Synonyms
Subdivisions
- Erosive Vulvovaginal Lichen Planus (most common)
- Classic Papulosquamous Lichen Planus
- Hypertrophic Lichen Planus
General Discussion
Lichen planus is an uncommon inflammatory disorder that affects the skin and mucous membranes of the mouth and genitals. [1] There are many subtypes of lichen planus that can affect various areas of the body including skin, oral mucosa, vulva and vaginal mucosa, esophagus, ocular mucosa, nails, and scalp. We will only be focusing on types affecting the vulva and vagina in our discussion here; however, it should be noted that if symptoms are occuring in other areas of the body it may help distinguish a diagnosis of lichen planus from other vulvovaginal conditions. In a retrospective review of 100 patients performed by the Mayo Clinic only 31% of patients experienced only vulvovaginal lichen planus.
Three subtypes of lichen planus affect the vulvovaginal tissue: classic, erosive, and hypertrophic. Erosive lichen planus is the most common type to affect the vulva and vaginia. Hypertrophic lichen planus is the most rare subtype. [1] Lichen Planus is a chronic condition with symptoms that can vary over time and has no known cure. However, many women respond to medical therapy.
LIchen planus is thought to be an autoimmune condition against the basal keratinocytes (cells in the skin layer). This produces the characteristic destructive patterns. Treatment is directed toward reducing the over-active immune process and allowing for natural healing.
Signs & Symptoms
Symptoms
General symptoms of vulvovaginal lichen planus include:
- Burning
- Pain with sex (dyspareunia)
- Generalized genital pain
- Itching (pruritus)
- Genital soreness
- Bleeding after sex
Signs
- Erosions: red, shiny, raw skin, often surrounded by a white border
- Scarring
- Narrowing of vaginal opening
- Fusion and scarring of clitoral hood
- Vaginal erosions
- Vaginal discharge if vaginal involvement present
Lesions and erosions vary based on the subtype:
Erosive Vulvovaginal Lichen Planus
Typically bright red erosions occur on the vulva or in the vagina. [2] Vaginal involvement is a key feature that differentiates lichen planus from other types of vulvovaginal skin disorders. The vagina may have patchy erosions and excessive amounts of discharge. The resulting discharge can cause contact irritation on the mucous tissues at the vaginal opening. Because of the intense inflammation associated with the disorder, scarring and vaginal strictures may occur. [1] In advanced diseases vulva and vaginal scarring, loss (or re-absorption) of the labia minora, narrowing of vaginal opening and burying of the clitoris can occur. [2]
Hypertrophic Lichen Planus
This rare condition is marked by thickened white skin patches on the perineum and vulva.
Classic Papulosquamous Lichen Planus
This rare condition is marked by violet to red very pruritic (itchy) small papules (tiny raised bumps) on the perineum and labia majora or outside surface of labia minora. [2]
Causes
LIchen planus is thought to be an autoimmune condition against the basal keratinocytes (cells in the skin layer). This produces the characteristic destructive patterns. Treatment is directed toward reducing the over-active immune process and allowing for natural healing.(5)
Affected Populations
Lichen Planus is a rare condition. The prevalence of the disorder in the general population is thought to be 0.5-2% but is estimated to affect 3-6% of patients seeking help in vulva speciality clinics. Lichen Planus tends to occur in women 50-60 and there is no established correlation between race or ethnicity. [1]
Diagnosis
The World Health Organization (WHO) has a set diagnostic criteria for oral lichen planus; however, none exists for vulvovaginal lichen planus. In 2012 an expert panel was formed to establish agreed upon criteria for erosive lichen planus that affects the vulva. This panel’s recommendations were published in 2013. [4] This criteria can be used as a guideline for providers but more research is needed to fully understand and diagnose vulvovaginal lichen planus.
Often a biopsy is recommended for diagnosis but interpretation can require the services of a specialty-trained pathologist. Indications for the biopsy include thickened white edges, non-healing sores, lack of response to therapy, and desire for definitive diagnosis. Such a biopsy should be a 4 mm punch biopsy performed at the edges of the lesion to include normal skin. (1) The biopsy can be important to guide treatment but also rule out precancerous or cancerous changes associated with human papillomavirus (HPV) infections (vulvar intraepithelial neoplasia, or VIN).
A consensus document developed by the Difficult Pathologic Diagnoses committee of the International Society of the Study of Vulvovaginal Diseases (ISSVD) advises that all five of the following clinicopathologic criteria be present for the diagnosis of erosive vulvar lichen planus:(6)
- Well-demarcated, glazed, red macule or patch on the vestibule, labia minora, and/or vagina
- Lesions located on nonkeratinized squamous epithelium, mucocutaneous junction, and/or adjacent hairless skin
- A band-like, inflammatory infiltrate closely applied to the epithelium
- Basal layer damage
- Absence of sclerosis
Standard Therapies
There is no cure for vulvovaginal lichen planus but it can be treated to manage symptoms and significantly improve quality of life. Once the lesions have shown response to initial, often daily, treatment, it is important to continue a maintenance regimen. Also it is recommended to avoid heat, scented pads or products, or restrictive clothing.
Vulva
Often the first line of treatment for the vulva is usually a topical ultra-potent corticosteroid (such as clobetasol) to reduce the immune response. Mild and moderate strength topical steroids may be used but they may be less likely to provide significant improvement or remission. Often a single type of treatment is sufficient. [2] Second line therapy is a topical medicine tacrolimus which also acts to suppress the overly-active immune response. This can be associated with a burning sensation and is best used by increasing the dose gradually.
Vagina
If vaginal erosions or other symptoms of the vagina are present hydrocortisone compounded vaginal suppositories may be prescribed. In patients with high risk of yeast infection weekly fluconazole may be prescribed to prevent a yeast infection while using a vaginal corticosteroid. In post menopausal patients local estrogen therapy may be needed as well. [2]
Vaginal dilators may be used to treat narrowing of the vagina and vaginal opening (introitus or vestibule). [3]
Investigational Therapies
Systemic (oral) corticosteroids can be used when there is limited response to topical treatment. [3][1]Topical photodynamic therapy (PDT) [1] is considered investigational.
Support Available
- Lichen Sclerosus & Vulval Cancer UK Awareness
https://lsvcukawareness.weebly.com/ - https://rarediseases.org/rare-diseases/lichen-planus/
- Vulvar Lichen Planus Support Group - Facebook
https://www.facebook.com/groups/1099455013507234/ - ISSVD Patient Handout - Lichen Planus
https://www.issvd.org/resources/vulvar-lichen-planus - Vulval Pain Society
https://vulvalpainsociety.org/
References
- 1.Jacques L, Kornik R, Bennett D, Eschenbach D. Diagnosis and Management of Vulvovaginal Lichen Planus, Obstetrical and Gynecological Survey 2020; Vol 75 No. 10
- Mauskar M, Marathe K, Venkatesan A, Schlosser B, Edwards L. Vulvar diseases conditions in adults and children, J Am Acad Dermatol 2020; Vol 82, No.6
- Simonetta C, Burns E, Guo M. Vulvar Dermatoses A Review and Update, Missouri Medicine 2015;7-8:301-307 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6170060/
- Simpson R, et al. Diagnostic criteria for erosive lichen planus affecting the vulva: an international electronic-Delphi consensus exercise, British Journal of Dermatology 2013; 169: 337-343
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3838629/ - The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease: a case-control study. AU Cooper SM, Ali I, Baldo M, Wojnarowska F SO Arch Dermatol. 2008;144(11):1432. PMID 19015417
- Clinicopathologic Diagnostic Criteria for Vulvar Lichen Planus. AU Day T, Wilkinson E, Rowan D, Scurry J, ISSVD Difficult Pathologic Diagnoses Committee* SO J Low Genit Tract Dis. 2020;24(3):317.
