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A 76-year-old woman with hypertension and hepatitis C presented with a 10-year history of sclerotic, atrophic, ivory-white plaques with violaceous borders on the upper part of her trunk and on her arms, waistline, and anogenital areas (Figure 1). The plaques were gradually extending and becoming more pruritic. A biopsy revealed findings diagnostic of lichen sclerosus (LS), including an atrophic epidermis, homogenized upper dermis, and sparse chronic inflammation in the middle dermis, below the homogenized collagen.
Figure 1. Before narrowband UV-B therapy. White, slightly indurated, atrophic plaque with thin scale and erythema at the periphery.
The patient experienced a modest response to topical tacrolimus therapy, followed by more substantial improvement with topical clobetasol propionate therapy. However, several months later, she returned with worsening pruritus and enlargement of the plaques on her neck, waist, and arms. The anogenital lesions were stable. A trial of calcipotriene ointment applied to the midchest region did not produce any appreciable benefit.
Lichen sclerosus is a chronic inflammatory disease that presents as white, atrophic plaques characteristically involving the anogenital area of prepubertal and postmenopausal women. The cause of LS is unknown, but most studies suggest that it is multifactorial. Recently, immunoreactivity to extracellular matrix protein 1 has been demonstrated in up to 74% of cases.1 Extragenital lesions occur in 15% to 20% of patients.2 They may be localized or widespread and typically affect the neck, inframammary area, shoulders, wrists, and inner part of the thighs. In contrast to genital LS, which is accompanied by itching, burning, and dysuria, extragenital LS is typically asymptomatic.3 However, progressive disease may cause discomfort and pruritus.
There is no known cure for LS, but therapy is often initiated with the hope of relieving symptoms and preventing disease progression. Therapeutic success is variable. Standard treatments include topical corticosteroids and calcineurin inhibitors, such as tacrolimus.4
In 2002, Kreuter et al5 described 10 patients who were successfully treated for extragenital LS with UV-A1 phototherapy. Since a UV-A1 source was not available to us, we initiated thrice-weekly therapy with narrowband UV-B (NBUV-B). This regimen resulted in almost complete resolution of pruritus after only 3 phototherapy sessions. Therefore, the patient discontinued all topical therapy. The plaques stopped expanding, and after 1 month they were less discolored and significantly less indurated. Within 3 months, the abdominal plaques had nearly cleared (Figure 2), and the other affected areas continued to improve, with loss of active violaceous changes at their periphery. The frequency of phototherapy was decreased to twice weekly and then discontinued at the request of the patient. Three months after discontinuation of phototherapy, the patient had no relapse of pruritus or enlargement of existing sclerotic areas. She reported that she was very pleased with the therapy and had not reinitiated any other treatments for this condition.
Figure 2. After 3 months of narrowband UV-B therapy. Near-resolution of the lichen sclerosus on the abdomen.
We describe a patient with progressive, severely symptomatic, extragenital LS that did not respond to multiple topical therapies but responded dramatically to NBUV-B treatment. Therapy with NBUV-B has been used successfully to treat several chronic inflammatory skin disorders, such as psoriasis and atopic dermatitis. It has also been reported to reduce the pruritus associated with various cutaneous and systemic conditions.6 Although its mechanism of action is not completely understood, it appears that NVUV-B has both anti-inflammatory and immunosuppressive effects.7
While several reports and small case series have demonstrated the effectiveness of UV-A1 therapy (340-400 nm) for extragenital LS,5,8,9 we could find no published studies in which NBUV-B (311-313 nm) was successfully or unsuccessfully used to treat this condition. Several studies have demonstrated that both UV-A1 and NBUV-B increase matrix-metalloproteinase levels in human skin and cultured dermal fibroblasts,9- 14 which may explain the effectiveness of UV-A1 in sclerosing skin diseases. The action of NBUV-B against many of these diseases may be limited by its reduced depth of penetration, relative to UV-A1. This limitation may not apply to LS, which affects only the epidermis and the superficial dermis. Indeed, in a recent comparative study by Kreuter et al,15 medium-dose UV-A1 therapy was shown to be statistically more effective than NBUV-B therapy for morphea. Our patient's response suggests that NBUV-B therapy can be beneficial in treating LS, producing not just symptomatic relief but modification of the disease course as well. The increased availability and favorable cost of NBUV-B in the United States compared with UV-A1, as well as the low adverse effect profile of this modality, make it an attractive alternative to other more conventional treatments of extragenital LS.
Clinicians, residents, and fellows are invited to submit cases of challenges in management and therapeutics to this section. Cases should follow the established pattern. Manuscripts should be prepared double-spaced with right margins nonjustified. Pages should be numbered consecutively with the title page separated from the text (see Instructions for Authors for information about preparation of the title page). Clinical photographs, photomicrographs, and illustrations must be sharply focused and submitted as separate JPG files with each file numbered with the figure number. Material must be accompanied by the required copyright transfer statement (see authorship form [http://archderm.ama-assn.org/misc/auinst_crit.pdf]). Preliminary inquiries regarding submissions for this feature may be submitted to George J. Hruza, MD (email@example.com). Manuscripts should be submitted via our online manuscript submission and review system (http://manuscripts.archdermatol.com).
Correspondence: Christopher S. Carlin, MD, Department of Dermatology, Medical College of Wisconsin, 9200 W Wisconsin Ave, Milwaukee, WI 53226 (firstname.lastname@example.org).
Financial Disclosure: None reported.
Author Contributions: All authors contributed substantially to the production of the manuscript for this article and take responsibility for the accuracy of the data. Study concept and design: Colbert, Chiang, and Carlin. Acquisition of data: Colbert, Chiang, Carlin, and Fleming. Analysis and interpretation of data: Colbert, Chiang, and Carlin. Drafting of the manuscript: Colbert, Chiang, and Carlin. Critical revision of the manuscript for important intellectual content: Colbert, Carlin, and Fleming. Statistical analysis: Colbert and Chiang. Administrative, technical, and material support: Colbert and Carlin. Study supervision: Fleming. Photographs: Carlin.
Accepted for Publication: May 4, 2006.
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. doi:10.1001/archderm.143.1.19