Diagnostic Considerations

The presentation of whitening of the skin with evidence of cutaneous atrophy and either loss of normal genital architecture in women or progressive phimosis in men may be enough to make the diagnosis. Atypical history and examination findings may lead to a broadened differential diagnosis.

Differential Diagnoses

Workup

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Terlou A, Santegoets LA, van der Meijden WI, Heijmans-Antonissen C, Swagemakers SM, van der Spek PJ, 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 Mar. 132 (3 Pt 1):658-66. [QxMD MEDLINE Link].
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Baldo M, Bailey A, Bhogal B, Groves RW, Ogg G, Wojnarowska F. T cells reactive with the NC16A domain of BP180 are present in vulval lichen sclerosus and lichen planus. J Eur Acad Dermatol Venereol. 2010 Feb. 24(2):186-90. [QxMD MEDLINE Link].
Zollinger T, Mertz KD, Schmid M, Schmitt A, Pfaltz M, Kempf W. Borrelia in granuloma annulare, morphea and lichen sclerosus: a PCR-based study and review of the literature. J Cutan Pathol. 2009 Dec 10. [QxMD MEDLINE Link].
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 Nov. 144(11):1432-5. [QxMD MEDLINE Link].
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 Derm Venereol. 2013 Mar 27. 93(2):238-41. [QxMD MEDLINE Link].
Bjekic M, Sipetic S, Marinkovic J. Risk factors for genital lichen sclerosus in men. Br J Dermatol. 2011 Feb. 164(2):325-9. [QxMD MEDLINE Link].
Gunthert AR, Faber M, Knappe G, Hellriegel S, Emons G. Early onset vulvar Lichen Sclerosus in premenopausal women and oral contraceptives. Eur J Obstet Gynecol Reprod Biol. 2008 Mar. 137(1):56-60. [QxMD MEDLINE Link].
Walls AC, Qureshi AA. Psoriasis and concomitant fibrosing disorders: lichen sclerosus, morphea, and systemic sclerosis. J Am Acad Dermatol. 2012 Nov. 67(5):1079-83. [QxMD MEDLINE Link].
Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus: an increasingly common problem. J Am Acad Dermatol. 2001 May. 44 (5):803-6. [QxMD MEDLINE Link].
Leibovitz A, Kaplun V V, Saposhnicov N, Habot B. Vulvovaginal examinations in elderly nursing home women residents. Arch Gerontol Geriatr. 2000 Aug 1. 31 (1):1-4. [QxMD MEDLINE Link].
Sherman V, McPherson T, Baldo M, Salim A, Gao XH, Wojnarowska F. The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study. J Eur Acad Dermatol Venereol. 2010 Feb 25. [QxMD MEDLINE Link].
Kirtschig G, Becker K, Günthert A, Jasaitiene D, Cooper S, Chi CC, et al. Evidence-based (S3) Guideline on (anogenital) Lichen sclerosus. J Eur Acad Dermatol Venereol. 2015 Oct. 29 (10):e1-43. [QxMD MEDLINE Link].
Smith SD, Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol. 2009 Nov-Dec. 26(6):725-9. [QxMD MEDLINE Link].
Diwan NG, Nair PA. Extragenital lichen sclerosus et atrophicus along the lines of Blaschko. Indian Dermatol Online J. 2015 Sep-Oct. 6 (5):342-4. [QxMD MEDLINE Link].
Jones RW, Sadler L, Grant S, Whineray J, Exeter M, Rowan D. Clinically identifying women with vulvar lichen sclerosus at increased risk of squamous cell carcinoma: a case-control study. J Reprod Med. 2004 Oct. 49(10):808-11. [QxMD MEDLINE Link].
Olejek A, Kozak-Darmas I, Kellas-Sleczka S, Jarek A, Wiczkowski A, Krol W, et al. Chlamydia trachomatis infection in women with lichen sclerosus vulvae and vulvar cancer. Neuro Endocrinol Lett. 2009. 30(5):671-4. [QxMD MEDLINE Link].
Borghi A, Corazza M, Minghetti S, Bianchini E, Virgili A. Dermoscopic Features of Vulvar Lichen Sclerosus in the Setting of a Prospective Cohort of Patients: New Observations. Dermatology. 2016. 232 (1):71-7. [QxMD MEDLINE Link].
Prowse DM, Ktori EN, Chandrasekaran D, Prapa A, Baithun S. Human papillomavirus-associated increase in p16INK4A expression in penile lichen sclerosus and squamous cell carcinoma. Br J Dermatol. 2008 Feb. 158(2):261-5. [QxMD MEDLINE Link].
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Lacarrubba F, Pellacani G, Verzì AE, Pippione M, Micali G. Extragenital lichen sclerosus: clinical, dermoscopic, confocal microscopy and histologic correlations. J Am Acad Dermatol. 2015 Jan. 72(1 Suppl):S50-2. [QxMD MEDLINE Link].
Neill SM, Lewis FM, Tatnall FM, Cox NH, British Association of Dermatologists. British Association of Dermatologists' guidelines for the management of lichen sclerosus 2010. Br J Dermatol. 2010 Oct. 163 (4):672-82. [QxMD MEDLINE Link].
Casey GA, Cooper SM, Powell JJ. Treatment of vulvar lichen sclerosus with topical corticosteroids in children: a study of 72 children. Clin Exp Dermatol. 2015 Apr. 40(3):289-92. [QxMD MEDLINE Link].
Funaro D, Lovett A, Leroux N, Powell J. 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. 2014 Jul. 71(1):84-91. [QxMD MEDLINE Link].
Borghi A, Corazza M, Minghetti S, Toni G, Virgili A. Avocado and soybean extracts as active principles in the treatment of mild-to-moderate vulvar lichen sclerosus: results of efficacy and tolerability. J Eur Acad Dermatol Venereol. 2014 Sep 3. [QxMD MEDLINE Link].
Feig JL, Gribetz ME, Lebwohl MG. Chronic lichen sclerosus successfully treated with intralesional adalimumab. Br J Dermatol. 2016 Mar. 174 (3):687-9. [QxMD MEDLINE Link].
Bousema MT, Romppanen U, Geiger JM, Baudin M, Vähä-Eskeli K, Vartiainen J, et al. Acitretin in the treatment of severe lichen sclerosus et atrophicus of the vulva: a double-blind, placebo-controlled study. J Am Acad Dermatol. 1994 Feb. 30 (2 Pt 1):225-31. [QxMD MEDLINE Link].
Borghi A, Corazza M, Minghetti S, Virgili A. Topical tretinoin in the treatment of vulvar lichen sclerosus: an advisable option?. Eur J Dermatol. 2015 Oct 1. 25 (5):404-9. [QxMD MEDLINE Link].
Terras S, Gambichler T, Moritz RK, Stücker M, Kreuter A. UV-A1 phototherapy vs clobetasol propionate, 0.05%, in the treatment of vulvar lichen sclerosus: a randomized clinical trial. JAMA Dermatol. 2014 Jun. 150(6):621-7. [QxMD MEDLINE Link].
Teske NM, Jacobe HT. Phototherapy for sclerosing skin conditions. Clin Dermatol. 2016 Sep-Oct. 34 (5):614-22. [QxMD MEDLINE Link].
Kreuter A, Gambichler T. Narrowband UV-B phototherapy for extragenital lichen sclerosus. Arch Dermatol. 2007 Sep. 143(9):1213. [QxMD MEDLINE Link].
Romero A, Hernandez-Nunez A, Cordoba-Guijarro S, Arias-Palomo D, Borbujo-Martinez J. Treatment of recalcitrant erosive vulvar lichen sclerosus with photodynamic therapy. J Am Acad Dermatol. 2007 Aug. 57(2 Suppl):S46-7. [QxMD MEDLINE Link].
Kreuter A, Tigges C, Gaifullina R, Kirschke J, Altmeyer P, Gambichler T. Pulsed high-dose corticosteroids combined with low-dose methotrexate treatment in patients with refractory generalized extragenital lichen sclerosus. Arch Dermatol. 2009 Nov. 145(11):1303-8. [QxMD MEDLINE Link].
Ellis E, Fischer G. Prepubertal-Onset Vulvar Lichen Sclerosus: The Importance of Maintenance Therapy in Long-Term Outcomes. Pediatr Dermatol. 2015 Jul-Aug. 32 (4):461-7. [QxMD MEDLINE Link].
Burger MP, Obdeijn MC. Complications after surgery for the relief of dyspareunia in women with lichen sclerosus. A case series. Acta Obstet Gynecol Scand. 2016 Jan 22. [QxMD MEDLINE Link].
Flynn AN, King M, Rieff M, Krapf J, Goldstein AT. Patient Satisfaction of Surgical Treatment of Clitoral Phimosis and Labial Adhesions Caused by Lichen Sclerosus. Sex Med. 2015 Dec. 3 (4):251-5. [QxMD MEDLINE Link].
[Guideline] Akel R, Fuller C. Updates in lichen sclerosis: British Association of Dermatologists guidelines for the management of lichen sclerosus 2018. Br J Dermatol. 2018 Apr. 178 (4):823-824. [QxMD MEDLINE Link].

Media Gallery

Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.
Typical lichen sclerosus histology demonstrating homogenized edematous papillary (upper) dermis and effaced epidermis.
More advanced vulvar lichen sclerosus; eroded areas need to be carefully examined and a biopsy sample should be taken to exclude coexistent squamous cell carcinoma. Courtesy of Wilford Hall Medical Center Dermatology slide files.
Male genital lichen sclerosus may present with a sclerotic ring at the edge of the prepuce or anywhere on the glans itself. Advanced disease at the urethral os may lead to urinary obstruction. Courtesy of Wilford Hall Medical Center Dermatology Slide files.
Late lichen sclerosus may show less edema in the upper dermis and more sclerosis throughout the dermis. Involvement of the lower dermis or fat may occur in lichen sclerosus/scleroderma overlap presentations.

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Author

Lisa K Pappas-Taffer, MD Assistant Professor of Clinical Dermatology, University of Pennsylvania School of Medicine; Attending Physician, Hospital of the University of Pennsylvania; Staff Physician, Veterans Affairs Medical Center

Lisa K Pappas-Taffer, MD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology, Atlantic Dermatologic Society, Medical Dermatology Society, Philadelphia Dermatological Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Jeffrey Meffert, MD † Former Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Joshua A Zeichner, MD Assistant Professor, Director of Cosmetic and Clinical Research, Mount Sinai School of Medicine; Chief of Dermatology, Institute for Family Health at North General

Joshua A Zeichner, MD is a member of the following medical societies: American Academy of Dermatology, National Psoriasis Foundation

Disclosure: Received consulting fee from Valeant for consulting; Received grant/research funds from Medicis for other; Received consulting fee from Galderma for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Pharmaderm for consulting; Received consulting fee from Onset for consulting.

Lichen Sclerosus Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

Tables

Contributor Information and Disclosures

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