Vulvar lichen sclerosus: breaking the silence

Early diagnosis and treatment play a substantial role in a patient’s prognosis and result in a decreased risk of malignancy and scarring.
Early diagnosis and treatment play a substantial role in a patient’s prognosis and result in a decreased risk of malignancy and scarring.

Lichen sclerosus is a chronic inflammatory skin condition that most commonly affects the vulva, groin, and perianal region of postmenopausal women.1,2 This disease is not limited to the anogenital skin of postmenopausal women, as it also less frequently affects men and children, and it develops in extragenital areas as well. The estimated prevalence ranges from 1 in 30 elderly women to 1 in 1,000 patients referred to a dermatologist.3 These numbers are likely underestimated because the disease is often misdiagnosed, never diagnosed due to the patient being asymptomatic, or in some instances, patients are reluctant to seek help due to embarrassment of symptoms.4 Vulvar lichen sclerosus typically presents in a bimodal fashion in prepubertal children and in postmenopausal women. It is more common in women, with a 5:1 female/male ratio.1,4 

Because these patients may initially present at the onset of symptoms to a wide variety of practices such as primary care, dermatology, gynecology, urology, pediatrics, or even the emergency department, it is important for practitioners to understand how to properly diagnose and treat those with this disease. Severe cases of vulvar lichen sclerosus can significantly impair the quality of life that patients have, as it often negatively affects women physically, mentally, and emotionally.5 The earlier that the disease is diagnosed and adequately treated, the less likely the disease will progress to irreversible scarring and malignancies such as squamous cell carcinoma.

Etiology of the disease 

The exact etiology of lichen sclerosus has not been ascertained; however, evidence points to an increased likelihood of an autoimmune and genetic component.4 In a study of 350 women with lichen sclerosus, researchers found that 21.5% had 1 or more autoimmune-related diseases, 21% had a family history of autoimmune disease, and 42% had autoimmune antibodies.4 The most common autoimmune diseases associated with lichen sclerosus are autoimmune thyroiditis, alopecia areata, vitiligo, and pernicious anemia.4

In addition to an autoimmune factor, it appears that genetics has a pathogenetic role as well. A study of 1,052 women with vulvar lichen sclerosus found that 12% of participants had a positive family history of lichen sclerosus.

Trauma and chronic irritation have also been linked to being contributory factors of vulvar lichen sclerosus.4 Cases of the skin condition have been reported in patients with genital jewelry, postsurgical procedures, and genital trauma.4 Furthermore, chronic irritation caused by urine exposure and reduced estrogen levels have also been associated with anogenital lichen sclerosus.4 

Physical findings and clinical presentation

Physical examination findings can vary widely in presentation and severity among patients (see Table 1).1 Typical vulvar lichen sclerosus characteristics may include hypopigmentation, atrophy, erythema, fissures, and purpura of the skin.6 These skin characteristics are often described as “parchment-like” or “cigarette paper” and present in an hourglass configuration surrounding the intraoital and perianal area.1,3 As the disease progresses, atrophy of the epidermis can cause development of scar tissue, resulting in the loss of vulvar architecture, labial fusion, phimosis of the clitoral hood, and intraoital constriction.7 All of these dermal skin changes are usually associated with symptoms of intense pruritus and pain, although it is possible for patients to be entirely asymptomatic. In mild cases, erythema may be the only sign present; however, in more severe cases, inflammation, subepithelial hemorrhages, and chronic ulceration may be evident.1

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Diagnosis 

Diagnosis is typically based on the characteristic clinical presentation and is preferably confirmed with histologic features of a 3-mm punch biopsy.3 Several other disorders that present with vulvar pruritus and/or similar presentations need to be considered when diagnosing this condition. A differential diagnosis should include, but is not limited to: localized scleroderma, cutaneous discoid lupus erythematous, atrophic lichen planus, lichen simplex chronicus, vitiligo, mucous membrane pemphigoid, anal fissures and hemorrhoids, candidiasis, estrogen deficiency, and vulvar intraepithelial neoplasia. Biopsy of suspected vulvar lichen sclerosus in adults is preferred during the initial visit rather than after empiric therapy because of: 

  1. the risk for atypia or malignancy; 
  2. the expansive list of possible differentials associated with vulvar pruritus and whitening can lead to misdiagnosis and/or delay in diagnosis; 
  3. steroid treatment within 2 weeks prior to biopsy may affect pathological accuracy; and 
  4. classic signs and symptoms may not be present, further complicating diagnosis.3

Adverse effects and associated comorbidities

Psychological. In addition to the adverse physical effects of vulvar lichen sclerosus, patients often experience psychological consequences. Frustration and despair are commonly reported.8 Quality of life tends to decline due to the difficulties associated with vulvar lichen sclerosus.8 Patients may have trouble sleeping, interacting socially with others, or even being productive in their everyday life.2 The various symptoms may be so severe that they may necessitate the need for psychosomatic or psychological therapy and counseling.

Sexual dysfunction. The atrophy, strictures, and scarring of the affected genitalia secondary to vulvar lichen sclerosus often result in sexual dysfunction, specifically dyspareunia.7 These anatomical changes may also lead to decreased lubrication and decreased sensation, resulting in a decreased ability to achieve orgasm.7 A study conducted by Dalziel established the adverse effect of vulvar lichen sclerosus on sexual function with a high rate of dyspareunia and reduced frequency of intercourse.7 When these patients were treated appropriately, sexual dysfunction improved but did not completely resolve.6,7 

Malignancy. The lesions associated with vulvar lichen sclerosus are not considered to be premalignant, but patients have an increased risk of developing squamous cell carcinoma (SCC).3 SCC tends to develop within long-standing lesions with irreversible changes in 3% to 10% of patients.1,9 Early detection, biopsy/excision, and treatment of vulvar lichen sclerosus may lead to a reduced risk of these patients developing SCC.3 

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