Lichen Sclerosus Treatment & Management

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.

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]

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.

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

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.

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.

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.