Lichen sclerosus (LS) is a chronic inflammatory condition of the skin; While it may occur in any part of the body it typically involves the vulvar and perianal regions in women. The cause of LS is not well understood, but it is thought to be an autoimmune process that breaks down the skin, perhaps exacerbated by chronic irritation of the affected area — all causing inflammatory changes that result in characteristic white patches and thinning of the skin in the vulva.
Most women with LS have itching in the vulvar region, and may also experience:
- pain when urine or water touches the area
- genital bleeding and blistering
- if left untreated, significant scarring of the genital areas and significant sexual dysfunction.
Women with LS may thus also experience discomfort during intercourse.
It is just s important to realize that an increased risk of squamous cell carcinoma may exist in vulvar LS. The precise increase in risk and what cofactors (such as human papilloma virus infection) may be involved are not yet completely defined, but yhe lifetime risk of vulvar cancer may be as high as 5% in women with LS and may be associated with a longer duration of the disease.
LS is a diagnosis made by looking at affected tissue under the microscope; therefore it can only be diagnosed with a biopsy. Skin biopsies of the vulva usually consist of a very small (5mm) piece of tissue which is taken in the doctor’s office under local anesthesia. The liberal use of biopsies is recommended because skin changes in the vulva can be difficult to diagnose, and some benign conditions can mimic malignant ones. Skin specimens may be sent to pathologists who specialize in skin disorders (so called, dermatopathologists).Ulcerative vulvar lesions may need to be biopsied more than once to screen for squamous cell carcinoma.
Epidermal hyperplasia and/or dysplasia associated with LS on vulvar biopsy specimens are associated with an increased risk of malignant transformation. Due to this increased risk of vulvar malignancy in these situations, women may be referred to specialists for care.
Treatment of LS usually consists of two goals: stop the inflammatory response and heal the broken-down tissue. To stop the inflammatory response topical steroids are typically employed. These may be higher on the dosage side such as Temovate, usually applied until the lesion is cleared and then the frequency or dosage is then decreased. Treatment however should continue indefinitely in order to stop any ongoing scarring. Topical testosterone was mainstay of treatment for female genital LS for decades, although it was never shown useful genital or extra genital cases. Recent studies suggest it may not be any more efficacious than placebo. Other treatments include topical progesterone, Vitamin D derivatives, and retinoids.
- Tacrolimus, which is an immunomodulator and acts like a non-steroidal anti-inflammatory agent, has been approved for the treatment of atopic eczema and has shown its efficacy in the treatment of vulvar sclerosis in a limited number of patients.
- Pimecrolimus cream 1% (Elidel®) is a topical agent Ongoing studies are comparing the effectiveness and safety of Elidel 1% cream versus an ultra-potent corticosteroid for the treatment for vulvar LS.
- Topical estrogens may also be prescribed to heal and build up the vulvar and vaginal tissue. Topical estrogens without alcohol should be prescribed. Changes in hygiene may also help to decrease symptoms.
It is better to avoid soaps, bubble baths, talc and anything with artificial colors and scents. Natural emollients should be used as often as necessary, and many women will get relief with Vaseline in addition to steroid use. In addition, cotton underwear is best. Allergic contact dermatitis may develop with any topical therapy, including steroids and should be considered when symptoms worsen in an unexplained fashion.
The bottom line: LS can be an uncomfortable skin condition of the vulva, but if it is properly treated, symptoms and side effects can be controlled. When it affects the vulva LS should be treated on a chronic and ongoing basis, regardless of the presence or severity of symptoms; otherwise, the resulting scarring and narrowing is irreversible and may interfere with urination and/or sexual intercourse. It is also important that women and there caregivers be aware that women with LS are at increased risk for vulvar cancers and thus the liberal use of skin biopsies and referrals to specialists be encouraged when new lesions occur or are difficult to manage. Standard therapies consist of treatment with steroids, but the newer therapies may be employed in difficult or unusual situations.