Lichen sclerosus (LS) is a chronic inflammatory dermatosis of unknown cause that most commonly affects the genitalia (vulvar and penile lichen sclerosus) but can occur at any skin site (extragenital lichen sclerosus).[1] Outdated terms for this conditions include lichen sclerosus et atrophicus, balanitis xerotica obliterans (glans penis presentation), and kraurosis vulvae (older description of vulvar presentation).
Although LS can occur in males or females of any age, it more commonly affects prepubertal or perimenopausal females or males between puberty and age 60 years. It is characterized by white, often atrophic, plaques associated with pruritus and pain that result in genital scarring and adhesion. Complications include pain, sexual or urinary dysfunction, reduced quality of life, and an increased risk (≤ 5%) of squamous cell carcinoma (SCC) with genital lichen sclerosus.
Guidelines for treatment of LS have been published by the British Association of Dermatologists (BAD),[2] the European Academy of Dermatology and Venereology (EADV),[3] and the EuroGuiDerm Centre for Guideline Development.[4]
In LS, inflammation and altered fibroblast function in the papillary dermis lead to fibrosis of the upper dermis. Genital skin and mucosa are affected most frequently, with only rare reports of oral presentations. It has been hypothesized that oral manifestations are underdiagnosed or misdiagnosed as oral lichen planus, or that environmental effects play a large role in disease expression.
Findings within LS tissue include the following:
Increased glut-1 and decreased vascular endothelial growth factor (VEGF) expression - These suggest a role for hypoxia and ischemia[5]
Higher expression of extracellular matrix (ECM) proteins and connective-tissue growth factor (CTGF) - Upregulation may induce accumulation of ECM proteins and maintain fibrosis in chronic vulval LS[6]
Altered expression of isocitrate dehydrogenase enzymes and aberrant hydroxymethylation - These indicate an epigenetic background for the pathogenesis of vulval LS[7]
Increased levels of Th1-specific cytokines, a dense T-cell infiltrate and enhanced BIC/miR-155 expression, and a microRNA involved in regulation of the immune response - These demonstrate an autoimmune phenotype in vulvar LS[8]
Distinct expression patterns of tissue remodeling–associated genes in boys with congenital phimosis and LS[9]
Decreased or absent epidermal expression of CD44 - This suggests that LS might result from epidermal damage of unknown origin, resulting in CD44-deficient dermal changes with hyaluronic acid accumulation[10]
Increased incidence of autoantibodies to EMC protein 1 and to BP180 antigen, with increased circulation of NC16A domain–specific T cells[11]
The etiology and pathogenesis of LS have not been defined but may include genetic, infectious, environmental, and hormonal factors.
Several older studies linked borrelial or other infections with LS, but most subsequent studies have disputed this finding, with polymerase chain reaction (PCR)-based studies showing no increased incidence of borrelial infection.[12]
Exploration of genetic and autoimmune factors has not identified consistent, reproducible patterns, though autoantibodies to ECM protein 1 have been reported. The level of circulating autoantibodies may be as high as 28%, comparable to the rate seen in bullous lichen planus.[13] Baldo et al reported that more than 40% of vulvar LS and lichen planus patients have reactive T cells to the NC16A domain of bullous pemphigoid antigen 180[11] ; however, others have suggested that the level of autoantibodies is poorly correlated to disease activity and treatment response. Women with LS have a higher rate of associated autoimmune disease (odds ratio, 4.3), especially for autoimmune thyroid disease, than men do.[14]
A genetic predisposition, based on family clustering, has been described.[15] Of patients with vulvar LS, 8-39% report a family history of the condition, whereas only 1% of male genital LS patients have a family history.[3]
Local irritation or trauma seems to play a role in some cases of LS, especially in genetically predisposed individuals.[16] However, the sequence of events that leads to the altered fibroblast function, microvascular changes, and hyaluronic acid accumulation in the upper dermis remains a subject of research.
Oral contraceptives in premenopausal women have been shown to give rise to a relative risk of 2.5, which suggests an altered hormonal axis as a possible contributory factor.[17]
LS may occur in association with other inflammatory conditions, including psoriasis.[18] This association is surprising because the histopathologic findings of LS and psoriasis are dissimilar.
A systematic review and meta-analysis of 21 case-control studies by Baglama et al found that both male and female LS patients had a higher incidence of certain dermatologic conditions (lichen planus, vitiligo, alopecia areata, atopic dermatitis, and psoriasis), certain cardiovascular risk factors (essential hypertension, obesity, dyslipidemia, and diabetes mellitus), genital warts, and hypothyroidism in comparison with control subjects.[19] However, there were no significant differences between males and females with respect to these associations.
LS is a relatively common dermatosis, though its true prevalence has not been established and is probably underestimated. This may be because patients are distributed among different specialties (eg, gynecology, dermatology, family practice, and urology), but it may also be because one third of cases can be asymptomatic.
The prevalence of genital LS in prepubertal girls has been reported to be 0.1% (1 in 900).[20] The prevalence of vulvar LS in elderly nursing home women in one study was found to be 3% (1 in 30).[21] Another study found the rate to be approximately 1.7% among women seen in a gynecology practice.
A Danish study covering the period from 1997 to 2022 found that the incidence of biopsy-confirmed vulvar LS rose from 5.0 per 100,000 person-years at the beginning of the period to 35.7 per 100,000 person-years at the end.[22] The risk of vulvar squamous precancer and SCC increased as well.
The prevalence of male LS is thought to be influenced by the circumcision rate.[23] Given that male genital LS is seen almost exclusively in uncircumcised or incompletely circumcised men and boys, it follows that the circumcision rate in a given population would have an impact on the rate of LS in this subset.
The prevalence of extragenital LS is unclear. A study of foreskins submitted after therapeutic circumcision for phimosis revealed many cases of unrecognized LS. Extragenital LS is much less common than genital LS and is rare in children.
Age-, sex-, and race-related demographics
Vulvar LS can occur at any age, and incidence increases with age. In females, the two peaks of onset are during the prepubertal period and during the perimenopausal/postmenopausal period. It is noteworthy that both periods are characterized by low-estrogen states. It is not clear whether a hormonal relationship is involved or whether the low-estrogen state promotes koebnerization due to reduced lubrication. In males, the incidence increases after puberty and then decreases again in older age (>60 y).
The female-to-male ratio has ranged from 3:1 to 10:1.[3]
Neither genital nor extragenital LS has been shown to have any racial predilection.
For patients with more acute cases of genital LS, the prognosis is good, especially for those in the pediatric age group, in whom the condition may resolve spontaneously. For patients with extragenital LS or chronic atrophic genital disease, however, the likelihood of improvement is low.
Many pediatric cases improve with puberty, though some authors have suggested that the rate of spontaneous resolution may actually be lower than 25%.[24]
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.
Extragenital cases and many genital cases are asymptomatic, except for cosmetic changes or pruritus. Recalcitrant cases, especially those associated with erosion or progressive scarring, may result in severe sexual dysfunction.
An increased risk of SCC may exist in genital disease, but the magnitude of the increase in risk and the cofactors (eg, human papillomavirus [HPV] infection or prior radiotherapy) that may be involved have not been fully defined. Some have cited figures as high as 5% for the lifetime risk of vulvar SCC in patients with LS.[25] Older age, longer duration of LS, and evidence of hyperplastic or early vulvar carcinoma in situ changes appear to be significant risk factors. A 2009 study linked coexisting chlamydial infection and LS with an increased risk of SCC, but other sexually transmitted illnesses (eg, HPV infection) were not completely addressed.[26]
In patients who have been treated for vulvar cancer, the presence or absence of LS does not appear to affect the timing of recurrence.
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 thus mandate an immediate reevaluation.
Extragenital lichen sclerosus (LS) may be asymptomatic (approximately one third of cases), or it may itch or be tender. The most common complaint in vulvar LS is itch, and the most common complaint in penile LS is sexual or urinary dysfunction.
Patients with vulvar LS usually present with progressive pruritus, dyspareunia, dysuria, or genital bleeding. Penile LS usually is preceded by pruritus, but patients may present with sudden phimosis of previously retractable foreskin, and urinary obstruction can result.
LS 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, though it should be kept in mind that other conditions (eg, chronic cutaneous lupus) may also show follicular plugs. With time, the plugs and dells disappear, leaving a plaque. Skin color is white, often with a shiny porcelain appearance. Telangiectases may be seen. The size of the plaque or plaques may vary widely, ranging 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.
Oral LS has been reported but is quite rare.[27] Given the high frequency of genital mucosal disease, it is perhaps surprising that more oral cases have not been reported. The rare cases that have been reported have mostly been in patients with widespread, generalized LS. Some authorities hage suggested that many cases of clinically diagnosed lichen planus may actually be LS and that isolated oral mucosal LS may not be as rare as has generally been thought.
Distribution of lesions on skin
Extragenital LS lesions (see the image below) may occur anywhere on the body, though the back and shoulders are the most common sites.
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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 they usually involve, and eventually obliterate, the labia minora and cause stenosis of the introitus. Often, an hourglass, butterfly, or figure-eight pattern involves the perivaginal and perianal areas, with minimal involvement of the perineum in between. (See the images below.)
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Lichen sclerosus demonstrating classic hourglass or figure 8 vulvar and perianal distribution. Courtesy of Wilford Hall Medical Center slide files.
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More advanced vulvar lichen sclerosus; eroded areas need to be carefully examined and a biopsy sample should be taken to exclude coexistent squamous c....
Male genital lesions usually are confined to the glans penis and the prepuce or foreskin remnants. Involvement of the penile shaft is much less common, and scrotal involvement is rare. The initial manifestation may be a sclerotic ring at the prepuce edge (see the image below).
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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 ur....
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 has been described in extragenital cases.[28]
Potential complications of male genital, female genital, and extragenital LS include the following:
Male genital - Painful erections, urinary obstruction, inability to retract the foreskin, and (rarely) squamous cell carcinoma (SCC)
Female genital - Dyspareunia, urinary obstruction, secondary infection from chronic ulceration, secondary infection related to steroid use, and SCC (rare, but not as rare as in male genital LS)
Extragenital - Cosmetic concerns, but only in extensive cases
The following issues also may arise:
Incomplete diagnosis - The patient may have LS, but a persistently ulcerated area or an area unresponsive to therapy may be a malignancy; another biopsy or multiple additional biopsies may be necessary
Inappropriate surveillance - Repeatedly refilling a patient's topical corticosteroids without reexamining the lesions may allow a malignancy to spread or may allow steroid adverse effects to develop
Confusion with child abuse - LS, especially when bullous and hemorrhagic or erosive, may be confused with child abuse; on the other hand, one case report suggested that LS either coexisted with child abuse or was related to 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 - With any topical therapy, including steroids, allergic contact dermatitis or irritant dermatitis may develop; these potential new problems should be considered when a patient who previously was doing well suddenly seems to worsen
Diagnosis of lichen sclerosus (LS) may often be made on the basis of clinical appearance, and ancillary examinations such as dermoscopy may help confirm the diagnosis.[29] Biopsy should be performed in questionable cases, and in the course of follow-up, biopsy specimens from nonhealing ulcerations or masses should be examined to exclude malignant transformation.
Despite the presence of autoantibodies described in several studies, an autoimmune workup (eg, antinuclear antibody [ANA] titer, vitamin B12 level, thyroid function testing) is still not generally recommended, because the frequency of multiple autoimmune diseases associated with LS is not high enough to justify the expense of screening all patients. For the same reason, Borrelia antibody titers are not recommended; they would not clearly influence therapy and, in most studies, are not strongly associated with LS, especially in the United States.
Imaging studies are not needed unless urinary obstruction secondary to severe, stenosing genital LS is present. In this situation, intravenous pyelography (IVP) might be appropriate.
Skin biopsy (punch preferred) is the primary study for confirming the diagnosis of LS. A punch biopsy in the most mature area of the lesion is usually diagnostic. In genital biopsies, snip excisions may suffice. Suturing the wound—especially with braided suture, which is less likely to poke sensitive skin—leads to more rapid healing than allowing self-granulation.
With ulcerative or vegetative genital lesions, biopsy may have to be done more than once to screen for squamous cell carcinoma (SCC). Epidermal hyperplasia 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. A study by Voss et al suggested that p53 immunochemistry and DNA methylation could serve as prognostic biomarkers for vulvar LS.[30]
A human papillomavirus (HPV)-associated increase in p16INK4A has been associated with increased cancer risk as well.[31] On the other hand, a study by Paolino et al suggested that HPV is not identified in most cases of LS-associated malignancy and that when it is identified, it may be a nononcogenic type.[32]
Classic LS is characterized by a lichenoid infiltrate in the dermoepidermal junction and 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 with classic LS. (See the images below.)
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 occ....
Dermoscopy and reflectance confocal microscopy (RCM) are noninvasive methods of recognizing typical histologic findings for different skin conditions in vivo. A study by Lacarruba et al discussed typical dermoscopic and RCM findings in LS, including the appearance of epidermal atrophy and follicular plugging.[33]
An evidence-based treatment guideline for lichen sclerosus (LS) was published by the British Association of Dermatologists (BAD) in 2010[34] ; this guideline was updated in 2018 (see Guidelines),[2] and a further update was under way as of November 2024. In 2015, an evidence-based guideline for anogenital LS was published by the European Academy of Dermatology and Venereology (EADV).[3] Table 1 of this 2015 consensus guideline summarized treatment responses achieved with different therapies in different sets of patients. The author's treatment practice is aligned with recommendations in these guidelines.
In October 2024, a EuroGuiDerm guideline on LS was published under the aegis of the EuroGuiDerm Centre for Guideline Development (see Guidelines).[4]
Genital lichen sclerosus
Some randomized controlled trials (RCTs) have been performed to evaluate treatments for genital LS, including topical steroids, acitretin, lasers,[35] and para-aminobenzoate.[36, 37] For a number of proposed treatments, however, efficacy data have been derived from case reports and small studies.
All genital LS cases, even if asymptomatic, should be treated with the goal of preventing scarring and its associated disfigurement, sexual and urinary dysfunction, and impaired quality of life. Therapeutic success is typically evaluated every 3 months during treatment. Efficacy is gauged on the basis of resolution of symptoms (pruritus and pain) and improvement of physical variables (eg, ulceration, hyperkeratosis, erythema, ecchymosis, atrophy, and depigmentation). Clinical photography is helpful for monitoring from visit to visit. It should be kept in mind that scarring will be permanent.
First-line therapy includes patient education and superpotent topical corticosteroids (eg, clobetasol propionate). Vulvar LS patients typically do not develop atrophy with prolonged use, owing to the resistant nature of modified mucous membranes of the labia and clitoris (in contrast to the perianal and hair-bearing skin of the labia majora, which can atrophy within 2-3 wk of use). Intralesional corticosteroid injections are also considered first-line therapy, though not used by the author.
Second-line therapies include the calcineurin inhibitors tacrolimus and pimecrolimus, which can be a helpful adjunct to topical corticosteroids for maintenance.
Third-line therapies that could be considered for treatment-resistant genital LS include topical or oral retinoids, steroid injections, cyclosporine (topical shown not to work), methotrexate, and hydroxyurea. An RCT (N = 66) by Zivanovic et al compared dual laser (Nd:YAG/Er:YAG) therapy (n = 44) for vulvar LS with topical steroid therapy (n = 22) and found that the former yielded comparable improvements and better patient satisfaction. Platelet-rich plasma (PRP) has been suggested as a potential alternative when treatment must be escalated.[38] For extragenital LS, phototherapy or methotrexate could be considered (treatment regimens analogous to those used for morphea).[35]
The author has used hydroxychloroquine with good results (< 6.5 mg/kg based on ideal body weight) as a systemic maintenance drug for both genital and extragenital lichen sclerosus. It is especially useful to help aid tapering of long-term immunosuppressant therapy. A retrospective study by Akpala et al found hydrochloroquine to be promising for anogenital LS.[39]
Topical testosterone, topical estrogen, topical progesterone, and hormone replacement therapy should not be used. Although these measures were extensively employed in the past, they are not supported by convincing evidence.
A small study (N = 23) by Borghi et al examined the use of topical avocado and soybean extracts as alternative treatments for mild-to-moderate vulvar LS in patients wishing to avoid corticosteroids.[40] The patients also received dietary supplements containing the same substances, along with vitamin E and para-aminobenzoic acid; most reported improvement. Another anecdotal therapy that has been described is intralesional injection of adalimumab.[41] A case report by Du et al noted significant relief of pruritus with dupilumab therapy.[42]
Extragenital lichen sclerosus
There is a lack of RCTs evaluating the efficacy of treatment for extragenital LS; recommendations are based on case reports and small uncontrolled studies.[3] Treatment is often extrapolated from the findings of studies addressing the treatment of genital LS or morphea.
It is important to discuss with patients their goals for care and then to treat LS in accordance with those goals. If patients are asymptomatic, deferring treatment is reasonable. If localized lesion treatment (based on cosmesis or symptoms) is desired, topical treatment with potent or ultrapotent topical corticosteroids is first-line therapy. If patients have extensive involvement, rapid progression, or a goal of preventing new lesions, phototherapy or systemic therapy should be offered.
It is important to discuss the status of the genitalia with the patient (in particular, to clarify that if scarring occurs, it will be permanent), as well as to explain the chronicity of the condition and the associated frequent recurrences that are likely if therapy is not maintained. However, patients should also be reassured that the condition can be treated into remission with continued therapy. Malignancy risk, greatest with ulcerative genital LS, should be addressed, and monthly self-examinations should be encouraged.[43] Yearly skin examinations by a dermatologist or gynecologist are important for skin cancer screening. Screening for sexual or urinary dysfunction should be performed at each visit.
Vulvar hygiene
Some studies have shown reductions in symptoms with the use of a bland moisturizer (10% reduction), and wearing silk underwear rather than cotton underwear is recommended; one study showed a 10% symptom improvement. Avoidance of mechanical irritation may include applying moisturizer to toilet paper after urination or defecation, using lubrication with sexual activity, and employing local treatment after known mechanical irritation.
Topical corticosteroids
No standardized regimen has been established for treating LS with topical corticosteroids, though most providers divide treatment into an initiation phase and a maintenance phase. For initial treatment, the 2015 EADV guidelines recommended once or twice daily for 3 months.[3] The author typically uses the tapering regimen recommended in the 2010 BAD guidelines[34] : once daily for 1 month, every other day for 1 month, and then two or three times weekly, with dosing escalated in the event of flares. The recommended approach to tapering involves reducing frequency of use rather than reducing potency.
One fingertip unit amount (extending from the tip of the finger to the distal interphalangeal [DIP] joint) should be used for each application, with no more than 10 g used monthly. Maintenance treatment is necessary, given that LS is a chronic condition with a high relapse rate. Per EADV expert opinion, some patients only require treatment once or twice monthly, whereas others may require it two or three times weekly. In view of the long-term safety data on the use of superpotent corticosteroid on mucosa, the author strongly encourages a minimum of 3 times a week as maintenance. If daily use is needed, the addition of calcineurin inhibitors should be considered.
The addition of a topical calcineurin inhibitor (eg, tacrolimus or pimecrolimus) as a second agent for maintenance is a recommended option if daily treatment is needed. The author prescribes every-other-day treatment with a superpotent topical steroid given three times a week and tacrolimus given the remaining days. There is no risk of skin atrophy. Tacrolimus works better than pimecrolimus. In general, tacrolimus has efficacy equivalent to that of a midstrength topical steroid.
Although concerns have been raised in the past about neoplasia induction and the long-term adverse effects with topical calcineurin inhibitor therapy, the consensus is that the risk of neoplasia in LS is due to endogenous inflammation rather than to therapies themselves. The main limiting factor for this class is the adverse effect of burning. In the author's experience, only approximately 25-50% of patients can tolerate these agents despite a 1-week trial.
Topical retinoid and acitretin
An RCT by Bousema et al found oral acitretin (20-30 mg/day for 16 wk) to be effective in comparison with placebo.[44] Short-contact topical retinoid therapy has been reported to be efficacious; however, its application is limited by irritation on nonintact skin.[45]
Phototherapy
In an RCT comparing the efficacy of home-administered medium-dose ultraviolet (UV)-A1 phototherapy four times weekly with that of once-daily application of clobetasol ointment in 30 women with vulvar LS, both groups showed significant improvement.[46] Case reports have described successful outcomes with psoralen plus UV-A (PUVA), narrowband UV-B, and photodynamic therapy.[47, 48, 49]
However, the risk of neoplasia in the setting of phototherapy (especially PUVA) is unclear. It should be noted that the risk of developing squamous cell carcinoma (SCC) in genital LS is no higher than 5% and that SCC development is rare in extragenital LS. The author has frequently used narrowband UV-B phototherapy in patients with extensive or progressive extragenital LS who want to prevent new lesion formation while the existing lesions are being treated.
Methotrexate
The efficacy of systemic corticosteroids plus methotrexate was supported by a retrospective study of 10 patients with extragenital LS.[50] This regimen has often been used in the treatment of generalized morphea, and in the author's experience, it works well at 15 mg/wk with folic acid 1 mg daily. For rapid progression, pulse dosing with methylprednisolone 1000 mg/wk intravenously (IV) on 3 sequential days per month for 3 months in conjunction with prednisone 1 mg/kg/day orally (PO) for 3 months as taper would be reasonable.
Outcomes
Genital LS may respond well to ultrapotent (superpotent) topical corticosteroids, though patients should be warned that the clinical appearance is not always reversed, even if symptoms are relieved. It has been widely reported that prepubertal LS in girls may resolve spontaneously, though some of these patients may go on to develop various types of vulvodynia in adulthood. Treatment is necessarily prolonged, and short-term treatments often lead to suboptimal control of findings and symptoms.[51]
Male patients with LS and the phimosis that often accompanies it may benefit from circumcision. For patients with vulvar LS, surgery has not been recommended unless an associated malignancy is present. Some articles have suggested that surgical release of clitoral phimosis and labial adhesions may benefit some patients with severe dyspareunia, though the numbers cited have been small and surgical complications have been noted.[52, 53] Extragenital lesions may be excised, but caution should be exercised, given that LS can arise in old surgical scars.
Various destructive procedures have been reported to be beneficial, though follow-up studies often have not shown the same efficacy as the original pilot reports. Not only tissue-vaporizing carbon dioxide lasers but also nonablative lasers (eg, pulsed dye and Er:YAG lasers) have been reported to benefit patients with LS. Cryotherapy of affected genital lesions has also been reported to reduce the area involved after one or more treatments.
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.
Periodically, a report suggests that areas of vulvar LS should be surgically excised or ablated with a laser as a prophylactic measure. Most authors disagree with this suggestion 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, though the use of ultrapotent topical steroids may render surgery unnecessary in such cases.
There are no reproducible studies relating to diet or reactions to any particular foods, spices, or flavorings in the context of LS. For this reason, no dietary recommendations or restrictions are currently proposed for LS patients.
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 subsequent studies have not validated either of these as causative.
If potent topical steroids are to be used, regular follow-up is required to monitor for the development of steroid atrophy. Female patients with genital LS should be monitored for any sign of secondary or associated genital malignancy. Cases of extragenital LS require no specific follow-up.
In October 2024, a EuroGuiDerm guideline on lichen sclerosus (LS) was published under the aegis of the EuroGuiDerm Centre for Guideline Development (see Guidelines).[4] Recommendations and suggestions for female genital LS, male genital LS, and extragenital LS included the following.
Genital lichen sclerosus: women
Main recommendations were as follows:
Cotreatment with emollients during standard therapy is recommended.
Potent or ultrapotent topical corticosteroids are recommended.
If LS is resistant to topical steroids and malignancy has been excluded, intralesional injection of steroids into hyperkeratotic lesions is suggested.
Topical calcineurin inhibitors are suggested as an alternative or additional choice if topical steroids are contraindicated or insufficiently effective.
Ultraviolet (UV)-A1 therapy is suggested as a second choice for treatment, with carcinogenicity and practicality considered.
If systemic therapy is required, acitretin is suggested, with teratogenicity considered.
Genital lichen sclerosus: girls
Main recommendations were as follows:
Cotreatment with emollients during standard therapy is recommended.
Potent or ultrapotent topical corticosteroids are recommended.
Topical calcineurin inhibitors are suggested as an alternative or additional choice if topical steroids are contraindicated or insufficiently effective.
Genital lichen sclerosus: men
Main recommendations were as follows:
Cotreatment with emollients during standard therapy is suggested.
Potent or ultrapotent topical corticosteroids are recommended.
Topical calcineurin inhibitors are suggested as an alternative or additional choice if topical steroids are contraindicated or insufficiently effective.
If systemic therapy is required, acitretin is suggested.
If guideline-conforming medical treatment for LS causing phimosis fails, circumcision (preferably with removal of the entire foreskin) is suggested.
If guideline-conforming medical treatment for LS causing scarring or shortening of the frenulum fails, either frenuloplasty plus intralesional triamcinolone or complete circumcision is suggested.
If urethral stricture from LS gives rise to mechanical problems with voiding or sexual intercourse, urethroplasty with oral mucosal grafts is suggested.
Genital lichen sclerosus: boys
Main recommendations were as follows:
Cotreatment with emollients during standard therapy is suggested.
Potent or ultrapotent topical corticosteroids are recommended.
Topical calcineurin inhibitors are suggested as an alternative or additional choice if topical steroids are contraindicated or insufficiently effective.
If guideline-conforming medical treatment for LS causing scarring or shortening of the frenulum fails, either frenuloplasty plus intralesional triamcinolone or complete circumcision is suggested.
Extragenital lichen sclerosus
Main recommendations were as follows:
Cotreatment with emollients during standard therapy is suggested.
Potent or ultrapotent topical corticosteroids are suggested.
UV therapy is recommended.
Methotrexate is suggested, with teratogenicity considered if systemic therapy is required for adults with genital or extragenital LS.
In 2018, the British Association of Dermatologists (BAD) issued updated guidelines on the management of LS in males and females.[2]
Studies have illustrated an increased incidence of tissue‐specific antibodies and associations with other autoimmune diseases, especially thyroid disease in female, but not in male, patients.
Female treatment guidelines
In adult female patients, after the diagnosis of anogenital LS is made, an initial 3‐month induction regimen is recommended. Clobetasol propionate 0.05% ointment should be applied once a day for a month, on alternative days for the next month, and then twice weekly for the third month, in combination with a soap substitute and a barrier preparation. This recommendation is based on randomized control trials that found clobetasol propionate 0.05% to be more effective than topical tacrolimus 0.1%, testosterone 2% in petrolatum, and UV-A1 home‐based phototherapy, and equally efficacious to mometasone furoate 0.1%. Two follow‐up visits, at 3 months and 6 months, are suggested.
After 3 months, about 70% of adult female patients achieve remission. Treatment failure may result from poor compliance or coexistent vulvodynia, and a biopsy might be required at this point to confirm the diagnosis. The topical steroid should be continued in a regular regimen once or twice per week for ongoing active lichen sclerosus disease.
Patients are followed every 6-12 months until there is symptom control, good sexual function, and no further alteration in architecture. They can then be discharged back to the general practitioner with annual checks and clear instructions on self‐monitoring. The guidelines state that topical steroids are to be used as needed thereafter, recommending a practical approach and suggesting that treatment be tapered to maintain symptom control and resolution of skin thickening and ecchymosis, but not pallor (which does not always resolve completely). Female children should be referred to specialized vulval services.The same adult treatment algorithm (clobetasol propionate 0.05%) appears to be effective and safe.
Male treatment guidelines
An association has also been noted between male LS and increased body mass index (BMI), coronary artery disease (CAD), diabetes mellitus (DM), and smoking. The observations that LS is rare in men circumcised at birth and that LS is documented in cases of urostomy, ileostomy, hypospadias, and hypospadias repair suggest that irritation from urinary occlusion may play a central role in LS development.
The algorithm for male patients is somewhat different. After the diagnosis of genital LS is made, clobetasol propionate 0.05% ointment is administered once daily for 1-3 months with an emollient soap substitute and barrier preparation. There is a lack of randomized controlled trials (RCTs) addressing management of male LS, but a large retrospective series demonstrated that 50% of male patients with LS responded to clobetasol propionate.
A follow‐up assessment of response is made at 3 months. If remission is achieved, then a follow‐up is planned for 6 months, followed by discharge back to the general practitioner with an emphasis on self‐examination. A repeat course of topical treatment for 1-3 months is recommended in those who relapse.
Treatment failure in adult and pediatric male patients can result from tight phimosis, which necessitates circumcision. Obesity and burying of the penis can also result in treatment failure, necessitating weight reduction and, in some cases, bariatric surgery and reconstructive penile surgery. Patients might have residual active disease even after circumcision and might then require a repeat course of a potent topical steroid. It is therefore wise to inform all male patients of this possible risk before they consent to undergoing a therapeutic circumcision.
Long‐term follow‐up in a specialist clinic is reserved for patients with troublesome symptoms, atypical disease, previous cancer, or any type of diagnosed or pathologically uncertain intraepithelial neoplasia. Persistent erosions, ulcers, and fixed erythematous areas must be urgently referred for tissue sampling to exclude intraepithelial neoplasia or invasive squamous cell carcinoma.
What is lichen sclerosus (LS)?What is the pathophysiology of lichen sclerosus (LS)?What are the findings within lichen sclerosus (LS) tissue?What causes lichen sclerosus (LS)?Which infections are associated with lichen sclerosus (LS)?Which genetic and autoimmune factors are associated with lichen sclerosus (LS)?What is the role of oral contraceptives in the etiology of lichen sclerosus (LS)?Which inflammatory conditions are associated with lichen sclerosus (LS)?What is the prevalence of lichen sclerosus (LS)?What is the prevalence of genital lichen sclerosus (LS) in prepubertal girls?What is the prevalence of vulvar lichen sclerosus (LS) in elderly women?What is the prevalence of lichen sclerosus (LS) in males?What is the prevalence of extragenital lichen sclerosus (LS)?What are the race-related demographics for lichen sclerosus (LS)?Is lichen sclerosus (LS) more common in males or females?What are the age-related demographics for lichen sclerosus (LS)?What is the prognosis of lichen sclerosus (LS)?What is the prognosis of lichen sclerosus (LS) in pediatric patients?What is the mortality rate for lichen sclerosus (LS)?What are the morbidities of lichen sclerosus (LS)?What is included in the patient education information for lichen sclerosus (LS)?What complaints are associated with extragenital lichen sclerosus (LS)?What are the symptoms of genital lichen sclerosus (LS)?What are the physical findings in lichen sclerosus (LS)?What are the physical findings in vulvar lichen sclerosus (LS)?What is the typical distribution of extragenital lichen sclerosus (LS)?What are the physical findings of female genital lichen sclerosus (LS)?What are the physical findings of male genital lichen sclerosus (LS)?What is the isomorphic (Koebner) phenomenon in relation to lichen sclerosus (LS)?What are complications of lichen sclerosus (LS)?What pitfalls are associated with lichen sclerosus (LS)?What are the diagnostic considerations in lichen sclerosus (LS)?What are the differential diagnoses for Lichen Sclerosus?How is lichen sclerosus (LS) diagnosed?Which study is diagnostic for lichen sclerosus (LS)?What is the role of imaging studies in the workup of lichen sclerosus (LS)?Which procedures are used in the workup of lichen sclerosus (LS)?What are the histologic findings in lichen sclerosus (LS)?Which types of microscopy are used in the workup of lichen sclerosus (LS)?What are the treatment guidelines for lichen sclerosus (LS)?What are the treatment approaches for genital lichen sclerosus (LS)?What is the first-line therapy for genital lichen sclerosus (LS)?What are the second-line therapies for genital lichen sclerosus (LS)?What are third-line therapies for treatment-resistant genital lichen sclerosus (LS)?What is the role of hormone therapy in the treatment of genital lichen sclerosus (LS)?What are the treatment options for extragenital lichen sclerosus (LS)?What long-term monitoring and patient education should be included in the treatment of lichen sclerosus (LS)?What personal hygiene habits can minimize symptoms in lichen sclerosus (LS)?How are topical corticosteroids used in the treatment of lichen sclerosus (LS)?What is the role of topical calcineurin inhibitors in the treatment of lichen sclerosus (LS)?How are retinoid and acitretin used in the treatment of lichen sclerosus (LS)?How is phototherapy used in the treatment of lichen sclerosus (LS)?How is methotrexate used in the treatment of lichen sclerosus (LS)?What is the of lichen sclerosus (LS)?What are the surgical options for lichen sclerosus (LS)?Which tissue destruction procedures are used in the treatment of lichen sclerosus (LS)?When is referral indicated for the surgical treatment of lichen sclerosus (LS)?When is surgical care indicated in the treatment of lichen sclerosus (LS)?What dietary restrictions are indicated in the treatment of lichen sclerosus (LS)?What activity restrictions are indicated in the treatment of lichen sclerosus (LS)?Which specialist consultations are indicated for the treatment of lichen sclerosus (LS)?What is included in the long-term monitoring of lichen sclerosus (LS)?What are the British Association of Dermatologists treatment guidelines for lichen sclerosus (LS) in females?What are the British Association of Dermatologists treatment guidelines for lichen sclerosus (LS) in males?What are the goals of drug treatment for lichen sclerosus (LS)?Which medications in the drug class Retinoid-like Agents are used in the treatment of Lichen Sclerosus?Which medications in the drug class Calcineurin Inhibitors are used in the treatment of Lichen Sclerosus?Which medications in the drug class Corticosteroids, Topical are used in the treatment of Lichen Sclerosus?
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
Disclosure: Nothing to disclose.
Specialty Editors
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
Disclosure: Nothing to disclose.
Chief Editor
William D James, MD, Emeritus Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier<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
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
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.
Extragenital lichen sclerosus demonstrating coalescing pitted white papules. Courtesy of Wilford Hall Medical Center slide files.
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 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.
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.
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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