1. What every clinician should know
Lichen planus is the most common chronic erosive vulvar dermatosis, often presenting in the peri- and postmenopausal period. Lichen planus is an inflammatory, mucocutaneous disorder that exhibits a wide range of morphologies. The most common, most morbid, and most difficult to treat is the erosive form.
Usually recognized on the skin or oral mucosa, lichen planus can affect the lower genital tract (vulva and vagina). Approximately 1% of the population has oral lichen planus, and of women with oral disease, 25% are estimated to have vulvovaginal disease. Because genital lesions are underrecognized or asymptomatic, incidence rates likely represent underestimates of the true extent of disease. Lichen planus presents less frequently to gynecologists and dermatologists than does lichen sclerosus.
Among women with erosive vulvovaginal lichen planus, over 50% will relate a personal or family history of an autoimmune disorder (most commonly, diabetes mellitus, thyroid disease and celiac disease). A genetic component to lichen planus has been suggested by data demonstrating the presence of the class II HLA DQB1*0201 allele in 80% of women with vulvovaginal lichen planus. This association, however, have not been consistently seen across diverse populations.
An association between vulvar malignancy and lichen planus has been reported in women with erosive lichen planus. The incidence of this rare condition is not, however, known.
2. Diagnosis and differential diagnosis
Lichen planus classically presents on mucous membranes as white, lacy, reticulate or fern-like striae (Wickham striae). Occasionally, the skin appears uniformly white (hence the confusion with other disorders). On occasion, the pruritic, purple, shiny papules of lichen planus can be seen on the vulva, although they often appear dusky pink, without scale, and poorly demarcated.
In erosive lichen planus, deep, painful, erythematous lesions appear in the posterior vestibule, often extending lateral to the labia minora. Over time, agglutination and resorption of the labial architecture occur. The vaginal epithelium can become erythematous, eroded, acutely inflamed and denuded. Erosive patches are extremely friable.
With time, eroded surfaces may adhere, leading to formation of synechiae and eventually complete vaginal obliteration. Increased vaginal discharge, burning, pruritus, dyspareunia and irritation are commonly reported. A subset of women with vulvovaginal lichen planus also present with chronic erosive lesions of the gingivae (and thus termed vulvovaginal gingival syndrome). Therefore, a complete mucocutaneous evaluation is warranted to rule out disease at distant sites.
In women suspected to have lichen planus, a biopsy should be performed and is useful to rule out immunobullous disorders often confused with erosive lichen planus. A band-like infiltrate of lymphocytes and colloid bodies will be seen in the basal layers of the epidermis. Because of the loss of vaginal epithelium, however, the biopsy may be relatively nonspecific.
Area to biopsy
Care should be taken to biopsy an area of Wickham striae or white epithelium surrounding an erosion. If only an erosion is present, a biopsy should be taken from the intact skin at the edge of the erosion. For women presenting with vaginal discharge, a vaginal pH and saline preparation should be performed. The vaginal pH is typically in the 5-6 range, with numerous inflammatory cells and immature parabasal and basal epithelial cells seen on microscopic examination. These findings are shared with patient who have desquamative inflammatory vaginitis (DIV), atrophic vaginitis as well as a number of other erosive or inflammatory vaginal disorders.
The differential diagnosis of vulvovaginal lichen planus includes other inflammatory disorders such as cicatricial pemphigoid, bullous pemphigoid and pemphigus vulgaris, as well as atrophy, infection with herpes simplex, severe vulvovaginal candidaisis and vulvar intraepithelial neoplasia. Commonly confused with lichen sclerosus, lichen planus, unlike lichen sclerosus, often involves the vaginal mucosa. It should be noted that recent evidence indicates an overlap disorder between the two based on clinical and histologic features. Such cases are often associated with squamous cell hyperplasia and are poorly responsive to ultrapotent topical steroids. Consultation with an expert in vulvovaginal disorders can be helpful in managing lichen planus.
Treatment, while not curative, often improves patients’ symptoms and sense of well-being. Erosive vulvovaginal disease, however, is more painful and difficult to manage than extragenital cutaneous disease. Once a diagnosis has been made, the first-line treatment for lichen planus is topical corticosteroids.
Ultrapotent topical steroids are used, with intralesional injections (e.g., triamcinolone acetonide) or oral steroids reserved for patients with severe erosions and exudation or refractory disease. When choosing a topical steroid, ointments are preferred. With an ointment, compared to a cream, the risk of burning is decreased, while potency and absorption are increased. Most patients benefit initially from the application of a topical steroid once to twice daily, with tapering to daily or every other day as symptoms improve. As symptoms continue to improve, switching to a less potent topical steroid is reasonable.
For women with vaginal involvement, hydrocortisone suppositories can be used. A 25-mg rectal suppository (or a compounded 25-mg intravaginal suppository) is inserted intravaginally twice daily. As symptoms improve, the dose can be reduced to twice weekly. In one study using this protocol, over 80% of women reported significant symptom reduction and over 75% improved objectively on examination. While patients with erosions and erythema saw improvement in this study, those with vaginal stenosis did not significantly improve.
As vaginal patency is a frequent concern among women with erosive lichen planus, instruction in the use of vaginal dilators should be encouraged. Further development of scars and adhesions can be prevented with regular use of dilators as demonstrated in a number of prospective studies. Coating the dilator with either steroid ointment or vaginal estrogen cream is recommended.
Other management options
There are numerous small trials of a number of other medical options for the management of lichen planus. These include the topical calcineruin inhibitors, the most studied of the alternatives to steroids and the ones most consistently associated with improvement in patient symptomatology. These agents, due an increased risk of malignancy in animal models, should be considered second-line therapy for lichen planus. A number of other agents, such as griseofulvin, dapsone, topical and oral cyclosporine, azathioprine, cyclophosphamide, oral retinoids, hydroxychloroquine, methotrexate, etanrecept, adalimumab and thalidomide have been reported in case reports or small open trials, most with varied or discouraging results.
Surgical management for scarring is advocated only for those women whose active disease is under control and whose adhesiolysis will be performed by a surgeon with expertise in the care of similar patients.
Over time, women with lichen planus are at risk for the development of a number of complications including scarring and adhesions that obliterate the labia minora and the vaginal canal. Loss of normal vulvar architecture occurs via resorption of the labia minora and clitoral hood. Vaginal synechiae formation and eventual vaginal stenosis make sexual intercourse impossible. Finally, women with lichen planus face the risk of vulvar squamous cell cancer.
Care should be taken to monitor not only local side effects (e.g., atrophy, striae, and steroid dermatitis) of ultrapotent topical steroids, but also systemic absorption leading to adrenal suppression. In women experiencing severe or acute exacerbations while using ultrapotent topical steroids, secondary infection with Candida or herpes simplex should be ruled out.
5. Prognosis and outcome
The prognosis for spontaneous remission of erosive vulvovaginal lichen planus is poor. Despite a number of available therapies, significant control of symptoms and restoration of sexual function remain difficult. The use of appropriate therapy and vaginal dilators appears to help prevent vaginal stenosis.
6. What is the evidence for specific management and treatment recommendations?
Cooper, SM, Haefner, HK, Abrahams-Gessel, S, Margesson, LJ. “Vulvovaginal lichen planus treatment: a survey of current practices”. Arch Dermatol. vol. 144. 2008. pp. 1250-1. (Prescribing practices among providers from the U.S., UK, and Brazil revealed geographic differences in treatment allocation with US providers more likely to prescribe ultrapotent topical steroids and calcineurin inhibitors.)
“Diagnosis and management of vulvar skin disorders. ACOG Practice Bulletin No. 93. American College of Obstetricians and Gynecologists”. Obstet Gynecol. vol. 111. 2008. pp. 1243-53.
Edwards, L. “Dermatologic causes of vaginitis: a clinical review”. Dermatol Clin. vol. 28. 2010. pp. 727-35. (Comprehensive overview of isolated vaginal disease (e.g., atrophic vaginitis and DIV) and multimucosal erosive dermatoses with vaginal involvement (erosive lichen planus as well as cicatricial pemphigoid and pemphigus vulgaris).
Mirowski, GW, Goddard, A. “Treatment of vulvovaginal lichen planus”. Dermatol Clin. vol. 28. 2010. pp. 717-25. (Comprehensive review of multiple treatment options and supportive measure and clinical outcomes for women with vulvovaginal lichen planus.)
Pipkin, C. “Erosive diseases of the vulva”. Dermatol Clin. vol. 28. 2010. pp. 737-51. (Excellent and compact review of the major erosive disorders involving the vagina.)
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- Lichen planus
- 1. What every clinician should know
- 2. Diagnosis and differential diagnosis
- 3. Management
- 4. Complications
- 5. Prognosis and outcome
- 6. What is the evidence for specific management and treatment recommendations?