Lichen Sclerosus. Review
Authors:
J. Štork
Authors place of work:
Dermatovenerologická klinika 1. LF UK a VFN přednosta prof. MUDr. Jiří Štork, CSc.
Published in the journal:
Čes-slov Derm, 95, 2020, No. 6, p. 195-205
Category:
Reviews
Summary
Lichen sclerosus is a chronic inflammatory skin disease affecting children and adults, mostly women, leading to atrophy of the epidermis, fibrosis and scarring, often accompanied by pruritus. It most often affects the anogenital area, where it causes functional impairment and is associated with an increased risk of vulvar skin cancer, however it can also occur in other localizations. The pathogenesis of the disease is unclear. Various factors are considered, including genetic predisposition and local irritation. The autoimmune nature of the disease, mediated by Th1 cell cytokines, accompanied by the increased expression of microRNA-155 and the production of antibodies to extracellular matrix glycoprotein 1, is most commonly proposed. The diagnosis is usually clinical, biopsy is mostly not necessary. The treatment of choice are strong topical corticosteroids (clobetasol), circumcision is often curative in men. For more extensive skin involvement, application of phototherapy is possible (especially UVA1, less UVB311), rarely systemic therapy with methotrexate and corticosteroids is used. Due to the chronic nature of the disease and the possible development of malignancy, long-term follow-up of patients is recommended.
Keywords:
lichen sclerosus – Pathogenesis – diagnosis – clinical manifestation – therapy – follow–up
Zdroje
1. AZEVEDO, R. S., ROMAÑACH, M. J., DE ALMEIDA, O. P. et al. Lichen sclerosus of the oral mucosa: clinicopathological features of six cases. Int J Oral Maxillofac Surg., 2009, 38, p. 855.
2. BALAKIRSKI, G., GROTHAUS, J., ALTENGARTEN, J. et al. Paediatric lichen sclerosus: a systematic review of 4516 cases. Br J Dermatol., 2020, 182, p. 231–233.
3. BALDO, M., BHOGAL, B., GROVES, R. W., POWELL, J., WOJNAROWSKA, F. Childhood vulval lichen sclerosus: autoimmunity to the basement membrane zone protein BP180 and its relationship to autoimmunity. Clin Exp Dermatol., 2010, 35, p. 543–545.
4. BRACCO, G. L., CARLI, P., SONNI, L. et al. Clinical and histologic effects of topical treatments of vulval lichen sclerosus. A critical evaluation. J Reprod Med., 1993, 38, p. 37–40.
5. BRODSKÁ, P., PIZINGER, K. Klinický případ: Bělavá ložiska s hemoragickými puchýři na hrudníku. Čes-slov Derm., 2011, 86 (6), s. 285–287.
6. BUNKER, C. B. Atopy, the barrier, urine and genital lichen sclerosus. Br J Dermatol., 2013, 169, p. 953.
7. CALONJE, E., LEWIS, F., BUNKER, CH. et al. Diseases of anogenital skin. P. 470 In: Calonje, E., Brenn, T., Lazar, A., Billings, S. McKee‘s Pathology of the Skin. 5th Ed., Elsevier, 2019, Hardcover ISBN: 9780702069833.
8. CLAY, F. E., CORK, M. J., TARLOW, J. K. et al. Interleukin 1 receptor antagonist gene polymorphism association with lichen sclerosus. Hum Genet, 1994, 94, p. 407–410.
9. COLBERT, R. L., CHIANG, M., CARLIN, C. S., FLEMING, M. Progressive extragenital lichen sclerosus successfully treated with narrowband UV-B phototherapy. Arch Dermatol., 2007, 143 (1), p. 19.
10. COOPER, S. M., GAO, X. H., POWELL, J. J. et al. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol., 2004, 140, p. 702–706.
11. EBERT, A. K., ROSCH, W. H., VOGT, T. Safety and tolerability of adjuvant topical tacrolimus treatment in boys with lichen sclerosus: a prospective phase 2 study. Eur Urol., 2008, 54, p. 932–937.
12. EDMONDS, E. V., HUNT, S., HAWKINS, D. et al. Clinical parameters in male genital lichen sclerosus: a case series of 329 patients. J Eur Acad Dermatol Venereol., 2012, 26, p. 730–737.
13. FERGUS, KB., LEE, AW., BARADARAN, N. et al. Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review. Urology, 2020, 135, p. 11−19.
14. FISTAROL, S. K., ITIN, P. H. Diagnosis and Treatment of Lichen Sclerosus. Dermatol., 2013, 14, p. 27–47.
15. Funaro, D., Lovett, A., Leroux, N. et al. 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., 201, 71, p. 84–91.
16. GOLDSTEIN, A. T., MARINOFF, S. C., CHRISTOPHER, K. et al. Prevalence of vulvar lichen sclerosus in a general gynecology practice. J Reprod Med., 2005, 50, p. 4772–4780
17. Goldstein, A. T., Creasey, A., Pfau, R., Phillips, D., Burrows, L. J. A double-blind, randomized controlled trial of clobetasol vs. pimecrolimus in patients with vulvar lichen sclerosus. J Am Acad Dermatol., 2011, 64, p. 99–104.
18. HASEGAWA, M., ISHIKAWA, O., ASANO, Y. et al. Diagnostic criteria, severity classification and guidelines of lichen sclerosus et atrophicus. Journal of Dermatology, 2018, 45, p.891–897.
19. HENGGE, U. R., 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, p. 1021–1028.
20. HERCOGOVÁ, J., TOMÁNKOVÁ, M., FROSSLOVÁ, D. et al. Sklerodermie, lichen sclerosus et atrophicus a protilátkx proti Borrelia burgdorferi. Čes--slov Derm.,1994, 69, s. 8–10.
21. HIGGINS, C. A., CRUICKSHANK, M. E. A population-based case-control study of aetiological factors associated with vulval lichen sclerosus. J Obstet Gynaecol., 2012, 32, p. 271–275.
22. HOFER, M. D., MEEKS, J. J., MEHDIRATTA, N., GRANIERI, M. A., CASHY, J., GONZALEZ, C. M. Lichen sclerosus in men is associated with elevated body mass index, diabetes mellitus, coronary artery disease and smoking. World J Urol., 2014, 32, p. 105–108.
23. CHUNG, A. S. J., SUAREZ, O. A. Current treatment of lichen sclerosus and stricture. World J Urol., 2019 on line, https://doi.org/10.1007/s00345-019-03030-z.
24. JACOBE, H. Extragenital lichen sclerosus. UpToDate 2019, Topic 15838 Version 13.0
25. KANTERE, D., ALVERGREN, G., GILLSTEDT, M. et al. Clinical features, complications and autoimmunity in male lichen sclerosus. Acta Derm Venereol., 2017, 97, p. 365–369.
26. KHACHEMOUNE, A., GULDBAKKE, K. K., EHRSAM, E. Infantile perineal protrusion. J Am Acad Dermatol., 2006, 54, p. 1046–1049.
27. KIRTSCHIG, G., BECKER, K., GÜNTHERT, A. et al. Evidence-based (S3) guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol., 2015, 10, e1–e43.
28. KIRTSCHIG, G. Lichen Sclerosus – Presentation, Diagnosis and Management. Dtsch Arztebl Int., 2016, 113, p. 336–343.
29. KISS, A., CSONTAI, A., PIROT, L., NYIRADY, P., MERKSZ, M., KIRALY, L. The response of balanitis xerotica obliterans to local steroid application compared with placebo in children. J Urol., 2001, 165, p. 219–220 (Level II).
30. KISS, A., KIRALY, L., KUTASY, B. et al. High incidence of balanitis xerotica obliterans in boys with phimosis: prospective 10-year study. Pediatr Dermatol., 2005, 22, p. 305–308.
31. KRAPF, J. M., MITCHELL, L., HOLTON, M. A. et al. Vulvar Lichen Sclerosus: Current Perspectives. International J Women‘s Health, 2020, 12, p. 11–20.
32. KREUTER, A., GAMBICHLER, T., AVERMAETE, A. et al. Low-dose ultraviolet A1 phototherapy for extragenital lichen sclerosus: results of a preliminary study. J Am Acad Dermatol., 2002, 46 (2), p. 251.
33. 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 (11), p. 1303.
34. KREUTER, A., WISCHNEWSKI, J., TERRAS, S. et al, Coexistence of lichen sclerosus and morphea: A retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center. J Am Acad Dermatol., 2012, Dec; 67(6), p. 1157–1162.
35. 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, p. 1061.
36. LEWIS, F. M., TATNALL, F. M., VELANGI, S. S. et al. British Association of dermatologists guidelines for the management of lichen sclerosus. 2018, Br J Dermatolol., 2018, 178, p. 839–853.
37. LONGINOTTI, M., SCHIEFFER, Y. M., KAUFMAN, R. H. Lichen sclerosus involving the vagina. Obstet Gynecol., 2005, 106 (5 Pt 2), p. 1217–1219.
38. LUTZ, V., FRANCÈS, C., BESSIS, D. et al. High Frequency of Genital Lichen Sclerosus in a Prospective Series of 76 Patients With Morphea. Arch Dermatol., 2012,148 (1), p. 24–28.
39. MEYRICK, T., RIDLEY, R. H., CM., MCGIBBON C. M. et al. Lichen sclerosus et atrophicus and autoimmunity: a study of 350 women. Br J Dermatol., 1988, 118, p. 41–46.
40. MORREL, B., VAN EERSEL, R., BURGER, C. W. et al. The long-term clinical consequences of juvenile vulvar lichen sclerosus: A systematic review. J Am Acad Dermatol., 2020, 82, p. 469–477.
41. MORRIS, B. J., KRIEGER, J. N. Penile inflammatory skin disorders and the preventive role of circumcision. Int J Prev Med., 2017, 8, p. 32.
42. NEILL, S. M., LEWIS, F. M., TATNALL, F. M. et al. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol., 2010, 163, p. 672–682.
43. NGUYEN, ALEXANDER T. M., HOLLAND, ANDREW J. A. Balanitis xerotica obliterans: an update for clinicians. European Journal of Pediatrics, 2020, 179, p. 9–16.
44. OYAMA, N., CHAN, I., NEILL, S. M. et al. Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet, 2003, 362, p. 118.
45. PATSATSI, A., KYRIAKOU, A., MANTAS, A. et al. Circulating anti-BP180 NC16a and anti-BP230 autoantibodies in patients with genital lichen sclerosus do not correlate with the disease activity and pruritus. Acta Dermato Venereol., 2014, 94, p. 711–712.
46. POWELL, J. J., WOJNAROWSKA, F. Lichen sclerosus. Lancet, 1999, 353, p. 1777.
47. POWELL, J., WOJNAROWSKA, F. Childhood vulvar lichen sclerosus: an increasingly common problem. J Am Acad Dermatol., 2001, 44, p. 803–806.
48. RAMRAKHA-JONES, V. S., PAUL, M., MCHENRY, P. et al. Nail dystrophy due to lichen sclerosus? Clin. Exp. Dermatol., 2001, 26, p. 507–509.
49. REGAUER, S., REICH, O., BEHAM-SCHMID, CH. Monoclonal gamma-T-Cell Receptor Rearrangement in Vulvar Lichen Sclerosus and Squamous Cell Carcinomas American Journal of Pathology, 2002, 160 (3), p. 1035–1045.
50. REN, L., ZHAO, Y., HUO, X. et al. MiR-155-5p promotes fibroblast cell proliferation and inhibits FOXO signaling pathway in vulvar lichen sclerosis by targeting FOXO3 and CDKN1B. Gene., 2018, 653, p. 43–50.
51. SAUDER, M. B., LINZON-SMITH, J., BEECKER, J. Extragenital bullous lichen sclerosus. J Am Acad Dermatol., 2014, 71 (5), p. 981.
52. SHELLEY, W. B., SHELLEY, E. D., AMURAO, C. V. Treatment of lichen sclerosus with antibiotics. International Journal of Dermatology, 2006, 45, p. 1104–1106.
53. SIMPKIN, S., OAKLEY, A. Clinical review of 202 patients with vulval lichen sclerosus: a possible association with psorasis. Australas J Dermatol., 2007, 48, p. 28–31.
54. SMITH, S. D., FISCHER, G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol., 2009, 26, p. 725–729.
55. ŠTORK, J. Bělavý pruh atrofie kůže čela. Čes-slov Derm., 1993, 68 (4), s. 229–230.
56. TERLOU, A., SANTEGOETS, L. A. M., VAN DER MEIJDEN W. I. et al. An autoimmune phenotype in vulvar lichen sclerosus and lichen planus: a Th1 response and high levels of MicroRNA-155. J Invest Dermatol., 2012,132, p. 658–666.
57. TRAN, D. A., TAN, X., MACRI, C. H. J. et al. Lichen Sclerosus: An autoimmunopathogenic and genomic enigma with emerging genetic and immune targets. Int. J. Biol. Sci. 2019, 15, p. 1429–1439.
58. VALÍČKOVÁ, J., KOCÁNOVÁ, E., BUČKOVÁ, H. Lichen sclerosus et atrophicus anogenitalis u dětí: retrospektivní analýza souboru nemocných z období 2006–2010. Čes-slov Dermatol., 2012, 87 (4), s. 141–144.
59. VIRGILI, A., GORGHI, A., TONI, G., MINGHETTI, S., CORAZZA, M. First randomized trial on clobetasol propionate and mometasone furoate in the treatment of vulvar lichen sclerosus: results of efficacy and tolerability. Br J Dermatol., 2014, 171, p. 388–396.
60. WALLACE, H. J. Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc., 1971, 57, p. 9–30.
61. WERNHAM, A. G. H., SHAH, F., VELANGI, S. Nivolumab PD-1 inhibitor immunotherapy associated with vulvar, perineal and perianal lichen sclerosus. Clin Exp Dermatol., 2019, 44, e22-e23.
62. WHIMSTER, I. W. An experimental approach to the problem of spottiness. Br J Dermatol., 1965, 77, p. 397.
63. ZENDELL, K., EDWARDS, L. Lichen Sclerosus With Vaginal Involvement: Report of 2 Cases and Review of the Literature. JAMA Dermatol., 2013, 149 (10), p. 1199–1202.
Štítky
Dermatology & STDs Paediatric dermatology & STDsČlánok vyšiel v časopise
Czech-Slovak Dermatology
2020 Číslo 6
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