvar lichen sclerosus - a review of the literature. Int. J. Environ. Res. Public Health. 2021; 18(13): 7153. https://doi.org/10.3390/ijerph18137153

12. Semenchak Y., Snarskaya E. Lichen sclerosus et atrophicus. Doctor. 2018; 29(8): 26–34. https://doi.org/10.29296/25877305-2018-08-07

13. Klaber R. Lichen sclerosus et atrophicans (Hallopeau). Proc. R. Soc. Med. 1937; 30(8): 977-9. https://doi.org/10.1177/003591573703000823

14. Farrell A.M., Dean D., Millard P.R., Charnock F.M., Wojnarowska F. Cytokine alterations in lichen sclerosus: an immunohistochemical study. Br. J. Dermatol. 2006; 155(5): 931-40. https://doi.org/10.1111/j.1365-2133.2006.07414.x

15. Simpkin S., Oakley A. Clinical review of 202 patients with vulval lichen sclerosus: A possible association with psoriasis. Australas. J. Dermatology. 2007; 48(1): 28-31. https://doi.org/10.1111/j.1440-0960.2007.00322.x

16. Cooper S.M., Ali I., Baldo M., Wojnarowska F. The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease. Arch. Dermatol. 2008; 144(11): 1432-5. https://doi.org/10.1001/archderm.144.11.1432

17. Clifton M.M., Garner I.B., Kohler S., Smoller B.R. Immunohistochemical evaluation of androgen receptors in genital and extragenital lichen sclerosus: Evidence for loss of androgen receptors in lesional epidermis. J. Am. Acad. Dermatol. 1999; 41(1): 43-6. https://doi.org/10.1016/s0190-9622(99)70404-4

18. Singh N., Ghatage P. Etiology, clinical features, and diagnosis of vulvar lichen sclerosus: a scoping review. Obstet. Gynecol. Int. 2020; 2020: 7480754. https://doi.org/10.1155/2020/7480754

19. Poindexter G., Morrell D. Anogenital pruritus: lichen sclerosus in children. Pediatr. Ann. 2007; 36(12): 785-91. https://doi.org/10.3928/0090-4481-20071201-07

20. Edwards L.R., Privette E.D., Patterson J.W., Tchernev G., Chokoeva A.A., Wollina U., et al. Radiation-induced lichen sclerosus of the vulva: First report in the medical literature. Wien. Med. Wochenschr. 2016; 167(3-4): 74-7. https://doi.org/10.1007/s10354-016-0525-3

21. Chattopadhyay S., Arnold J.D., Malayil L., Hittle L., Mongodin E.F., Marathe K.S., et al. Potential role of the skin and gut microbiota in premenarchal vulvar lichen sclerosus: A pilot case-control study. PLoS One. 2021; 16(1): e0245243. https://doi.org/10.1371/journal.pone.0245243

22. Kirtschig G., Becker K., Günthert A., Jasaitiene D., Cooper S., Chi C.C., et al. Evidence-based (S3) guideline on (anogenital) lichen sclerosus. J. Eur. Acad. Dermatol. Venereol. 2015; 29(10): e1-e43. https://doi.org/10.1111/jdv.13136

23. Pranteda G., Muscianese M., Grimaldi M., Fidanza L., Pranteda G., Narcisi A. Lichen sclerosus et atrophicus induced by carbamazepine: a case report. Int. J. Immunopathol. Pharmacol. 2013; 26(3): 791-4. https://doi.org/10.1177/039463201302600326

24. Baldo M., Ali I., Wojnarowska F. The contribution of drugs to lichen sclerosus. Clin. Exp. Dermatol. 2014; 39(2): 234. https://doi.org/10.1111/ced.12264

25. Arlen A., Wang M., Vash-Margita A. Lichen sclerosus in prepubertal girls: an uncommon but treatable cause of lower urinary tract symptoms. Urology. 2020; 137: e1-e2. https://doi.org/10.1016/j.urology.2019.12.029

26. Cooper S.M., Gao X.H., Powell J.J., Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch. Dermatol. 2004; 140(6): 702-6. https://doi.org/10.1001/archderm.140.6.702

27. Lee A., Bradford J., Fischer G. Long-term management of adult vulvar lichen sclerosus. JAMA Dermatol. 2015; 151(10): 1061. https://doi.org/10.1001/jamadermatol.2015.0643

28. Bercaw-Pratt J., Boardman L., Simms-Cendan J. Clinical recommendation: pediatric lichen sclerosus. J. Pediatr Adolesc. Gynecol. 2014; 27(2): 111-6. https://doi.org/10.1016/j.jpag.2013.11.004

29. Mashayekhi S., Flohr C., Lewis F. The treatment of vulval lichen sclerosus in prepubertal girls: a critically appraised topic. Br. J. Dermatol. 2017; 176(2): 307-16. https://doi.org/10.1111/bjd.15202

30. Wood P., Bevan T. Lesson of the week child sexual abuse enquiries and unrecognised vulval lichen sclerosus et atrophicus. BMJ. 1999; 319(7214): 899-900. https://doi.org/10.1136/bmj.319.7214.899

31. Casey G., Cooper S., Powell J. Treatment of vulvar lichen sclerosus with topical corticosteroids in children: a study of 72 children. Clin. Exp. Dermatol. 2014; 40(3): 289-92. https://doi.org/10.1111/ced.12519

32. Smith Y., Quint E. Clobetasol propionate in the treatment of premenarchal vulvar lichen sclerosus. Obstet. Gynecol. 2001; 98(4): 588-91. https://doi.org/10.1097/00006250-200110000-00011

33. Smith S., Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr. Dermatol. 2009; 26(6): 725-9. https://doi.org/10.1111/j.1525-1470.2009.01022.x

34. Ridley C. Lichen sclerosus et atrophicus. Arch. Dermatol. 1987; 123(4): 457-60. https://doi.org/10.1001/archderm.123.4.457

35. Warrington S., de San Lazaro C. Lichen sclerosus et atrophicus and sexual abuse. Arch. Dis. Child. 1996; 75(6): 512-6. https://doi.org/10.1136/adc.75.6.512

36. Li Y., Xiao Y., Wang H., Li H., Luo X. Low-concentration topical tacrolimus for the treatment of anogenital lichen sclerosus in childhood: maintenance treatment to reduce recurrence. J. Pediatr. Adolesc. Gynecol. 2013; 26(4): 239-42. https://doi.org/10.1016/j.jpag.2012.11.010

37. Boms S., Gambichler T., Freitag M., Altmeyer P, Kreuter A. Pimecrolimus 1% cream for anogenital lichen sclerosus in childhood. BMC Dermatol. 2004; 4(1): 14. https://doi.org/10.1186/1471-5945-4-14

38. Neill S., Ridley C. Management of anogenital lichen sclerosus. Clin. Exp. Dermatol. 2001; 26(8): 637-43. https://doi.org/10.1046/j.1365-2230.2001.00907.x

39. Carli P. Topical cyclosporine in the treatment of vulvar lichen sclerosus: clinical, histologic, and immunohistochemical findings. Arch. Dermatol. 1992; 128(11): 1548. https://doi.org/10.1001/archderm.1992.01680210128025

40. Zarochentseva N.V., Dzhidzhiya L.K. Lichen sclerosus et atrophicus: modern view on the problem. Rossiyskiy vestnik akushera-gynekologa. 2018; 6: 41–50. https://doi.org/10.17116/rosakush20181806141