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Lichen Sclerosus et Atrophicus Clinical Presentation

  • Author: Jeffrey Meffert, MD; Chief Editor: William D James, MD  more...
 
Updated: Feb 24, 2016
 

History

Extragenital lichen sclerosus may be asymptomatic or it may itch, although itching is not usual. Vulvar lichen sclerosus usually presents with progressive pruritus, dyspareunia, dysuria, or genital bleeding. Penile lichen sclerosus usually is preceded by pruritus but may present with sudden phimosis of previously retractable foreskin, and urinary obstruction can result.

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Physical

Pertinent physical findings are limited to the skin.

Skin primary lesion

Lichen sclerosus usually begins as white, polygonal papules that coalesce into plaques. Evenly spaced dells or comedolike plugs correspond to obliterated appendiceal ostia. These may be easily identified with dermoscopy, keeping in mind that other conditions such as chronic cutaneous lupus may also show follicular plugs. With time, the plugs and dells will disappear and leave a smooth, porcelain-white plaque. Skin color is white, often with a shiny porcelain appearance. Telangiectases and follicular plugs may be seen. The size of the plaque or plaques may vary widely from a few millimeters resembling lichen nitidus to the entire upper trunk.

Vulvar lichen sclerosus may progress to gradual obliteration of the labia minora and stenosis of the introitus. The most common variation occurs when the inflammation is intense enough to cause separation of a large area of epidermis, creating blisters or large, occasionally hemorrhagic, bullae. Because this occurs more often in genital cases, it may be confused with the trauma of sexual abuse or other genital ulcerative disease.

Given the high frequency of genital mucosal disease, it is surprising that more oral cases have not been reported. Those rare cases reported are usually seen in patients with widespread, generalized lichen sclerosus. Some believe that many cases of clinically diagnosed lichen planus may actually be lichen sclerosus and that isolated oral mucosal lichen sclerosus may not be as rare as is thought.

Skin distribution

Extragenital lesions may occur anywhere on the body, although the back and shoulders are reported most commonly. Note the image below:

Extragenital lichen sclerosus demonstrating coales Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.

Female genital lesions may be confined to the labia majora but usually involve and eventually obliterate the labia minora and stenose the introitus. Often, an hourglass, butterfly, or figure-8 pattern involves the perivaginal and perianal areas, with minimal involvement of the perineum in between. Note the images below:

Lichen sclerosus demonstrating classic hourglass o Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
More advanced vulvar lichen sclerosus; eroded area 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 lesions usually are confined to the glans penis and the prepuce or foreskin remnants. Penile shaft involvement is much less common, and scrotal involvement is rare. The initial manifestation may be a sclerotic ring at the prepuce edge. Note the image below:

Male genital lichen sclerosus may present with a s 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.

The isomorphic (Koebner) phenomenon is described in this condition, with the resultant lesions in old surgical scars, burn scars, sunburned areas, and areas subject to repeated trauma. Distribution of lichen sclerosus along the lines of Blaschko was described in an extragenital case.

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Causes

The etiology and pathogenesis of lichen sclerosus is unknown but may include genetic, infectious, environmental, and hormonal factors.

Several older studies have linked borrelial or other infections with lichen sclerosus, yet most other studies have disputed this, with polymerase chain reaction–based studies showing no increased incidence of borrelial infection.[4]

Genetic and autoimmune factors have been explored without identification of consistent, reproducible patterns, although autoantibodies to ECM1 have now been reported by several independent authors. Increased circulating autoantibodies may be as high as 28%, comparable to the rate seen in bullous lichen planus.[5] Baldo et al reported that more than 40% of vulvar lichen sclerosus and lichen planus patients have reactive T cells to the NC16A domain of bullous pemphigoid antigen 180[6] ; however, other studies suggest that the level of autoantibodies is poorly correlated to disease activity and response to treatment. Women with lichen sclerosus have a higher rate of associated autoimmune disease (odds ratio, 4.3), especially for autoimmune thyroid disease, compared with men.[7]

Local irritation or trauma seems to play a role in some cases of lichen sclerosus, especially in genetically predisposed individuals.[8] However, the sequence of events that leads to the altered fibroblast function, microvascular changes, and hyaluronic acid accumulation in the upper dermis continues to be researched.

Oral contraceptives in premenopausal women have been shown to give a relative risk of 2.5, which suggests an altered hormonal axis as a possible contributory factor.[9]

Lichen sclerosus may occur in association with other inflammatory conditions including psoriasis.[10] This is surprising because the histopathological findings of lichen sclerosus and psoriasis are so dissimilar.

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Contributor Information and Disclosures
Author

Jeffrey Meffert, MD 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.

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: Nothing to disclose.

Additional Contributors

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.

References
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  2. 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. 2007 May. 16(5):416-20. [Medline].

  3. 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. [Medline].

  4. 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. [Medline].

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  7. 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. [Medline].

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  10. 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. [Medline].

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  12. 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. [Medline].

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  18. Shi L, Miao F, Zhang LL, Zhang GL, Wang PR, Ji J, et al. Comparison of 5-Aminolevulinic Acid Photodynamic Therapy and Clobetasol Propionate in Treatment of Vulvar Lichen Sclerosus. Acta Derm Venereol. 2016 Jan 18. [Medline].

  19. 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. [Medline].

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  25. 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. [Medline].

  26. 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. [Medline].

  27. 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. [Medline].

  28. 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].

  29. 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. [Medline].

 
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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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