Lichen sclerosus et atrophicus (better known as just lichen sclerosus, LS) is a chronic inflammatory skin disorder that results in significant compromised architectural changes in the vulva and anal area. LS affects approximately one in 70 women, usually presenting in girls before their first menstrual period and in women after menopause. It can lead to loss of vulvar anatomical structure, depigmentation, scarring, fissures, and narrowing of the vaginal opening.
Vulvar irritation is the most common presenting symptom, although LS may be asymptomatic and found incidentally on examination. With progression of untreated disease, ongoing scratching leads to erosions and scarring. The chronic nature of the disease can significantly compromise quality of life. Proper diagnosis, treatment, maintenance, and regular follow-up are key to the successful management of LS.
The exact cause of LS in unknown, but several theories have been posed. A strong association with autoimmune disorders has been reported in up to 35% of LS patients, and experts believe it behaves much like an autoimmune disease. LS is associated with autoimmune disorders such as alopecia areata, vitiligo, psoriasis, Hashimoto’s thyroiditis, Graves disease, diabetes type I, and pernicious anemia.
There seems to be a genetic component to LS as family clustering has been demonstrated. Higher rates of LS have been reported among family members, particularly twins.
LS is not infectious, but infection with both bacterial and viral pathogens has been implicated in its causation, such as the Borrelia bacterium (which causes Lyme disease) and the human papilloma virus (HPV) and hepatitis C virus (HCV).
Lichen sclersosus causes substantial discomfort, and even pain, in the vulva and around the anus, with persistent itching, burning, stinging, and lingering soreness. Riding a bike or wearing tight clothing can make these symptoms worse. In addition vulvar itching, there may be vaginal burning. Repeated scratching and rubbing lead to blistering, bruising, and bleeding of the skin. As mentioned, if untreated LS progresses, erosions and scarring develop, and in advanced cases, the scarring can tighten the skin around the urethra, anus, and opening of the vagina. This leads to difficult urination, constipation, and painful intercourse.
Of all the quality of life domains impacted in LS patients, sexual function has been found to be most affected. Women with LS report significantly lower sexual desire, arousal, lubrication, orgasm, and satisfaction. Improvement in sexual function depends on the success of treatment and improvement in the woman’s genital self-image.
There is often a delay in diagnosis of lichen sclerosus due to its initial asymptomatic nature and lack of awareness not only among patients but also physicians. Other reasons for delay in diagnosis are embarrassment on the part of the patient because of the private nature of the disease and the failure to examine the vulva.
Diagnosis depends on careful examination of the vulva and recognition of the different signs of LS at the different stages of disease. The diagnosis is confirmed with a vulvar biopsy, performed in the office under local anesthesia.
Ivory-white patches first appear on the vulva. As these patches enlarge and fuse, the now white, de-pigmented vulvar skin becomes smooth, shiny, thin, and/or transparent. Sometimes the skin may look “crinkled.” Alternately, the affected skin may become hyper-pigmented.
As the LS worsens, the skin starts to crack and split, because it loses its pliability, so painful fissures develop. As itching continues, further scratching causes the breakage of blood vessels under the skin, so red blotches are seen. At the most advanced stage of LS, the labia of the vulva flatten out and fuse at the midline. This fusion can cover the clitoris and narrow the vaginal opening, affecting sexual function.
Lichen sclerosus cannot be cured, but it can be well controlled. First-line therapy is potent topical steroid ointment, Clobetasol Propionate ointment. Clobetasol ointment relieves the itching and inflammation in 60-90% of women with LS when used daily for the first month. It is then applied every other day for the second month, and twice per week for a third month. Depending on response durability, there may or may not be a need for maintenance therapy thereafter.
There is no standard second-line therapy for LS, although Carbon (CO2) dioxide laser therapy, Mona Lisa Touch, has been studied for use in women whose LS doesn’t respond well, or any longer, to clobetasol ointment, or women who prefer not to use ointments for an extended period of time. It appears to provide good symptomatic improvement.
It is well established that women with LS have up to a 7% chance of developing vulvar cancer. Women who remain on regular (not as-needed) maintenance clobetasol ointment therapy decrease their risk of developing vulvar cancer by nearly 100%. It is not known if regular maintenance CO2 laser therapy offers a similar vulvar cancer risk reduction. However, the CO2 laser’s beneficial effect on symptoms and on vulvar skin integrity justifies its use in clobetasol-resistant women.
We at Adaptive Gynecology have treated many women with vulvar lichen sclerosus and can help with this frustrating and quality of life limiting condition.
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