Almost every fifth woman at least once in her life experiences tangible subjective disorders in the vulva, and this may well be vulvar lichen sclerosus. Unfortunately, this disorder often goes undiagnosed and therefore untreated, but not only does it cause significant physical, emotional, and sexual discomfort, it can also lead to permanent genital damage or even squamous cell carcinoma. In order to minimize complications and improve the patient's quality of life, practitioners must have the skills to recognize and manage lichen.
Definition, epidemiology of vulvar lichen sclerosus
Vulvar sclerosus sclerosus (SALS) is a chronic, inflammatory skin condition that can occur anywhere on the body in men, women, and children, but most commonly affects the anogenital region of adult women. Although there are currently no reliable treatments for SALV, the disease can be effectively managed.
Prevalence
The true prevalence of SALV is difficult to determine because the disease often goes unnoticed and is not always diagnosed. The most accurate estimates show that the prevalence of SAL among the female population is 1 per 1000 women and is 10 times higher than the prevalence of SAL in men. SALV peaks in the prepubertal and postmenopausal period, with an increase in the prevalence of SALV of up to 1 in 30 in postmenopausal women.
Etiology
Although the causes of SALV are not currently known, experts have identified strong associations between SALV and autoimmune disease, genetic factors, and low levels of endogenous sex hormones. Thorstensen and Birenbaum found that up to 20% of women with PAL have co-morbid autoimmune diseases, such as thyroid disease, vitiligo, and alopecia areata. Other scientific studies have shown that 44%-74% of women with SALV have circulating autoantibodies. The high prevalence of SALV among first-degree relatives supports the theory that there is a genetic component to this condition. Up to 12% of patients with SALS have a positive family history of the disease. Low endogenous estrogen levels are also not uncommon in patients with SALV. This association is supported by the significantly higher incidence of SALV among postmenopausal women. However, no change in symptoms was observed during menstruation, pregnancy, use of oral contraceptives or hormonal therapy.
Main characteristics of vulvar lichen sclerosus
Symptoms
SALS may be aggressive or asymptomatic for long periods of time. The hallmark of SALV is vulvar itching, which can be mild and intermittent or intense and persistent. This condition results in cracks in the vulvar area, which can occur spontaneously or with the help of physical irritation from scratching and/or sexual intercourse. Fissures of the vulva and subsequent scarring often result in dysuria, dyspareunia, pain during defecation, and rectal bleeding.
Expertise
SALV causes characteristic skin color changes in the vulva. The skin becomes shiny, white, in the form of corrugated tissue paper with lesions localized in the pubic area, perineum. Skin changes occur only on the external genital organs without involving the vagina and internal genital organs in the process. Flat, shiny white plaques are surrounded by red, purple, or violet-tinted borders, suggesting atrophy and scarring caused by years of SALV. Structural changes such as reduction and flattening of the labia, clitoral involvement, narrowing of the vaginal opening may occur in the absence or ineffective treatment of SALV. Secondary skin lesions are usually present and may include vulvar edema, erythema, excoriations, and fissures. Attachment of infection can increase inflammation with blistering. Deep erosions are not usually seen in SALV unless it is accompanied by significant scratching or vulvar intraepithelial neoplasia (VIN) or squamous cell carcinoma (SCC) develops.
Diagnostic testing for vulvar lichen sclerosus
Biopsy of the vulva
Vulvar biopsy is the gold standard for confirming pathologies such as SALV, RCC, and INV. Biopsy is mandatory if malignant transformation is suspected, if there is no response to adequate treatment, in the presence of extragenital foci of LES, pigmented areas observed in INV or RCC, and if second-line therapy is required. A biopsy before initiating treatment with topical corticosteroids is recommended because these drugs distort the results of the biopsy. With a typical clinical picture, a biopsy may not be performed. However, since PALW is a chronic, long-term condition that requires long-term treatment and follow-up, a confirmatory biopsy prior to treatment is recommended.
Microscopy for urogenital microflora
Although urogenital microbiota are not usually required for the diagnosis of SALV, they may be useful in ruling out concomitant conditions such as bacterial vaginosis, trichomoniasis, vulvovaginal candidiasis, herpes simplex virus, and other STIs. Taking material for STIs is indicated for pathological discharge, inflammation, cracks or excoriations in the vulva. Puffiness, hyperemia, white cheesy plaque in the area of the labia are often easily identified as vulvovaginal candidiasis. However, SALV should be kept in mind, especially if the infection recurs or does not respond to antifungal therapy.
Possible complications of vulvar lichen sclerosus
Complications from the progression of SALS cause patients to become frustrated, anxious, and embarrassed by the physical and emotional impact of the condition. The next exacerbation or often recurring chronic symptoms of genital itching, irritation, burning and pain can significantly impair the patient's quality of life. Left untreated or inadequately treated, irreversible structural changes develop, causing sexual dysfunction and difficult relationships.
Furthermore, SALS has a lifetime risk of developing 3-5% of RCC cases. Approximately two-thirds of vulvar cancers occur in close proximity to SALV. Vulvar cancer is usually not diagnosed immediately, although patients may have vulvar symptoms for many years. This is primarily due to untimely referral and insufficient skills of health workers in diagnosing such conditions of the genital organs.
Pharmacotherapy for vulvar lichen sclerosus
First line therapy
Topical ultrapotent corticosteroids
The most widely accepted and recommended gold standard for the treatment of SALV is the topical application of ultrapotent corticosteroid ointments, especially 0.05% clobetasol propionate ointment. The anti-inflammatory properties of clobetasol are most effective in the treatment of SALV, which is reflected in the reduction of inflammation and the prevention of progression of the condition and subsequent scarring. The mucous membranes of the vulva are relatively resistant to steroids, which suggests the use of ointments with maximum steroid potency to achieve the effect. Ointments have fewer additives than creams and are therefore less likely to cause skin irritation.
Initial treatment of acute inflammation should be timely and aggressive. Clobetasol propionate 0.05% ointment is recommended to be applied topically 2 times a day for 4 weeks, followed by 1 time per day for 3 months and then in a dosed non-daily application for a long time (maintenance treatment). Patients should apply the corticosteroid ointment sparingly, strictly only on dry, affected areas, and should continue to use the dose once or twice a week even during remission. If symptoms increase with a decrease in the frequency of lubrication, the frequency of application should be increased until the symptoms begin to disappear. Because relapses can be chronic and lead to atrophy and scarring, long-term maintenance therapy is recommended and is considered safer.
In a study by Gurumurthy et al. reported that when testing ointment clobetasol propionate, complete remission was achieved in 66% of patients, and in another 30% - a partial response to treatment. The scars have not progressed. Conversely, there was no improvement in 75% of patients who received no treatment, and scarring progression was observed in 35%. Although rare, side effects of long-term topical anogenital corticosteroid therapy include skin thinning, rebound reactions, striae, fungal infections, and adrenal suppression due to systemic absorption. Bradford and Fisher found that all side effects quickly resolved as the activity of topical corticosteroids decreased.Second line therapy
Topical Calcineurin Inhibitors (TCIs)TICs are currently recommended as second-line therapy for SALV. Pimecrolimus 1% cream is an immunosuppressant that inhibits T cell activation and thus significantly reduces the itching, burning, and inflammation associated with SALV. Although TECs can provide effective symptom relief, topical clobetasol is superior to pimecrolimus in terms of reducing inflammation and improving the clinical picture. Although pimecrolimus cream has an acceptable safety profile and does not cause skin atrophy, its use is associated with an increased risk of RCC due to local immune suppression. As a result, TECs should be administered under the supervision of a specialist who can monitor the potential risk of SALV malignancy. Given the proven efficacy and safety of topical corticosteroid ointments, experts agree
Retinoids
Some evidence does indicate that retinoids may be effective for treating hyperkeratosis or scarring that does not respond to corticosteroids.
Sedatives
Prescribed to suppress the main symptom of SALV, itching, which causes the patient to scratch the skin, especially at night, when they are not aware of their actions. These complications can be prevented if patients use sedatives, antihistamines, especially at bedtime. In addition, the lipophilic properties of antihistamines allow these drugs to cross the blood-brain barrier, resulting in a sedative effect. However, the use of antihistamines as a hypnotic is not recommended for more than 3 consecutive days due to the development of patient tolerance to the sedative effect. Long-term use of antihistamines can lead to side effects such as daytime sleepiness, dry mouth, dizziness, and memory problems.<
Non-drug treatment
In addition to drug therapy, patients should be informed of the need to avoid skin irritants. These non-pharmacological interventions can improve symptom management and improve quality of life. Eliminating potential skin irritants such as soap, sanitary pads, excessive brushing, and wearing tight clothing can reduce local inflammation. Measures such as cool packs of gel, use of SITZ baths, and daily application of petroleum jelly can improve patient comfort, all of which provide temporary relief of symptoms. Emollients free of potential allergens such as propylene glycol and lanolin can also minimize local inflammation.
Moisturizers are effective daily safe excipients. Emollients increase the moisture content of the stratum corneum, which enhances the weakened skin barrier function and reduces subclinical inflammation. A study by Simonart et al. concluded that more than 50% of women who used a daily moisturizer along with topical corticosteroids maintained remission for a median of 58 months. In this study, more than two-thirds of women stopped using topical corticosteroids while remaining on long-term emollients.
Surgical treatment
Surgery to remove diseased vulvar tissue is not indicated in uncomplicated cases of SALV. The surgical method is generally reserved for cases of malignancy of the SALS or the development of severe scarring and adhesions that interfere with genitourinary and/or sexual function. In the postoperative period, the use of dilators that reduce the recurrence of narrowing of the vaginal inlet is often justified. With the development of RCC of the vulva, the most appropriate specialist for surgical treatment is a gynecologist-oncologist. Mechanical problems associated with scars and adhesions are effectively eliminated with the help of a gynecologist-surgeon.
Alternative Therapy
Dermatologists and gynecologists use several alternative treatments for SALV with varying degrees of success. Some of these alternative treatments include photodynamic therapy, ultraviolet phototherapy, cryotherapy, and laser vaporization. Although these procedures improve symptoms in many patients, there is anecdotal evidence that these alternative therapies contribute to the slow progression of the disease. Therefore, alternative therapies should be the subject of further research before they can be recommended for patients with SALW.
Long-term patient follow-up is required in cases where persistent symptoms persist and/or local skin infiltration is present, if the patient has a history of SCC or VIN, if VIN is suspected, in treatment-resistant cases, when using more than 30 g topical corticosteroids per day for a 6-month period and when using second-line therapy.
SALV can have significant psychological and sexual effects on women and their partners. Therefore, according to indications, a patient with SALV should be consulted by a psychotherapist. It is necessary to rule out depression. Counseling must be done in the right way. In addition, many experts also encourage the participation of sexologists and sexologists who are more highly qualified in counseling patients with SALW to help patients with sexual health issues.
Due to the chronic nature and propensity for a progressive course of SALS, it is critical that patients understand the importance of maintenance therapy with topical corticosteroids. Patients should receive an explanation of the warnings in the instructions for topical corticosteroids (TGCS), which may prevent the patient from following the doctor's orders. Although side effects have been associated with long-term use of THCS, these effects are very rare. Frequent follow-up of patients with SALV ensures early detection of any side effects.
In addition, patients should be educated on the proper use of THCS, with emphasis on thorough handwashing, avoiding contact with sensitive areas of the body such as the eyes. Patients should also be aware that symptoms may improve with intermittent THCS treatment, but previous scarring is irreversible.
To increase health promotion and disease prevention, all women should be aware of the need and procedure for self-examination of the vulva. In-house monthly self-tests help detect potential changes early. Using a mirror, the patient should carefully examine the pubis, labia, labia minora, perineum, and anus for any noticeable changes, incl. skin color and any new rashes. In addition to individual interviews, the physician should provide patients with written instructions describing available pharmacological and non-pharmacological procedures.
Unfortunately, in many cases, the pathology of the vulva goes unnoticed in the absence of adequate treatment. Women may feel uncomfortable discussing issues related to their genitals when visiting a doctor. In this regard, health workers should take the initiative in such cases. Talking to patients about disease prevention enables women to take control of their own health and improve their quality of life.
According to the materials of the journal "Dermatology"
Unfortunately, many people do not even realize that their sexual problems are not a matter of whispering with a friend or discussing with a sex therapist, but a reason to go to an aesthetic medicine clinic and without much difficulty - and most importantly, quickly and forever - get rid of these problems. Modern medicine has many different opportunities to improve the intimate health of patients, make their sex life brighter and richer:
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