A Review Article on Lichen Sclerosus, Affecting Ano-Genital Area in Females
Main Article Content
Abstract
Objective: To collect detailed up-to-date knowledge, using systematic review of the medical literature, that is specific to the Dermatologic condition of Lichen Sclerosus (LS), which mainly affects the ano-genital area of female patients. This article will review: The disease epidemiology, pathogenesis, clinical features, complications, differential diagnosis, diagnostic methods, histopathology, psychosocial impact, and treatment options.
Background: LS is a chronic dermatologic condition. The condition was described at the end of the 19th century. LS incidence is higher in females than in males, affecting mainly the female ano-genital area. Diverse and crippling complications exist, including malignant transformation. Clinical features progress towards severe vulvar anatomic distortion, which results in devastating psychosocial implications. Mainstay treatments are both medical and surgical; recent randomized controlled trials promote the use of topical Calcineurin inhibitors.
Methods: I used a meticulous strategy across databases, led by pre-specified keywords, followed by the application of database-specific filters to scrutinize the hierarchy of available medical literature, from guidelines, systematic reviews and randomized controlled trials to medical papers with weak evidence. Some articles were requested from the British library.
Results: I used around 70 references; the most relevant data were extracted. This article is divided into sections of topics, starting from an introduction to a conclusion.
Conclusion: This article will enable the medical researcher to obtain a detailed perspective of the condition LS. Thus, a researcher can seek strongest evidence for his original research.
Article Details
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
Abdelbaky, A. M., Aluru, P., Keegan, P., & Greene, D. R. (2012). Development of male genital lichen sclerosus in penile reconstruction skin grafts after cancer surgery: An unreported complication. BJU Int, 109(5), 776–9.
Aberer, E., Kollegger, H., Kristoferitsch, W., & Stanek, G. (1988). Neuroborreliosis in morphea and lichen sclerosus et atrophicus. J Am Acad Dermatol, 19, 820–5.
Al-niaimi, F., & Lyon, C. (2013). Peristomal lichen sclerosus: The role of occlusion and urine exposure? Br J Dermatol, 168(3), 643–6.
Ansink, A. C., Krul, M. R. L., de Weger, R. A., Kleyne, J. A., Pijpers, H., Van Tinteren H., … Heintz, A. P. (1994). Human papillomavirus, lichen sclerosus, and squamous cell carcinoma of the vulva: Detection and prognostic significance. Gynecol Oncol, 52, 180–4.
August, P. J., & Milward, T. M. (1980). Cryosurgery in the treatment of lichen sclerosus et atrophicus of the vulva. Br J Dermatol, 103, 667–70.
Bonin, S., Gubertini, N., & Trevisan, G. (2011). Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis. Dermatol Rep, 3(2).
Bousema, M. T., Romppanen, U., Geiger, J. M., Baudin, M., Vähä-Eskeli, K., Vartiainen, J., & Vuopala, S. (1994). Acitretin in the treatment of severe lichen sclerosus et atrophicus of the vulva: A double-blind, placebo-controlled study. J Am Acad Dermatol, 30, 225–31.
Brown, B. C., McKenna, S. P., Siddhi, K., McGrouther, D. A., & Bayat. A. (2008). The hidden cost of skin scars: Quality of life after skin scarring, J Plast Reconstr Aesthet Surg, 61(9), 1049–58.
Bunker, C. B., & Neill, S. M. (2010). The genital, perianal and umbilical regions. In T. B., S. B., N. C., C. G. (Eds.), Rook’s textbook of dermatology (8th ed.) (pp. 3705–3806). Oxford, UK: Wiley-Blackwell.
Bussen, S. S. (2009). Melanocytic proliferations associated with lichen sclerosus in adolescence. Arch Gynecol Obstet, 280(6), 1039–40.
Carli, P., Cattaneo, A., Taddei, G., & Gianotti, B. (1992). Topical cyclosporine in the treatment of vulvar lichen sclerosus: Clinical, histologic and immunohistochemical findings. Arch Dermatol, 128, 1548–9.
Christman, M. S., Chen, J. T., & Holmes, N. M. (2009). Obstructive complications of lichen sclerosus. J Pediatr Urol, 5(3), 165–9.
Cox, N. H., Mitchell, J. N. S., & Morley, W. N. (1986). Lichen sclerosus et atrophicus in non-identical female twins. Br J Dermatol, 115, 743–6.
Darier, J. (1892). Lichen sclerosus. Ann Dermatol Syphiligr, 3, 833.
Drut, R. M., Gómez, M. A., Drut, R., & Lojo, M. M. (1998). Human papillomavirus is present in some cases of childhood penile lichen sclerosus: An in situ hybridization and SP-PCR study. Pediatr Dermatol, 15, 85–90.
eMedicine (n.d.). http://emedicine.com
Evers, A. W., Duller, P., Van de kerkhof, P. C., van der Valk, P. G., de Jong, E.M., Gerritsen, M. J., … Kraaimaat, F. W. (2008). The Impact of Chronic Skin Disease on Daily Life (ISDL): A generic and dermatology-specific health instrument. Br J Dermatol, 158(1), 101–8.
Farrell, A. M., Dean, D., Millard, P. R., Charnock, F. M., & Wojnarowska, F. (2006). Cytokine alterations in lichen sclerosus: An immunohistochemical study. Br J Dermatol, 155, 931–40.
Farrell, A. M., Marren, P. M., & Wojnarowska, F. (2000). Genital lichen sclerosus associated with morphoea or systemic sclerosis: Clinical and HLA characteristics. Br J Dermatol, 143, 598–603.
Fischer, G., & Rogers, M. (1997). Treatment of childhood vulvar lichen sclerosus with potent topical corticosteroid. Pediatr Dermatol, 14, 235–8.
Garzon, M. C., & Paller, A. S. (1999). Ultrapotent topical corticosteroid treatment of childhood lichen sclerosus. Arch Dermatol, 135, 525–8.
Goodfield, M. J. D., Jones, S. K., & Veale, D. J. (2010). The ‘connective tissue diseases’. In T. B., S. B., N. C., C. G. (Eds.), Rook’s textbook of dermatology (8th ed.) (pp. 2475–612). Oxford, UK: Wiley-Blackwell.
Guerrero, D., Guarch, R., Ojer, A., Casas, J. M., Méndez-Meca, C., Esteller, M., … Puras, A. (2011). Differential hypermethylation of genes in vulvar cancer and lichen sclerosus coexisting or not with vulvar cancer. Int J Cancer, 128(12), 2853–64.
Günthert, A. R., Faber, M., Knappe, G., Hellriegel, S., & Emons, G. (2008). Early onset vulvar lichen sclerosus in premenopausal women and oral contraceptives. Eur J Obstet Gynecol Reprod Biol, 137(1), 56–60.
Gurumurthy, M., Morah, N., Gioffre, G., & Cruickshank, M. E. (2012). The surgical management of complications of vulvar lichen sclerosus. Eur J Obstet Gynecol Reprod Biol, 162(1), 79–82.
Hagedorn, M., Buxmeyer, B., Schmitt, Y., & Bauknecht, T. (2002). Survey of genital lichen sclerosus in women and men. Arch Gynecol Obstet, 266(2), 86–91.
Hallopeau, H. (1887). Lichen plan, atrophique. Ann Dermatol Syphiligr, 8, 790–4.
Hassanein, A. M., Mrstik, M. E., Hardt, N. S., Morgan, L. A., & Wilkinson, E. J. (2004). Malignant melanoma associated with lichen sclerosus in the vulva of a 10-year-old. Pediatr Dermatol, 21(4), 473–6.
Hillemans, P., Untch, M., Prove, F., Baumgartner, R., Hillemanns, M., & Korell, M. (1999). Photodynamic therapy of vulvar lichen sclerosus with 5-aminolevulinic acid. Obstet Gynecol, 93, 71–4.
Hong, J., Koo, B., & Koo, J. (2008). The psychosocial and occupational impact of chronic skin disease. Dermatol Ther, 21(1), 54–9.
Howard, A., Dean, D., Cooper, S., Kirtshig, G., & Wojnarowska, F. (2004). Circulating basement membrane zone antibodies are found in lichen sclerosus of the vulva. Australas J Dermatol, 45, 12–15.
Kartamaa, M., & Reitamo, S. (1997). Treatment of lichen sclerosus with carbon dioxide laser vaporization. Br J Dermatol, 136, 356–9.
Kirtschig, C. C., Baldo, G., Brackenbury, M., Lewis, F., & Wojnarowska, F. (2011). Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev, (12):CD008240.Chi.
Kiss, A., Kiraly, L., Kutasy, B., & Merksz, M. (2005). High incidence of balanitis xerotica obliterans in boys with phimosis: Prospective 10-year study. Pediatr Dermatol, 22, 305–8.
Koblenzer, C. S. (2005). The emotional impact of chronic and disabling skin disease: A psychoanalytic perspective. Dermatol Clin, 23(4), 619–27.
Lascano, E. F., Montes, L. F., & Mazzini, M. A. (1964). Tissue changes in lichen sclerosus in children following local application of oestrogens. Br J Dermatol, 76, 496–7.
Mann, P. R., & Cowan, M. A. (1973). Ultrastructural changes in four cases of lichen sclerosus et atrophicus. Br J Dermatol, 89, 223–31.
Meyrick Thomas, R. H., & Kennedy, C. T. C. (1986). The development of lichen sclerosus et atrophicus in monozygotic twin girls. Br J Dermatol, 114, 377–9.
Meyrick-Thomas, R. H., Ridley, C. M., McGibbon, D. H., & Black, M. M. (1988). Lichen sclerosus and autoimmunity – A study of 350 women. Br J Dermatol, 118, 41–46.
Mills, S. E. 2009. Sternberg’s diagnostic surgical pathology. USA: LWW.
Nasca, M. R., Innocenzi, D., & Micali, G. (2006). Association of penile lichen sclerosus and oncogenic human papilloma virus infection. Int J Dermatol, 45, 681–3.
Neill, S., Tatnall, F., & Cox, N. (2002). Guidelines for the management of lichen sclerosus. Br J Dermatol, 147(4), 640–649.
Neill, S. M., Lewis, F. M., Tatnall, F. M., & Cox, N. H. (2010). British Association of Dermatologists' guidelines for the management of lichen sclerosus 2010. Br J Dermatol, 163(4), 672–82.
Oyama, N., Chan, I., Neill, S. M., Hamada, T., South, A. P., Wessagowit, V., … McGrath, J. A. (2003). Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Lancet, 362, 118–23.
Penneys, N. S. (1984). Treatment of lichen sclerosus with potassium paraaminobenzoate. J Am Acad Dermatol, 10, 1039–42.
Pinelli, S., D'erme, A. M., & Lotti, T. (2013). Management of sexual dysfunction due to vulvar lichen sclerosus in postmenopausal women. Dermatol Ther, 26(1), 79–82.
Powell, J., & Wojnarowska, F. (2001). Childhood vulvar sclerosus: An increasingly common problem. J Am Acad Dermatol, 44, 803–6.
Prowse, D. M., Ktori, E. N., Chandrasekaran, D., Prapa, A., & Baithun, S. (2008). Human papillomavirus-associated increase in p16INK4A expression in penile lichen sclerosus and squamous cell carcinoma. Br J Dermatol, 158(2), 261–5.
Pugliese, J. M., Morey, A. F., & Peterson, A. C. (2007). Lichen sclerosus: Review of the literature and current recommendations for management. J Urol, 178, 2268–76.
Regauer, S., Reich, O., & Beham-Schmid, C. (2002). Monoclonal gamma-T-cell receptor rearrangement in vulvar lichen sclerosus and squamous cell carcinomas. Am J Pathol, 160, 1035–1045.
Regauer, S., & Reich, O. (2005). Early vulvar lichen sclerosus: A histological challenge. Histopathology, 47, 340–7.
Ridley, C. M. (1987). Lichen sclerosus et atrophicus. Arch Dermatol, 23, 457–60.
Ruan, L., Xie, Z., Wang, H., Jiang, J., Shi, H., & Xu, J. (2010). High-intensity focused ultrasound treatment for non-neoplastic epithelial disorders of the vulva. Int J Gynaecol Obstet, 109(2), 167–70.
Sander, C. S., Ali, I., Dean, D., Thiele, J. J., & Wojanrowaska, F. (2004). Oxidative stress is implicated in the pathogenesis of lichen sclerosus. Br J Dermatol, 151, 627–35.
Scurry, B., & Cohen, A. (1998). Ki67 expression in lichen sclerosus of vulva in patients with and without associated squamous cell carcinoma. Histopathology, 32(5), 399–404.
Senturk, N., Aydin, F., Birinci, A., Yildiz, L., Canturk, T., Durupinar, B., & Turanli, A. (2004). Coexistence of HLA B08 and HLA B18 in four siblings with lichen sclerosus. Dermatology, 208, 64–6.
Shasi, P. B., Chapman, H. T., Evans, D. T., & Jaleel, H. (2010). Psychological and psychiatric morbidity in lichen sclerosus in a cohort recruited from a genitourinary medicine clinic. Int J STD AIDS, 21(1), 17–8.
Shelley, W. B., Shelley, E. D., & Amurao, C. V. (2006). Treatment of lichen sclerosus with antibiotics. Int J Dermatol, 45(9), 1104–6.
Sherman, V., McPherson, T., Baldo, M., Salim, A., Gao, X. H., & Wojnarowska, F. (2010). The high rate of familial lichen sclerosus suggests a genetic contribution: An observational cohort study. J Eur Acad Dermatol Venereol.
Simpkin, S., & Oakley, A. (2007). Clinical review of 202 patients with vulval lichen sclerosus: A possible association with psoriasis. Australas J Dermatol, 48, 28–31.
Smith, S. D., & Fischer, G. (2009). Paediatric vulval lichen sclerosus. Australas J Dermatol, 50(4), 243–8.
Stanley L., Vinay, K., & Ramzi, S. (2010). Robbins and Cotran pathologic basis of disease (8th ed.). Philadelphia, PA: Saunders/Elsevier.
Strittmatter, H. J., Hengge, U. R., & Blecken, S. R. (2006). Calcineurin antagonists in vulvar lichen sclerosus. Arch Gynecol Obstet, 274(5), 266–70.
Taylor, A. H., Guzail, M., & Al-azzawi, F. (2008). Differential expression of oestrogen receptor isoforms and androgen receptor in the normal vulva and vagina compared with vulval lichen sclerosus and chronic vaginitis. Br J Dermatol, 158(2), 319–28.
Thomas, B. (2009). Disorders of the genitalia, perineum, and anus. In W. Klaus & A. Richard (Eds.), Fitzpatrick's color atlas and synopsis of clinical dermatology (6th ed.) (pp. 1046–66). USA: McGraw-Hill Professional.
Van de nieuwenhof, H. P., Meeuwis, K. A., Nieboer, T. E., Vergeer, M. C., Massuger, L. F., & De hullu, J. A. (2010). The effect of vulvar lichen sclerosus on quality of life and sexual functioning. J Psychosom Obstet Gynaecol, 31(4), 279–84.
Wallace, H. J., & Whimster, I. (1951). Vulval atrophy and leukoplakia. Br J Dermatol, 63, 241–57.
Warrington, S. A., & de San Lazaro, C. (1996). Lichen sclerosus et atrophicus and sexual abuse. Arch Dis Child, 75, 512–6.
Wehbe-alamah, H., Kornblau, B. L., Haderer, J., & Erickson, J. (2012). Silent no more! The lived experiences of women with lichen sclerosis. J Am Acad Nurse Pract, 24(8), 499–505.
Wikipedia, the Free Encyclopedia. (n.d.). Lichen sclerosus. Retrieved from http://en.wikipedia.org/wiki/Lichen_sclerosus#Psychology