eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Lichen Sclerosus et Atrophicus

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio

Updated: Jan 29, 2009

Introduction

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 vulvar 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.

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 effect of cell-mediated cytotoxicity has been better defined at the biochemical level.⁴ 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 have been reported.

Frequency

International

The population rate is unknown. Male genital lichen sclerosus 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

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. 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.

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.

Clinical

History

Extragenital lichen sclerosus may be asymptomatic or it may itch, although itching is not common. 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.

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. 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 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. 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 lichen sclerosus 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 lichen sclerosus is unknown. Over the years, a number of etiologies have been proposed. Several studies have linked borrelial or other infections with lichen sclerosus,⁵ yet other studies have disputed this. This concept has reemerged with European reports demonstrating Borrelia more readily in lichen sclerosus cases using "focus-floating microscopy" than conventional histology.⁶ 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.⁷

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. A lichen sclerosus relative risk factor of 2.5 was seen in premenopausal women placed on oral contraceptives, which, once again, brings up an altered hormonal axis as a possible contributory factor.⁸

Differential Diagnoses

Other Problems to Be Considered

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

Workup

Laboratory 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 lichen sclerosus 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 lichen sclerosus 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 lichen sclerosus, especially in the United States.

Imaging Studies

Imaging studies are not needed unless urinary obstruction secondary to severe, stenosing genital lichen sclerosus 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 lichen sclerosus 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 as is a human papillomavirus–associated increase in p16INK4A.⁹

Histologic Findings

Classic lichen sclerosus 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 lichen sclerosus.

Treatment

Medical Care

Asymptomatic extragenital lichen sclerosus 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 lichen sclerosus 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 lichen sclerosus 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 lichen sclerosus 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 lichen sclerosus, 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 lichen sclerosus, 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.

Surgical Care

Circumcision may benefit male lichen sclerosus 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 lichen sclerosus has arisen in old surgical scars.

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 lichen sclerosus. Cryotherapy of affected genital lesions is also reported to reduce the area involved after one or a series of treatments.¹⁰ Narrow-band UVB,¹¹ psoralen plus UVA (PUVA), and photodynamic therapy¹² using a photosensitizer with laser light activation are also reported to be anecdotally beneficial by various authors.

Consultations

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

Diet

One study suggests patients with lichen sclerosus might have a higher rate of positive patch tests to spices and flavorings.¹³ Older literature suggests that diets that lower the acidity in the urine may benefit female genital lichen sclerosus. These latter studies were not reproducible, and no dietary recommendations currently are proposed.

Activity

Lichen sclerosus–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 lichen sclerosus in girls, but neither of these has been validated as the cause of lichen sclerosus in later studies.

Medication

Topical testosterone was mainstay of treatment for female genital lichen sclerosus for decades, although it was never shown useful in male genital or extragenital cases. More 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 reliably 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 lichen sclerosus in both sexes and in all age groups.¹⁴ These have been used  long term (weeks to months) or daily, without significant adverse cutaneous effects, although patients should always be monitored regularly adverse effects.

Corticosteroids

Reducing inflammation helps reduce symptoms and, in some cases, helps resolve the lesion.^15,16

Clobetasol (Temovate)

Class I superpotent topical steroid, Suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction. Other superpotent steroids would be expected to have the same results; however, most published studies showing benefit of such medications have used clobetasol.
Pulse dosing (2 consecutive d/wk) may be used long-term, even in genital cases. On off-days, a milder steroid or emollient alone may be used. No additional benefit in using potent and super-potent steroids >qd. Especially in genital applications, bid application of such a medication will more quickly lead to steroid adverse effects.

Dosing

Adult

Apply qd for 12 wk or less if symptoms are improved

Pediatric

Administer as in adults; genital use for 3 mo or longer is reported without adverse effects

Interactions

None reported

Contraindications

Documented hypersensitivity; viral or fungal skin infections

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May suppress adrenal function in prolonged therapy; observe for evidence of steroid atrophy; consider biopsy to exclude malignancy in patients with genital lichen sclerosus who have good response yet have a single persistent lesion involved or ulcerated area

Hydrocortisone (LactiCare HC, Westcort, Dermacort, Cortaid, DermaGel)

An adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. It has mineralocorticoid and glucocorticoid effects resulting in anti-inflammatory activity.

Dosing

Adult

Apply sparingly to affected areas bid/qid

Pediatric

Apply as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity; viral, fungal, and bacterial skin infections

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Prolonged use, applying over large surface areas, application of potent steroids, and occlusive dressings may increase systemic absorption of corticosteroids and may cause Cushing's syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria

Retinoids

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

Acitretin (Soriatane)

Retinoic acid analog, like etretinate and isotretinoin. Etretinate is main metabolite and has demonstrated clinical effects close to those seen with etretinate. Mechanism of action is unknown. Has been reported to benefit both symptoms and induce resolution of lesion. Although not well studied, combining a medication in this class with a topical corticosteroid as described above may be useful in refractory cases.

Dosing

Adult

25 or 50 mg/d PO given as single dose with main meal initial; 25-50 mg/d PO after initial response to treatment; terminate therapy when lesions have resolved sufficiently

Pediatric

Not established

Interactions

Increases toxicity of methotrexate (avoid concomitant use); interferes with effects of microdosed progestin minipill; concomitant use with tetracycline product may lead to pseudotumor cerebri; coadministration with alcohol may enhance synthesis of etretinate, which has much longer half-life than acitretin (>120 d)

Contraindications

Documented hypersensitivity; pregnancy must not be planned for at least 3 y after cessation of drug, many recommend medication be avoided if patient is planning to ever be pregnant, especially if patient consumes alcohol, which encourages conversion to teratogenic metabolite that may persist indefinitely

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Do not use in severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; perform AST, ALT, and LDH tests prior to initiation of acitretin therapy at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated

Isotretinoin (Accutane)

Oral agent that treats serious dermatologic conditions. Synthetic 13-cis isomer of the naturally occurring tretinoin (trans -retinoic acid). Both agents are structurally related to vitamin A.
Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization. Has been reported to benefit both symptoms and may help resolution of lesion itself. Although not well studied, combining a medication in this class with a topical corticosteroid as described above may be useful in refractory cases.

Dosing

Adult

0.5-1 mg/kg/d PO; duration of therapy will likely be at least 4-6 mo

Pediatric

Administer as in adults

Interactions

Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; acitretin may reduce plasma levels of carbamazepine

Contraindications

Documented hypersensitivity; pregnancy

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

May decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; diabetes patients may experience problems in controlling blood sugar; avoid exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occur

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.

Tacrolimus (Protopic)

Mechanism of action in atopic dermatitis is not known. Reduces itching and inflammation by suppressing release of cytokines from T cells. Also inhibits transcription for genes that encode IL-3, IL-4, IL-5, GM-CSF, and TNF-alpha, all of which are involved in the early stages of T-cell activation. Additionally, may inhibit release of preformed mediators from skin mast cells and basophils and may down-regulate expression of FCeRI on Langerhans cells. Indicated in dermatitis patients as young as 2 y. Drugs of this class are more expensive than topical corticosteroids. Available as an ointment in concentrations of 0.03% and 0.1%.

Dosing

Adult

Apply thin layer to affected skin areas bid and rub in gently and completely; continue treatment for 1 wk after clearing of signs and symptoms

Pediatric

<2 years: Not established
2-15 years: Apply 0.03% ointment bid to affected area(s)
>15 years: Administer as adults

Interactions

None reported

Contraindications

Documented hypersensitivity to tacrolimus or components of ointment

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Patients may experience a burning sensation during first few days of application; skin can become photosensitive, and patients should be cautioned about exposure to direct or artificial sunlight and to use sunscreen
Safety and efficacy in infected atopic dermatitis is not known
Application under occlusion, which may promote systemic exposure, has not been evaluated (do not use with occlusive dressings)
Absorption following topical applications is minimal (relative to systemic administration), but is excreted in human milk and, thus, a decision should be made whether to discontinue nursing or to discontinue drug, taking into account importance of drug to mother (potential for serious adverse reactions in nursing infants from tacrolimus should also be a concern)
Caution with conditions that suppress the immune system (eg, AIDS, cancer)
Possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections; other adverse effects include headache, sore throat, flulike symptoms, fever, and cough

Pimecrolimus (Elidel)

First nonsteroid cream approved in the United States for mild-to-moderate atopic dermatitis. Derived from ascomycin, a natural substance produced by Streptomyces hygroscopicus var. ascomyceticus. Selectively inhibits production and release of inflammatory cytokines from activated T cells by binding to cytosolic immunophilin receptor macrophilin-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release. Cutaneous atrophy not observed in clinical trials, a potential advantage over topical corticosteroids. Indicated only after other treatment options have failed

Dosing

Adult

Apply topically to affected areas bid

Pediatric

<2 years: Not established
>2 years: Administer as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Potential exacerbation of existing infection at site of application; may cause burning and irritation; caution with conditions that suppress the immune system (eg, AIDS, cancer); possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections; other adverse effects include headache, sore throat, flulike symptoms, fever, and cough

Follow-up

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 lichen sclerosus 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 lichen sclerosus.¹⁷ The epidemiology of lichen sclerosus patients who develop squamous cell carcinoma shows that older age, longer duration of lichen sclerosus, 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 lichen sclerosus should be educated on what changes (eg, ulceration) might indicate malignant transformation and mandate an immediate reevaluation.

Miscellaneous

Medicolegal Pitfalls

• Incomplete diagnosis: The patient may have lichen sclerosus, 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 patient's topical corticosteroids without reexamining them may allow a malignancy to spread or may allow steroid side effects to develop.
• Child abuse issues: lichen sclerosus, especially when bullous and hemorrhagic or erosive, may be confused with child abuse. On the other hand, one case report suggested lichen sclerosus 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

Media file 1: Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.

Media file 2: Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.

Media file 3: Typical lichen sclerosus histology demonstrating homogenized edematous papillary (upper) dermis and effaced epidermis.

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.

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.

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.

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. 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].

5. 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].

6. 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].

7. 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].

8. 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].

9. 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].

10. 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].

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

12. 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].

13. 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].

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

15. 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].

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

17. 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].

18. 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].

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Keywords

lichen sclerosus, lichen sclerosus et atrophicus, kraurosis vulvae, balanitis xerotica obliterans

Contributor Information and Disclosures

Author

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-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.

Medical Editor

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: Nothing to disclose.

Pharmacy Editor

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
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: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

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.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

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