THE CUTTING EDGE

SECTION EDITOR: GEORGE J. HRUZA, MD; ASSISTANT SECTION EDITORS: MICHAEL P. HEFFERNAN, MD; SUMMER R. YOUKER, MD

Progressive Extragenital Lichen Sclerosus

Successfully Treated With Narrowband
UV-B Phototherapy
Rand L. Colbert, MD; Melissa P. Chiang, MD; Christopher S. Carlin, MD; Matthew Fleming, MD;
Medical College of Wisconsin, Milwaukee

The Cutting Edge: Challenges in Medical and Surgical Therapeutics

REPORT OF A CASE SOLUTION

A 76-year-old woman with hypertension and hepatitis
C presented with a 10-year history of sclerotic, atro-
phic, 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 gradu-
ally extending and becoming more pruritic. A biopsy re-
vealed findings diagnostic of lichen sclerosus (LS), in-
cluding an atrophic epidermis, homogenized upper
dermis, and sparse chronic inflammation in the middle
dermis, below the homogenized collagen.
The patient experienced a modest response to topi-
cal tacrolimus therapy, followed by more substantial im-
provement with topical clobetasol propionate therapy.
However, several months later, she returned with wors-
ening 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.
CLINICAL CHALLENGE
Lichen sclerosus is a chronic inflammatory disease that
presents as white, atrophic plaques characteristically in-
volving the anogenital area of prepubertal and postmeno-
pausal women. The cause of LS is unknown, but most
studies suggest that it is multifactorial. Recently, immu-
noreactivity to extracellular matrix protein 1 has been
demonstrated in up to 74% of cases.1 Extragenital le-
sions occur in 15% to 20% of patients.2 They may be lo-
calized or widespread and typically affect the neck, in-
framammary 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 typi-
cally 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 pre-
venting disease progression. Therapeutic success is vari-
able. Standard treatments include topical corticoste-
roids and calcineurin inhibitors, such as tacrolimus.4
In 2002, Kreuter et al5 described 10 patients who were suc-
cessfully treated for extragenital LS with UV-A1 photo-
therapy. 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 reso-
lution of pruritus after only 3 phototherapy sessions. There-
fore, 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 im-
prove, with loss of active violaceous changes at their pe-
riphery. The frequency of phototherapy was decreased to
twice weekly and then discontinued at the request of the
patient. Three months after discontinuation of photo-
therapy, the patient had no relapse of pruritus or enlarge-
ment of existing sclerotic areas. She reported that she was
very pleased with the therapy and had not reinitiated any
other treatments for this condition.
COMMENT
We describe a patient with progressive, severely symp-
tomatic, extragenital LS that did not respond to mul-
Figure 1. Before narrowband UV-B therapy. White, slightly indurated,
atrophic plaque with thin scale and erythema at the periphery.

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©2007 American Medical Association. All rights reserved.

Figure 2. After 3 months of narrowband UV-B therapy. Near-resolution of
the lichen sclerosus on the abdomen.
tiple 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 dem-
onstrated 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 success-
fully or unsuccessfully used to treat this condition. Sev-
eral studies have demonstrated that both UV-A1 and
NBUV-B increase matrix-metalloproteinase levels in hu-
man skin and cultured dermal fibroblasts,9-14 which may
explain the effectiveness of UV-A1 in sclerosing skin dis-
eases. The action of NBUV-B against many of these dis-
eases may be limited by its reduced depth of penetra-
tion, relative to UV-A1. This limitation may not apply
to LS, which affects only the epidermis and the superfi-
cial 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 mo-
dality, make it an attractive alternative to other more con-
ventional treatments of extragenital LS.
Accepted for Publication: May 4, 2006.
Correspondence: Christopher S. Carlin, MD, Depart-
ment of Dermatology, Medical College of Wisconsin, 9200
W Wisconsin Ave, Milwaukee, WI 53226 (ccarlin@mcw
.edu).
Author Contributions: All authors contributed substan-
tially 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. Acqui-
sition of data: Colbert, Chiang, Carlin, and Fleming. Analy-
sis and interpretation of data: Colbert, Chiang, and Car-
lin. Drafting of the manuscript: Colbert, Chiang, and Carlin.
(REPRINTED) ARCH DERMATOL/ VOL 143, JAN 2007
20
Downloaded from www.archdermatol.com at Ohio State University, on January 16, 2007
©2007 American Medical Association. All rights reserved.
Critical revision of the manuscript for important intellec-
tual content: Colbert, Carlin, and Fleming. Statistical analy-
sis: Colbert and Chiang. Administrative, technical, and ma-
terial support: Colbert and Carlin. Study supervision:
Fleming. Photographs: Carlin.
Financial Disclosure: None reported.
REFERENCES
1. Oyama N, Chan I, Neill SM, et al. Autoantibodies to extracellular matrix protein 1
in lichen sclerosus. Lancet. 2003;362:118-123.
2. Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet. 1999;353:1777-1783.
3. Funaro D. Lichen sclerosus: a review and practical approach. Dermatol Ther. 2004;
17:28-37.
4. Böhm M, Frieling U, Luger T, Bonsmann G. Successful treatment of anogenital
lichen sclerosus with topical tacrolimus. Arch Dermatol. 2003;139:922-924.
5. Kreuter A, Gambichler T, Avermaete A, et al. Low-dose ultraviolet A1 photo-
therapy for extragenital lichen sclerosus: results of a preliminary study. J Am
Acad Dermatol. 2002;46:251-255.
6. Samson Yashar S, Gielczyk R, Scherschun L, Lim H. Narrowband ultraviolet B
for vitiligo, pruritus, and inflammatory dermatoses. Photodermatol Photoimmu-
nol Photomed. 2003;19:164-168.
7. Sigmundsdottir H, Johnston A, Gudjonsson E, Valdimarsson H. Narrowband-
UVB decreases the production of proinflammatory cytokines by stimulated T cells.
Arch Dermatol Res. 2005;297:39-42.
8. Brenner M, Herzinger T, Berking C, Plewig G, Degitz K. Phototherapy and pho-
tochemotherapy of sclerosing skin diseases. Photodermatol Photoimmunol
Photomed. 2005;21:157-165.
9. Gruss C, Reed JA, Atlmeyer P, McNutt NS, Kerscher M. Induction of interstitial
collagenase (MMP-1) by UVA-1 phototherapy in morphea fibroblasts. Lancet.
1997;350:1295-1296.
10. Chung JH, Seo JY, Lee MK, et al. Ultraviolet modulation of human macrophage
metalloelastase in human skin in vivo. J Invest Dermatol. 2002;119:507-512.
11. Fisher GJ, Choi HC, Bata-Csorgo Z, et al. Ultraviolet irradiation increases matrix
metalloproteinase-8 protein in human skin in vivo. J Invest Dermatol. 2001;
117:219-226.
12. Brenneisen P, Oh J, Wlaschek M. Ultraviolet B wavelength dependence for the
regulation of two major matrix-metalloproteinases and their inhibitor TIMP-1 in
human dermal fibroblasts. Photochem Photobiol. 1996;64:877-885.
13. Wlaschek M, Heinen G, Poswig A, et al. UVA-induced autocrine stimulation of
fibroblast-derived collagenase/MMP-1 by interrelated loops of interleukin-1 and
interleukin-6. Photochem Photobiol. 1994;59:550-556.
14. Petersen MJ, Hansen C, Craig S. Ultraviolet A irradiation stimulates collagenase pro-
duction in cultured human fibroblasts. J Invest Dermatol. 1992;99:440-444.
15. Kreuter A, Hyun J, Stucker M, et al. A randomized controlled study of low-dose
UVA-1, medium-dose UVA-1, and narrowband UVB in the treatment of localized
scleroderma. J Am Acad Dermatol. 2006;54:440-447.
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