Associated comorbidities. Patients with vulvar lichen sclerosus may also have bladder, pain, and bowel comorbidities.5 Bladder comorbidities that were self-reported more  frequently among patients with vulvar lichen sclerosus than in the general population include urinary incontinence and stress urinary incontinence.5 Pain disorders such as vulvar pain, interstitial cystitis, fibromyalgia, and temporomandibular joint pain syndrome were also self-reported significantly more frequently among patients with vulvar lichen sclerosus than in the general population.5 This trend was equivalent for gastrointestinal disorders such as inflammatory bowel diseases, chronic constipation, and irritable bowel syndrome.5 Practitioners should be aware of these associated comorbidities and appropriately screen for them among their patients with vulvar lichen sclerosus. 

Management 

Although there is no cure for vulvar lichen sclerosus, treatment can provide significant relief of symptoms and prevent progression of the condition. Early diagnosis is imperative because the disease can lead to irreversible scarring and anatomical changes if left untreated. 

Topical ultrapotent corticosteroids are considered the gold standard of treatment for patients with vulvar lichen sclerosus. In one study, ultrapotent corticosteroids resulted in the improvement of symptoms in nearly all participants, complete relief of symptoms in about 70%, and complete remission of skin changes in about 20%.4 The use of topical clobetasol propionate 0.05% has been examined in several studies and appears to provide therapeutic benefit and improve the quality of life in patients with vulvar lichen sclerosus.2 Initial course of treatment is usually 6 to 12 weeks, with ointment being applied nightly to the affected skin. 

If patients do not respond to this treatment after an adequate amount of time, dosage is appropriate, and patient compliance is confirmed, practitioners should investigate other likely diagnoses. Possible contact allergy, secondary infection, and malignancy should also be considered.4 Once these have been ruled out, patients can use topical calcineurin inhibitors as a 2nd-line therapy. 

Maintenance therapy is recommended for patients with vulvar lichen sclerosus even when they report complete relief of symptoms after initial treatment.10 Maintenance therapy should consist of mometasone furoate, 0.1%, ointment applied 2 to 3 times weekly.10 This is a less potent corticosteroid that provides greater anti-inflammatory benefits and has a longer duration of action compared with those corticosteroids of similar potency.3 

Topical corticosteroids are less effective on thickened hypertrophic plaques. Practitioners should consider intralesional injections of triamcinolone hexacetonide or triamcinolone acetonide for patients with lesions of this type.3 Various other therapies have also been studied in the treatment of vulvar lichen sclerosus; however, when compared with topical clobetasol propionate 0.5%, they are less efficacious, less convenient for patients, or have increased side effects associated with their use. These therapies, which include UV-A1 phototherapy, photodynamic therapy, oral acitretin, and topical progesterone/testosterone, should only be used as 3rd-line therapy.

Surgical intervention should only be used in a limited number of circumstances, such as in patients with malignancy or to correct irreversible scarring and adhesions. Intraoital surgical widening can be used to correct micturition difficulties or sexual dysfunction caused by the subsequent anatomical changes.4 

Prognosis

Although vulvar lichen sclerosus cannot be cured, it is highly treatable. Early diagnosis, early treatment, and patient compliance play a substantial role in the patient’s prognosis and result in a decreased risk of malignancy and scarring.9 It is estimated that 96% of patients will respond to topical corticosteroids initially, but relapse is likely without long-term maintenance therapy.9 Patients often see an increase in quality of life and decrease in sexual dysfunction with adequate treatment of this condition. Long-term follow-up is strongly encouraged and recommended due to the chronic nature of this condition and associated risk for malignancy. Patients should be advised that they need to follow up for evaluation at least once annually after their condition is well-managed and under control.3 

Conclusion

Vulvar lichen sclerosus is a chronic inflammatory skin condition that often leads to a decreased quality of life if left undiagnosed and subsequently untreated. The etiology of the disease is still undetermined, but it is thought that genetic and autoimmune factors may predispose patients to the condition. Generally, the disease is diagnosed based on characteristic clinical presentation; however, biopsy is recommended to confirm the diagnosis. Women are not only affected physically by the disease, but mentally and emotionally as well. It is important for practitioners to be aware of the adverse effects and associated comorbidities when diagnosing and treating patients with vulvar lichen sclerosus. Earlier detection and subsequent adequate management can lead to a reduction of these adverse outcomes. Topical ultrapotent corticosteroids remain the gold standard of treatment; however, various 2nd-line therapies can be used among patients whose condition is refractory to the topical ultrapotent corticosteroids. The disease is highly treatable, but long-term maintenance therapy and lifetime follow-up are imperative among all patients with this chronic and debilitating condition.

Melissa Morgan, MPA, PA-C, is a physician assistant with North Atlanta Primary Care, and Lisa Daitch, MPAS, PA-C, is clinical medicine II course coordinator at Augusta University in Georgia.

References

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