Vulvar lichen planus

INTRODUCTION — Lichen planus is a relatively uncommon inflammatory dermatologic condition with subtypes that can affect the skin, mucosa, nails, and scalp. Vulvar lichen planus is a subtype of lichen planus that is characterized by erosive, papular, or hypertrophic lesions on the vulva, with or without concomitant vaginal involvement.

Erosive lichen planus (also known as mucosal lichen planus) can result in severe tissue destruction that leads to vulvar pain and urinary and sexual impairment. Erosive lichen planus also can affect other mucosal sites, such as the oral cavity, nasal mucosa, esophagus, larynx, conjunctiva, and urethra [1].

High-quality studies to guide treatment of vulvar lichen planus are lacking. Superpotent topical corticosteroids are well accepted as the first-line treatment for erosive disease.

Vulvar lichen planus will be discussed here. Other clinical presentations of lichen planus are reviewed separately. (See "Lichen planus", section on 'Clinical features' and "Oral lichen planus: Pathogenesis, clinical features, and diagnosis".)

EPIDEMIOLOGY — Lichen planus is estimated to affect 0.5 to 2 percent of the population; estimates have varied based upon geographic location and diagnostic criteria [2]. The incidence and prevalence of vulvar lichen planus has not been clearly established, but vulvar disease may be a common manifestation of lichen planus in women. In one series of 37 women diagnosed with lichen planus, vulvar lesions were present in 51 percent [3].

ETIOLOGY — The etiology is unknown. The characteristic skin lesions of lichen planus are thought to arise from a T-cell mediated autoimmune response against basal keratinocytes. The disorder is often associated with other autoimmune diseases [4].

CLINICAL MANIFESTATIONS — Vulvar lichen planus often occurs in women 50 to 60 years of age, though younger and older women can be affected [5-7]. Women with vulvar lichen planus frequently present with complaints of vulvar pain, burning, pruritus, soreness, or dyspareunia (sometimes with post-coital bleeding) [8,9]. Another prominent symptom that may be present is an irritating vaginal discharge that does not respond to standard therapies for vaginitis. However, this clinical scenario is not specific to lichen planus, as it occurs with other vulvar disorders (table 1).

The coexistence of vulvar lichen planus with cutaneous lichen planus, oral lichen planus, or lichen planopilaris (including frontal fibrosing alopecia) is common (picture 1A-F) [3,10-12]. (See "Lichen planus" and "Oral lichen planus: Pathogenesis, clinical features, and diagnosis" and "Lichen planopilaris".)

Signs and symptoms of vulvar lichen planus can be constant or intermittent. A minority of women are asymptomatic or have only minimal symptoms [13].

Four types of lichen planus affect the vulva: erosive lichen planus, papulosquamous lichen planus, hypertrophic lichen planus, and lichen planopilaris [3,14,15].

Erosive lichen planus — Erosive lichen planus is the most common type of vulvar lichen planus seen in the secondary care setting. The desquamative, erosive, chronic dermatitis often also involves the vagina. Recurrent exacerbations, slow healing, and scarring are common. Scarring can cause significant anatomic disruption, including severe stenosis of the vagina and urethral obstruction (picture 2). Dyspareunia, apareunia, difficulty with urination, and dysuria are the major symptoms.

Lesions are characterized by well-demarcated, glazed, brightly erythematous patches or erosions with white striae or a serpentine, white border along the margin (Wickham striae) (picture 3A-B). Occasionally, a violaceous border is noted. The lesions may occur on the labia minora and vestibule as isolated lesions on an otherwise normal vulva, or they may be associated with marked architectural destruction, including loss of the labia minora and narrowing of the introitus. Small, localized lesions on the labia majora are less common [13]. Anal involvement is rare [16]:

●Vaginal involvement – Vaginal involvement has been reported in up to 70 percent of patients with erosive lichen planus; in contrast, vaginal involvement is rare in lichen sclerosus [17,18] (see "Vulvar lichen sclerosus: Clinical manifestations and diagnosis"). The vaginal epithelium in lichen planus is friable, easily bleeding upon speculum insertion or with coitus. It may have small areas of inflammation and increased vaginal discharge, or it may be massively inflamed and denuded with a seropurulent exudate, pseudomembrane, or serosanguinous vaginal discharge [13,19]. In severe cases, adhesions and synechiae develop, which can lead to narrowing or obliteration of the vagina.

Vaginal involvement can occur in the absence of any vulvar involvement, and vaginal synechiae/adhesions can form in the absence of any symptomatology if the patient is not sexually active or menstruating. The Papanicolaou smear may show atypia if the cervix is affected [20].

●Vulvo-vaginal-gingival syndrome – The vulvo-vaginal-gingival (VVG) syndrome is a variant of erosive lichen planus that involves the epithelium of the vulva, vestibule, vagina, and mouth (picture 4); additional sites (eg, skin, esophagus) may also be involved [19,21-24]. Although all three areas can be affected, the lesions may not be concurrent. The gingival epithelium is usually involved, and erosions, white plaques, or a whitish and lace-like reticular pattern may occur on the buccal mucosa, tongue, and palate. Scarring and stricture formation are common and a major cause of long-term morbidity [24]. VVG is particularly resistant to treatment.

Erosive lichen planus also can affect other mucosal sites, such as the nasal mucosa, esophagus, larynx, conjunctiva, and urethra; concomitant involvement in these sites is often missed [1]. (See 'Clinical evaluation' below.)

Papulosquamous lichen planus — Papulosquamous lichen planus consists of small, intensely pruritic papules with a violaceous hue that arise on keratinized and perianal skin. It is associated, occasionally, with milky striae on the inner aspects of the labia [25]. Resolution of papulosquamous lesions is often followed by postinflammatory hyperpigmentation [5].

Hypertrophic lichen planus — Hypertrophic lichen planus characteristically exhibits hyperkeratotic, rough lesions on the perineum and perianal region (picture 5) [13]. The appearance is similar to vulvar intraepithelial neoplasia or invasive squamous cell carcinoma. This type of lichen planus is rare and can be difficult to diagnose clinically because it resembles other hypertrophic vulvar lesions [25].

Lichen planopilaris — Lichen planopilaris is an uncommon follicular variant of lichen planus that presents with perifollicular erythema and hyperkeratosis as well as scarring alopecia. It typically presents on the scalp; however, a patient with lichen planopilaris on the labia majora and mons pubis has been reported [15]. (See "Lichen planopilaris".)

DIAGNOSIS

Clinical evaluation — The diagnosis of the erosive and papulosquamous variants of vulvar lichen planus is primarily based upon the recognition of characteristic clinical manifestations. Erythematous erosions with a glazed appearance on the vulva demonstrating Wickham striae (white striae within involved areas or a serpentine white border) are highly suggestive of erosive lichen planus and pruritic violaceous papules are consistent with the papulosquamous variant, particularly when accompanied by papulosquamous lichen planus in other body sites and postinflammatory hyperpigmentation. However, a biopsy of lesions suspicious for erosive vulvar lichen planus or papulosquamous vulvar lichen planus should be obtained to rule out other disorders if there is any uncertainty regarding the clinical diagnosis [9]. A biopsy is usually indicated in patients with suspected hypertrophic lichen planus because this variant can closely resemble squamous cell carcinoma. (See 'Biopsy' below.)

In addition to examination of the vulva and vagina, the physical examination of patients with suspected vulvar lichen planus should include an evaluation of other mucosal and cutaneous surfaces, including the oral cavity, scalp, nails, anus, and entire skin surface. Patients with vulvar lichen planus frequently have other manifestations of lichen planus, and the detection of lichen planus in other body areas increases suspicion for the diagnosis (picture 1A-F). Examination of the oral cavity also allows for the detection of patients with the vulvo-vaginal-gingival (VVG) syndrome. (See "Lichen planus", section on 'Clinical features' and 'Erosive lichen planus' above.)

The clinical evaluation should also include a review of systems for signs or symptoms suggestive of involvement of hidden mucosal areas, such as the nasal mucosa, esophagus (eg, dysphagia, odynophagia), larynx (eg, hoarseness, stridor), conjunctiva (eg, foreign body sensation or visual changes), urethra (eg, hematuria, dysuria), and anus. Erosive lichen planus in these areas is not often recognized and can result in functional impairments if untreated.

Biopsy — A biopsy is usually recommended for vulvar lichen planus, particularly in the erosive and hypertrophic forms of disease. Patients with classic clinical features of papulosquamous lichen planus (ie, small, violaceous, pruritic papules) may not require a biopsy for confirmation. (See 'Clinical manifestations' above.)

The identification of classic histologic features of lichen planus in biopsy specimens from patients with clinical features suggestive of vulvar lichen planus confirms the diagnosis. Classic histologic features of lichen planus include:

●Irregular acanthosis of the epidermis (in sites of keratinized skin)

●Vacuolar change of the epidermal basal cell layer

●A band-like dermal infiltrate of lymphocytes in the upper dermis

●Apoptotic keratinocytes scattered within the epidermis

In addition, plasma cells are often present in biopsy specimens from mucosal lesions.

However, the failure to detect classic histologic features of lichen planus in patients with vulvar disease does not rule out the diagnosis. Classic histologic features of lichen planus are identified in only 70 to 80 percent of patients with vulvar lichen planus [7,11,26].

A 4 mm punch biopsy of an involved area is performed to obtain tissue for histologic examination. In patients with erosive disease, typical features of lichen planus are most likely to be found at the margins of ulcers [27]. Therefore, a specimen taken from a site of erosive disease should include intact epithelium adjacent to the ulcer. (See "Skin biopsy techniques".)

Immunofluorescence studies are typically reserved for erosive presentations in which an autoimmune blistering disease is in the differential diagnosis. If performed, direct immunofluorescence of vulvar lichen planus may show shaggy staining of the basement membrane zone, suggesting deposition of fibrinogen, IgM, cytoid bodies, and, occasionally, granular IgG or IgA [28].

Proposed diagnostic criteria — Additional study is necessary to validate diagnostic criteria for the various presentations of vulvar lichen planus:

●A 2012, electronic, Delphi consensus exercise attempted to identify the important diagnostic features of erosive lichen planus of the vulva through a series of surveys administered to a multidisciplinary group of 73 experts in vulvovaginal disease [9]. The following nine criteria were identified by at least 75 percent of the participants as findings supportive of a diagnosis of the disease:

•Presence of well-demarcated erosions or glazed erythema at the vaginal introitus

•Presence of a hyperkeratotic, white border to erythematous areas or erosions ± Wickham striae in surrounding skin

•Symptoms of pain or burning

•Scarring or loss of normal architecture

•Presence of vaginal inflammation

•Involvement of other mucosal sites

•Presence of a well-defined, inflammatory band in the superficial connective tissue that involves the dermoepidermal junction

•Presence of an inflammatory band that consists predominantly of lymphocytes

•Signs of basal cell layer degeneration (eg, Civatte bodies, abnormal keratinocytes, or basal apoptosis)

There was some disagreement among participants about whether at least three supportive features or at least four supportive features are required for a diagnosis of erosive lichen planus of the vulva. One study of 72 women with erosive vulvovaginal lichen planus found that 97 percent had at least three of the nine diagnostic criteria [29].

●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 [30]:

•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 consisting of a:

-Degenerative pattern – Apoptotic bodies, vacuolar change, and/or squamatization

And/or

-Regenerative pattern – Increased nucleus-to-cytoplasm ratio, mitoses, diminished epithelial maturation

•Absence of sclerosis

●A retrospective, single-center study compared the clinical and pathologic features of 243 females with clinically suspected vulvar lichen planus with 50 patients with biopsy-confirmed lichen sclerosus and 50 patients with culture-proven, chronic vulvovaginal candidiasis to support potential diagnostic criteria. This study found that the presence of at least two clinical features (erosions, glazed erythema, oral involvement, pain/burning sensation, and hyperkeratotic border) correlated with histopathologic diagnosis of vulvar lichen planus with 100 percent sensitivity. The specificity of at least two clinical features was 92 percent when compared with vulvar lichen sclerosus and 88 percent when compared with chronic vulvovaginal candidiasis. One limitation of this study was that other, more common, clinical mimics of vulvar lichen planus (eg, genitourinary syndrome of menopause) were not included [31].

DIFFERENTIAL DIAGNOSIS — Other inflammatory and erosive diseases of the vulva may mimic lichen planus. Some examples include:

●Lichen sclerosus causes architectural distortion and labial adhesions, but unlike vulvar lichen planus, rarely affects the vagina. The classic presentation consists of white, atrophic papules or plaques on the vulva that may become hemorrhagic, purpuric, hyperkeratotic, bullous, or eroded (picture 6A-B). Erosive lesions due to lichen sclerosus respond to therapy better than those related to lichen planus. (See "Vulvar lichen sclerosus: Clinical manifestations and diagnosis".)

●Autoimmune bullous diseases (eg, mucous membrane pemphigoid and pemphigus) can have clinical vulvovaginal features identical to erosive lichen planus; immunofluorescence studies on biopsy specimens are necessary to make a definitive diagnosis [32]. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid".)

●Plasma cell vulvitis is an uncommon erosive disease that results in tender, velvety, raised erythematous lesions of the vestibule and, rarely, the clitoris; oral and vaginal involvement are unusual (picture 7) [33-35]. On histologic examination, plasma cells contribute to one-half of the dermal infiltrate.

●Desquamative inflammatory vaginitis (DIV) is an intensely inflammatory vaginitis, also called lichenoid vaginitis. The purulent vaginal discharge has an elevated pH and consists of sheets of white blood cells and parabasal cells, indicative of desquamating vaginal epithelium. There may be bacterial overgrowth, except for Lactobacillus, which is reduced or absent. Whether DIV is a form of lichen planus [36] or caused by an unidentified bacterium [37] is controversial. (See "Vaginitis in adults: Initial evaluation".)

●Vulvar lichen planus can be confused clinically and pathologically with vulvar intraepithelial neoplasia (VIN), differentiated type [38]. (See "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)".)

●Behçet syndrome causes recurrent oral and genital ulcerations and ocular inflammation (picture 8). These three major criteria are often associated with the minor criteria of arthritis, thrombophlebitis, acneiform skin eruptions, ulcerative colitis, and neurologic involvement [39]. (See "Clinical manifestations and diagnosis of Behçet syndrome".)

●Crohn disease may affect the vulva or perineum; the vulvar manifestations may precede or follow bowel involvement by many years. Vulvar signs of Crohn disease include edema of the labia majora, labia minora, and prepuce; edematous perianal skin tags; ulcers; fissures; sinuses; and fistulae (picture 9). Vulvar edema may be an isolated and unilateral finding [40]. (See "Perianal Crohn disease".)

●Erythema multiforme and Stevens-Johnson syndrome produce lesions of the mouth, eye, and genitals consisting of painful, shallow ulcers and bullae on the labia and surrounding skin. The acute onset helps to differentiate this disorder from erosive lichen planus. (See "Erythema multiforme: Pathogenesis, clinical features, and diagnosis" and "Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis".)

●A lichenoid drug eruption can be indistinguishable clinically and histologically from lichen planus [41]. A positive history of medication use and disappearance of skin lesions following withdrawal of the medication suggest this possibility. (See "Lichenoid drug eruption (drug-induced lichen planus)".)

●Genitourinary syndrome of menopause (vulvovaginal atrophy or atrophic vaginitis) is common in peri- or postmenopausal women, in women with hypoestrogenism, and may coexist in women with vulvar lichen planus. The clinical features can be similar to erosive lichen planus; however, hyperkeratosis and Wickham striae are not evident. Histology should not show any of the features of lichen planus. (See "Genitourinary syndrome of menopause (vulvovaginal atrophy): Clinical manifestations and diagnosis".)

NATURAL HISTORY — The oral and genital lesions of lichen planus, especially erosive disease, are persistent and tend to be resistant to therapy [3,42]. In contrast, the natural history of most cases of cutaneous lichen planus elsewhere on the body is spontaneous remission within a couple of years. (See "Lichen planus".)

Associated malignancy — The relationship between lichen planus and squamous cell malignancy is uncertain; the best evidence is for an association between oral malignancy and oral lichen planus, which causes chronic inflammation of the oral mucosa [43]. A systematic review of observational studies that included 7806 patients with oral lichen planus (and no dysplasia on initial biopsy) found an overall transformation rate of approximately 1 percent [43].

A slightly increased risk of vulvar malignancy in women with vulvar lichen planus has been postulated, but not confirmed in large series [44-50]. A study reviewing all cases of vulvar lichen planus in one institution over 11 years (127 females) found that premalignant and malignant transformation was rare, with only three patients developing a high-grade squamous intraepithelial lesion (2 percent). However, two of these patients later developed invasive vulvar squamous cell carcinoma [51].

Vulvar squamous cell carcinoma that develops in patients with vulvar lichen planus may demonstrate aggressive features. In a retrospective study of 38 women diagnosed with both vulvar lichen planus and vulvar squamous cell carcinoma, inguinal metastases were present at the time of presentation in 16 women (42 percent) and squamous cell carcinoma was the cause of death in 14 women (37 percent) [52]. All cancers in this series arose in non-hair-bearing vulvar mucosa and the majority were in the vestibular area between the clitoris and urethra. Although data are insufficient to determine the impact of early and aggressive treatment of vulvar lichen planus on the risk for malignancy, data from this study highlight the vulvar sites of highest risk [52].

MANAGEMENT — Data on the treatment of vulvar lichen planus are limited. The approach to treatment is largely guided by uncontrolled studies and reports of clinical experience.

Erosive vulvar lichen planus — The physical discomfort and disfigurement that result from erosive vulvar lichen planus justify a treatment approach aimed at rapidly reducing signs and symptoms of the disease. Consultation with a clinician experienced in the assessment and treatment of patients with erosive vulvar lichen planus often is of value for the management of patients with this disease.

Adjunctive measures — Although medical therapy is the mainstay of treatment of vulvar lichen planus, other interventions play important roles in patient management:

●Patient education and support – Although patients with vulvar lichen planus can experience periods of remission with treatment, there is no cure. Clinicians should educate patients about the chronic course of vulvar lichen planus and the need for continued treatment after symptoms improve. Patients who understand the expected course of the disease may be more likely to adhere to clinical follow-up and prescribed treatments. Written, patient-directed information and referral to local or national patient support groups can be a useful supplement to verbal communication.

In addition, because vulvar lichen planus can be emotionally distressing and can have a significant negative effect on patient quality of life [53], addressing the psychologic and emotional concerns of the patient is an important component of treatment. Resources for psychologic support or counseling should be provided if needed.

●Vulvar care – Factors that irritate the vulvar skin may exacerbate symptoms. In addition to discouraging scratching of the vulva, we encourage patients to engage in vulval hygiene practices that may reduce risk for irritation. Our recommendations for vulvar hygiene reviewed in a table (table 2).

●Review of medications – Lichenoid drug eruptions (also known as drug-induced lichen planus) are cutaneous or mucosal eruptions that clinically and histologically resemble idiopathic lichen planus and occur as a result of drug exposure. Lichenoid drug eruptions have been associated with a wide variety of medications and may occur weeks to several months or longer after initiation of the causative medication [54]. Most of the literature on lichenoid drug eruptions focuses on cutaneous lichenoid drug eruptions (the most common form of lichenoid drug eruption) and oral lichenoid drug eruptions. There is a paucity of data on drug-induced lichenoid eruptions of the vulva leaving uncertainty about the relationship between drugs and this presentation of disease. (See "Lichenoid drug eruption (drug-induced lichen planus)".)

Based upon the known association of drugs with lichenoid eruptions of the skin and oral mucosa, we routinely review the medications of patients diagnosed with vulvar lichen planus to determine whether drugs that have been associated with lichenoid eruptions are present. Drugs that have been linked to lichenoid eruptions are reviewed separately. (See "Lichenoid drug eruption (drug-induced lichen planus)", section on 'Medications'.)

If it is feasible to discontinue a drug that has been linked to lichenoid drug eruptions, we proceed with a trial of drug cessation [55]. Oral lichenoid drug reactions typically resolve within weeks to months after the inciting drug is stopped although delayed responses may occur [56]. We have treated several women with erosive vulvar lichen planus refractory to topical therapy who experienced improvement in symptoms after the discontinuation of NSAIDs or hydrochlorothiazide. We have also observed the development of flares of vulvar lichen planus during NSAID treatment.

First-line therapy — The treatment of erosive lichen planus of the vulva (with or without vaginal involvement) is challenging. The paucity of data on the efficacy of treatments for lichen planus precludes definitive conclusions about the best approach to therapy. Most commonly, treatment is initiated with a superpotent topical corticosteroid ointment, a generally well-tolerated local therapy.

However, other approaches to initial treatment may be reasonable. In patients presenting with severe disease, where application of topical therapy would be intolerable, systemic glucocorticoid therapy may also be used. Other clinicians have advocated using systemic glucocorticoid therapy more routinely [57].

Topical corticosteroids — Although topical corticosteroids are the mainstay of therapy for erosive vulvar lichen planus [8] and have demonstrated efficacy for oral lichen planus in a randomized trial [58], randomized trials in vulvar lichen planus are lacking.

Efficacy — The use of topical corticosteroid therapy is supported by the findings of an uncontrolled prospective study of 114 women with erosive lichen planus of the vulva [7]. In the study, a variety of topical corticosteroid therapies were used for initial treatment, with most patients receiving either clobetasol 0.05% ointment or a combined preparation containing clobetasone butyrate, oxytetracycline, and nystatin. Of the 89 women who received clobetasol 0.05% ointment as a first-line treatment, 94 percent experienced good or partial symptomatic improvement and 71 percent had complete resolution of symptoms during treatment. Good responses were also observed among patients treated with the combined preparation; good or partial improvement occurred in all 14 treated women, and 13 women became symptom free during treatment.

Retrospective studies also suggest that topical corticosteroid is effective in some patients; however, treatment regimens and reported success rates have varied widely [6,59,60]. In one retrospective study a response to treatment was documented in 85 of 129 patients (66 percent) treated with a very potent topical corticosteroid as first-line therapy. The measures used to define response were unclear.

Administration — Our topical corticosteroid regimen for vulvar lichen planus consists of an initial treatment course to induce remission followed by a plan for long-term maintenance therapy with a less intense treatment regimen. Our typical treatment plan is as follows:

●The patient performs nightly application of a super high-potency topical corticosteroid (eg, clobetasol propionate 0.05% ointment or halobetasol propionate 0.05% ointment (table 3)) to the affected area. As a guide, half of one finger-tip-unit (the amount of ointment expressed from a tube with a nozzle of 5 mm in diameter, applied from the distal skin-crease to the tip of the index finger) should be sufficient to cover both labia majora and minora and the perianal skin.

●The patient returns for clinical reevaluation of the response after two to three months:

•If there is satisfactory improvement (ie, symptom resolution and healing of all erosions), we commence maintenance therapy.

•If no improvement is evident after eight weeks with persistent erosions, treatment with a systemic glucocorticoid or systemic glucocorticoid-sparing agent may be required. (See 'Refractory disease' below.)

Maintenance therapy is necessary after remission is achieved because symptoms often recur once treatment is discontinued. The goal of the maintenance phase is to maintain improvement while reducing the frequency and potency of topical corticosteroid treatment to minimize risk for local corticosteroid side effects. This is performed as a stepwise process that eventually reveals the lowest dose and frequency that sustains remission.

The first step in the transition to a maintenance regimen is usually the reduction of the frequency of application of the superpotent topical corticosteroid from application every night to application every other night. Treatment is tailored to the patient's response; most patients need to continue application of a topical corticosteroid one to three times per week indefinitely [25]. Consultation with a dermatologist can be helpful for managing topical corticosteroid therapy. Lower-potency topical corticosteroids may also be utilized, as can combination preparations containing both a topical corticosteroid and an antimicrobial. (See "Topical corticosteroids: Use and adverse effects".)

If relapse occurs during the maintenance phase, we restart treatment with a superpotent topical corticosteroid nightly, as in the initial course of treatment. Once the disease is in remission, we taper the frequency and potency of treatment to the lowest effective maintenance regimen.

Skin atrophy is a potential side effect of topical corticosteroid therapy that is most likely to occur with the use of high-potency topical corticosteroids on areas of relatively thin skin; however, the vulvar area is relatively resistant to corticosteroid side effects. The least intense topical corticosteroid regimen for long-term maintenance treatment is preferred. The adverse effects of topical corticosteroids are reviewed in greater detail separately. (See "Topical corticosteroids: Use and adverse effects", section on 'Adverse effects'.)

Second-line therapy — Topical tacrolimus (a topical calcineurin inhibitor) is an alternative local therapy that is used in the treatment of vulvar lichen planus. Similar to topical corticosteroids, high-quality data on the efficacy of topical tacrolimus for this indication are lacking.

Topical tacrolimus — Topical tacrolimus may improve vulvar lichen planus via its immunosuppressive properties. Potential disadvantages of topical tacrolimus therapy include greater cost in comparison to some topical corticosteroids and the frequent occurrence of local burning or tingling sensations with application of this therapy [26]. However, burning sensations often resolve with continued use of this therapy [26].

Efficacy — Support for the use of topical tacrolimus for erosive vulvar lichen planus primarily stems from retrospective studies and case reports [6,26,61-63]. As an example, a telephone questionnaire administered to 16 patients whose records indicated that they received topical tacrolimus for erosive vulvar lichen planus after failure to respond to other therapies revealed that 15 patients (94 percent) recalled symptomatic improvement within three months (mean response time 4.2 weeks) and noted partial or complete resolution of lesions [26]. In addition, of 13 patients who returned for clinical follow-up, review of medical records indicated that 10 patients had no clinical evidence of erosions on examination.

No randomized trials have compared the efficacy of topical tacrolimus and topical corticosteroids in erosive vulvar lichen planus. The results of one small retrospective study suggested greater patient satisfaction after treatment with topical tacrolimus than after treatment with topical clobetasol; however, additional studies are needed to validate this finding [63]. There are few data on the efficacy of topical pimecrolimus, an additional topical calcineurin inhibitor [6,64]. The findings of a small series suggest that topical pimecrolimus may be beneficial [64].

Administration — Clinicians vary on the approach to topical tacrolimus therapy. Our instructions for use of topical tacrolimus aim to minimize the burning sensation that may occur early in the course of treatment. Although most of the literature on topical tacrolimus therapy for vulvar lichen planus describes the use of tacrolimus 0.1% ointment, we usually initiate treatment with tacrolimus 0.03% ointment in an attempt to improve tolerance. We begin with every other day treatments, gradually working up to twice daily as tolerated. Daily sitz baths for the first week of therapy or application of topical tacrolimus on top of a layer of petrolatum or a topical corticosteroid ointment for the first few weeks of treatment also can help alleviate burning.

We typically reevaluate patients for initial signs of a response to treatment after one to two months, and if the 0.03% formulation is tolerated, the dose can be increased to 0.1% ointment. Once remission is achieved, the frequency of application is gradually reduced to a maintenance regimen, which in some patients may be application one to three times per week [63].

Severe erosive lichen planus — Patients who present with severe erosive vulvar lichen planus that prevents the application of topical therapy due to pain may benefit from an alternative initial approach to treatment. In our experience, initiating treatment with a systemic glucocorticoid can be beneficial for these patients.

We prescribe a four- to six-week course of oral prednisone (40 to 60 mg per day, tapered over four to six weeks) and commence topical corticosteroid therapy after one week [65]. Twice-daily sitz baths in water followed by the application of a greasy emollient (such as petroleum jelly) may also help restore the epithelial barrier.

An alternative approach is the use of intramuscular triamcinolone. Intramuscular triamcinolone (1 mg/kg) may be given as a single dose or as a series of injections separated by one month.

Although systemic glucocorticoid therapy can improve erosive vulvar lichen planus, systemic glucocorticoids are associated with a wide variety of adverse effects that make long term treatment with these agents unfavorable. The adverse effects are reviewed in detail separately. (See "Major adverse effects of systemic glucocorticoids".)

Refractory disease — There are few data to guide the treatment of patients who fail topical therapy, manifested by persistent painful erosions or tender lacy papules and plaques. Prior to considering an alternative approach to treatment we assess for factors that may exacerbate symptoms or inhibit healing, including:

●Incorrect application of topical medication

●Exposure to skin irritants or nighttime scratching

●Superinfection by bacteria, Candida, or herpes simplex virus (see 'Treatment of concurrent infection' below)

●Vulvar pain that persists despite clinical resolution of vulvar lichen planus (this may indicate neuropathic pain possibly precipitated by lichen planus [66]) (see "Vulvar pain of unknown cause (vulvodynia): Treatment")

If an exacerbating factor responsible for the persistence of symptoms is not identified, treatment with systemic agents can be attempted. A variety of systemic immunomodulatory and immunosuppressive agents (eg, methotrexate, mycophenolate mofetil, oral or intramuscular glucocorticoids, hydroxychloroquine, acitretin, minocycline, cyclosporine, and others) have been utilized in individual patients [57,60,67], but data are insufficient to confirm efficacy of specific interventions.

Vaginal involvement — Similar to vulvar disease, our first-line treatment for vaginal involvement is local administration of a corticosteroid.

Intravaginal corticosteroids — Corticosteroids are administered vaginally to women with vaginal involvement. In one case series, 16 of 17 patients noted improvement with hydrocortisone 25 mg suppositories inserted into the vagina twice daily for two months [68]. In another series of 60 patients treated with 25 mg hydrocortisone suppositories, 80 percent improved [69].

Treatment will be guided by the medications available in the country of practice. In our experience, prednisolone foam (20 mg metered dose as an aerosol foam) or hydrocortisone acetate 10% foam (125 mg metered dose) prescribed on alternate nights at bedtime for six weeks can be effective. Subsequently, treatment is reduced to a maintenance regimen of the lowest dose that controls symptoms (usually one to two applications per week).

Where hydrocortisone suppositories are available, these may be prescribed nightly at bedtime for 14 days (50 mg for early and mild disease or 100 mg for severe disease), followed by applications on alternate nights for an additional 14 days. The dose is then tapered to find the lowest maintenance dose that controls symptoms, which may be as low as 25 mg every other week.

Of note, medication that leaks from the vagina may cause vulvar irritation and burning. The vulvar skin can be protected by coating the perivaginal area with petroleum jelly or zinc oxide, or a tampon can be cut in half and inserted in the distal vagina as a "plug" to control leakage.

Other therapies — The best approach to the treatment of severe vaginal lichen planus that fails to respond to intravaginal corticosteroids unclear. If the response to topical corticosteroid therapy is insufficient, oral or intramuscular glucocorticoids and tacrolimus suppositories are additional therapeutic options for vaginal involvement:

●Oral or intramuscular glucocorticoids – Either oral prednisone 40 to 60 mg daily, tapered over four to six weeks, or intramuscular triamcinolone, 60 to 80 mg once per month for three to four months, may be tried before local maintenance therapy is initiated.

Systemic glucocorticoid therapy may exacerbate underlying medical disorders, such as diabetes and hypertension. Coordination of care with the patient's primary care clinician may be beneficial. (See "Major adverse effects of systemic glucocorticoids".)

●Tacrolimus – An alternative to systemic glucocorticoids is compounded tacrolimus 2 mg suppositories nightly for 30 days. Once symptoms improve, treatment is gradually tapered to the lowest frequency sufficient to maintain disease control. Monitoring tacrolimus drug levels is unnecessary with topical therapy.

Adhesions and scarring — Vulvar or vaginal involvement by lichen planus can lead to anatomic distortion and functional limitations secondary to the formation of adhesions and scarring. Dilators and surgery can be useful for improving these complications after mucosal inflammation is controlled with medical therapy. Follow-up care is necessary to maintain benefit after these procedures:

●Dilators – Dilator therapy (with rigid dilators) can be effective for distending mild to moderate vaginal synechiae that interfere with intromission or speculum insertion. We determine the correct dilator size during an office visit and show the patient how to insert it and then have her demonstrate that she can perform the insertion. Application of topical 5% lidocaine around the vaginal orifice 15 minutes before insertion and application of a lubricant such as petrolatum to the dilator can be helpful.

In our experience, the dilator should be used several times per week, for 5 to 10 minutes, with the woman gently rotating the dilator during this time. Once she can comfortably insert the dilator at home (typically after about three months of regular use), she should commence using the next larger size of dilator. Medical maintenance therapy for vaginal lichen planus (eg, intravaginal hydrocortisone) should be continued during dilator treatment.

Once satisfactory vaginal dilatation has been achieved, use of a dilator can be continued two to three times weekly to maintain a patent, functional vagina. Women who are able to resume weekly intercourse may be able to stop dilator use.

●Surgery – Severe vaginal synechiae require surgical release. All surgical approaches to vaginal occlusion must be followed by diligent therapy with dilators, topical therapy to maintain control of the inflammation of lichen planus, topical estrogen, and topical corticosteroids. Isolated vaginal procedures without this follow-up therapy invariably result in recurrent vaginal occlusion [25]. Although topical estrogen has no therapeutic role in treatment of the lichen planus, its contribution to epithelial integrity of the vagina cannot be underestimated. Many women with lichen planus are postmenopausal, making the estrogen an important adjunct to restore suppleness, elasticity, and moisture to the area.

Emerging therapy — Photodynamic therapy (PDT) may be an emerging treatment option for vulvar and vaginal lichen planus. A observer-blinded randomized trial that compared one session of vulvovaginal hexyl 5-aminolevulinate hydrochloride (HAL)-PDT to daily application of clobetasol propionate 0.05% ointment (with or without intravaginal hydrocortisone acetate 1% foam) in 40 women with genital erosive lichen planus found similar mean reductions in clinical scores in the PDT and corticosteroid groups after six weeks (25 versus 22 percent) [70]. PDT is painful; all patients were sedated during treatment. Additional study is needed to confirm the efficacy of PDT.

Treatment of hypertrophic and papulosquamous lichen planus — Hypertrophic and papulosquamous lichen planus involving the vulva is more responsive to therapy than erosive lichen planus. A two-week course of a moderate or superpotent topical corticosteroid, as described above, usually results in complete remission or significant improvement of the disease. The adjunctive measures used for erosive vulvar lichen planus are also employed for hypertrophic and papulosquamous disease. (See 'Adjunctive measures' above.)

Hyperkeratotic lesions may fail to respond well to topical corticosteroids due to the limited ability of the drugs to penetrate a thickened stratum corneum. We have found intralesional corticosteroid therapy useful for these cases. The procedure is similar to that used for vulvar lichen sclerosus. (See "Vulvar lichen sclerosus: Management", section on 'Superpotent topical corticosteroids'.)

Treatment of vulvo-vaginal-gingival syndrome — Vulvovaginal disease is treated as discussed above. Treatment of oral lesions is reviewed separately. (See "Oral lichen planus: Management and prognosis".)

Treatment of concurrent infection — An associated bacterial or fungal infection should be treated with antimicrobials concurrently with corticosteroid therapy. Antibiotic selection should be based upon culture and sensitivity results. We prescribe oral fluconazole for vulvar candidiasis to avoid the irritant effects of topical creams.

●(See "Candida vulvovaginitis in adults: Treatment of acute infection".)

●(See "Candida vulvovaginitis in adults: Recurrent infection".)

OUTCOME — Most women report improvement or resolution of symptoms with initial therapy [6,7,69]. Symptomatic improvement corresponds with healing of erosions and reticulation on physical examination, but scarring is not reversible. As an example, a series in which 89 women with erosive lichen planus of the vulva were treated with 0.05% clobetasol ointment reported 71 percent had a good response, 24 percent had a partial response, and 5 percent had a poor response to initial treatment [7]. The response rate was similar during maintenance therapy. A reliable estimate of the efficacy of second-line therapies could not be made because each agent was used by only one to seven patients.

Burning and itching that persist after therapy and despite the clinical appearance of skin improvement suggest superimposed vulvar pain (see "Vulvar pain of unknown cause (vulvodynia): Treatment"). Symptoms with characteristic skin changes suggest relapse.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Lichen planus" and "Society guideline links: Vulvar dermatitis".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Lichen planus (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Lichen planus refers to a relatively uncommon inflammatory dermatologic condition that may be generalized or isolated to the vulva. (See 'Introduction' above.)

●The four types of vulvar lichen planus are erosive lichen planus, papulosquamous lichen planus, hypertrophic lichen planus, and lichen planopilaris. Clinical manifestations include irritating vaginal discharge, vulvar soreness, intense pruritus, burning, and dyspareunia. A few patients are asymptomatic or have only minimal symptoms. (See 'Clinical manifestations' above.)

●Erosive lichen planus is the most severe form of vulvar lichen planus and can result in marked architectural destruction, including loss of the labia minora and narrowing of the introitus. The vagina is often involved, as well. (See 'Erosive lichen planus' above.)

●The diagnosis of vulvar lichen planus is based upon the presence of characteristic clinical manifestations and biopsy findings. A biopsy is recommended for all patients with suspected erosive or hypertrophic lichen planus. (See 'Diagnosis' above.)

●The management of all chronic vulvar disorders requires patient education, behavioral modification, support, and medication. (See 'Adjunctive measures' above.)

●For most women with erosive vulvar lichen planus, we suggest a superpotent topical corticosteroid for first-line therapy (table 3) (Grade 2C). Patients who present with severe erosive vulvar lichen planus that prevents the application of topical therapy may be given oral or intramuscular glucocorticoids as initial therapy. (See 'Erosive vulvar lichen planus' above.)

●For women with vaginal lichen planus, we suggest intravaginal corticosteroids given as foam or suppositories as first-line therapy (Grade 2C). Other therapeutic options include tacrolimus suppositories and systemic glucocorticoid therapy. (See 'Vaginal involvement' above.)

●Vaginal involvement with lichen planus can lead to the formation of adhesions and scarring. After mucosal inflammation is controlled with medical therapy, dilators and surgery can be useful for improving these complications. Surgery is necessary for the treatment of severe vaginal synechiae. (See 'Adhesions and scarring' above.)

ACKNOWLEDGMENTS — The UpToDate editorial staff acknowledges Elizabeth G Stewart, MD, and Susan M Cooper, MB ChB, MRCGP, FRCP, MD, who contributed to earlier versions of this topic review.