Vulvar Lichen Sclerosus
Sunday, February 05, 2012
Lichen Sclerosus (LS) is a dermatologic disorder that can affect both men and women as well as young children, but it is most common in women.
Photo: Lara J. Burrows, MD, MSc
More recent prevalence rates indicate that LS is relatively common, affecting one in seventy adult women. There is a bimodal peaked incidence in premenarchal girls and in menopause women with the average age of diagnosis being 51 years of age.
The etiology of LS has not been completely elucidated, however it is most likely an autoimmune disorder. It is highly associated with other auto-immune disorders including autoimmune thyroid disease, alopecia areata, viteligo, pernicious anemia, and Lichen Planus. There may be a genetic predisposition as well. Women with LS have a 4-6% risk of developing vulvar carcinoma and LS has been found in greater than 60% of cases of squamous carcinoma of the vulva. Therefore, women who have LS should be counseled that they need routine surveillance and vulvar examinations.
Lichen Sclerosus typically occurs in the vulva, although it can be found on other parts of the body such as the upper arms and breasts. Extra genital lesions may occur in about 10% of female patients. Lichen Sclerosus may involve the labia minora and inner portion of the labia majora, interlabial sulcus, clitoris, and the perianal region, but almost never involves the vagina. On examination, white atrophic plaques (“cigarette paper”) can be seen, as well as depigmentation, submucosal hemorrhage and scarring with narrowing of the introitus and distortion of or loss of normal vulvar architecture. Over time, scarring and decreased elasticity of the skin may predispose to fissures. Sometimes, scar tissue forms between the clitoral prepuce and the glans clitoris leading to clitoral phimosis.
A biopsy should be performed to confirm the diagnosis as the histopathologic changes of LS are distinctive and make biopsy a useful diagnostic tool. It is essential that the biopsy is performed prior to starting corticosteroids as the histologic changes of LS can resolve with the application of corticosteroids. While some patients are asymptomatic, most give a history of pruritis or pain. Additionally, women with LS are less likely to be sexually active than women without LS.
There is no cure for LS, although symptoms can be managed. After histologic confirmation of the diagnosis and the biopsy site has healed, the mainstay of treatment is ultrapotent topical corticosteroids, such as clobetasol propionate applied daily until all active disease has resolved (for example, the patient is no longer complaining of itching). To facilitate absorption of the topical medication, patients may be instructed to soak in warm water before applying the medication. Patients should be seen regularly after initiating therapy to confirm improvement and to taper the medication as ultrapotent topical corticosteroids should not be used on a daily basis indefinitely. Areas of ulceration that do not resolve after appropriate treatment with corticosteroids must be biopsied to rule out vulvar intraepithelial neoplasia or carcinoma.
Once improvement has been demonstrated, the frequency may be tapered down to once or twice per week, usually titrated to the patient’s symptoms. It is imperative that the clinician counsel patients that LS is a chronic disease and that treatment only when symptomatic is not sufficient as there can be active disease without symptoms. Thus, patients require lifetime therapy for this disorder. Frequently, patients are managed with a once weekly application of clobetasol and an annual thorough vulvar examination with vulvoscopy as indicated.
In summary, vulvar LS is a dermatologic disorder which typically presents with itching and in advanced cases can causes significant distortion of vulvar anatomy. Vulvar LS connotes an increased risk of vulvar cancer. Although it is a chronic condition, most patients respond well to treatment with topical ultrapotent corticosteroids. Patient should be followed with yearly vulvar examinations.
Dr. Lara Burrows is director of The Vulvar and Vaginal Disorders Center at Summa Akron City Hospital and a physician with Summa Physicians Inc. – Gynecology. She is fellowship-trained in female pelvic medicine and reconstructive surgery and is an elected member of the International Society for the Study of Vulvovaginal Disorders and a member of the International Society for the Study of Women’s Sexual Health. She is one of a few physicians in the country selected to participate in the National Vulvodynia Association’s treatment registry.
MD News January/February 2012, Cleveland/Akron/Canton