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DOI: 10.1055/a-1887-5399
Sklerodermie – „die harte Haut“
Scleroderma: “the hard skin”Zusammenfassung
Unter dem Begriff Sklerodermie ist eine heterogene Gruppe von Erkrankungen zusammengefasst, die eine kutane Sklerose gemeinsam haben. Die einzelnen Erkrankungen variieren in der Beteiligung betroffener Strukturen wie Haut, Fettgewebe, Muskulatur, Gelenkstrukturen, Knochen, innerer Organe und der damit einhergehenden Krankheitsausprägung. Es lassen sich grundsätzlich zwei verschiedene Subtypen unterscheiden: die lokalisierte Sklerodermie (LoS) und die systemische Sklerose (SSc), die sich jeweils in weitere Subgruppen unterteilen lassen. Es handelt sich um zwei verschiedene Erkrankungen, die sich in der Ausprägung der Hautsklerosierung, der Beteiligung innerer Organe, im Antikörper-Profil, im Krankheitsmanagement sowie der Prognose unterscheiden. Darüber hinaus gibt es Krankheitsbilder, die eine Sklerodermie imitieren und daher als sklerodermie-artige Erkrankungen oder auch als Pseudosklerodermien bezeichnet werden. Um die richtige Diagnose stellen zu können, ist ein standardisiertes diagnostisches Vorgehen bedeutsam. Die frühe Diagnosestellung ist wichtig, um frühzeitige eine Therapie einleiten zu können, und so funktionelle kutane und extrakutane Einschränkungen und auch kosmetische Schäden gering halten zu können.
Abstract
The term scleroderma covers a heterogeneous group of diseases sharing the characteristic feature of cutaneous sclerosis. The individual diseases vary in the involvement of affected structures such as skin, adipose tissue, muscles, joint structures, bones, internal organs and the associated disease expression. Basically, two different subtypes can be distinguished: localised scleroderma (LoS) and systemic sclerosis (SSc), each of which are subdivided into further subgroups. These are two distinct diseases that differ in the extent of skin sclerosis, involvement of internal organs, antibody profile, disease management and prognosis. In addition, there are clinical pictures that mimic scleroderma and are therefore referred to as scleroderma-like diseases or pseudoscleroderma. In order to make the correct diagnosis, a standardised diagnostic procedure is significant. Early diagnosis is important in order to initiate early treatment and thus minimise functional cutaneous and extracutaneous impairment as well as cosmetic damage.
Schlüsselwörter
Sklerodermie - lokalisierte Sklerodermie - zirkumskripte Sklerodermie - Morphea - systemische SkleroseKey words
scleroderma - systemic sclerosis - circumscribed scleroderma - morphea - localised sclerodermaPublication History
Article published online:
11 August 2022
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Literatur
- 1 Ferreli C, Gasparini G, Parodi A. et al. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review. Clin Rev Allergy Immunol 2017; 53: 306-336 DOI: 10.1007/s12016-017-8625-4.
- 2 Hawk A, English JC. Localized and systemic scleroderma. Semin Cutan Med Surg 2001; 20: 27-37 DOI: 10.1053/sder.2001.23093.
- 3 Knobler R, Moinzadeh P, Hunzelmann N. et al. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol 2017; 31: 1401-1424 DOI: 10.1111/jdv.14458.
- 4 Giuggioli D, Colaci M, Cocchiara E. et al. Erratum to “From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution”. Dermatol Res Pract 2018; 2018: 6984282 DOI: 10.1155/2018/6984282.
- 5 Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis. J Am Acad Dermatol 2011; 64: 217-228 quiz 229-30 DOI: 10.1016/j.jaad.2010.05.045.
- 6 Hunzelmann N, Genth E, Krieg T. et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology (Oxford) 2008; 47: 1185-1192 DOI: 10.1093/rheumatology/ken179.
- 7 Plewig G, Ruzicka T, Kaufmann R. et al. Braun-Falco’s Dermatologie, Venerologie und Allergologie. Berlin, Heidelberg: Springer Berlin Heidelberg; 2018
- 8 Moinzadeh P, Kreuter A, Krieg T. et al. Morphea or localized scleroderma and extragenital lichen sclerosus. Hautarzt 2018; 69: 892-900 DOI: 10.1007/s00105-018-4266-7.
- 9 Kreuter A, Krieg T, Worm M. et al. AWMF Guideline no. 013/066. Diagnosis and therapy of circumscribed scleroderma. J Dtsch Dermatol Ges 2009; 7: S1-S14 DOI: 10.1111/j.1610-0387.2009.07178.x.
- 10 Kreuter A, Krieg T, Worm M. et al. German guidelines for the diagnosis and therapy of localized scleroderma. J Dtsch Dermatol Ges 2016; 14: 199-216 DOI: 10.1111/ddg.12724.
- 11 Christen-Zaech S, Hakim MD, Afsar FS. et al. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol 2008; 59: 385-396 DOI: 10.1016/j.jaad.2008.05.005.
- 12 Avancini J, Valente NYS, Romiti R. Generalized lenticular atrophoderma of Pasini and Pierini. Pediatr Dermatol 2015; 32: 389-391 DOI: 10.1111/pde.12434.
- 13 Zulian F, Athreya BH, Laxer R. et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology (Oxford) 2006; 45: 614-620 DOI: 10.1093/rheumatology/kei251.
- 14 Leitenberger JJ, Cayce RL, Haley RW. et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol 2009; 145: 545-550 DOI: 10.1001/archdermatol.2009.79.
- 15 Marzano AV, Menni S, Parodi A. et al. Localized scleroderma in adults and children. Clinical and laboratory investigations on 239 cases. Eur J Dermatol 2003; 13: 171-176
- 16 Herrick AL, Ennis H, Bhushan M. et al. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken) 2010; 62: 213-218 DOI: 10.1002/acr.20070.
- 17 Chiang K-L, Chang K-P, Wong T-T. et al. Linear scleroderma “en coup de sabre”: initial presentation as intractable partial seizures in a child. Pediatr Neonatol 2009; 50: 294-298 DOI: 10.1016/S1875-9572(09)60081-4.
- 18 Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 2007; 56: 257-263 DOI: 10.1016/j.jaad.2006.10.959.
- 19 Orozco-Covarrubias L, Guzmán-Meza A, Ridaura-Sanz C. et al. Scleroderma ‘en coup de sabre’ and progressive facial hemiatrophy. Is it possible to differentiate them?. J Eur Acad Dermatol Venereol 2002; 16: 361-366 DOI: 10.1046/j.1468-3083.2002.00442.x.
- 20 Jappe U, Hölzle E, Ring J. Parry-Romberg syndrome. Summary and new knowledge based on an unusual case. Hautarzt. 1996; 47: 599-603 DOI: 10.1007/s001050050475.
- 21 Lehman TJ. The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma en coup de sabre. Mistaken diagnosis or overlapping conditions?. J Rheumatol 1992; 19: 844-845
- 22 Bielsa I, Ariza A. Deep morphea. Semin Cutan Med Surg 2007; 26: 90-95 DOI: 10.1016/j.sder.2007.02.005.
- 23 Blaszczyk M, Krysicka-Janiger K, Jabłońska S. Primary atrophic profound linear scleroderma. Report of three cases. Dermatology 2000; 200: 63-66 DOI: 10.1159/000018321.
- 24 Malandrini A, Dotti MT, Federico A. Selective ipsilateral neuromuscular involvement in a case of facial and somatic hemiatrophy. Muscle Nerve 1997; 20: 890-892 DOI: 10.1002/(sici)1097-4598(199707)20:7<890:aid-mus16>3.0.co;2-w.
- 25 Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease. Best Pract Res Clin Rheumatol 2012; 26: 449-458 DOI: 10.1016/j.berh.2012.08.001.
- 26 Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol 2006; 18: 606-613 DOI: 10.1097/01.bor.0000245727.40630.c3.
- 27 Weibel L, Harper JI. Linear morphoea follows Blaschko’s lines. Br J Dermatol 2008; 159: 175-181 DOI: 10.1111/j.1365-2133.2008.08647.x.
- 28 Arkachaisri T, Vilaiyuk S, Li S. et al. The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures. J Rheumatol 2009; 36: 2819-2829 DOI: 10.3899/jrheum.081284.
- 29 Arkachaisri T, Vilaiyuk S, Torok KS. et al. Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof-of-concept study. Rheumatology (Oxford) 2010; 49: 373-381 DOI: 10.1093/rheumatology/kep361.
- 30 Krieg T, Takehara K.. Skin disease: a cardinal feature of systemic sclerosis. Rheumatology (Oxford) 2009; 48iii14-8 DOI: 10.1093/rheumatology/kep108.
- 31 Sato S, Fujimoto M, Kikuchi K. et al. Soluble CD4 and CD8 in serum from patients with localized scleroderma. Arch Dermatol Res 1996; 288: 358-362 DOI: 10.1007/BF02507103.
- 32 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 DOI: 10.1016/j.jaad.2005.11.1063.
- 33 Marrani E, Foeldvari I, Lopez JA. et al. Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach. Semin Arthritis Rheum 2018; 48: 495-503 DOI: 10.1016/j.semarthrit.2018.03.003.
- 34 Weibel L, Sampaio MC, Visentin MT. et al. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Br J Dermatol 2006; 155: 1013-1020 DOI: 10.1111/j.1365-2133.2006.07497.x.
- 35 Fett N, Werth VP. Update on morphea: part II. Outcome measures and treatment. J Am Acad Dermatol 2011; 64: 231-242 quiz 243-4 DOI: 10.1016/j.jaad.2010.05.046.
- 36 Fett NM. Morphea: evidence-based recommendations for treatment. Indian J Dermatol Venereol Leprol 2012; 78: 135-141 DOI: 10.4103/0378-6323.93628.
- 37 van den Hoogen F, Khanna D, Fransen J. et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72: 1747-1755 DOI: 10.1136/annrheumdis-2013-204424.
- 38 LeRoy EC, Black C, Fleischmajer R. et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15: 202-205
- 39 Bellando-Randone S, Guiducci S, Matucci-Cerinic M. Very early diagnosis of systemic sclerosis. Pol Arch Med Wewn 2012; 122: 18-23
- 40 Sticherling M. Systemic sclerosis-dermatological aspects. Part 1: Pathogenesis, epidemiology, clinical findings. J Dtsch Dermatol Ges 2012; 10: 705-718 quiz 716 DOI: 10.1111/j.1610-0387.2012.07999.x.
- 41 Pearson DR, Werth VP, Pappas-Taffer L. Systemic sclerosis: Current concepts of skin and systemic manifestations. Clin Dermatol 2018; 36: 459-474 DOI: 10.1016/j.clindermatol.2018.04.004.
- 42 Alivernini S, De Santis M, Tolusso B. et al. Skin ulcers in systemic sclerosis: determinants of presence and predictive factors of healing. J Am Acad Dermatol 2009; 60: 426-435 DOI: 10.1016/j.jaad.2008.11.025.
- 43 Nihtyanova SI, Brough GM, Black CM. et al. Clinical burden of digital vasculopathy in limited and diffuse cutaneous systemic sclerosis. Ann Rheum Dis 2008; 67: 120-123 DOI: 10.1136/ard.2007.072686.
- 44 Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol 2012; 8: 469-479 DOI: 10.1038/nrrheum.2012.96.
- 45 Pakozdi A, Nihtyanova S, Moinzadeh P. et al. Clinical and serological hallmarks of systemic sclerosis overlap syndromes. J Rheumatol 2011; 38: 2406-2409 DOI: 10.3899/jrheum.101248.
- 46 Kucharz EJ, Kopeć-Mędrek M. Systemic sclerosis sine scleroderma. Adv Clin Exp Med 2017; 26: 875-880 DOI: 10.17219/acem/64334.
- 47 Smith V, Herrick AL, Ingegnoli F. et al. Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud’s phenomenon and systemic sclerosis. Autoimmunity Reviews 2020; 19: 102458 DOI: 10.1016/j.autrev.2020.102458.
- 48 Mierau R, Moinzadeh P, Riemekasten G. et al. Frequency of disease-associated and other nuclear autoantibodies in patients of the German Network for Systemic Scleroderma: correlation with characteristic clinical features. Arthritis Res Ther 2011; 13: R172 DOI: 10.1186/ar3495.
- 49 Trang G, Steele R, Baron M. et al. Corticosteroids and the risk of scleroderma renal crisis: a systematic review. Rheumatol Int 2012; 32: 645-653 DOI: 10.1007/s00296-010-1697-6.
- 50 Ebata S, Yoshizaki A, Oba K. et al. Safety and efficacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-initiated, randomised, placebo-controlled trial. The Lancet Rheumatology 2021; 3: e489-e497 DOI: 10.1016/S2665-9913(21)00107-7.
- 51 Tashkin DP, Roth MD, Clements PJ. et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 2016; 4: 708-719 DOI: 10.1016/S2213-2600(16)30152-7.
- 52 Pope JE, Bellamy N, Seibold JR. et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 2001; 44: 1351-1358 DOI: 10.1002/1529-0131(200106)44:6<1351:AID-ART227>3.0.CO;2-I.
- 53 van den Hoogen FH, Boerbooms AM, Swaak AJ. et al. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 1996; 35: 364-372 DOI: 10.1093/rheumatology/35.4.364.
- 54 Kowal-Bielecka O, Fransen J, Avouac J. et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017; 76: 1327-1339 DOI: 10.1136/annrheumdis-2016-209909.
- 55 Denton CP, Hughes M, Gak N. et al. BSR and BHPR guideline for the treatment of systemic sclerosis. Rheumatology (Oxford) 2016; 55: 1906-1910 DOI: 10.1093/rheumatology/kew224.
- 56 Fabri M, Hunzelmann N. Differentialdiagnose der Sklerodermien und Pseudosklerodermien. J Dtsch Dermatol Ges 2007; 5 DOI: 10.1111/j.1610-0387.2007.06311_supp.x.
- 57 Distler O, MD., B. Highland K., et al. Nintedanib for systemic sclerosis – associated interstitial lung disease. N Engl J Med 2019; 380: 2518-2528 DOI: 10.1056/NEJMoa1903076.
- 58 Khanna, DineshLucero, Eleonora et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. The Lancet Respiratory Medicine 8: 963-974 DOI: 10.1016/S2213-2600(20)30318-0.