Lichen Sclerosus

A 50-year-old woman presented to dermatology clinic for evaluation of a pruritic eruption that started on her medial thighs and spread to her abdomen and breasts over the course of 7 to 8 months. Her primary care provider had treated her with triamcinolone ointment, without improvement. On further questioning, she reported vulvar itching and discomfort for a long time. 

On examination, she was noted to have several 1 cm to 5 cm oval, white, scaly, sclerotic plaques with erythematous borders located on the medial thighs, breasts, and lower abdomen (Figures 1 and 2). Similar findings were noted on the medial surfaces of the labia majora (Figure 3). A diagnosis of extragenital and vulvar lichen sclerosus (LS) was made. 

Figure 1. White wrinkled sclerotic plaques with overlying scale on the medial thigh.

The initial treatment was clobetasol ointment once daily to all lesions. At the follow-up appointment 3 months later, the patient noted no improvement. Narrowband UVB phototherapy 3 times a week was prescribed. 

At the 1 month follow-up, the patient was found to have responded dramatically to light therapy and it was suggested that she continue this treatment, along with topical clobetasol. She continued to improve over the next few months but was then lost to follow-up.

Figure 2. Similar lesions on breast and abdomen.

Discussion. LS is a chronic, often relapsing and remitting, inflammatory condition affecting genital and, less commonly, extragenital skin. The typical lesions of LS are polygonal white sclerotic papules and plaques involving the vulvar and sometimes perianal areas.¹ Extragenital LS can be found on the breasts, thighs, and neck.² LS is seen 5 times more often in women, however it can cause significant dysfunction in both sexes as a result of chronic itching, dysuria, dyspareunia, and even obliteration of anatomic structures. In rare cases, squamous cell carcinoma may develop in lesions of LS.³ This is more likely to occur in patients whose LS is severe and poorly controlled.⁴

Figure 3. White, wrinkled sclerotic changes in a "figure of 8" distribution on the vulvar and perianal area. 

While the exact etiology has not been elucidated, an autoimmune pathogenesis in genetically susceptible individuals has been postulated.¹ First-line treatment for LS consists of 4 to 6 weeks of daily ultra-potent topical steroids, such as clobetasol ointment.⁵ The ointment vehicle is preferred over the cream vehicle, especially on the vulva, because it is less likely to cause irritation.⁶ Frequency of application is decreased as the eruption improves. Patients should be monitored for steroid atrophy of surrounding skin, but this generally does not occur within the LS lesions.⁴ Topical tacrolimus has also been efficacious for patients with genital LS.⁷ Refractory extragenital LS has responded to narrowband UVB light therapy in several case reports, although these data have not been confirmed with large-scale, randomized, controlled clinical trials.²

References:

1. Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14(1):27-47. 

2. Colbert RL, Chiang MP, Carlin CS, Fleming M. Progressive extragenital lichen sclerosus successfully treated with narrowband UV-B phototherapy. Arch Dermatol. 2007;143(1):19-20. 

3. Margesson LJ. Practice gaps “down there” failures in education, physical examination, recognition, diagnosis, therapy, follow-up care, and cancer surveillance in lichen sclerosus. JAMA Dermatol. 2013;149(10):1203. 

4. Brodrick B, Belkin ZR, Goldstein AT. Influence of treatments on prognosis for vulvar lichen sclerosus: Facts and controversies. Clin Dermatol. 2013;31(6):
780-786.

5. Zendell K, Edwards L. Lichen sclerosus with vaginal involvement: report of 2 cases and review of the literature. JAMA Dermatol. 2013;149(10):1199-1202. 

6. Ference JD, Last AR. Choosing topical corticosteroids. Am Fam Physician. 2009;79(2):135-140.

7. Kim GW, Park HJ, Kim HS, et al. Topical tacrolimus ointment for the treatment of lichen sclerosus, comparing genital and extragenital involvement. J Dermatol. 2012;39(2):145-150.