Lichen Sclerosus

Background

Lichen sclerosus (LS) is a chronic inflammatory dermatosis of unknown cause that most commonly affects the genitalia (vulvar and penile lichen sclerosus), but it can occur at any skin site (extragenital lichen sclerosus). Outdated terms for this conditions include lichen sclerosus et atrophicus, balanitis xerotica obliterans (glans penis presentation), and kraurosis vulvae (older description of vulvar presentation). Lichen sclerosus can occur in males or females of any age, but 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 and/or urinary dysfunction, reduced quality of life, and an increased risk of squamous cell carcinoma (<5%) with genital lichen sclerosus.

• References

Pathophysiology

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 lichen sclerosus (LS) tissue include the following:

• Increased glut-1 and decreased vascular endothelial growth factor (VEGF) expression - Suggesting a role for hypoxia and ischemia)^[1]
• Higher expression of extracellular matrix (ECM) proteins and connective-tissue growth factor (CTGF) - Up-regulation may induce accumulation of ECM proteins and maintain fibrosis in chronic vulval lichen sclerosus^[2]
• Altered expression of isocitrate dehydrogenase enzymes and aberrant hydroxymethylation - Indicating an epigenetic background for the pathogenesis of vulval lichen sclerosus)^[3]
• 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 - Demonstrating an autoimmune phenotype in vulvar lichen sclerosus^[4]
• Distinct expression patterns of tissue remodelling–associated genes in boys with congenital phimosis and lichen sclerosus^[5]
• Decreased or absent epidermal expression of CD44 - Suggesting lichen sclerosus might result from epidermal damage of unknown origin, resulting in CD44-deficient dermal changes with hyaluronic acid accumulation^[6]
• Increased incidence of autoantibodies to EMC protein 1 and to BP180 antigen, with increased circulation of NC16A domain–specific T cells^[7]

• References

Etiology

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

Several older studies have linked borrelial or other infections with lichen sclerosus, yet most other studies have disputed this, with polymerase chain reaction–based studies showing no increased incidence of borrelial infection.^[8]

Genetic and autoimmune factors have been explored, without identification of consistent, reproducible patterns, although autoantibodies to extracellular extracellular matrix (ECM) protein 1 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.^[9] Baldo et al reported that more than 40% of vulvar lichen sclerosus and lichen planus patients have reactive T cells to the NC16A domain of bullous pemphigoid antigen 180^[7] ; however, other studies suggest that the level of autoantibodies is poorly correlated to disease activity and response to treatment. Women with lichen sclerosus have a higher rate of associated autoimmune disease (odds ratio, 4.3), especially for autoimmune thyroid disease, compared with men.^[10]

Local irritation or trauma seems to play a role in some cases of lichen sclerosus, especially in genetically predisposed individuals.^[11] However, the sequence of events that leads to the altered fibroblast function, microvascular changes, and hyaluronic acid accumulation in the upper dermis continues to be researched.

Oral contraceptives in premenopausal women have been shown to give a relative risk of 2.5, which suggests an altered hormonal axis as a possible contributory factor.^[12]

Lichen sclerosus may occur in association with other inflammatory conditions, including psoriasis.^[13] This is surprising because the histopathological findings of lichen sclerosus and psoriasis are dissimilar.

• References

Epidemiology

Frequency

Lichen sclerosus (LS) is a relatively common dermatosis, although the true prevalence of lichen sclerosus is unknown and likely underestimated. This may be because patients are distributed among different specialties (gynecology, dermatology, family practice, urology), but also because one third of cases can be asymptomatic.

The prevalence of genital lichen sclerosus in prepubertal girls is 0.1% (1 in 900).^[14]

The prevalence of vulvar lichen sclerosus in elderly nursing home women in one study was found to be 3% (1 in 30).^[15]

One study found the rate to be approximately 1.7% among women seen in a gynecology practice. The prevalence of male lichen sclerosus is thought to be influenced by the rate of circumcision, as male genital lichen sclerosus is seen almost exclusively in uncircumcised or incompletely circumcised men and boys. Therefore, the rate of circumcision in a given population would thus influence the rate in this subset.

The prevalence of extragenital lichen sclerosus is unclear as a study of foreskins submitted after therapeutic circumcision for phimosis revealed many cases of unrecognized lichen sclerosus. Extragenital lichen sclerosus is much less common than genital lichen sclerosus and is rare in children.

Race

Lichen sclerosus, both genital and extragenital, has no known racial predilection. A genetic predisposition, based on family clustering, is apparent.^[16] . Of patients with vulvar lichen sclerosus, 8-39% report a family history of the condition, while only 1% of male genital lichen sclerosus patients have a family history.^[17]

Sex

The female-to-male ratio is 3:1-10:1.^[17]

Age

Vulvar lichen sclerosus can occur at any age, with increasing incidence with age. In females, the two peaks of onset are during prepubertal and perimenopausal/postmenopausal ages. Interestingly, both are low estrogen states. It is unclear if there is a hormonal relationship or whether this low estrogen state promotes koebnerization due to reduced lubrication. In males, the incidence increases after puberty and then decreases again in older age (>60 years).

• References

Prognosis

Prognosis is good for more acute genital cases of lichen sclerosus (LS), especially for those in the pediatric age group, in whom it may resolve spontaneously. Prognosis for improvement is poor for extragenital cases and for chronic atrophic genital disease.

Many pediatric cases improve with puberty. In contrast, some authors suggest the rate of spontaneous resolution may be lower than 25%.^[18]

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.

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.

An increased risk of squamous cell carcinoma may exist in genital disease, but the precise increase in risk and what cofactors (human papillomavirus infection or prior radiotherapy) may be involved are not yet completely defined. In patients who have been treated for vulvar cancer, the presence or absence of lichen sclerosus does not appear to affect the timing of recurrence.

• References

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.

• References

History

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 lichen sclerosus is itch and sexual/urinary dysfunction in penile lichen sclerosus.

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.

• References

Physical Examination

Pertinent physical findings are limited to the skin.

Skin primary lesion

Lichen sclerosus (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, keeping in mind that other conditions such as chronic cutaneous lupus may also show follicular plugs. With time, the plugs and dells disappear and leave a smooth, porcelain-white plaque. Skin color is white, often with a shiny porcelain appearance. Telangiectases and follicular plugs may be seen. The size of the plaque or plaques may vary widely from a few millimeters, resembling lichen nitidus, to the entire upper trunk.

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

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

Skin distribution

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

View Image

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

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

View Image

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

View Image

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. Penile shaft involvement is much less common, and scrotal involvement is rare. The initial manifestation may be a sclerotic ring at the prepuce edge. Note the image below:

View Image

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 lichen sclerosus along the lines of Blaschko has been described in extragenital cases.^[19]

• References

Complications

Following are male genital, female genital, and extragenital complications of lichen sclerosus (LS):

• 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.^[20] 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. A 2009 study linked coexistent chlamydia infection and lichen sclerosus with increased risk of squamous cell carcinoma, but other sexually transmitted illnesses, such as human papillomavirus infection, were not completely addressed.^[21]
• Extragenital: Complications include cosmetic concerns, but only in extensive cases.

The following issues also may arise:

• 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 adverse 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 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: 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.

• References

Approach Considerations

Diagnosis of lichen sclerosus (LS) may often be made on clinical appearance, and ancillary examinations such as dermoscopy may help confirm the diagnosis.^[22] Biopsy should be performed in questionable cases, and, in follow up, biopsy samples from nonhealing ulcerations or masses should be examined to exclude malignant transformation.

• References

Laboratory Studies

Skin biopsy (punch preferred) is the primary study to perform for the diagnosis of lichen sclerosus (LS). Despite the presence of autoantibodies described in several studies, an autoimmune workup (eg, antinuclear antibody, vitamin B-12 levels, thyroid function tests) is still not generally recommended because the frequency of multiple autoimmune diseases associated with lichen sclerosus is not high enough to justify the expense of screening all patients. 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.

• References

Imaging Studies

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

• References

Procedures

Punch biopsy in the most mature area of the lesion usually is diagnostic. In genital biopsies, snip excisions may suffice. Suturing the wound, especially when using braided suture, which is less likely to poke sensitive skin, leads to more rapid healing than allowing self-granulation.

Ulcerative or vegetative genital lesions may need to undergo biopsy more than once to screen for squamous cell carcinoma. Epidermal hyperplasia and/or dysplasia associated with lichen sclerosus (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, as is a human papillomavirus–associated increase in p16INK4A.^[23] Contrary to this study, Paolino, et al (2013) suggest that human papillomavirus is not identified in most cases of lichen sclerosus–associated malignancy and, when it is, may be a nononcogenic type.^[24]

• References

Histologic Findings

Classic lichen sclerosus (LS) demonstrates a lichenoid infiltrate in the dermoepidermal 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. Note the images below.

View Image

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

View Image

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....

Both dermoscopy and confocal microscopy are in vivo methods of recognizing typical histologic findings for different skin conditions. Lacarruba et al (2015) discuss typical findings in lichen sclerosus, including the appearance of epidermal atrophy and follicular plugging.^[25]

• References

Approach Considerations

An evidence-based treatment guideline was published in the British Journal of Dermatology for lichen sclerosus (LS) in 2010^[26] and more recently, in 2015 from the European Academy of Dermatology and Venereology.^[17] Of note, Table 1 of this 2015 consensus guideline summarizes treatment responses achieved with different therapies in women, men, girls, boys, extragenital, and long term in studies. The author's treatment practice is aligned with recommendations in these guidelines.

Genital lichen sclerosus

Multiple randomized controlled trials (RCTs) evaluating topical interventions, two RCTs evaluating acitretin, and one RCT evaluating para-aminobenzoate exist.^{[27, 28]} The efficacy data for other agents include case reports and small studies. All genital lichen sclerosus cases should be treated, even if asymptomatic, with the goal of preventing scarring and its associated disfigurement, sexual and urinary dysfunction, and reduction in quality of life. Treatment success is typically evaluated every 3 months when actively modifying treatment. Efficacy is gaged by the patient's resolution of symptoms (pruritus and pain) and improved variables on physical examination (reduced ulceration, hyperkeratosis, erythema, ecchymosis, atrophy, and depigmentation). Clinical photography is helpful for monitoring from visit to visit, and scarring is permanent.

First-line therapy includes patient education and super-potent topical corticosteroids (eg, clobetasol propionate). It should be recognized that vulvar lichen sclerosus 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 perianal and hair-bearing skin of the labia majora, which can atrophy within 2-3 wk of use). Although not used by the author, intralesional corticosteroid injections are also considered first-line therapy.

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 in treatment-resistant genital lichen sclerosus could include topical or oral retinoids, steroid injections, cyclosporin (topical shown not to work), methotrexate, or hydroxyurea. For extragenital lichen sclerosus, phototherapy or methotrexate could be considered (treatment regimens analogous to those used for morphea). The author uses 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.

Topical testosterone, topical estrogen, topical progesterone, and hormone replacement therapy should not be used. Although extensively used in the past, there is no evidence base for their use. Topical avocado and soybean extracts as alternative treatments for mild-to-moderate lichen sclerosus have been used in patients wishing to avoid corticosteroids.^[29] The patients also received dietary supplements containing the same substances, along with vitamin E and para-aminobenzoic acid. In a small study of 23 patients, most reported improvement. Other anecdotal therapies include intralesional injection of adalimumab.^[30]

Extragenital lichen sclerosus

There are no RCTs evaluating the efficacy of treatment for extragenital lichen sclerosus, and recommendations are based on case reports and small uncontrolled studies.^[17] Treatment is often extrapolated from data from studies in genital lichen sclerosus and studies in the treatment of morphea. It is important to discuss with patients goals of care, and treat according to the patient's 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. For those with extensive involvement, rapid progression, or a goal of preventing new lesions, phototherapy or systemic therapy should be offered.

• References

Medical Care

Patient education

It is important to discuss the status of the patient's genitalia with the patient (specifically with scarring, ie, that it is permanent), the chronicity of the condition, and the associated frequent recurrences if therapy is not maintained. However, patients should be reassured that the condition can be treated into remission with continued therapy. Malignancy risk, greatest with ulcerative genital lichen sclerosus (LS), should be discussed and monthly self-examinations should be encouraged. 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

Studies exist showing 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 can include putting moisturizer on toilet paper following urination or defecation, using lubrication with sexual activity, and treating locally following known mechanical irritation.

Topical corticosteroids

There is no standardized treatment regimen, although most providers have an initiation phase and a maintenance phase. For initial treatment, the European Academy of Dermatology and Venereology consensus group recommends once or twice daily for 3 months.^[17] The author typically uses the tapering regimen recommended in the 2010 British Journal of Dermatology guidelines,^[26] which includes once daily for 1 month, every other day for 1 month, then 2-3 times per week, with dosing escalation with flares. Tapering is recommended by reducing frequency of use rather than reducing potency. One fingertip unit amount (extending from tip of finger to distal interphalangeal joint) should be used for each application, with no more than 10 g used monthly. Maintenance treatment is necessary, as this is a chronic condition with a high relapse rate. Per European Academy of Dermatology and Venereology expert opinion, some patients only require treatment once or twice monthly, while others may need treatment 2-3 times a week. Given long-term safety data for use of superpotent corticosteroid on mucosa, the author strongly encourages a minimum of 3 times a week as maintenance. If daily use is needed, consideration for the addition of calcineurin inhibitors is recommended.

Topical calcineurin inhibitors (tacrolimus, pimecrolimus)

This is a recommended option for a second agent for maintenance if daily treatment is needed. The author prescribes every-other-day treatment with the goal of a super-potent topical steroid 3 times a week and tacrolimus the remaining days. There is no risk for skin atrophy. Tacrolimus works better than pimecrolimus. In general, tacrolimus has the equivalent efficacy to a midstrength topical steroid. Although in the past there was a question about neoplasia induction and the long-term safety of topical calcineurin inhibitor therapy, consensus is that the risk of neoplasia in lichen sclerosus is due to endogenous inflammation rather than 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 them despite a 1-week trial.

Topical retinoid and acitretin (Soriatane)

One RCT found oral acitretin (20-30 mg/day for 16 wk) was found to be effective compared with placebo in one trial.^[31] Short-contact topical retinoid therapy is reported to have efficacy; however, it is limited by irritation on nonintact skin.^[32]

Phototherapy

Regarding genital lichen sclerosus, a RCT comparing the efficacy of home-administered medium-dose ultraviolet (UV)–A-1 was given 4 times a week with once-daily application of clobetasol ointment in 30 women with vulvar lichen sclerosus, with both groups having significant improvement.^[33] Case reports for psoralen plus UVA, narrowband UVB, and photodynamic therapy successes have been reported.^{[34, 35, 36]} However, the risk of neoplasia in the setting of phototherapy, especially psoralen plus UVA, is unclear. Of note, the risk of developing a squamous cell carcinoma in genital lichen sclerosus is less than 5%, while squamous cell carcinoma development in extragenital lichen sclerosus is rare. The author uses narrowband UVB phototherapy in patients with extragenital lichen sclerosus with extensive or progressive disease in which patients want to prevent new lesion formation while treating the present lesions.

Methotrexate

The efficacy of systemic corticosteroids plus methotrexate is supported by a retrospective study of 10 patients with extragenital lichen sclerosis.^[37] This regimen is often 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 1000 mg per week intravenous methylprednisolone 3 sequential days per month for 3 months with 1 mg/kg per day oral prednisone for 3 months as taper would be reasonable in conjunction.

Outcomes

Genital lichen sclerosus may respond to ultrapotent (superpotent) 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 develop various types of vulvodynia in adulthood. Treatment is necessarily prolonged, and short-term treatments often lead to suboptimal control of findings and symptoms.^[38]

• References

Surgical Care

Circumcision may benefit male patients with lichen sclerosus (LS) and the phimosis that may accompany it. Vulvar surgery has not been recommended unless an associated malignancy is present. More recent articles have suggested that surgical release of clitoral phimosis and labial adhesions may improve some patients with severe dyspareunia, although the numbers were small and some surgical complications were noted.^{[39, 40]} 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.

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 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 ultrapotent topical steroids may obviate the need for surgery in such cases.

• References

Consultations

Consultations with the following specialists may be helpful:

• 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

• References

Diet

There are no reproducible studies relating to diet or reactions to any particular foods, spices, or flavorings. For that reason, no dietary recommendations or restrictions currently are proposed.

• References

Activity

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

• References

Long-Term Monitoring

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 (LS) patients for any sign of secondary or associated genital malignancy. Extragenital cases require no specific follow-up.

• References

Guidelines Summary

The British Association of Dermatologists has issued updated guidelines on the management of lichen sclerosus in males and females.^[41]

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 lichen sclerosus 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 ultraviolet 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.

Female patients must be followed up every 6-12 months until there is symptom control, good sexual function, and no further alteration in architecture. At this time, the patient can be discharged back to her 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 the treatment be tapered to maintain symptom control and resolution of skin thickening and ecchymosis, but not pallor, as pallor is known not always to resolve completely. Female children should be referred to specialized vulval services, and the same adult treatment algorithm using clobetasol propionate 0.05% appears to be effective and safe to follow.

Male treatment guidelines

An association has also been noted between male lichen sclerosus and increased body mass index, coronary artery disease, diabetes mellitus, and smoking. The fact that lichen sclerosus is rare in men circumcised at birth, and that lichen sclerosus is documented in cases of urostomy, ileostomy, hypospadias, and hypospadias repair, suggests that irritation from urinary occlusion may play a central role in lichen sclerosus development.

The algorithm for male patients is somewhat different. After making the diagnosis of genital lichen sclerosus, clobetasol propionate 0.05% ointment must be commenced once daily for 1-3 months with an emollient soap substitute and barrier preparation. There are no randomized controlled trials for male lichen sclerosus management, but a large retrospective series demonstrated that 50% of male patients with lichen sclerosus responded to clobetasol propionate. A follow‐up assessment of response must be done at 3 months. If remission is achieved, then a follow‐up is planned for 6 months, followed by discharging the patient back to his 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 requires circumcision. Obesity and burying of the penis can also result in treatment failure; this necessitates 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.

• References

Medication Summary

The goals of pharmacotherapy in lichen sclerosus (LS) are to reduce morbidity and to prevent complications.

• References

Class Summary

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

• References

Class Summary

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.

• References

Class Summary

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 topical immunomodulator 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. 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. 

• References