AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that results in white plaques with epidermal atrophy. LS 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 vulvar disease, but the precise increase in risk and what cofactors (human papilloma virus 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.

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 LS 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). 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. The exact role of these antibodies and the changes seen in the microvasculature are currently under investigation. Systemic disease or involvement of other organ systems, unlike scleroderma, is not described, although many more authors are describing LS and scleroderma as closely related entities; many cases of coexistent LS/scleroderma have been reported.

Frequency

International

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

Mortality/Morbidity

LS 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. 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

LS, both genital and extragenital, has no known racial predilection.

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 LS. Extragenital LS is rare in children.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

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

Physical

• Pertinent physical findings are limited to the skin.
• Skin primary lesion
• • LS usually begins as white, polygonal papules that coalesce into plaques. Evenly spaced dells or comedolike plugs correspond to obliterated appendiceal ostia. With time, the plugs and dells will disappear and leave a smooth, porcelain-white plaque. The size of the plaque or plaques may vary widely from a few millimeters resembling lichen nitidus to the entire upper trunk.
  • Vulvar LS 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 LS. Some believe that many cases of clinically diagnosed lichen planus may actually be LS and that isolated oral mucosal LS 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. 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.
  • 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.
  • 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 LS along the lines of Blaschko was described in an extragenital case.
• Skin color is white, often with a shiny porcelain appearance. Telangiectases and follicular plugs may be seen.

Causes

The cause of LS is unknown. Over the years, a number of etiologies have been proposed. Several studies have linked borrelial or other infections with LS, yet other studies have disputed this and the current reports along this line are few. 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. Local irritation seems to play a role in some cases, but 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.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Child abuse (sexual)
Genital ulcerative disease
Macular atrophy
Pinta
Atrophie blanche

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Skin biopsy (punch preferred) is the primary study to perform. Despite the presence of autoantibodies described in several studies, an autoimmune (AI) workup (eg, antinuclear antibody, vitamin B-12 levels, thyroid function tests) is still not generally recommended because the frequency of multiple AI diseases associated with LS is not high enough to justify the expense of screening all patients. Work is now underway to identify any patient subsets or particular presentations of LS that would warrant AI screening. For the same reason, Borrelia antibody titers are not recommended, as they would not clearly influence therapy and, in most studies, are not strongly associated with LS, especially in the United States.

Imaging Studies

• Imaging studies are not needed unless urinary obstruction secondary to severe, stenosing genital LS is present. Intravenous pyelogram might be appropriate in this situation.

Procedures

• Punch biopsy in the most mature area of the lesion usually is diagnostic. In genital biopsies, snip excisions may suffice. Suturing the wound leads to more rapid healing than allowing self-granulation. Ulcerative or vegetative genital lesions may need to be biopsied more than once to screen for squamous cell carcinoma. Epidermal hyperplasia and/or dysplasia associated with LS on vulvar biopsy specimens is associated with an increased risk of malignant transformation. Overexpression of wild-type p53 is also associated with increased cancer risk.

Histologic Findings

Classic LS demonstrates a lichenoid infiltrate in the dermal-epidermal junction, compact hyperkeratosis with stratum corneum, which often is thicker than the greatly effaced epidermis. Remarkable edema in the papillary (upper) dermis is replaced by a dense, homogenous fibrosis as the lesion matures. Extensive and deeper biopsies may show areas more consistent with scleroderma than classic LS.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

• Extragenital cases: Asymptomatic extragenital LS usually requires no treatment as control of pruritus other than resolution of the lesion, which is a more realistic goal of therapy. Also, these lesions rarely itch.
• Genital LS may respond to potent topical corticosteroids although the patient should be warned that the clinical appearance does not always reverse, even if symptoms are relieved. It is widely reported that prepubertal LS in girls may resolve spontaneously although some of these patients may go on to suffer from various types of vulvodynia in adulthood. Periodically a report will suggest that areas of vulvar LS be surgically excised or ablated with a laser as a prophylactic measure. Most authors dispute this concept and do not recommend mutilating gynecologic surgery for what, in most patients, is a benign disorder. It is true, however, that circumcision may resolve male genital LS, although the use of potent topical steroids may obviate the need for surgery in such cases.
• The calcineurin inhibitors (ie, tacrolimus, pimecrolimus) have been found to help some patients, especially with genital LS, but they do not work as fast or as effectively as potent topical corticosteroids. They may have a role in maintenance therapy, although several case reports have described that they were not effective.
• A variety of destructive procedures have been reported to be of benefit, although follow-up studies often do not show the same efficacy as original pilot reports. Not just tissue-vaporizing carbon dioxide lasers, but also nonablative lasers such as the pulsed dye and Er:YAG lasers, have been reported to benefit persons with LS. Cryotherapy of affected genital lesions is also reported to reduce the area involved with one or a series of treatments. Photodynamic therapy using a photosensitizer with laser light activation is also reported to be beneficial.

Surgical Care

Circumcision may benefit male LS and the phimosis that may accompany it. Vulvar surgery is not recommended unless an associated malignancy is present. Extragenital lesions may be excised but some caution should be taken as LS has arisen in old surgical scars.

Consultations

• Dermatologist
• Gynecologist - If dysplasia or malignancy are identified or suspected on biopsy. Ulcerated areas should be examined repeatedly and frequently.
• Urologist - If LS is complicated by symptomatic phimosis and circumcision is required
• Pediatrician and/or social services personnel - If coexistent child abuse is suspected

Diet

• Older literature suggests that diets that lower the acidity in the urine may benefit female genital LS. These studies were not reproducible and no dietary recommendations currently are proposed.

Activity

• LS-associated dyspareunia or painful erections may limit sexual activity. No specific activity limits or exercises are recommended. An author in the 1930s suggested that tight underwear and bicycle seats were the cause of LS in girls, but neither of these has been validated as the cause of LS in later studies.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Topical testosterone was mainstay of treatment for female genital LS for decades, although it was never shown useful in male genital or extragenital cases. Recent studies suggest it may not be any more efficacious than placebo. Systemic therapies, including anti-Borrelia antibiotics, potassium benzoate, penicillamine, and systemic steroids, have not proven effective. Systemic retinoids have been useful in limited studies with isotretinoin, etretinate, and acitretin. Topical corticosteroids, especially in the superpotent class, have been found to be useful in genital LS in both sexes and in all age groups.

Drug Category: Corticosteroids

Reducing inflammation helps reduce symptoms and, in some cases, helps resolve the lesion.

Drug Category: Retinoids

Mechanism of action for successful effects systemic retinoids have had in LS (usually studied in female genital cases) is not clear but may have to do with down-regulation of fibroblast function.

Drug Category: Topical calcineurin inhibitors (topical immune modulators)

Many have concerns about the use of potent topical corticosteroids in occluded areas such as the genitals. They often do not work as well or as fast as the corticosteroids when used as monotherapy, but this class of medications may have a role either as maintenance medications after steroid-driven improvement or in conjunction with them in a pulse-steroid regimen (ie, superpotent corticosteroid on weekend days and a TIM during the week). Some believe this may delay or obviate the onset of tachyphylaxis and reduce the risk of steroid atrophy. This is an off-label use for both medications. Recently, some have raised concerns about the use of these medications long term, especially in young children and infants. The exact risks of long-term use, if any, remain to be defined, but the prescriber should be aware of the issues.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Inpatient Care

• Inpatient care generally is not required unless for surgery for malignancy or to relieve urinary obstruction is planned.

Further Outpatient Care

• If potent topical steroids are to be used, regular follow-up is required to monitor for the occurrence of steroid atrophy. Monitor female LS patients for any sign of secondary or associated genital malignancy. Extragenital cases require no specific follow-up.

Transfer

• A patient requiring surgical intervention (circumcision or cancer surgery) may require transfer to another specialist if the dermatologist or primary care physician is not competent in the procedure required.

Complications

• Male genital: Complications can include painful erections, urinary obstruction, an inability to retract the foreskin, and squamous cell carcinoma (rare).
• Female genital: Complications include dyspareunia, urinary obstruction, secondary infection from chronic ulceration, secondary infection related to steroid use, and squamous cell carcinoma (rare, but not as rare as male cases). Some estimates are as high as 5% for the lifetime risk of vulvar squamous cell carcinoma in patients with LS. The epidemiology of LS patients who develop squamous cell carcinoma shows that older age, longer duration of LS, and evidence of hyperplastic/early vulvar carcinoma in situ changes to be significant risk factors.
• Extragenital: Complications include cosmetic concerns, but only in extensive cases.

Prognosis

• Prognosis is good for more acute genital cases, especially for those in pediatric age group that may resolve spontaneously.
• Prognosis for improvement is poor for extragenital cases and for chronic atrophic genital disease.

Patient Education

• Education relating to sexual dysfunction and dyspareunia may be required. Patients with genital LS should be educated on what changes (eg, ulceration) might indicate malignant transformation and mandate an immediate reevaluation.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Incomplete diagnosis: The patient may have LS, but a persistently ulcerated area or an area not responsive to therapy may be a malignancy. Another biopsy or additional biopsies may be necessary.
• Inappropriate surveillance: Repeatedly refilling a patients steroid without reexamining them may allow a malignancy to spread or may allow steroid side effects to develop.
• Child abuse issues: LS, especially when bullous and hemorrhagic or erosive, may be confused with child abuse. On the other hand, one case report suggested LS either coexisted with child abuse or was the result of the trauma associated with the repeated sexual attacks.
• Suboptimal therapy: Topical testosterone, despite the extensive literature describing its use, may not be more effective than placebo and can be associated with virilization.
• New problem: Allergic contact dermatitis may develop with any topical therapy, including steroids. Irritant dermatitis may likewise develop. Consider these when a patient who previously was doing well suddenly seems to worsen.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
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Media file 2:  Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.
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Media file 3:  Typical lichen sclerosus histology demonstrating homogenized edematous papillary (upper) dermis and effaced epidermis.
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Media file 4:  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.
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Media file 5:  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.
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Media file 6:  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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REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• Bornstein J, Heifetz S, Kellner Y, et al. 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].
• Chan I, Oyama N, Neill SM, et al. Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Clin Exp Dermatol. Sep 2004;29(5):499-504. [Medline].
• 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].
• Fischer G, Rogers M. Treatment of childhood vulvar lichen sclerosus with potent topical corticosteroid. Pediatr Dermatol. May-Jun 1997;14(3):235-8. [Medline].
• Fung MA, LeBoit PE. Light microscopic criteria for the diagnosis of early vulvar lichen sclerosus: a comparison with lichen planus. Am J Surg Pathol. Apr 1998;22(4):473-8. [Medline].
• Jones RW, Sadler L, Grant S, et al. 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].
• Kowalewski C, KozĹ‚owska 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].
• Lindhagen T. Topical clobetasol propionate compared with placebo in the treatment of unretractable foreskin. Eur J Surg. Dec 1996;162(12):969-72. [Medline].
• Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol. Mar 1995;32(3):393-416; quiz 417-8. [Medline].
• 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].
• Stucker M, Grape J, Bechara FG, et al. The outcome after cryosurgery and intralesional steroid injection in vulvar lichen sclerosus corresponds to preoperative histopathological findings. Dermatology. 2005;210(3):218-22. [Medline].
• Thomas RH, Ridley CM, McGibbon DH, et al. Anogenital lichen sclerosus in women. J R Soc Med. Dec 1996;89(12):694-8. [Medline].
• Wallace HJ. Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc. 1971;57(1):9-30. [Medline].

Article Last Updated: Dec 15, 2006