Lichen Sclerosus

  • Ramón VirasoroEmail author
  • Gerald H. Jordan
Part of the Current Clinical Urology book series (CCU)


Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin. The incidence in the Western world is underreported. In men, LS peaks between the ages of 30 and 60 years. Both genders can be affected, although genital involvement is much more common in women. Possible etiologies for LS are the Koebner phenomenon, genetic susceptibility and autoimmunity, oxidative stress, and infection. The clinical presentation is as white patches that seem to coalesce into “plaques” that can affect the prepuce and glans. It is not clear whether LS spreads by direct extension into the fossa navicularis and a portion of the anterior urethra or if urethral involvement is secondary to LS-induced meatal stenosis and subsequent inflammation of the glands of Littré. The classic radiographic appearance of LS anterior urethral stricture is a saw-toothed pattern. Surgical management of LS is primarily by staged urethroplasty.


Human Leukocyte Antigen Human Leukocyte Antigen Class Lichen Planus Bullous Pemphigoid Alopecia Areata 
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  1. 1.
    Bernard P, Prost T, Durepaire N, et al. The major cicatricial pemphigoid antigen is a 180-KD protein that shows immunologic cross-reactivity with the bullous pemphigoid antigen. J Invest Dermatol. 1992;99:174–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Oyama N, Chan I, Neill SM, et al. Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet. 2003;362:118–23.PubMedCrossRefGoogle Scholar
  3. 3.
    Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol. 2003;28:128–33.PubMedCrossRefGoogle Scholar
  4. 4.
    Wallace HJ. Lichen sclerosus et atrophicus. Trans St John’s Dermatol Soc. 1971;57:9–30.Google Scholar
  5. 5.
    Kizer WS, Prarie T, Morey AF. Balanitis xerotica obliterans: epidemiologic distribution in an equal access health care system. South Med J. 2003;96:9.PubMedCrossRefGoogle Scholar
  6. 6.
    Nelson DM, Peterson AC. Lichen sclerosus: epidemiologic distribution in an equal access health care system. J Urol. 2011;185:522–5.PubMedCrossRefGoogle Scholar
  7. 7.
    Dogliotti M, Bentley-Phillips CB, Schmaman A. Lichen sclerosus et atrophicus in the Bantu. Br J Dermatol. 1974;91:81–5.PubMedCrossRefGoogle Scholar
  8. 8.
    Jacyk WK, Isaac F. Lichen sclerosus et atrophicus in Nigerians. J Natl Med Assoc. 1979;71:387–8.PubMedGoogle Scholar
  9. 9.
    Datta C, Dutta SK, Chaudauri A. Histopathological and immunological studies in a cohort of balanitis xerotica obliterans. J Indian Med Assoc. 1993;91:146–8.PubMedGoogle Scholar
  10. 10.
    Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol. 1995;32:393–416.PubMedCrossRefGoogle Scholar
  11. 11.
    Powell J, Wojnarowska F. Lichen sclerosus. Lancet. 1999;353:177–83.Google Scholar
  12. 12.
    Walkden V, Chia Y, Wojnarowska F. The association of squamous cell carcinoma of the vulva and lichen sclerosus: implications for management and follow up. J Obstet Gynaecol. 1997;17:551–3.PubMedCrossRefGoogle Scholar
  13. 13.
    Depasquale I, Park AJ, Bracka A. The treatment of balanitis xerotica obliterans. BJU Int. 2000;86:459–65.PubMedCrossRefGoogle Scholar
  14. 14.
    Nasca MR, Innocenzi D, Micali G. Penile cancer among patients with genital lichen sclerosus. J Am Acad Dermatol. 1999;41:911–4.PubMedCrossRefGoogle Scholar
  15. 15.
    Micali G, Nasca MR, Innocenzi D. Lichen sclerosus of the glans is significantly associated with penile carcinoma. Sex Transm Infect. 2001;77:226.PubMedCrossRefGoogle Scholar
  16. 16.
    Barbagli G, Palminteri E, Mirri F, et al. Penile carcinoma in patients with genital lichen sclerosus: a multicenter survey. J Urol. 2006;175:1359–63.PubMedCrossRefGoogle Scholar
  17. 17.
    Stühmer A. Balanitis xerotica obliterans (post operationem) und ihre beziehungem zur Kraurosis glandis et preaeputii. Penis Arch Derm Syph. 2008;156:613–23.CrossRefGoogle Scholar
  18. 18.
    Freeman C, Laymon CW. Balanitis xerotica obliterans. Arch Derm Syphilol. 1941;44:547–61.CrossRefGoogle Scholar
  19. 19.
    Laymon CW, Freeman C. Relationship of balanitis xerotica obliterans to lichen sclerosus et atrophicus. Arch Derm Syphilol. 1944;49:57–9.CrossRefGoogle Scholar
  20. 20.
    Weir RF. Icthyosis of the tongue and vulva. NY State J Med. 1875;4:246.Google Scholar
  21. 21.
    Hallopeau H. Lichen plan scléreux. Ann Derm Syph (2nd series). 1889;20:447–9.Google Scholar
  22. 22.
    Darier J. Lichen plan scléreux. Ann Derm Syph. 1892;23:833–7.Google Scholar
  23. 23.
    Unna PG. Kartenblattförmige sklerodermie. In: Lehrbuch der speziell path. Anatomie (8 lief). Berlin: A Hirschwald; 1894. p. 112.Google Scholar
  24. 24.
    Westberg F. Ein Fall von mit weissen flecken Einherhehender, bisher nicht bekannten. Dermatose Monatschr Prakt Dermatol. 1901;33:355–61.Google Scholar
  25. 25.
    Von Zombusch LR. Über Lichen albus, eine bisher unbeschrieben Erkrankung. Arch Dermatol Syph (Berlin). 1906;82:339.CrossRefGoogle Scholar
  26. 26.
    Montgomery FH, Ormsby OS. “White spot disease” (morphea guttata) and lichen planus sclerosus et atrophicus. J Cutan Dis. 1907;25:1–16.Google Scholar
  27. 27.
    Csillag J. Dermatitis lichenoides chronica atrophicans. Ikonographia Dermatol. 1909;4:147.Google Scholar
  28. 28.
    Breisky A. Über Kraurosis vulvae. Z Heilkr. 1885;6:69.Google Scholar
  29. 29.
    Friedrich EG. Lichen sclerosus. J Reprod Med. 1976;17:147–54.PubMedGoogle Scholar
  30. 30.
    Friedrich EG. New nomenclature for vulvar disease. Obstet Gynecol. 1976;47:122–4.Google Scholar
  31. 31.
    Howard A, Dean D, Cooper S, et al. Circulating basement membrane zone antibodies are found in lichen sclerosus of the vulva. Australas J Dermatol. 2004;45:12–5.PubMedCrossRefGoogle Scholar
  32. 32.
    Baldo M, Bailey A, Bhogal B, et al. T cells reactive with the NC16A domain of BP180 are present in vulval lichen sclerosus and lichen planus. J Eur Acad Dermatol Venereol. 2010;24:186–90.PubMedCrossRefGoogle Scholar
  33. 33.
    Sander CS, Ali I, Dean D, et al. Oxidative stress is implicated in the pathogenesis of lichen sclerosus. Br J Dermatol. 2004;151:627–35.PubMedCrossRefGoogle Scholar
  34. 34.
    Pass CJ. An unusual variant of lichen sclerosus et atrophicus: delayed appearance in a surgical scar. Cutis. 1984;33:405–6.PubMedGoogle Scholar
  35. 35.
    Cockerell EG, Knox JM, Rogers SF. Lichen sclerosus et atrophicus. Obstet Gynecol. 1960;15:554–9.PubMedGoogle Scholar
  36. 36.
    Milligan A, Graham-Brown RA, Burns DA. Lichen sclerosus et atrophicus following sunburn. Clin Exp Dermatol. 1988;13:36–7.PubMedCrossRefGoogle Scholar
  37. 37.
    Fallic ML, Faller G, Klauber GT. Balanitis xerotica obliterans in monozygotic twins. Br J Urol. 1997;79:810.CrossRefGoogle Scholar
  38. 38.
    Thomas RHM, Kennedy CT. The development of lichen sclerosus et atrophicus in monozygotic twin girls. Br J Dermatol. 1986;114:277–9.Google Scholar
  39. 39.
    Cox NH, Mitchell JNS, Wn M. Lichen sclerosus et atrophicus in non-identical female twins. Br J Dermatol. 1986;115:743–6.PubMedCrossRefGoogle Scholar
  40. 40.
    Shirer JA, Ray MC. Familial occurrence of lichen sclerosus et atrophicus. Arch Dermatol. 1987;123:485–8.PubMedCrossRefGoogle Scholar
  41. 41.
    Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138:370–9.PubMedGoogle Scholar
  42. 42.
    Meyrick Thomas RH, Ridley CM, Black MM, et al. The association between lichen sclerosus et atrophicus and autoimmune related diseases in males. Br J Dermatol. 1983;109:661–4.PubMedCrossRefGoogle Scholar
  43. 43.
    Meyrick Thomas RH, Ridley CM, Black MM, et al. The association between lichen sclerosus et atrophicus and autoimmune related disease in males: an addendum (letter). Br J Dermatol. 1984;111:371–2.CrossRefGoogle Scholar
  44. 44.
    Azurdia MR, Luzi G, Byren I, et al. Lichen sclerosus in adult men: a study to HLA association and susceptibility to autoimmune disease. Br J Dermatol. 1999;140:79–83.PubMedCrossRefGoogle Scholar
  45. 45.
    Garcia-Bravo B, Sánchez-Pedrero P, Rodríguez-Pichardo A, et al. Lichen sclerosus et atrophicus. A study of 76 cases and their relation to diabetes. J Am Acad Dermatol. 1988;19:482–5.PubMedCrossRefGoogle Scholar
  46. 46.
    Faergemann J. Lichen sclerosus et atrophicus generalisata, alopecia areata, and polymyalgia rheumatica found in the same patient. Cutis. 1979;23:757–8.PubMedGoogle Scholar
  47. 47.
    Cunliffe WJ, Newell DJ, Hall R, et al. Vitiligo, thyroid disease, and autoimmunity. Br J Dermatol. 1968;80:135–9.PubMedCrossRefGoogle Scholar
  48. 48.
    Ditkowsky SP, Falk AB, Baker N, et al. Lichen sclerosus et atrophicus in childhood. Am J Dis Child. 1956;91:52–4.Google Scholar
  49. 49.
    Panet-Raymond G, Dirard C. Lichen sclerosus et atrophicus. Can Med J. 1972;106:1332–4.Google Scholar
  50. 50.
    Lewis GM. Scleroderma: Lichen sclerosus et atrophicus? Arch Dermatol. 1961;84:146–8.CrossRefGoogle Scholar
  51. 51.
    Kahana M, Levy A, Schewach-Millet M, et al. Appearance of lupus erythematous in a patient with lichen sclerosus et atrophicus of the elbows (letter). J Am Acad Dermatol. 1985;12:127–9.PubMedCrossRefGoogle Scholar
  52. 52.
    Goolamali SK, Barnes EW, Irvine WJ, et al. Organ-specific antibodies in patients with lichen sclerosus. Br Med J. 1974;4:78–9.PubMedCentralPubMedCrossRefGoogle Scholar
  53. 53.
    Poskitt L, Wojnarowska F. Lichen sclerosus as a cutaneous manifestation of thyroid disease (letter). J Am Acad Dermatol. 1993;28:665.PubMedCrossRefGoogle Scholar
  54. 54.
    Edmonds EV, Oyama N, Chan I, Francis N, McGrath JA, Bunker CB. Extracellular matrix protein 1 autoantibodies in male genital lichen sclerosus. Br J Dermatol. 2011;165(1):218–9.PubMedCrossRefGoogle Scholar
  55. 55.
    Chan I. The role of extracellular matrix protein 1 in human skin. Clin Exp Dermatol. 2004;29:52–6.PubMedCrossRefGoogle Scholar
  56. 56.
    Hamada T. Lipoid proteinosis. Clin Exp Dermatol. 2002;27:624–9.PubMedCrossRefGoogle Scholar
  57. 57.
    Bunker CB. Re: Sanjay Kulkarni, Guido Barbagli, Deepak Kirpekar, et al. Lichen sclerosus of the male genitalia and urethra: surgical options and results in a multicenter international experience with 215 patients. Eur Urol. 2009;55:945–56.CrossRefGoogle Scholar
  58. 58.
    Buechner SA, Winkelmann RK, Lautenschlager S, et al. Localized scleroderma associated with Borrelia burgdorferi infection. Clinical, histologic, and immunohistochemical observations. J Am Acad Dermatol. 1993;29:190–6.PubMedCrossRefGoogle Scholar
  59. 59.
    Shono S, Imura M, Ota M, et al. Lichen sclerosus et atrophicus, morphea and coexistence of both diseases. Histological studies using lectins. Arch Dermatol. 1991;127:1352–6.PubMedCrossRefGoogle Scholar
  60. 60.
    Aberer E, Neumann R, Stanek G. Is localized scleroderma a Borrelia infection? Lancet. 1995;2:278.Google Scholar
  61. 61.
    Weide B, Waltz T, Garbe C. Is morphea caused by Borrelia burgdorferi? A review. Br J Dermatol. 2000;142:636–44.PubMedCrossRefGoogle Scholar
  62. 62.
    Edmonds E, Mavin S, Francis N, Ho-Yen D, Bunker C. Borrelia burgdorferi is not associated with genital lichen sclerosus in men. Br J Dermatol. 2009;160(2):459–60.PubMedCrossRefGoogle Scholar
  63. 63.
    McCammon KA, Stewart L, Metro M, Virasoro R. Anterior urethra – Lichen sclerosus. In: Jordan GH, Chapple C, Heyns C, editors. International consultation of urological disease on urethral strictures 2011, ICUD/SIU Press.Google Scholar
  64. 64.
    Akporiaye LE, Jordan GH, Devine Jr CJ. Balanitis xerotica obliterans (BXO). AUA Updates Series. 1997;16:162.Google Scholar
  65. 65.
    Virasoro R, Kahn AG, Secin FP. Balanitis xerotica obliterante (Article in Spanish). Rev Arg de Urol. 2003;68:125–30.Google Scholar
  66. 66.
    Chalmers RJG, Burton PA, Bennet RF. Lichen sclerosus et atrophicus. Arch Dermatol. 1984;120:1025–7.PubMedCrossRefGoogle Scholar
  67. 67.
    Riddell L, Edwards A, Sherrard J. Clinical features of lichen sclerosus in men attending a department of genitourinary medicine. Sex Transm Infect. 2000;76:311–3.PubMedCrossRefGoogle Scholar
  68. 68.
    Neill SM, Lewis FM, Tatnall FM, et al. British Association of dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol. 2010;163:672–82.PubMedCrossRefGoogle Scholar
  69. 69.
    Luesley D, Downey G. Topical tacrolimus in the management of lichen sclerosus. BJOG. 2006;113:832–4.PubMedCrossRefGoogle Scholar
  70. 70.
    Bunker CB. Comments on the British Association of Dermatologists guidelines for the management of lichen sclerosus. Br J Dermatol. 2011;164:878–99.CrossRefGoogle Scholar
  71. 71.
    Kulkarni S, Barbagli G, Kirpekar D, Mirr F, Lazzeri M. Lichen sclerosus of the male genitalia and urethra: surgical options and results in a multicenter international experience with 215 patients. Eur Urol. 2009;55:945–56.PubMedCrossRefGoogle Scholar
  72. 72.
    Morey AF, Lin HC, DeRosa CA, Griffith BC. Fossa navicularis reconstruction: impact of stricture length on outcomes and assessment of extended meatotomy (first stage Johanson) maneuver. Urol Int. 2007;79(1):8–12.Google Scholar
  73. 73.
    Malone P. A new technique for meatal stenosis in patients with lichen sclerosus. J Urol. 2004;172:949–52.PubMedCrossRefGoogle Scholar
  74. 74.
    Cohney BC. A penile flap procedure for the relief of meatal stricture. Br J Urol. 1963;35:182.PubMedCrossRefGoogle Scholar
  75. 75.
    Blandy JP, Tresidder GC. Meatoplasty. Br J Urol. 1967;39:633.PubMedCrossRefGoogle Scholar
  76. 76.
    Brannen GE. Meatal reconstruction. J Urol. 1976;116:319.PubMedGoogle Scholar
  77. 77.
    Devine Jr CJ. Surgery of the urethra. In: Walsh PC, Gittes RF, Perlmutter AD, et al., editors. Campbell’s urology. 5th ed. Philadelphia: WB Saunders; 1986. p. 2853.Google Scholar
  78. 78.
    De Sy WA. Aesthetic repair of meatal stricture. J Urol. 1984;132:678.PubMedGoogle Scholar
  79. 79.
    Duckett JW. Transverse preputial island flap technique for repair of severe hypospadias. Urol Clin North Am. 1980;7:423.PubMedGoogle Scholar
  80. 80.
    Virasoro R, Eltahawy EA, Jordan GH. Long-term follow-up for reconstruction of strictures of the fossa navicularis with a single technique. BJU Int. 2007;100(5):1143–5.PubMedGoogle Scholar
  81. 81.
    Jordan GH. Reconstruction of the fossa navicularis. J Urol. 1987;138:102.PubMedGoogle Scholar
  82. 82.
    Venn SN, Mundy AR. Urethroplasty for balanitis xerotica obliterans. Br J Urol. 1998;81:735.PubMedCrossRefGoogle Scholar
  83. 83.
    Armenakas NA, Morey AF, McAninch JW. Reconstruction of resistant strictures of the fossa navicularis and meatus. J Urol. 1998;160:359.PubMedCrossRefGoogle Scholar
  84. 84.
    Nahas BW, Hart AJ. Letter to the editor. Re: reconstruction of resistant strictures of the fossa navicularis and meatus. J Urol. 1999;161:924.PubMedCrossRefGoogle Scholar
  85. 85.
    Bracka A. Letter to the editor. Re: reconstruction of resistant strictures of the fossa navicularis and meatus. J Urol. 1999;162:1389.PubMedCrossRefGoogle Scholar
  86. 86.
    Burger RA. The buccal mucosal graft for urethral reconstruction: a preliminary report. J Urol. 1992;147(3):662–4.PubMedGoogle Scholar
  87. 87.
    Simonato A, Gregori A, Lissiani A, et al. The tongue as an alternative donor site for graft urethroplasty: a pilot study. J Urol. 2006;175:589–92.PubMedCrossRefGoogle Scholar
  88. 88.
    Das SK, et al. Lingual mucosal graft urethroplasty for anterior urethral strictures. Urology. 2009;73:105–8.PubMedCrossRefGoogle Scholar
  89. 89.
    Dubey D, Sehgal A, Srivastava A, Mandhani A, Kapoor R, Kumar A. Buccal mucosal urethroplasty for balanitis xerotica obliterans related urethral strictures: the outcome of 1 and 2-stage techniques. J Urol. 2005;173(2):463–6.PubMedCrossRefGoogle Scholar

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© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of UrologyEastern Virginia Medical SchoolNorfolkUSA

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