Lichen Sclerosus et Atrophicus 

  • Author: Jeffrey Meffert, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 21, 2011
 

Background

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy. Lichen sclerosus has both genital and extragenital presentations. It is reported under a variety of other appellations such as lichen sclerosus et atrophicus (dermatological literature), balanitis xerotica obliterans (glans penis presentation), and kraurosis vulvae (older description of vulvar presentation). An increased risk of squamous cell carcinoma may exist in genital disease, but the precise increase in risk and what cofactors (human papillomavirus infection or prior radiotherapy) may be involved are not yet completely defined. In large series, genital presentations, both vulvar and penile, outnumber extragenital reports by more than 5:1.

Next

Pathophysiology

Inflammation and altered fibroblast function in the papillary dermis leads to fibrosis of the upper dermis. Genital skin and mucosa are affected most frequently, but extragenital lichen sclerosus does occur, and even rare oral presentations are reported. Several studies have recently identified the presence of autoantibodies to the glycoprotein extracellular matrix protein 1 (ECM1).[1] This may be associated with histologic evidence of vasculitis in some cases and may lead to reduplication of the basement membrane in blood vessel walls.[2] The exact role of these antibodies and the changes seen in the microvasculature are currently under investigation.[3] The role that hypoxia and ischemia have in the initial cellular and vascular damage is supported by the finding of increased glut-1 and decreased vascular endothelial growth factor (VEGF) expression in affected skin.[4]

The effect of cell-mediated cytotoxicity has been better defined at the biochemical level.[5] Systemic disease or involvement of other organ systems, unlike scleroderma, is not described, although many more authors are describing lichen sclerosus and scleroderma as closely related entities; many cases of coexistent lichen sclerosus/scleroderma having been reported.

Previous
Next

Epidemiology

Frequency

International

The population rate is unknown. Male genital lichen sclerosus is seen almost exclusively in uncircumcised or incompletely circumcised men and boys. The rate of circumcision in a given population would thus influence the rate in this subset.

Mortality/Morbidity

Lichen sclerosus has no associated increased mortality unless the patient develops a malignancy in the area. Cancer arising in extragenital presentations is described only rarely and may be coincident with other factors. Many pediatric cases will improve with puberty, although other expressions of vulvodynia may persist. In contrast, some authors suggest the rate of spontaneous resolution may be lower than 25%.[6] Extragenital cases and many genital cases are asymptomatic except for the cosmetic aspect or pruritus. Recalcitrant cases, especially those associated with erosion or progressive scarring, may result in severe sexual dysfunction.

Race

Lichen sclerosus, both genital and extragenital, has no known racial predilection. A genetic predisposition, based on family clustering, is apparent.[7]

Sex

The male-to-female ratio is 1:6, with female genital cases making up the bulk of reports.

Age

Up to 15% of cases are in children with the majority being vulvar presentations. A study of foreskins submitted after therapeutic circumcision for phimosis revealed many cases of unrecognized lichen sclerosus. Extragenital lichen sclerosus is rare in children.

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Jeffrey Meffert, MD  Assistant 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, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Ponciano D Cruz Jr, MD  Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: RCTS Consulting fee Independent contractor; Mary Kay Cosmetics Honoraria Consulting; Galderma Grant/research funds Principal Investigator

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

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, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Chan I, Oyama N, Neill SM, Wojnarowska F, Black MM, McGrath JA. Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Clin Exp Dermatol. Sep 2004;29(5):499-504. [Medline].

  2. Regauer S, Liegl B, Reich O, Beham-Schmid C. Vasculitis in lichen sclerosus: an under recognized feature?. Histopathology. Sep 2004;45(3):237-44. [Medline].

  3. Kowalewski C, Kozlowska A, Chan I, et al. Three-dimensional imaging reveals major changes in skin microvasculature in lipoid proteinosis and lichen sclerosus. J Dermatol Sci. Jun 2005;38(3):215-24. [Medline].

  4. Li YZ, Wu Y, Zhang QH, Wang Y, Zhen JH, Li SL. Hypoxia-ischaemia is involved in the pathogenesis of vulvar lichen sclerosus. Clin Exp Dermatol. Dec 2009;34(8):e531-6. [Medline].

  5. Hunger RE, Bronnimann M, Kappeler A, Mueller C, Braathen LR, Yawalkar N. Detection of perforin and granzyme B mRNA expressing cells in lichen sclerosus. Exp Dermatol. May 2007;16(5):416-20. [Medline].

  6. Smith SD, Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol. Nov-Dec 2009;26(6):725-9. [Medline].

  7. 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. Feb 25 2010;[Medline].

  8. De Vito JR, Merogi AJ, Vo T, et al. Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases. J Cutan Pathol. Aug 1996;23(4):350-8. [Medline].

  9. Eisendle K, Grabner T, Kutzner H, Zelger B. Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch Dermatol. May 2008;144(5):591-8. [Medline].

  10. 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. Nov 2008;144(11):1432-5. [Medline].

  11. 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. Dec 10 2009;[Medline].

  12. Bjekic M, Sipetic S, Marinkovic J. Risk factors for genital lichen sclerosus in men. Br J Dermatol. Feb 2011;164(2):325-9. [Medline].

  13. 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. Mar 2008;137(1):56-60. [Medline].

  14. O'Mahony C, Yesudian PD, Stanley M. Imiquimod use in the genital area and development of lichen sclerosus and lichen planus. Int J STD AIDS. Mar 2010;21(3):219-21. [Medline].

  15. 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. Feb 2008;158(2):261-5. [Medline].

  16. Stucker M, Grape J, Bechara FG, Hoffmann K, Altmeyer P. The outcome after cryosurgery and intralesional steroid injection in vulvar lichen sclerosus corresponds to preoperative histopathological findings. Dermatology. 2005;210(3):218-22. [Medline].

  17. Kreuter A, Gambichler T. Narrowband UV-B phototherapy for extragenital lichen sclerosus. Arch Dermatol. Sep 2007;143(9):1213. [Medline].

  18. 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. Aug 2007;57(2 Suppl):S46-7. [Medline].

  19. Vermaat H, Smienk F, Rustemeyer T, Bruynzeel DP, Kirtschig G. Anogenital allergic contact dermatitis, the role of spices and flavour allergy. Contact Dermatitis. Oct 2008;59(4):233-7. [Medline].

  20. Fischer G, Rogers M. Treatment of childhood vulvar lichen sclerosus with potent topical corticosteroid. Pediatr Dermatol. May-Jun 1997;14(3):235-8. [Medline].

  21. 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. Nov 2009;145(11):1303-8. [Medline].

  22. Bornstein J, Heifetz S, Kellner Y, Stolar Z, Abramovici H. Clobetasol dipropionate 0.05% versus testosterone propionate 2% topical application for severe vulvar lichen sclerosus. Am J Obstet Gynecol. Jan 1998;178(1 Pt 1):80-4. [Medline].

  23. Lindhagen T. Topical clobetasol propionate compared with placebo in the treatment of unretractable foreskin. Eur J Surg. Dec 1996;162(12):969-72. [Medline].

  24. 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. Oct 2004;49(10):808-11. [Medline].

  25. 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. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.