Dermatology

Lichen Sclerosus et Atrophicus (lichen sclerosus [preferred term], lichen planus atrophicus, lichen planus sclerosus, kraurosis vulva, balanitis xerotica obliterans, vulvar dystrophy)

Are You Confident of the Diagnosis?

Characteristic findings on physical examination

The most common presenting symptom in patients with lichen sclerosus is pruritus, often worse at night. Pain, dysuria, urinary retention, and dyspareunia due to fissures and/or erosions is not uncommon. Painful defecation due to perianal fissures can result in constipation (often the presenting symptom in young girls) and stool retention. Some patients (7% of children, 1% of adults) may be asymptomatic, even in the setting of advanced disease.

Classic lichen sclerosus is characterized by porcelain white (hypo/depigmented) patches and plaques with epidermal wrinkling characterized by a crinkled “cigarette paper” appearance; shininess, waxiness and hyperkeratosis are also common (Figure 1). Additional findings include fissures, erosions, superficial ulcers and purpura; bullae are less common. Lichenification, the result of scratching, may also be present and can confuse the clinical picture.

Figure 1.

Localized mild vulvar lichen sclerosus

The most frequently affected sites of vulvar lichen sclerosus include the medial aspects of the labia majora, interlabial creases, labia minora, clitoral hood, and clitoris. Skin changes may extend to the genitocrural creases, creases at the inferior aspect of the labia majora, perineal body and perianal skin. Perianal lesions occur in 30% to 60% of women (Figure 2). In girls and women, a figure-of-eight or hourglass configuration with vulvar and perianal involvement is common. Lichen sclerosus can affect the vaginal introitus (also called the vaginal vestibule) but unlike lichen planus, lichen sclerosus does not extend past the hymen to involve the vagina.

Figure 2.

Perianal involvement in a patient with mild vulvar lichen sclerosus

Lichen sclerosus affects the glans penis, prepuce (foreskin) and less often the penile shaft. Perianal disease is extremely rare in males. Lichen sclerosus can, however, affect the epithelium immediately around the urethral meatus and also the lower part of the urethra.

Lichen sclerosus can present with variable degrees of agglutination scarring characterized by loss of tissue mass, tissue resorption and destruction

of normal vulvar architecture of the clitoral hood, labia minora, posterior fourchette and vaginal introitus (Figure 3). Agglutination of the clitoral hood may result in a complete burial of the clitoris such that it is no longer visible (though still palpable) on examination.

Figure 3.

Vulvar lichen sclerosus with labia minora agglutination

Similarly, the labia minora may be reduced in size or completely absent. Scarring of the introitus may result in decreased size of the introitus (both superiorly above the level of the urethra and at the posterior fourchette). In severe cases, the introitus may be almost completely sealed (Figure 4), thereby compromising the patient's ability to urinate.

Figure 4.

Vulvar lichen sclerosus with severe scarring and reduced introital aperture.

Scarring due to lichen sclerosus in boys and men leads to adhesions between the underside of the foreskin and the glans and a gradual tightening of the foreskin, which can result in phimosis (inability to retract the foreskin due to stenosis and tightening). Stenosis and obstruction of the urethral meatus may also occur. Lichen sclerosus is the most common cause of phimosis in boys and accounts for 11% to 30% of cases of phimosis in adults.

Active or resolving lichen sclerosus can present with patchy hyperpigmentation. This pigmentation can exhibit variable shades of brown/black/gray pigmentation and can be strikingly irregular and varied. Biopsy may be required to differentiate post-inflammatory hyperpigmentation from genital lentiginosis (melanosis) and atypical melanocytic proliferations including melanoma.

Extragenital lichen sclerosus occurs in 6% to 20% of women but is rare in men. Hypopigmented/white, waxy, wrinkled papules and plaques, often with follicular plugging, preferentially affect the neck, upper back, breasts, axillae, abdomen/waist, and thighs; extrageintal lesions are usually asymptomatic (Figure 5). Lichen sclerosus koebnerizes, which explains preferential involvement of areas subjected to repeated friction as well as sites of previous trauma/scars. Lichen sclerosus has rarely been reported to affect the scalp, face, mouth (manifesting as white firm plaques on the lips, buccal mucosa, dorsal tongue, attached gingivae) and nails.

Figure 5.

Ivory waxy papules typical of extragenital lichen sclerosus

Expected results of diagnostic studies

Diagnostic biopsy is ideal but may not always be practical or necessary. For pediatric patients, a clinical diagnosis of lichen sclerosus with a trial of topical corticosteroids is a reasonable initial approach; biopsy may be required if the patient does not respond to treatment as anticipated.

Classic histopathologic findings of lichen sclerosus include an atrophic epidermis with loss of rete ridges, hyperkeratosis and a band-like lymphocytic inflammatory infiltrate in the upper to mid-dermis. Interface dermatitis and dermal melanophages are also seen. The papillary and upper reticular dermis initially shows edema but eventually the collagen becomes more dense, homogeneous and deeply pink. This homogenized collagen typically sits above the lymphocytic infiltrate. Use of topical corticosteroids results in normalization of dermal collagen hyalinization and resolution of the lymphocytic inflammation (Figure 6).

Figure 6.

Histopathology of vulvar lichen sclerosus reveals atrophy of the epidermis with loss of rete ridges, homogenization of the papillary dermal collagen and lymphocytic bandlike infiltrate.

Evidence of squamous cell hyperplasia with acanthosis of the epidermis represents an increased risk for developing squamous cell carcinoma and warrants further investigation.

Biopsy should be performed if 1) there is suspicion for malignancy, 2) the disease fails to respond to appropriate treatment, 3) there is concern for possible overlap with morphea (for extragenital lesions) or 4) there is pigmentation concerning for atypical melanocytic proliferation.

A thorough review of systems should be obtained followed by directed laboratory testing for evaluation of potential associated systemic illnesses (ie, thyroid disease).

Diagnosis Confirmation

Lichen planus: Vulvar lichen planus most commonly presents as erosions; no hypo/depigmentation but post-inflammatory hyperpigmentation on cutaneous sites can be dramatic; vaginal involvement is common; concomitant oral lichen planus (reticular, erythematous, erosive) is common

Mucous membrane pemphigoid presents as erosions, bullae, purpura on background of hypopigmented scarring; oral mucosal (desquamative gingivitis) and cutaneous involvement (head, neck, upper trunk) may occur simultaneously; may require histopathology and immunofluorescence microscopy to distinguish from lichen sclerosus.

Lichen simplex chronicus: Lichenification with superimposed excoriations or erosions; labia majora are most commonly involved; hypopigmentation may occur in patients with darker skin types although hyperpigmentation is more common.

Extramammary Paget's disease: Widespread erythematous erosions with intervening islands of white hyperkeratosis; associated pruritus may be severe; no epidermal wrinkling; no hypo/depigmentation.

Morphea: Circumscribed indurated sclerotic plaques with ivory/yellow centers and lilac borders; variable dermal atrophy; hair may be absent; may exhibit decreased sweating in affected skin; typically no epidermal wrinkling or fissuring; may have overlapping clinical and histologic features with lichen sclerosus.

Vitiligo: Hypo/depigmentation without changes in skin texture; no fissures, erosions or purpura; typically asymptomatic; non-scarring vulvar intraepithelial neoplasia (squamous cell carcinoma in situ, Bowen's disease). Typically presents as a localized area of persistent hyperkeratosis, erythema, erosion or ulcer; may occur in the context of lichen sclerosus; may be distinguishable from hyperkeratotic or erosive lichen sclerosus only on histopathology.

Squamous cell carcinoma: Typically presents as a localized area of persistent hyperkeratosis, erythema, erosion or ulcer; may occur in the context of genital lichen sclerosus; may be distinguishable from hyperkeratotic or erosive lichen sclerosus only on histopathology.

Pinworm (Eneterobius vermicularis) infection: Presents with intense nocturnal perianal pruritus, excoriations only on examination, no hypo/depigmentation or epidermal wrinkling; positive tape test.

Sexual abuse: May present with purpura, erosions or fissures; no associated hypo/depigmentation or epidermal atrophy; may occur in the setting of lichen sclerosus; suspected cases should be managed by providers with specialized training in sexual abuse evaluation and intervention.

Who is at Risk for Developing this Disease?

Lichen sclerosus is common. Exact prevalence is unknown. Based on data obtained from referrals to vulvar specialty clinics, prevalence is estimated to be between 1/300 and 1/1000 women. Genital lichen sclerosus has a bimodal distribution for peak incidence - prepubertal and midlife (peri- and postmenopause in women) for both males and females.

Mean age for presentation of pediatric lichen sclerosus is 5 years. Adult lichen sclerosus is more common than pediatric disease. Lichen sclerosus is rare in circumcised males.

Familial predisposition has been reported in lichen sclerosus; one study demonstrated that 12% of 1000 vulvar lichen sclerosus patients in the UK had a positive family history of lichen sclerosus. The inheritance pattern has not been established.

One study of human leukocyte antigen (HLA) associations demonstrated that lichen sclerosus patients had a statistically significant difference in expression of DQ7, DQ8, or DQ9 antigens compared to controls. Another study demonstrated that haplotype DRB1*12/DQB1*0301/04/09/010 confers susceptibility to lichen sclerosus while haplotype DRB1*0301/04/DQB1*0201/02/03 seems to offer protection.

What is the cause of the disease?

Etiology and Pathophysiology

Lichen sclerosus is a chronic inflammatory, lymphocyte-mediated dermatosis that is believed to be autoimmune in nature. Exact pathogenesis and target antigen are not known.

Circulating IgG antibodies to basement membrane zone components (BP180, BP230) have been detected in 30% of vulvar lichen sclerosus patients.

Circulating autoantibodies to extracellular matrix protein 1 (ECM1), has been demonstrated in 67% of patients with lichen sclerosus.

Multiple studies have demonstrated a strong association between lichen sclerosus and other autoimmune disorders, namely autoimmune thyroid disease, pernicious anemia, vitiligo and alopecia areata. A substantial number of women with lichen sclerosus may have another autoimmune disease, a family history of autoimmune disease or autoimmune antibodies.

Given the peak incidence of genital lichen sclerosus before puberty and after menopause, a potential role for hormones in the pathogenesis of lichen sclerosus has been postulated. Reduced numbers of androgen receptors in vulvar lichen sclerosus has been shown in multiple studies. Studies of estrogen receptor (ER) isoform expression have shown that ER-beta is absent in normal tissues but highly expressed in vulvar lichen sclerosus while ER-alpha was not expressed in the fibrovascular layer of diseased vulvar tissue.

Lichen sclerosus exhibits koebnerization and has been shown to occur in areas of trauma, irritation, as well as after sunburn or radiation therapy.

A possible role for Borrelia burgdorferi infection in the etiology of extragenital lichen sclerosus has been suggested in Europe based on polymerase chain reaction (PCR) amplification data. Controversy surrounds this finding in Europe where Borrelia infection is endemic. Multiple studies have shown no association in the United States. There are no data to suggest a role for Borrelia in the pathogenesis of genital lichen sclerosus.

Systemic Implications and Complications

A thorough review of systems should be obtained followed by directed laboratory testing for evaluation of potential associated systemic illnesses (ie, thyroid disease).

Severe agglutination can result in loss and destruction of normal vulvar architecture. Narrowing of the vaginal introitus with potential compromise of effective micturition can occur; fortunately, agglutination tends to progress slowly, but very advanced scarring may result in acute urinary obstruction and retention, which mandates emergent surgical lysis of the adhesed structures to open the introitus (Figure 4, Figure 7).

Figure 7.

Vulvar lichen sclerosus with severe scarring after surgical lysis of agglutination.

Agglutination in males can result in phimosis with associated pain and urinary dysfunction.

Malignant transformation has been reported in patients with poorly controlled or untreated vulvar lichen sclerosus. The exact risk of neoplastic change is not known. The risk of developing squamous cell carcinoma is estimated to be 2% to 5% for vulvar lichen sclerosus. Risk factors include elderly age (possibly a surrogate marker for longer duration of disease), hyperkeratotic clinical lesions and squamous dypslasia on histopathology. If it occurs, vulvar squamous cell carcinoma tends to develop rapidly.

Squamous cell carcinoma is estimated to occur in 5% of penile lichen sclerosus cases.

Clitoral hood agglutination may result in the formation of a clitoral pseudocyst characterized by accumulation of keratin debris with subsequent pain. Surgical excision (subtotal or total) may be indicated.

Treatment Options

Topical medical treatment

Super-potent/potent topical corticosteroid ointments (eg, clobetasol propionate 0.05% ointment)

Topical calcineurin inhibitors (tacrolimus 0.1% ointment, pimecrolimus 1% cream)

Topical retinoids (tretinoin 0.025% cream)

Topical cyclosporine solution

Calcipotriol 0.005% ointment

Progesterone ointment

Systemic medical treatment

Acitretin

Methotrexate

Pulsed methylprednisolone with methotrexate

Cyclosporine

Stanozolol

Potassium para-aminobenzoate

Calcitriol

Hydroxychloroquine

Hydroxycarbamide (hydroxyurea)

Surgical treatment

Circumcision in males with persistent coronal adhesions and/or phimosis

Urethral dilation, meatoplasty or meatotomy for urethral meatal stenosis

Excision of vulvar intraepithelial neoplasia or squamous cell carcinoma

Lysis of adhesions and scarring in patients with severe introital narrowing

Circumcision (subtotal or total) for females with symptomatic clitoral pseudocyst

Physical treatment

Phototherapy (UVA1, psoralen-UVA, narrowband UVB)

Cryotherapy

Photodynamic therapy

Carbon dixoide (CO2) laser therapy

Optimal Therapeutic Approach for this Disease

General Principles

Multidisciplinary care, which may include dermatology, gynecology, urology, pediatrics (for children) and sex therapy counseling, is essential to the optimal care of patients with lichen sclerosus.

Goals of treatment include reduction of symptoms and normalization of skin changes.

There are very few randomized, controlled trials to support the therapeutic approach to lichen sclerosus. There is very limited literature on the use of systemic agents in the treatment of lichen sclerosus.

Asymptomatic patients with clinical evidence of active lichen sclerosus should be treated.

Genital lichen sclerosus typically is very responsive to topical corticosteroid treatment. Extragenital lichen sclerosus is generally resistant to treatment.

Patients should be evaluated for concomitant infection (impetigo, candidiasis, herpes simplex virus infection, etc) and other dermatologic (allergic or irritant contact dermatitis, lichen planus, etc) or gynecologic (atrophic vaginitis, bacterial vaginosis, etc) conditions upon initial evaluation and during all subsequent encounters. Any identified pathology should be treated, preferentially with oral medication to avoid potential for developing allergic or irritant contact dermatitis.

Patient education and teaching is essential to achieving correct application of topical medications to the vulva. The patient's vision, mobility and habitus should be considered when formulating a treatment plan; patients must be able to see and reach the targeted treatment areas. Patients should be provided with a hand mirror and shown the areas for medication application during the examination. Ideally, patients should also be provided with written instructions (detailing how much medication to apply at what frequency) as well as a diagram or clinical photograph with the areas to be treated highlighted to optimize the chances for successful topical therapy.

Patient educational handouts on vulvar lichen sclerosus are available through the International Society for the Study of Vulvovaginal Diseases (www.issvd.org).

Non-sedating (loratadine 10mg, fexofenadine 180mg, cetirizine 10mg daily in morning) and sedating antihistamines (diphenhydramine 25 to 50mg, hydroxyzine 10 to 50mg, doxepin 10 to 30mg daily at bedtime) can be used for associated pruritus while topical medications are initiated.

Topical Medical Treatment

Super-potent/potent topical cortisteroid ointments (eg, clobetasol propionate 0.05%) are considered the first-line, gold standard treatment of choice for both genital and extragenital lichen sclerosus. Ointment formulations are preferred over creams as ointments tend to contain fewer potential allergens/irritants, act as an adjunctive barrier on erosions and fissures and are not water-soluble (and therefore less likely to be diluted by sweat, vaginal discharge and urine).

Initially, topical corticosteroid ointment is applied once daily at bedtime or twice daily, depending on the severity of disease and associated symptoms. Once symptoms improve and the skin texture returns to normal, the frequency of topical corticosteroid use can be tapered to every other night at first and then less often as tolerated. With continued improvement, the potency of topical corticosteroid used may also be reduced as tolerated. Potential adverse effects of topical corticosteroids include erythema, telangiectasia and atrophy (more common on the labia majora and buttocks than the modified mucous membranes) and less commonly, allergic or irritant contact dermatitis.

Complete or partial relief of symptoms has been reported in 95% of women with vulvar lichen sclerosus after 3 months of daily use of super-potent topical corticosteroids.

Topical corticosteroid use in men and boys with penile lichen sclerosus is safe and effective with improvement in discomfort, skin tightness and urinary flow noted. Topical corticosteroid use may also decrease the need for circumcision.

Use of vaginal dilators with lubricants and topical estrogen can be helpful to address introital narrowing/scarring.

Topical calcineurin inhibitors, tacrolimus and pimecrolimus, applied twice daily may be used as second-line therapy for those patients who do not respond to or experience adverse effects from topical corticosteroids. There is concern for increased risk of malignancy with use of topical calcineurin inhibitors, and squamous cell carcinoma has been reported in patients using topical calcineurin inhibitors for vulvar lichen sclerosus.

Tretinoin 0.025% cream has been used once daily, 5 days per week for 1 year in the treatment of vulvar lichen sclerosus in an open uncontrolled study. Symptoms, clinical and histopathologic parameters improved. Adverse effects (irritation, pain) did occur but did not cause discontinuation. Results were maintained at 4 to 13 months after treatment.

Topical cyclosporine (200mg/day of cyclosporine oral solution, 50mg 4 times daily for 8 weeks) had minimal impact on vulvar lichen sclerosus in a single small pilot study. Cost may be prohibitive.

Positive impact of calcipotriol 0.005% ointment applied twice daily under occlusion to extragenital lichen sclerosus lesions for 12 weeks has been documented in a single case report. Irritation may limit use of calcipotriol ointment on genital skin.

There is a single report of topical progesterone being beneficial in the treatment of vulvar lichen sclerosus.

Despite its historical use, topical testosterone currently has no role in the treatment of genital lichen sclerosus.

Systemic Medical Treatment

Systemic medical treatment should be considered in patients who have severe disease or disease that does not respond to topical therapy after consideration of potential adverse effects.

A double-blind, placebo-controlled study of acitretin (20 to 30mg/day for 16 weeks) for the treatment of refractory vulvar lichen sclerosus showed a higher number of responders for acitretin (63%) compared with placebo (25%). A more recent study of acitretin for the treatment of refractory penile lichen sclerosus revealed that partial or complete response was achieved in 72.8% of the acitretin group versus 18.8% of controls. Cheilitis, skin peeling, pruritus, paronychia, hair shedding and mild elevation of liver enzymes was common. Acitretin is teratogenic and should not be used in women of child-bearing potential.

Systemic retinoids may have a role in hyperkeratotic or hypertrophic disease that is refractory to superpotent topical corticosteroids.

Methotrexate (10mg/week, oral) was effective in one patient with extensive refractory lichen sclerosus (extragenital and genital). Softening of lesions was noted by 3 weeks of treatment, and an excellent response was reported after 5 months. Complete resolution of all cutaneous lesions occurred by 8 months and remission was maintained during the 6-month follow-up period. Transient liver enzyme elevation was the only adverse effect reported.

Pulsed high-dose methylprednisolone (1000mg single dose given intravenously for 3 consecutive days monthly) in combination with low-dose oral methotrexate (15mg/week) was utilized in seven patients with refractory generalized lichen sclerosus. Improvement was initially noted after the third month of treatment. Over at least 6 months of treatment, individual nonvalidated clinical scores improved, and relapse did not occur during the 3-month follow-up period. Adverse effects included nausea, headache and liver enzyme elevation, all of which resolved with cessation of treatment.

Oral cyclosporine (3 to 4mg/kg/day tapered over 3 months) was used in an open label, uncontrolled trial of five patients with refractory vulvar lichen sclerosus. At the end of 3 months of treatment, symptoms and clinical findings (erythema, erosion) were improved. The impact of treatment on histopathology was not assessed. Adverse effects included nausea, mild hypertrichosis and mucositis but did not result in interruption of treatment. Other potential adverse effects include immunosuppression with increased risk of infection, hypertension, electrolyte abnormalities and renal dysfunction.

Stanozolol (2mg oral twice daily for 3 months) was used in five patients with penile lichen sclerosus. At 3 months of treatment, moderate to marked improvement in symptoms and clinical findings was noted in all patients. Mild transient elevation of liver enzymes was noted in 1 patient.

The use of potassium para-aminobenzoate (4 to 24 g daily in divided doses) has been reported in five patients with refractory lichen sclerosus at various sites. Clinical improvement was noted in all patients with flattening of skin lesions and reduction or resolution of symptoms.

Oral calcitriol (0.5mcg once daily) was used in a single patient with refractory generalized cutaneous lichen sclerosus. Improvement of lesions was noted after 6 months of treatment and persisted for 1 year after discontinuation of treatment. Hypercalciuria, which resolved with dose reduction, was the only adverse effect noted.

Hydroxychloroquine has been utilized for widespread bullous extragenital lichen sclerosus in two reports with mixed results.

Hydroxycarbamide (hydroxyurea, 1g daily) has been reported in a postmenopausal woman with refractory vulvar lichen sclerosus. Associated soreness and pruritus improved within 1 month and resolved completely within 3 months. Clinical findings also remitted. Potential adverse effects include gastrointestinal disturbance, mucositis, renal dysfunction, skin ulceration and secondary leukemia, which may limit its utility.

Surgical Treatment

Large studies provide good evidence for surgical intervention for severe irreversible phimosis and/or urethral meatus stenosis due to lichen sclerosus. A multicenter study of more than 200 males demonstrated that circumcision, meatotomy, circumcision with meatotomy and urethroplasty were successful in the vast majority of treated patients. Circumcision does not guarantee remission of lichen sclerosus, and lichen sclerosus has been shown to koebnerize in circumcision scars.

In pediatric lichen sclerosus, topical corticosteroids should be used prior to circumcision in all cases of phimosis. Circumcision should be performed in corticosteroid-refractory cases.

First-line treatment of urethral meatal stenosis is urethral dilation or meatoplasty, often with topical corticosteroid use implemented at the time of surgery.

Surgery is not indicated for patients with uncomplicated vulvar lichen sclerosus. Surgical treatment is indicated for functionally significant vulvar and vaginal scarring once active inflammation has resolved; patients should be monitored for at least 6 months for recurrence of active disease before undergoing surgery. Regular use of super-potent/potent topical corticosteroids after vulvar surgery is essential to prevent flares of lichen sclerosus (given the koebnerization phenomenon) and to minimize the risk of re-agglutination and stenosis.

Surgical intervention is also indicated for squamous cell carcinoma and other cutaneous malignancies in the setting of lichen sclerosus.

Physical Treatment

The use of phototherapy (narrowband UVB, psoralen-UVA and UVA1) for extragenital lichen sclerosus has been reported in case reports and small case series. UVA1 has demonstrated the most success in reducing clinical signs of sclerosis and symptoms. UVA1 was used in 7 women with vulvar lichen sclerosus refractory to topical corticosteroids; initial improvement was noted in 5 subjects but most required ongoing treatment with topical corticosteroids.

Cryotherapy was performed in one small study and provided relief of pruritus in 75% of patients with vulvar lichen sclerosus; 50% of subjects had relief for 3 years. Patients should be cautioned regarding pain associated with the procedure as well as risk of scarring and dyschromia. Data are limited, and long-term efficacy is not known.

Photodynamic therapy using topical aminolevulinic acid and argon laser for 1 to 3 treatments provided significant improvement in a majority of patients with vulvar lichen sclerosus in one small open study. Photodynamic therapy can cause significant discomfort due to associated desquamation and inflammation.

Carbon dixoide (CO2) laser therapy has been employed for meatal stenosis due to penile lichen sclerosus but is not considered standard therapy. CO2 may cause scarring.

Patient Management

With appropriate medication use and monitoring, disease control can be achieved in 3 to 4 months in most patients, but treatment regimens should be tailored for individual patients.

Thirty grams of corticosteroid ointment should be an adequate amount for once-daily treatment of genital skin for 3 months. Maintenance therapy with 30g used over 6 to 12 months is considered safe.

Once disease and symptom control have been achieved, patients may be maintained on topical corticosteroids. Some clinicians recommend less frequent use of super-potent topical corticosteroid (clobetasol propionate 0.05% ointment) 2 to 3 times per week. Others recommend use of a low-to-medium potency topical corticosteroid (triamcinolone acetonide 0.1% ointment) once daily.

Long-term safety studies confirmed that super-potent topical corticosteroids can be safely used on genital skin for at least 12 months. Directed application of topical corticosteroids is essential, however, as striae may occur with inadvertent application of corticosteroids to the inguinal crura, inferior abdomen/mons, medial thighs and buttocks. When using topical corticosteroids daily, patients should be monitored every month to assess for disease improvement as well as potential complications (atrophy, steroid erythema, secondary infection, contact dermatitis, etc).

Patients who fail to improve despite correct medication use should be re-evaluated to ensure that the initial diagnosis was indeed correct and that there is no additional superimposed pathologic process (allergic/irritant contact dermatitis, infection, malignant transformation).

The risk of developing vulvar squamous cell carcinoma in patients with lichen sclerosus is estimated to be between 2% to 5%. The risk of vulvar squamous cell carcinoma is 300 times greater for women with lichen sclerosus compared to women without lichen planus. Approximately 60% of vulvar squamous cell carcinomas occur in the context of lichen sclerosus. Patients with poorly controlled vulvar lichen sclerosus may be at greater risk for malignancy. Areas of persistent erythema, erosion or hyperkeratosis can herald dysplastic change, and there should be a low threshold to biopsy such lesions in patients with lichen sclerosus.

Verrucous carcinoma, basal cell carcinoma, melanoma and Merkel cell carcinoma have rarely been reported in patients with vulvar lichen sclerosus; there appears to no increased frequency relative to the general population.

For penile lichen sclerosus, the risk of squamous cell carcinoma is estimated to be 5%. Approximately 40% of penile squamous cell carcinomas occur in the setting of lichen sclerosus.

The risk of malignancy in pediatric-onset lichen sclerosus is not known.

It is not known if effective treatment of genital lichen sclerosus reduces the risk of malignant transformation.

For some pediatric patients, lichen sclerosus tends to improve or remit at puberty. Lichen sclerosus remains active in adolescence in a significant portion of pediatric patients (75% in two studies) and can persist into adulthood.

For vulvar lichen sclerosus, increasing age (>70 years) is associated with poor response to treatment and failure to remit.

Even when lichen sclerosus is well-controlled on maintenance regimens, patients should be seen in follow-up every 6 to 12 months to monitor disease activity and potential complications (atrophy, scarring, dysplasia).

Unusual Clinical Scenarios to Consider in Patient Management

Vulvovaginal candidiasis, tinea cruris, herpes simplex virus infection, and secondary bacterial infection can complicate genital lichen sclerosus and its treatment. Appropriate testing (fungal culture, skin scraping for KOH, viral culture, bacterial culture) should be performed in patients who do not respond to treatment as expected or who present with worsening pruritus, fissuring, erosions or pain.

Estrogen deficiency can cause atrophy and fissuring of vulvar skin as well as introital erythema and erosions; therefore, estrogen deficiency can complicate the diagnosis and treatment of lichen sclerosus. Local hormone replacement therapy can be used as a beneficial adjuvant for vulvar lichen sclerosus.

Areas of persistent erythema, erosion or hyperkeratosis may represent malignant change; there should be a low threshold for biopsying these lesions especially if the patient is not responding appropriately to treatment.

Lichen sclerosus and lichen planus can demonstrate clinical and histologic overlap. Lichen sclerosus and vulvovaginal lichen planus can occur simultaneously in the same patient. Vaginal examination is imperative as lichen planus can affect the vagina, whereas lichen sclerosus does not, and if not treated can result in vaginal scarring and obliteration.

Lichen sclerosus (genital and/or extragenital) and morphea (localized scleroderma) can occur in the same patient. Cutaneous biopsy may also demonstrate morphea/lichen sclerosus overlap with features of lichen sclerosus observed in addition to collagen alteration in the deep dermis and entrapment of ecrrine coils more typical of morphea.

Patients may develop persistent vulvar pain (secondary vulvodynia) despite adequate resolution of skin changes and symptoms of lichen sclerosus; treatment with topical anesthetics (lidocaine ointment 5%) or systemic neuropathic pain modulators (amitriptyline, gabapentin, pregabalin, etc) may be considered after other causes of vulvar pain have been excluded.

Men with penile lichen sclerosus may similarly develop penile dysesthesia (burning, pain), which is treated similarly to secondary vulvodynia. Men and women with genital lichen sclerosus may develop decreased interest in sexual activity and sexual dysfunction. Patients and their partners should be encouraged to express any concerns; referral to a qualified sex therapist should be made.

What is the Evidence?

Neill, SM, Lewis, FM, Tatnall, RM, Cox, NH. "British Association of Dermatologists' guidelines for the management of lichen sclerosus 2010". Br J Dermatol. vol. 163. 2010. pp. 672-82.

(This update guideline provides a distilled comprehensive review of the current literature for the genital and extragenital lichen sclerosus with a focus on disease sequelae and evidence-based treatment strategies.)

Jones, RW, Curry, J, Neill, S, MacLean, AB. "Guidelines for the follow-up of women with vulvar lichen sclerosus in specialist clinics". Am J Obstet Gynecol. vol. 198. 2008. pp. 496.e1-3.

(This manuscript provides a review of vulvar lichen sclerosus and recommendations for appropriate long-term monitoring of patients based on complexity of disease.)

Poindexter, G, Morrell, DS. "Anogenital pruritus: lichen sclerosus in children". Pediatr Ann. vol. 36. 2007. pp. 785-91.

(The authors provide a thorough review of the clinical presentation and treatment of pediatric lichen sclerosus.)

Smith, SD, Fischer, G. "Childhood onset of vulvar lichen sclerosus does not resolve at puberty: a prospective case series". Pediatr Derm. vol. 26. 2009. pp. 725-9.

(This article prospectively addresses the natural course of pediatric-onset vulvar lichen sclerosus.)

Cooper, SM, Ali, I, Baldo, M, Wojnarowska, F. "The association of lichen sclerosus and erosive lichen planus of the vulva with autoimmune disease: a case-control study". Arch Dermatol. vol. 144. 2008. pp. 1432.5.

(This case-control study of more than 1000 women assesses the prevalence of personal or family history of autoimmune disease and the frequency of circulating autoantibodies.)

Dalziel, KL, Wojnarowska, F. "Long-term control of vulvar lichen sclerosus after treatment with a potent topical steroid cream". J Reprod Med. vol. 38. 1993. pp. 25-7.

(This retrospective study demonstrates the safety of potent topical cortisteroids in the chronic treatment of vulvar lichen sclerosus.)

Bradford, J, Fischer, G. "Long-term management of vulval lichen sclerosus in adult women". Aust N Z J Obstet Gynaecol. vol. 50. 2010. pp. 148-52.

(This retrospective chart review of 129 adult patients with vulvar lichen sclerosus details the efficacy of long-term treatment with moderate potency topical corticosteroids and the frequency of disease complications [scarring, malignancy].)

Yesudian, PD. "The role of calcineurin inhibitors in the management of lichen sclerosus". Am J Clin Dermatol. vol. 10. 2009. pp. 313-8.

(This review focuses on the utility of topical calcineurin inhibitors for anogenital lichen sclerosus with attention given to the current body of knowledge regarding efficacy, safety and adverse effects.)
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