Synonyms
Subdivisions
- Anogenital
- Extragenital
General Discussion
Lichen sclerosus is a common inflammatory disorder that affects the skin in the areas of the anus and genitals, and the most common condition among women seen in specialty clinics. The incidence in a general gynecology clinic of biopsy proven lichen sclerosus is 1.7% (6). This skin condition tends to affect women in states of low estrogen, with higher prevelence in girls before puberty and women in menopause.
Lichen sclerosus is thought to be an autoimmune disorder and associated with inflammation in the dermis layer of the skin. The presenting symptoms are itching (pruritus) and soreness, potentially progressing to scarring and painful intercourse.
The incidence of lichen sclerosus is increasing and early treatment is directed toward improving symptoms and maintaining normal skin architecture.
Signs & Symptoms
General symptoms of lichen sclerosus include:
- Itching (pruritus)
- Pain with sex (dyspareunia)
- Generalized genital irritation
- Perianal skin irritation, cracking and itching; painful defecation
- Pain with emptying of bladder
General signs of lichen sclerosus include:
- White areas on skin of the labia minora and majora, often thickening progressively over time
- Skin thinning and susceptibility to easily splitting and bleeding
- Shrinking of the vaginal opening with progressive disease
- Fusion and scarring of the clitoral hood
- Skin breakdown associated with scratching
- Loss of vulvar architecture, with absorption of the labia minora into the labia majora
Causes
The cause of lichen sclerosus is unknown but thought to be an autoimmune condition because women with lichen sclerosus often have other auto-immmune conditions such as alopecia (baldness), vitiligo(white skin patches), thyroid disorders, pernicious anemia, and diabetes mellitus. There is also thought to be a genetic component and it is seen to be grouped among family members.
Lichen sclerosus can be associated with hormone factors. While the condition is more often seen in women with low estrogen levels, treatment with hormone medications including estrogen or testosterone has not been shown to be effective treatment.
Affected Populations
Lichen sclerosus is a common condition in women seeking care for vulvar skin conditions and is more prevalent in women with low estrogen levels.
Diagnosis
The criteria for lichen sclerosus remains the constellation of signs and symptoms, and often do not require a biopsy for diagnosis. However, a biopsy can be useful for diagnosis and to confirm there is no malignant transformation, either vulvar intraepithelial neoplasia (VIN), or squamous cell carcinoma (SC). A biopsy can be recommended for diagnosis but early lesions can be non-diagnostic.
Diagnosis from symptoms can often take up to 5 years, therefore seeing a practitioner knowledgeable in vulvar conditions is important when symptoms are present. Often the services of a specialty trained pathologist are required for diagnosis and the microscopic diagnostic criteria include thinning of the skin, areas of thickened collagen, loss of normal skin layer patterns (rete pegs), loss of normal architecture, and generally the presence of inflammatory cells.
Standard Therapies
There is no cure for lichen sclerosus but it can be successfully treated to manage symptoms, to avoid loss of minora architecture, reduce the likelihood of scarring and vaginal narrowing, and to reduce risk of VIN. The risk of vulvar skin cancer is increased but less than 5%, and higher in women who do not maintain regular treatment.
The initial treatment consists of high-potency steroid applied sparingly to affected areas, using a mirror to see and a cotton swab to apply. The initial frequency can be a clinical judgment based on severity at presentation starting with once to twice daily, followed with a taper as it improves. A common regimen is 1-2 times daily until signs and symptoms improve, followed by the taper. Many women require treatment even at a low frequency for their entire lives after diagnosis. Symptoms usually return if therapy is not maintained. (5) Often flares will occur which require an increase in application frequency and then tapering to a point of being able to maintain good results.
Close follow-up is recommended to monitor results of the medication use. A biopsy should be done if it appears the lichen sclerosus is not responding to therapy to confirm diagnosis and that there are no precancerous or cancerous cells. Re-evaluation is recommended to confirm correct steroid application and to rule out secondary infection.
Second-line therapy includes tacrolimus which is applied once or twice a day. It can cause more irritation to the skin, therefore, if used, the dose strength must be gradually increased. (5)
Vaginal dilators may be used to treat narrowing of the vagina and vaginal opening (introitus or vestibule). In some cases lubricants and moisturizers may also be helpful. If significant stenosis or narrowing has occurred, surgical release of adhesions could be required by a surgeon experienced in treatment of lichen sclerosus.
Investigational Therapies
UVA1 phototherapy (PDT) is considered investigational.
Support Available
- Lichen Sclerosus & Vulval Cancer UK Awareness
https://lsvcukawareness.weebly.com/ - Vulvar Lichen Sclerosus Support Group - Facebook
https://www.facebook.com/Lichen.Sclerosus.Sclerosis/ - ISSVD Patient Handout - Lichen Sclerosus
https://www.issvd.org/wp-content/uploads/2020/09/VulvarLichenSclerosus2020.pdf - Vulval Pain Society
https://vulvalpainsociety.org/
References
- Childhood vulvar lichen sclerosus: an increasingly common problem. AU Powell J, Wojnarowska F SO J Am Acad Dermatol. 2001;44(5):803.
- Lichen Sclerosus: Incidence and Risk of Vulvar Squamous Cell Carcinoma. AU Bleeker MC, Visser PJ, Overbeek LI, van Beurden M, Berkhof J SO Cancer Epidemiol Biomarkers Prev. 2016;25(8):1224. Epub 2016 Jun 2.
- Lichen sclerosus: a review and practical approach. AU Funaro D SO Dermatol Ther. 2004;17(1):28.
- Dtsch Arztebl Int. 2016 May; 113(19): 337–343.Published online 2016 May 13. doi: 10.3238/arztebl.2016.0337
- A double-blind, randomized prospective study evaluation topical clobetasol versus topical tacrolimusAU Funaro D, Lovett A, Leroux N, Powell J SO J Am Acad Dermatol. 2014 Jul;71(1):84-91. Epub 2014 Apr 3.
- Prevalence of vulvar lichen sclerosus in a general gynecology practice. AU Goldstein AT, Marinoff SC, Christopher K, Srodon M SO J Reprod Med. 2005;50(7):477.
