Ghoreschi, K.

Rocken, M.

English

Universität München: Elektronischen Publikationen

Phototherapy of sclerosing skin diseases

Open Access LMU ( Ludwig-Maximilians-Univ. München)

EditorialDermatology 2002;205:219–220DOI: 10.1159/000065857Phototherapy of SclerosingSkin DiseasesKamran Ghoreschi Martin RöckenDepartment of Dermatology, Ludwig-Maximilians University of Munich, GermanyKamran GhoreschiDepartment of DermatologyLudwig Maximilian University, Munich (Germany)Tel. +49 89 5160 6205, Fax +49 89 5160 6206E-Mail Kami.Ghoreschi@lrz.uni-muenchen.deABCFax + 41 61 306 12 34E-Mail karger@karger.chwww.karger.com© 2002 S. Karger AG, Basel1018–8665/02/2053–0219$18.50/0Accessible online at:www.karger.com/journals/drmSclerosing skin diseases such as localized sclerodermaor lichen sclerosus frequently result in significant morbid-ity that requires treatment. Until 10 years ago, no general-ly effective treatment was available for these diseases. In1992, extracorporeal photopheresis was suggested astreatment for systemic sclerosis, but further studies arerequired to confirm this early report [1–3]. Two years lat-er, psoralen + UVA, in PUVA bath photochemotherapy,was proposed as effective and safe treatment for localizedscleroderma [4]. Since then, it has been shown that allthree modes of photochemotherapy, oral PUVA, creamPUVA and PUVA bath photochemotherapy can improvelocalized scleroderma, but the PUVA bath seems to be themost efficient mode [5–8]. In addition, phototherapy withUVA1 irradiation may resolve established sclerosis,whether applied as high-dose UVA1 (100–120 J/cm2) orat doses around 30 J/cm2 [8–11]. Due to the nature ofphototherapy, placebo-controlled studies cannot be per-formed. Meta-analysis of the various studies confirmsthat sclerosis resolves either partially or completely inabout 60% of patients receiving 36 treatments of PUVAbath therapy at slightly suberythematous doses or 36treatments of UVA1. The mechanisms underlying thetherapeutic principle remain enigmatic. Molecular analy-sis of resolving plaques shows a strong increase in theexpression of matrix metalloproteinase mRNA such ascollagenases and a reduction of procollagen [12–14]. Fur-thermore, phototherapy has a strong influence on thefunctioning of immune cells in the skin [15] suggestingthat the therapeutic effect relies on the modulation of col-lagen synthesis.Based on the success of phototherapy in the treatmentof localized scleroderma and eczema [7], several groupsanalyzed the role of either UVA1 or photochemotherapyin lichen sclerosus. The data of a manuscript reported onpages 245–248 as well as two case reports and one moreextended description show that some patients with lichensclerosus may improve when receiving either creamPUVA or UVA1 therapy [16–18]. However, the numberof patients reported until now is relatively small, and thetherapeutic modalities, including the mode of photothera-py and the number of treatments, vary widely. Together,the data suggest that phototherapy may be helpful in atleast some patients with lichen sclerosus. Similarly, wefound that 30 treatments of PUVA bath photochemother-apy or of UVA1 phototherapy may help in selectedpatients with extragenital lichen sclerosus, but the resultswere less convincing than in the treatment of localizedscleroderma.Importantly, while phototherapy is the only treatmenthelpful in scleroderma, the situation is different in lichensclerosus. More than 80% improve significantly whentreated with clobetasol propionate cream, and most pa-tients with persistent lesions of lichen sclerosus did re-ceive inadequate treatment with topical corticosteroids[19, 20]. Therefore, we consider PUVA bath photochemo-therapy or medium-dose UVA1 phototherapy as first-linetherapy of localized scleroderma and topical steroids as220 Dermatology 2002;205:219–220 Ghoreschi/Röckenfirst-line therapy of extragenital or genital lichen sclero-sus. The same rules apply to the treatment of children[21], especially as localized scleroderma may cause con-strictions [5]. The data reported here and earlier [16–18]suggest that selected patients with lichen sclerosus mayprofit from either cream PUVA or UVA1. Detailed stud-ies are required to characterize these patients more pre-cisely and to define the exact role of phototherapy in thetreatment of lichen sclerosus.References1 Rook AH, Freundlich B, Jegasothy BV, PerezMI, Barr WG, Jimenez SA, Rietschel RL, Win-troub B, Kahaleh MB, Varga J, et al: Treatmentof systemic sclerosis with extracorporeal photo-chemotherapy: Results of a multicenter trial.Arch Dermatol 1992;128:337–346.2 Cribier B, Faradji T, Le Coz C, Oberling F,Grosshans E: Extracorporeal photochemother-apy in systemic sclerosis and severe morphea.Dermatology 1995;191:25–31.3 Enomoto DN, Mekkes JR, Bossuyt PM, YongSL, Out TA, Hoekzema R, de Rie MA, Schel-lekens PT, ten Berge IJ, de Borgie CA, Bos JD:Treatment of patients with systemic sclerosiswith extracorporeal photochemotherapy (pho-topheresis). J Am Acad Dermatol 1999;41:915–922.4 Kerscher M, Volkenandt M, Meurer M, Leh-mann P, Plewig G, Röcken M: Treatment oflocalised scleroderma with PUVA bath photo-chemotherapy. Lancet 1994;343:1233.5 Scharffetter-Kochanek K, Goldermann R, Leh-mann P, Hölzle E, Goerz G: PUVA therapy indisabling pansclerotic morphoea of children.Br J Dermatol 1995;132:830–831.6 Kerscher M, Meurer M, Sander C, VolkenandtM, Lehmann P, Plewig G, Röcken M: PUVAbath photochemotherapy for localized sclero-derma: Evaluation of 17 consecutive patients.Arch Dermatol 1996;132:1280–1282.7 Lüftl M, Degitz K, Plewig G, Röcken M: Pso-ralen bath plus UVA therapy: Possibilities andlimitations. Arch Dermatol 1997;133:1597–1603.8 Grundmann-Kollmann M, Ochsendorf F,Zollner TM, Spieth K, Sachsenberg-Studer E,Kaufmann R, Podda M: PUVA-cream photo-chemotherapy for the treatment of localizedscleroderma. J Am Acad Dermatol 2000;43:675–678.9 Kerscher M, Dirschka T, Volkenandt M: Treat-ment of localised scleroderma by UVA1 photo-therapy. Lancet 1995;346:1166.10 Stege H, Berneburg M, Humke S, Klammer M,Grewe M, Grether-Beck S, Boedeker R, Diep-gen T, Dierks K, Goerz G, Ruzicka T, Krut-mann J: High-dose UVA1 radiation therapy forlocalized scleroderma. J Am Acad Dermatol1997;36:938–944.11 Kerscher M, Volkenandt M, Gruss C, ReutherT, von Kobyletzki G, Freitag M, Dirschka T,Altmeyer P: Low-dose UVA phototherapy fortreatment of localized scleroderma. J Am AcadDermatol 1998;38:21–26.12 Scharffetter K, Wlaschek M, Hogg A, Bolsen K,Schothorst A, Goerz G, Krieg T, Plewig G:UVA irradiation induces collagenase in humandermal fibroblasts in vitro and in vivo. ArchDermatol Res 1991;283:506–511.13 Gruss C, Reed JA, Altmeyer P, McNutt NS,Kerscher M: Induction of interstitial collage-nase (MMP-1) by UVA-1 phototherapy in mor-phea fibroblasts. Lancet 1997;350:1295–1296.14 Brinckmann J, Neess CM, Gaber Y, Sobhi H,Notbohm H, Hunzelmann N, Fietzek PP,Muller PK, Risteli J, Gebker R, Scharffetter-Kochanek K: Different pattern of collagencross-links in two sclerotic skin diseases: Li-podermatosclerosis and circumscribed sclero-derma. J Invest Dermatol 2001;117:269–273.15 Morita A, Werfel T, Stege H, Ahrens C, Kar-mann K, Grewe M, Grether-Beck S, Ruzicka T,Kapp A, Klotz LO, Sies H, Krutmann J: Evi-dence that singlet oxygen-induced human Thelper cell apoptosis is the basic mechanism ofultraviolet-A radiation phototherapy. J ExpMed 1997;186:1763–1768.16 von Kobyletzki G, Freitag M, Hoffmann K,Altmeyer P, Kerscher M: Balneophotochemo-therapy with 8-methoxypsoralen in lichen scle-rosis et atrophicus. Hautartz 1997;48:488–491.17 Kreuter A, Jansen T, Stucker M, Herde M,Hoffmann K, Altmeyer P, von Kobyletzki G:Low-dose ultraviolet-A1 phototherapy for li-chen sclerosus et atrophicus. Clin Exp Derma-tol 2001;26:30–32.18 Kreuter A, Gambichler T, Avermaete A,Happe M, Bacharach-Buhles M, Hoffmann K,Jansen T, Altmeyer P, von Kobyletzki G: Low-dose ultraviolet A1 phototherapy for extrageni-tal lichen sclerosus: Results of a preliminarystudy. J Am Acad Dermatol 2002;46:251–255.19 Dalziel KL, Millard PR, Wojnarowska F: Thetreatment of vulval lichen sclerosus with a verypotent topical steroid (clobetasol propionate0.05%) cream. Br J Dermatol 1991;124:461–464.20 Bornstein J, Heifetz S, Kellner Y, Stolar Z,Abramovici H: Clobetasol dipropionate 0.05%versus testosterone propionate 2% topical ap-plication for severe vulvar lichen sclerosus. AmJ Obstet Gynecol 1998;178:80–84.21 Garzon MC, Paller AS: Ultrapotent topical cor-ticosteroid treatment of childhood genital li-chen sclerosus. Arch Dermatol 1999;135:525–528.

Abramovici H: Clobetasol dipropionate 0.05% versus testosterone propionate 2% topical application for severe vulvar lichen sclerosus.

Altmeyer P, von Kobyletzki G: Low-dose ultraviolet-A1 phototherapy for lichen sclerosus et atrophicus. Clin Exp Dermatol

Altmeyer P: Low-dose UVA phototherapy for treatment of localized scleroderma.

Balneophotochemotherapy with 8-methoxypsoralen in lichen sclerosis et atrophicus. Hautartz

de Borgie CA, Bos JD: Treatment of patients with systemic sclerosis with extracorporeal photochemotherapy (photopheresis).

et al: Treatment of systemic sclerosis with extracorporeal photochemotherapy: Results of a multicenter trial. Arch Dermatol

Evidence that singlet oxygen-induced human T helper cell apoptosis is the basic mechanism of ultraviolet-A radiation phototherapy.

Grosshans E: Extracorporeal photochemotherapy in systemic sclerosis and severe morphea. Dermatology

High-dose UVA1 radiation therapy for localized scleroderma.

Hölzle E, Goerz G: PUVA therapy in disabling pansclerotic morphoea of children.

Induction of interstitial collagenase (MMP-1) by UVA-1 phototherapy in morphea fibroblasts.

Paller AS: Ultrapotent topical corticosteroid treatment of childhood genital lichen sclerosus. Arch Dermatol

Plewig G: UVA irradiation induces collagenase in human dermal fibroblasts in vitro and in vivo. Arch Dermatol Res

Podda M: PUVA-cream photochemotherapy for the treatment of localized scleroderma.

Psoralen bath plus UVA therapy: Possibilities and limitations. Arch Dermatol

PUVA bath photochemotherapy for localized scleroderma: Evaluation of 17 consecutive patients. Arch Dermatol

ScharffetterKochanek K: Different pattern of collagen cross-links in two sclerotic skin diseases: Lipodermatosclerosis and circumscribed scleroderma.

Treatment of localised scleroderma by UVA1 phototherapy.

Treatment of localised scleroderma with PUVA bath photochemotherapy. Lancet

von Kobyletzki G: Lowdose ultraviolet A1 phototherapy for extragenital lichen sclerosus: Results of a preliminary study.

Wojnarowska F: The treatment of vulval lichen sclerosus with a very potent topical steroid (clobetasol propionate

Kamran Ghoreschi

Martin Röcken

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