Download PDF
Aktuelle Rheumatologie 2015; 40(02): 132-138
DOI: 10.1055/s-0034-1395535
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Therapie der Hautbeteiligung bei der Sklerodermie – aktuelle Empfehlungen

Skin Therapy in Scleroderma – Current Recommendations
D. Belz
1   Klinik und Poliklinik für Dermatologie und Venerologie, Uniklinik Köln, Köln
,
P. Moinzadeh
1   Klinik und Poliklinik für Dermatologie und Venerologie, Uniklinik Köln, Köln
,
N. Hunzelmann
1   Klinik und Poliklinik für Dermatologie und Venerologie, Uniklinik Köln, Köln
› Author Affiliations
Further Information

Publication History

Publication Date:
07 January 2015 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Die systemische Sklerose (Systemische Sklerodermie, SSc) und die zirkumskripte Sklerodermie sind durch die charakteristische namensgebende Fibrosierung der Haut gekennzeichnet. Neben der Fibrose finden sich jedoch bei beiden Erkrankungen eine Reihe weiterer Symptome an der Haut wie z. B. digitale Ulzerationen und Teleangiektasien bei der SSc. Trotz Gemeinsamkeiten in der Pathophysiologie der Fibrose dieser Erkrankungen unterscheidet sich der therapeutische Ansatz erheblich. In der vorliegenden Arbeit sollen aktuelle Therapieempfehlungen der verschiedenen Formen der Hautbeteiligung beider Erkrankungen vorgestellt werden.

Abstract

Systemic sclerosis (SSc) and localised scleroderma (morphea) are characterised by the typical eponymous fibrosis of the skin. In addition to fibrosis, there are in both diseases a number of other symptoms of the skin such as digital ulceration and telangiectasia in patients with SSc. Despite the similarities in the pathophysiology of the fibrosis of these diseases, the therapeutic approach differs considerably. In the present work the current therapy recommendations of the skin involvement are presented.

Schlüsselwörter

systemische Sklerose - zirkumskripte Sklerodermie - Therapie der Hautbeteiligung - digitale Ulzerationen - Fibrose

Key words

systemic sclerosis - morphea - skin therapy - digital ulcer - fibrosis
 

  • Literatur

  • 1 Galluccio F, Walker UA, Nihtyanova S et al. Registries in systemic sclerosis: a worldwide experience. Rheumatology (Oxford) 2011; 50: 60-68
    CrossrefPubMedGoogle Scholar
  • 2 LeRoy EC, Black C, Fleischmajer R et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15: 202-205
    PubMedGoogle Scholar
  • 3 Hunzelmann N. Systemic scleroderma. Hautarzt 2013; 64: 299-310
    CrossrefPubMedGoogle Scholar
  • 4 Gambichler T, Terras S, Kreuter A. Treatment regimens, protocols, dosage, and indications for UVA1 phototherapy: facts and controversies. Clin Dermatol 2013; 31: 438-454
    CrossrefPubMedGoogle Scholar
  • 5 Hunzelmann N, Moinzadeh P, Genth E et al. German Network for Systemic Scleroderma Centers. High frequency of corticosteroid and immunosuppressive therapy in patients with systemic sclerosis despite limited evidence for efficacy. Arthritis Res Ther 2009; 11: R30
    CrossrefPubMedGoogle Scholar
  • 6 Kowal-Bielecka O, Landewé R, Avouac J et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009; 68: 620-628
    CrossrefPubMedGoogle Scholar
  • 7 Walker KM, Pope J participating members of the Scleroderma Clinical Trials Consortium (SCTC); Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails-a consensus of systemic sclerosis experts. Semin Arthritis Rheum 2012; 42: 42-55
    CrossrefPubMedGoogle Scholar
  • 8 Kowal-Bielecka O, Bielecki M, Kowal K. Recent advances in the diagnosis and treatment of systemic sclerosis. Pol Arch Med Wewn 2013; 123: 51-58
    PubMedGoogle Scholar
  • 9 Van Laar JM, Farge D, Sont JK et al. EBMT/EULAR Scleroderma Study Group . Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 2014; 311: 2490-2498
    CrossrefPubMedGoogle Scholar
  • 10 Sticherling M. Systemic sclerosis – focus on dermatological aspects. Part 2: diagnostics, therapy. J Dtsch Dermatol Ges 2012; 10: 783-791
    PubMedGoogle Scholar
  • 11 Poole JL. Musculoskeletal rehabilitation in the person with scleroderma. Curr Opin Rheumatol 2010; 22: 205-212
    CrossrefPubMedGoogle Scholar
  • 12 Maddali-Bongi S, Landi G, Galluccio F et al. The rehabilitation of facial involvement in systemic sclerosis: efficacy of the combination of connective tissue massage, Kabat’s technique and kinesitherapy: a randomized controlled trial. Rheumatol Int. 2011; 31: 895-901
    CrossrefPubMedGoogle Scholar
  • 13 Bali G, Aberer E. Iloprost therapy in systemic sclerosis. Hautarzt 2003; 54: 845-851
    CrossrefPubMedGoogle Scholar
  • 14 Iorio ML, Masden DL, Higgins JP. Botulinum toxin A treatment of Raynaud's phenomenon: a review. Semin Arthritis Rheum. 2012; 41: 599-603
    CrossrefPubMedGoogle Scholar
  • 15 Amanzi L, Braschi F, Fiori G et al. Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions. Rheumatology (Oxford) 2010; 49: 1374-1382
    CrossrefPubMedGoogle Scholar
  • 16 Seibold JR, Denton CP, Furst DE et al. Bosentan prevents occurrence but does not speed healing of digital ulcers in patients with systemic sclerosis (SSc). Arthritis Rheum 2005; 52 (suppl)
    PubMedGoogle Scholar
  • 17 Matucci-Cerinic M, Denton CP, Furst DE et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 2011; 70: 32-38
    CrossrefPubMedGoogle Scholar
  • 18 Tingey T, Shu J, Smuczek J et al. Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res (Hoboken) 2013; 65: 1460-1471
    CrossrefPubMedGoogle Scholar
  • 19 Herrick AL. Contemporary management of Raynaud’s phenomenon and digital ischaemic complications. Curr Opin Rheumatol 2011; 23: 555-561
    CrossrefPubMedGoogle Scholar
  • 20 Moore ZE, Cowman S. Wound cleansing for pressure ulcers. Cochrane Database Syst Rev 2013;
    PubMedGoogle Scholar
  • 21 Balin SJ, Wetter DA, Andersen LK et al. Calcinosis cutis occurring in association with autoimmune connective tissue disease: the mayo Clinic experience with 78 patients, 1996-2009. Arch Dermatol 2012; 148: 455-462
    CrossrefPubMedGoogle Scholar
  • 22 Wu JJ, Metz BJ. Calcinosis cutis of juvenile dermatomyositis treated with incision and drainage. Dermatol Surg 2008; 34: 575-577
    PubMedGoogle Scholar
  • 23 Minami A, Suda K, Kaneda K et al. Extensive subcutaneous calcification of the forearm in systemic lupus erythematosus. J hand Surg Br 1994; 19: 638-641
    CrossrefPubMedGoogle Scholar
  • 24 Saddic N, Miller JJ, Miller 3rd OF et al. Surgical debridement of painful fingertip calcinosis cutis in CREST syndrome. Arch Dermatol 2009; 145: 212-213
    PubMedGoogle Scholar
  • 25 Halachmi S, Gabari O, Cohen S et al. Telangiectasis in CREST syndrome and systemic sclerosis: correlation of clinical and pathological features with response to pulsed dye laser treatment. Lasers Med Sci 2014; 29: 137-140
    CrossrefPubMedGoogle Scholar
  • 26 Murray AK, Moore TL, Richards H et al. Pilot study of intense pulsed light for the treatment of systemic sclerosis-related telangiectases. Br J Dermatol 2012; 167: 563-569
    CrossrefPubMedGoogle Scholar
  • 27 Kreuter A, Hunzelmann N. S2k Leitlinie Diagnostik und Therapie der zirkumskripten Sklerodermie. AWMF-Register-Nummer (013-066) (noch nicht publiziert)
    PubMedGoogle Scholar
  • 28 Fett N. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies. Clin Dermatol 2013; 31: 432-437
    CrossrefPubMedGoogle Scholar
  • 29 Mancuso G, Berdondini RM. Localized scleroderma: response to occlusive treatment with tacrolimus ointment. Br J Dermatol 2005; 152: 180-182
    CrossrefPubMedGoogle Scholar
  • 30 Stefanaki C, Stefanaki K, Kontochristopoulos G et al. Topical tacrolimus 0,1% ointment in the treatment of localized scleroderma. An open label clinical and histological study. J Dermatol 2008; 35: 712-718
    CrossrefPubMedGoogle Scholar
  • 31 Kroft EB, Groeneveld TJ, Seyger MM et al. Efficacy of topical tacrolimus 0,1% in active plaque morphea: randomized, double-blind, emollient-controlled pilot study. Am J Clin Dermatol 2009; 10: 181-187
    CrossrefPubMedGoogle Scholar
  • 32 Cunningham BB, Landells ID, Langman C et al. Topical calcipotriene for morphea/linear scleroderma. J Am Acad Dermatol 1998; 39: 211-215
    CrossrefPubMedGoogle Scholar
  • 33 Kreuter A, Gambichler T, Avermaete A et al. Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. Pediatr Dermatol 2001; 18: 241-245
    CrossrefPubMedGoogle Scholar
  • 34 de Rie MA, Enomoto DN, de Vries HJ et al. Evaluation of medium-dose UVA1 phototherapy in localized scleroderma with the cutometer and fast transform method. Dermatology 2003; 207: 289-301
    PubMedGoogle Scholar
  • 35 Sator PG, Radakovic S, Schulmeister K et al. Medium-dose is more effective than low-dose ultraviolet A1 phototherapy for localized scleroderma as shown by 20-MHz ultrasound assessment. J Am Acad Dermatol 2009; 60: 786-791
    CrossrefPubMedGoogle Scholar
  • 36 Kreuter A, Hyun J, Stücker M et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol 2006; 54: 440-447
    CrossrefPubMedGoogle Scholar
  • 37 Zulian F, Martini G, Vallongo C et al. Methotrexate treatment in juvenile localized scleroderma: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum 2011; 63: 1998-2006
    CrossrefPubMedGoogle Scholar
  • 38 Kreuter A, Gambichler T, Breuckmann F et al. Pulsed high-dose corticosteroids combined with low-dose methotrexate in severe localized scleroderma. Arch Dermatol 2005; 141: 847-852
    CrossrefPubMedGoogle Scholar
  • 39 Uziel Y, Feldman BM, Krafchik BR et al. Methotrexate and corticosteroid therapy for pediatric localized scleroderma. J Pediatr 2000; 136: 91-95
    CrossrefPubMedGoogle Scholar
  • 40 Weibel L, Sampaio MC, Visentin MT et al. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphea) in children. Br J Dermatol 2006; 155: 1013-1020
    CrossrefPubMedGoogle Scholar
  • 41 Fitch PG, Rettig P, Burnham JM et al. Treatment of pediatric localized scleroderma with methotrexate. J Rheumatol 2006; 33: 609-614
    PubMedGoogle Scholar
  • 42 Cox D, O’Regan G, Collins S et al. Juvenile localized scleroderma: a retrospective review of response to systemic treatment. Ir J Med Sci 2008; 177: 343-346
    CrossrefPubMedGoogle Scholar
  • 43 Kroft EB, Creemers MC, van den Hoogen FH et al. Effectiveness, side-effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: an inception cohort study. Br J Dermatol 2009; 160: 1075-1082
    CrossrefPubMedGoogle Scholar
  • 44 Joly P, Bamberger N, Crickx B et al. Treatment of severe forms of localized scleroderma with oral corticosteroids: follow-up study on 17 patients. Arch Dermatol 1994; 130: 663-664
    CrossrefPubMedGoogle Scholar