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Склероатрофический лихен: современные представления и гипотезы
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Khryanin AA, Sokolovskaia AV, Bocharova VK. Scleroatrophic lichen: current view and hypotheses. Gynecology. 2022;24(3):212–218. DOI: 10.26442/20795696.2022.3.201694
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Ключевые слова: склероатрофический лихен, плоскоклеточный рак, аутоиммунное заболевание, диагностика, лечение, активированная глицирризиновая кислота
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Scleroatrophic lichen (SL) is a chronic inflammatory skin disease of unknown etiology characterized by scar formation. The most frequent localization of SL is the anogenital area. In females, the most common anogenital symptoms are pain, itching, dysuria and dyspareunia. Male patients often present with complaints of a white rash on the penile skin, itching, painful erections and urination, and bleeding or ulceration during intercourse. Although clinical descriptions of SL date back to the mid-20th century, the pathophysiological mechanism remains unclear to this day. The main hypotheses of the pathophysiology of SL are infectious, autoimmune, and chronic irritation theories (occlusive exposure to various irritants, such as urine, leads to chronic skin injury that causes the disease). It is assumed that SL quite often goes undiagnosed for a long time. However, sometimes dermatoses such as eczema in the anogenital area are misdiagnosed by specialists as SL. In this article, the authors discuss in detail the existing hypotheses of SL development, its clinical manifestations, and current methods of diagnostics and therapy.
Keywords: scleroatrophic lichen, squamous cell carcinoma, autoimmune disease, diagnosis, treatment, activated glycyrrhizic acid
2. Pugliese JM, Morey AF, Peterson AC. Lichen sclerosus: review of the literature and current recommendations for management. J Urol. 2007;178(6):2268-76.
3. Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47.
4. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association of Dermatologists guidelines for the management of lichen sclerosus 2010.
Br J Dermatol. 2010;163(4):672-82. DOI:10.1111/j.1365-2133.2010.09997.x
5. Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol. 2003;28(2):128-33. DOI:10.1046/j.1365-2230.2003.01211.x
6. Edmonds EV, 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;26:730-7.
7. Pass CJ. An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar. Cutis. 1984;33:405-8.
8. Milligan A, Graham-Brown RA, Burns DA. Lichen sclerosus et atrophicus following sunburn. Clin Exp Dermatol. 1988;13:36-7.
9. Kirk PS, Yi Y, Hadj-Moussa M, et al. Diversity of patient profile, urethral stricture, and other disease manifestations in a cohort of adult men with lichen sclerosus. Investig Clin Urol. 2016;57:202-7.
10. 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.
11. Uemura S, Hutson JM, Woodward AA, et al. Balanitis xerotica obliterans with urethral stricture after hypospadias repair. Pediatr Surg Int. 2000;16:144-5.
12. Edmonds E, Barton G, Buisson S, et al. Gene expression profiling in male genital lichen sclerosus. Int J Exp Pathol. 2011;92:320-5.
13. Powell J, Wojnarowska F, Winsey S, et al. Lichen sclerosus premenarche: autoimmunity and immunogenetics. Br J Dermatol. 2000;142:481-4.
14. 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.
15. Gambichler T, Terras S, Kreuter A, et al. Altered global methylation and hydroxymethylation status in vulvar lichen sclerosus: further support for epigenetic mechanisms. Br J Dermatol. 2014;170:687-93.
16. Fergus KB, Lee AW, Baradaran N, et al. Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review. Urology. 2020;135:11-9. DOI:10.1016/j.urology.2019.09.034
17. Gambichler T, Skrygan M, Czempiel V, et al. Differential expression of connective tissue growth factor and extracellular matrix proteins in lichen sclerosus. J Eur Acad Dermatol Venereol. 2012;26(2):207-12. DOI:10.1111/j.1468-3083.2011.04037.x
18. Feig JL, Gribetz ME, Lebwohl MG. Chronic lichen sclerosus successfully treated with intralesional adalimumab. Br J Dermatol. 2016;174:687-9.
19. Lowenstein EB, Zeichner JA. Intralesional adalimumab for the treatment of refractory balanitis xerotica obliterans. JAMA Dermatol. 2013;149:23-4.
20. Al-Niaimi F, Lyon C. Peristomal lichen sclerosus: the role of occlusion and urine exposure? Br J Dermatol. 2013;168:643-6.
21. Doiron PR, Bunker CB. Obesity-related male genital lichen sclerosus. J Eur Acad Dermatol Venereol. 2017;31(5):876-9. DOI:10.1111/jdv.14035
22. Welsh B, Howard A, Cook K. Vulval itch. Aust Fam Physician. 2004;33(7):505-10.
23. Cooper SM, Gao XH, Powell JJ, Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol. 2004;140:702-6.
24. Хрянин А.А., Маркарьян Д.Р., Гарманова Т.Н., Бочарова В.К. Перианальный зуд: возможные причины и тактика ведения пациентов. Клиническая дерматология и венерология. 2020;19(6):891-8 [Khryanin AA, Markaryan DR, Garmanova TN, Bocharova VK. Perianal pruritus: possible causes and management of patients. Russian Journal of Clinical Dermatology and Venereology. 2020;19(6):891-8 (in Russian). DOI:10.17116/klinderma202019061891
25. Edmonds EV, 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;26:730-7.
26. Becker K. Lichen sclerosus in boys. Dtsch Arztebl Int. 2011;108(4):53-8.
27. Kostner S. EBM Service. Phimose mit Talgretetionszyste. Z Allg Med. 2014;90:339-41.
28. Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus. The course after puberty. J Reprod Med. 2002;47:706-9.
29. Kirtschig G. Lichen Sclerosus-Presentation, Diagnosis and Management. Dtsch Arztebl Int. 2016;113(19):337-43. DOI:10.3238/arztebl.2016.0337
30. Yalici-Armagan B, Bostan E, Akdogan N, Ersoy-Evans S. Paediatric lichen sclerosus et atrophicus: A retrospective analysis of 38 paediatric patients. Int J Clin Pract. 2021;75(10):e14661. DOI:10.1111/ijcp.14661
31. 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(4):839-53. DOI:10.1111/bjd.16241
32. Kirtschig G, Becker K, Günthert A, et al. Evidence-based (S3) guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol. 2015;10:e1-43.
34. 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.
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;67:1157-62.
35. Kreuter A, Kryvosheyeva Y, Terras S, et al. Association of autoimmune diseases with lichen sclerosus in 532 male and female patients. Acta Derm Venereol. 2013;93:238-41.
36. 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.
37. Chi CC, Kirtschig G, Baldo M, et al. Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev. 2011;12:CD008240.
38. Lee A, Bradford J, Fischer G. Long-term management of adult vulval lichen sclerosus. A prospective cohort study of 507 women. JAMA Dermatol. 2015;151(10):1061-7. DOI:10.1001/jamadermatol.2015.0643
39. Virgili A, Minghetti S, Borghi A, Corazza M. Proactive maintenance therapy with a topical corticosteroid for vulvar lichen sclerosus: preliminary results of a randomized study. Br J Dermatol. 2013;168:1316-24.
40. Bunker CB, Porter WM. Dermatoses of the male genitalia: squamous carcinoma and other malignant neoplasms. In: Rook's Textbook of Dermatology. Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer D, eds. 9th ed., vol. 3. Chichester: John Wiley&Sons, 2016;111:29-31.
41. Lewis F. Dermatoses of the female genitalia: inflammatory dermatoses of the vulva: lichen sclerosus. In: Rook's Textbook of Dermatology. Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer D, eds. 9th ed., vol. 3. Chichester: John Wiley&Sons, 2016;112:6-9.
42. Turnbull N, Shim T, Patel N, et al. Primary melanoma of the penis in 3 patients with lichen sclerosus. JAMA Dermatol. 2016;152:226-7.
43. Хрянин А.А., Соколовская Е.В., Маркарьян Д.Р., и др. Заболевания кожи промежности и перианальной области. Под ред. А.А. Хрянина. М.: ГЭОТАР-Медиа, 2022 [Khryanin AA, Sokolovskaya EV, Markaryan DR, et al. Pod red. AA Khryanina. Zabolevaniia kozhi promezhnosti i perianal'noi oblasti Moscow: GEOTAR-Media, 2022
(in Russian)].
44. Лишай склеротический и атрофический. Проект клинических рекомендаций 2020. Общероссийская общественная организация «Российское общество дерматовенерологов и косметологов», Российское общество акушеров-гинекологов [Lishai skleroticheskii i atroficheskii. Proekt klinicheskikh rekomendatsii 2020. Obshcherossiiskaia obshchestvennaia organizatsiia "Rossiiskoe obshchestvo dermatovenerologov i kosmetologov", Rossiiskoe obshchestvo akusherov-ginekologov (in Russian)].
45. Virgili A, Minghetti S, Borghi A, Corazza M. Long-term maintenance therapy for vulvar lichen sclerosus: the results of a randomized study comparing topical vitamin E with an emollient. Eur J Dermatol. 2013;23:189-94.
46. Simonart T, Lahaye M, Simonart JM. Vulvar lichen sclerosus: effect of maintenance treatment with a moisturizer on the course of the disease. Menopause. 2008;15:74-7.
47. Dalziel KL. Effect of lichen sclerosus on sexual function and parturition. J Reprod Med. 1995;40:351-4.
________________________________________________
1. Smith YR, Haefner HK. Vulvar lichen sclerosus: pathophysiology and treatment. Am J Clin Dermatol. 2004;5:105-25.
2. Pugliese JM, Morey AF, Peterson AC. Lichen sclerosus: review of the literature and current recommendations for management. J Urol. 2007;178(6):2268-76.
3. Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14:27-47.
4. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association of Dermatologists guidelines for the management of lichen sclerosus 2010.
Br J Dermatol. 2010;163(4):672-82. DOI:10.1111/j.1365-2133.2010.09997.x
5. Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol. 2003;28(2):128-33. DOI:10.1046/j.1365-2230.2003.01211.x
6. Edmonds EV, 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;26:730-7.
7. Pass CJ. An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar. Cutis. 1984;33:405-8.
8. Milligan A, Graham-Brown RA, Burns DA. Lichen sclerosus et atrophicus following sunburn. Clin Exp Dermatol. 1988;13:36-7.
9. Kirk PS, Yi Y, Hadj-Moussa M, et al. Diversity of patient profile, urethral stricture, and other disease manifestations in a cohort of adult men with lichen sclerosus. Investig Clin Urol. 2016;57:202-7.
10. 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.
11. Uemura S, Hutson JM, Woodward AA, et al. Balanitis xerotica obliterans with urethral stricture after hypospadias repair. Pediatr Surg Int. 2000;16:144-5.
12. Edmonds E, Barton G, Buisson S, et al. Gene expression profiling in male genital lichen sclerosus. Int J Exp Pathol. 2011;92:320-5.
13. Powell J, Wojnarowska F, Winsey S, et al. Lichen sclerosus premenarche: autoimmunity and immunogenetics. Br J Dermatol. 2000;142:481-4.
14. 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.
15. Gambichler T, Terras S, Kreuter A, et al. Altered global methylation and hydroxymethylation status in vulvar lichen sclerosus: further support for epigenetic mechanisms. Br J Dermatol. 2014;170:687-93.
16. Fergus KB, Lee AW, Baradaran N, et al. Pathophysiology, Clinical Manifestations, and Treatment of Lichen Sclerosus: A Systematic Review. Urology. 2020;135:11-9. DOI:10.1016/j.urology.2019.09.034
17. Gambichler T, Skrygan M, Czempiel V, et al. Differential expression of connective tissue growth factor and extracellular matrix proteins in lichen sclerosus. J Eur Acad Dermatol Venereol. 2012;26(2):207-12. DOI:10.1111/j.1468-3083.2011.04037.x
18. Feig JL, Gribetz ME, Lebwohl MG. Chronic lichen sclerosus successfully treated with intralesional adalimumab. Br J Dermatol. 2016;174:687-9.
19. Lowenstein EB, Zeichner JA. Intralesional adalimumab for the treatment of refractory balanitis xerotica obliterans. JAMA Dermatol. 2013;149:23-4.
20. Al-Niaimi F, Lyon C. Peristomal lichen sclerosus: the role of occlusion and urine exposure? Br J Dermatol. 2013;168:643-6.
21. Doiron PR, Bunker CB. Obesity-related male genital lichen sclerosus. J Eur Acad Dermatol Venereol. 2017;31(5):876-9. DOI:10.1111/jdv.14035
22. Welsh B, Howard A, Cook K. Vulval itch. Aust Fam Physician. 2004;33(7):505-10.
23. Cooper SM, Gao XH, Powell JJ, Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol. 2004;140:702-6.
24. Khryanin AA, Markaryan DR, Garmanova TN, Bocharova VK. Perianal pruritus: possible causes and management of patients. Russian Journal of Clinical Dermatology and Venereology. 2020;19(6):891-8 (in Russian). DOI:10.17116/klinderma202019061891
25. Edmonds EV, 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;26:730-7.
26. Becker K. Lichen sclerosus in boys. Dtsch Arztebl Int. 2011;108(4):53-8.
27. Kostner S. EBM Service. Phimose mit Talgretetionszyste. Z Allg Med. 2014;90:339-41.
28. Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus. The course after puberty. J Reprod Med. 2002;47:706-9.
29. Kirtschig G. Lichen Sclerosus-Presentation, Diagnosis and Management. Dtsch Arztebl Int. 2016;113(19):337-43. DOI:10.3238/arztebl.2016.0337
30. Yalici-Armagan B, Bostan E, Akdogan N, Ersoy-Evans S. Paediatric lichen sclerosus et atrophicus: A retrospective analysis of 38 paediatric patients. Int J Clin Pract. 2021;75(10):e14661. DOI:10.1111/ijcp.14661
31. 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(4):839-53. DOI:10.1111/bjd.16241
32. Kirtschig G, Becker K, Günthert A, et al. Evidence-based (S3) guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol. 2015;10:e1-43.
34. 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.
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;67:1157-62.
35. Kreuter A, Kryvosheyeva Y, Terras S, et al. Association of autoimmune diseases with lichen sclerosus in 532 male and female patients. Acta Derm Venereol. 2013;93:238-41.
36. 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.
37. Chi CC, Kirtschig G, Baldo M, et al. Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev. 2011;12:CD008240.
38. Lee A, Bradford J, Fischer G. Long-term management of adult vulval lichen sclerosus. A prospective cohort study of 507 women. JAMA Dermatol. 2015;151(10):1061-7. DOI:10.1001/jamadermatol.2015.0643
39. Virgili A, Minghetti S, Borghi A, Corazza M. Proactive maintenance therapy with a topical corticosteroid for vulvar lichen sclerosus: preliminary results of a randomized study. Br J Dermatol. 2013;168:1316-24.
40. Bunker CB, Porter WM. Dermatoses of the male genitalia: squamous carcinoma and other malignant neoplasms. In: Rook's Textbook of Dermatology. Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer D, eds. 9th ed., vol. 3. Chichester: John Wiley&Sons, 2016;111:29-31.
41. Lewis F. Dermatoses of the female genitalia: inflammatory dermatoses of the vulva: lichen sclerosus. In: Rook's Textbook of Dermatology. Griffiths CEM, Barker J, Bleiker T, Chalmers R, Creamer D, eds. 9th ed., vol. 3. Chichester: John Wiley&Sons, 2016;112:6-9.
42. Turnbull N, Shim T, Patel N, et al. Primary melanoma of the penis in 3 patients with lichen sclerosus. JAMA Dermatol. 2016;152:226-7.
43. Khryanin AA, Sokolovskaya EV, Markaryan DR, et al. Pod red. AA Khryanina. Zabolevaniia kozhi promezhnosti i perianal'noi oblasti Moscow: GEOTAR-Media, 2022
(in Russian).
44. Lishai skleroticheskii i atroficheskii. Proekt klinicheskikh rekomendatsii 2020. Obshcherossiiskaia obshchestvennaia organizatsiia "Rossiiskoe obshchestvo dermatovenerologov i kosmetologov", Rossiiskoe obshchestvo akusherov-ginekologov (in Russian).
45. Virgili A, Minghetti S, Borghi A, Corazza M. Long-term maintenance therapy for vulvar lichen sclerosus: the results of a randomized study comparing topical vitamin E with an emollient. Eur J Dermatol. 2013;23:189-94.
46. Simonart T, Lahaye M, Simonart JM. Vulvar lichen sclerosus: effect of maintenance treatment with a moisturizer on the course of the disease. Menopause. 2008;15:74-7.
47. Dalziel KL. Effect of lichen sclerosus on sexual function and parturition. J Reprod Med. 1995;40:351-4.
1 ФГБОУ ВО «Новосибирский государственный медицинский университет» Минздрава России, Новосибирск, Россия;
2 РОО «Ассоциация акушеров-гинекологов и дерматовенерологов», Новосибирск, Россия;
3 ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Минздрава России, Санкт-Петербург, Россия
*khryanin@mail.ru
________________________________________________
Alexey A. Khryanin*1,2, Asia V. Sokolovskaia1, Valentina K. Bocharova3
1 Novosibirsk State Medical University, Novosibirsk, Russia;
2 Association of Obstetrician-Gynecologists and Dermatologists, Novosibirsk, Russia;
3 Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia
*khryanin@mail.ru