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Lupus Erythematosus

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Braun-Falco´s Dermatology

Abstract

Lupus erythematosus (LE) is an inflammatory autoimmune disease characterized by high clinical heterogeneity and a variable course. In systemic lupus erythematosus (SLE), there is the possibility of the development of life-threatening involvement of multiple organs that can affect the skin, musculosceletal system, kidneys, and cardiovascular and central nervous systems. The disease can also be limited to the skin, and is then called cutaneous lupus erythematosus (CLE). Together with an (immune) genetic background, various environmental factors, such as smoking, medication, hormones, infections, and above all ultraviolet (UV) radiation can lead to an initial manifestation or exacerbation of the disease. New findings on the pathogenesis of CLE have also led to further therapeutic options for this autoimmune disease.

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References

  • Albrecht J, Taylor L, Berlin JA et al (2005) The CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index): an outcome instrument for cutaneous lupus erythematosus. J Invest Dermatol 125:889–894

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Aringer M, Costenbader K, Daikh D et al. (2019) 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol 71:1400–1412. https://doi.org/10.1002/art.40930

  • Baima B, Sticherling M (2001) Apoptosis in different cutaneous manifestations of lupus erythematosus. Br J Dermatol 144:958–966

    Article  CAS  PubMed  Google Scholar 

  • Biazar C, Sigges J, Patsinakidis N et al (2013) Cutaneous lupus erythematosus: first multicenter database analysis of 1002 patients from the European Society of Cutaneous Lupus Erythematosus (EUSCLE). Autoimmune Rev 12:444–454

    Article  Google Scholar 

  • Cancro MP, D’Cruz DP, Khamashta MA (2009) The role of B lymphocyte stimulator (BLyS) in systemic lupus erythematosus. J Clin Invest 19:1066–1073

    Article  CAS  Google Scholar 

  • Costner MI, Sontheimer RD, Provost TT (2003) Lupus erythematosus. In: Sontheimer RD, Provost TT (eds) Cutaneous manifestations of rheumatic diseases. Williams & Wilkins, Philadelphia, pp 15–64

    Google Scholar 

  • Crowson AN, Magro C (2001) The cutaneous pathology of lupus erythematosus: a review. J Cutan Pathol 28:1–23

    Article  CAS  PubMed  Google Scholar 

  • Dalle Vedove C, Simon JC, Girolomoni G (2012) Medikamenteninduzierter Lupus erythematodes unter besonderer Berücksichtigung von Hautmanifestationen und Anti-TNFα-Therapeutika. J Dtsch Dermatol Ges 10:889–897

    PubMed  PubMed Central  Google Scholar 

  • Fairley JL, Oon S, Saracino AM et al. (2020) Management of cutaneous manifestations of lupus erythematosus: A systematic review. Semin Arthritis Rheum 50:95–127. https://doi.org/10.1016/j.semarthrit.2019.07.010

  • Fischer-Betz R (2012) Rheumatische Erkrankungen in der Schwangerschaft. Internist (Berl) 53:1047–1053

    Article  CAS  Google Scholar 

  • Franceschini F, Cavazzana I (2005) Anti-Ro/SSA and La/SSB antibodies. Autoimmunity 38:55–63

    Article  CAS  PubMed  Google Scholar 

  • Furie R, Petri M, Zamani O et al (2011) A phase III, randomized, placebo-controlled study of belimumab, a monoclonal antibody that inhibits B lymphocyte stimulator, in patients with systemic lupus erythematosus. Arthritis Rheum 63:3918–3930

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gilliam JN, Sontheimer RD (1981) Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol 4:471–475

    Article  CAS  PubMed  Google Scholar 

  • Jessop S, Whitelaw D, Jordaan F (2009) Drugs for discoid lupus erythematosus. Cochrane Database Syst Rev 4:CD002954

    Google Scholar 

  • Kuhn A, Amler S, Beissert S et al (2010a) Revised Cutaneous Lupus Erythematosus Disease Area and Severity Index (RCLASI): a modified outcome instrument for cutaneous lupus erythematosus. Br J Dermatol 163:83–92

    Article  CAS  PubMed  Google Scholar 

  • Kuhn A, Ruland V, Bonsmann G (2010b) Photosensitivity, phototesting and photoprotection in cutaneous lupus erythematosus. Lupus 19:1036–1046

    Article  CAS  PubMed  Google Scholar 

  • Kuhn A, Ruland V, Bonsmann G (2011) Hautmanifestationen des Lupus erythematodes. Z Rheumatol 70:213–227

    Article  CAS  PubMed  Google Scholar 

  • Kuhn A, Ruland V, Bonsmann G (2012) Cutaneous lupus erythematosus: update of therapeutic options part I, II. J Am Acad Dermatol 65:e179–e193, e195–e213

    Article  Google Scholar 

  • Kuhn A, Bonsmann G, Anders HJ et al (2015) Diagnose und Therapie des systemischen Lupus erythematodes. Dtsch Arztebl 112:423–432

    Google Scholar 

  • Kuhn A, Landmann A, Bonsmann G (2016a) The skin in autoimmune diseases: unmet needs. Autoimmune Rev 15:948–954

    Article  CAS  Google Scholar 

  • Kuhn A, Landmann A, Patsinakidis N et al (2016b) Fumaric acid ester treatment in cutaneous lupus erythematosus (CLE): a prospective, open-label, phase II pilot study. Lupus 25:1357–1364

    Article  CAS  PubMed  Google Scholar 

  • Kuhn A, Wenzel J, Bijl M (2016c) Lupus erythematosus revisited. Semin Immunopathol 38:97–112

    Article  CAS  PubMed  Google Scholar 

  • Kuhn A, Aberer E, Bata-Csörgő Z et al. (2017) S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol 31:389–404. https://doi.org/10.1111/jdv.14053

    Google Scholar 

  • Lee-Kirsch MA, Gong M, Schulz H et al (2006) Familial chilblain lupus, a monogenic form of cutaneous lupus erythematosus, maps to chromosome 3p. Am J Hum Genet 79:731–737

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Manzi S, Sanchez-Guerrero J, Merrill JT et al (2012) Effects of belimumab, a B lymphocyte stimulator-specific inhibitor, on disease activity across multiple organ domains in patients with systemic lupus erythematosus: combined results from two phase III trials. Ann Rheum Dis 71:1833–1838

    Article  CAS  PubMed  Google Scholar 

  • Marmor MF, Kellner U, Lai TY et al (2016) Recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 revision). Ophthalmology 123:1386–1394

    Article  PubMed  Google Scholar 

  • Massone C, Kodama K, Salmhofer W et al (2005) Lupus erythemtaosus panniculitis (lupus profundus): clinical, histopathological and molecular analysis of nine cases. J Cutan Pathol 32:396–404

    Article  PubMed  Google Scholar 

  • Melles RB, Marmor MF (2014) The risk of toxic retinopathy in patients on long-term hydroxychloroquine therapy. JAMA Ophthalmol 132:1453–1460

    Article  PubMed  Google Scholar 

  • Merrill JT, Neuwelt CM, Wallace DJ et al (2010) Efficacy and safety of rituximab in moderately-to-severely active systemic lupus erythematosus: the randomized, double-blind, phase II/III systemic lupus erythematosus evaluation of rituximab trial. Arthritis Rheum 62:222–233

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Navarra SV, Guzman RM, Gallacher AE et al (2011) Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomized, placebo-controlled, phase 3 trial. Lancet 377:721–731

    Article  CAS  PubMed  Google Scholar 

  • Ochsendorf FR (2010) Einsatz von Antimalariamitteln in der Dermatologie. J Dtsch Dermatol Ges 8:829–846

    PubMed  PubMed Central  Google Scholar 

  • Petri M, Orbai AM, Alarcón GS et al (2012) Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 64:2677–2686

    Article  PubMed  PubMed Central  Google Scholar 

  • Petty AJ, Cardones AR, Marano AL (2020) Analysis of clinical characteristics of drug-induced cutaneous lupus erythematosus in men. J Am Acad Dermatol 7:S0190–9622(20)30366–2. https://doi.org/10.1016/j.jaad.2020.03.002

  • Renner R, Sticherling M (2008) Incidental cases of subacute cutaneous lupus erythematosus in association with malignancy. Eur J Dermatol 18:700–704

    PubMed  Google Scholar 

  • Renner R, Sticherling M (2009) The different faces of cutaneous lupus erythematosus. G Ital Dermatol Venereol 144:135–147

    CAS  PubMed  Google Scholar 

  • Risselada AP, Kallenberg CG (2006) Therapy-resistant lupus skin disease successfully treated with rituximab. Rheumatology 45:915–916

    Article  CAS  PubMed  Google Scholar 

  • Rovin BH, Furie R, Latinis K et al (2012) Efficacy and safety of rituximab in patients with active proliferative lupus nephritis: the lupus nephritis assessment with rituximab study. Arthritis Rheum 64:1215–1226

    Article  CAS  PubMed  Google Scholar 

  • Ruland V, Haust M, Stilling RM et al (2013) Updated analysis of standardized photoprovocation in patients with cutaneous lupus erythematosus. Arthritis Care Res (Hoboken) 65:767–776

    Article  CAS  Google Scholar 

  • Schmitt V, Meuth AM, Amler S et al (2010) Lupus erythematosus tumidus is a separate subtype of cutaneous lupus erythematosus. Br J Dermatol 162:64–73

    Article  CAS  PubMed  Google Scholar 

  • Shinada S, Wallace DJ (2004) Laboratory features of cutaneous lupus erythematosus. In: Kuhn A, Lehmann P, Ruzicka T (eds) Cutaneous lupus erythematosus. Springer, Berlin, pp S 301–S 322

    Google Scholar 

  • Smith CD, Cyr M (1988) The history of lupus erythematosus. From Hippocrates to Osler. Rheum Dis Clin N Am 14:1–14

    Article  CAS  Google Scholar 

  • Sontheimer RD, Henderson CL, Grey RH (2009) Drug-induced subacute cutaneous lupus erythematosus: a paradigm for bedside-to-bench patient-oriented translational clinical investigation. Arch Dermatol Res 301:65–70

    Article  PubMed  Google Scholar 

  • Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277

    Article  CAS  PubMed  Google Scholar 

  • Terrier B, Amoura Z, Ravaud P et al (2010) Safety and efficacy of rituximab in systemic lupus erythematosus: results from 136 patients from the French AutoImmunity and Rituximab registry. Arthritis Rheum 62:2458–2466

    Article  CAS  PubMed  Google Scholar 

  • Toberer F, Sykora J, Göttel D et al (2013) Apoptotic signal molecules in skin biopsies of cutaneous lupus erythematosus: analysis using tissue microarray. Exp Dermatol 22:656–659

    Article  PubMed  Google Scholar 

  • Uthman I, Taher A, Abbas O et al (2008) Successful treatment of refractory skin manifestations of systemic lupus erythematosus with rituximab: report of a case. Dermatology 216:257–259

    Article  PubMed  Google Scholar 

  • Worm M et al (2020) Diagnostik und Therapie des kutanen Lupus erythematodes. https://www.awmf.org/leitlinien/detail/anmeldung/1/ll/013-060.html

  • Xie F, Frewen J, Divekar P (2020) Three cases of subacute cutaneous lupus erythematosus associated with malignancy: a late paraneoplastic phenomenon. Clin Exp Dermatol 45:607–608. https://doi.org/10.1111/ced.14176

  • Yuki EFN, Silva CA, Aikawa NE et al. (2019) Thalidomide and Lenalidomide for Refractory Systemic/Cutaneous Lupus Erythematosus Treatment: A Narrative Review of Literature for Clinical Practice. J Clin Rheumatol 2019 Oct 29. https://doi.org/10.1097/RHU.0000000000001160

First Describer

  • Bechet PE (1942) Lupus erythematosus hypertrophicus et profundus. Arch Dermatol Syphilol 45:33–39

    Article  Google Scholar 

  • Brocq L (1921) Précis-atlas de pratigue dermatologique. Doin, Paris, pp 468–477

    Google Scholar 

  • Cazenave PLA (1851) Lupus érythemateaux (érythéme centrifuge). Ann Mal Peau Syph 3:297–299

    Google Scholar 

  • Cazenave A, Schedel HE (1833) Abrégé pratique des maladies de la peau, 2nd edn. Béchet, Paris

    Google Scholar 

  • Gougerot H, Burnier R (1930) Lupus érythémateux tumidus. Bull Soc Fr Dermatol Syphiligr 37:1291–1292

    Google Scholar 

  • Hebra F (1845) Versuch einer auf pathologische Anatomie gegründeten Einteilung der Hautkrankheiten. K. K. Ges Aerzte Wien 1:34–52, 141–155, 211–231

    Google Scholar 

  • Hoffmann E (1909) Isolierter Lupus erythematodes tumidus der Gesichtshaut. Derm Z 16:159–160

    Article  Google Scholar 

  • Hutchinson J (1888) Harveian lectures on lupus. The varieties of common lupus. Br Med J 1:113–118

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Irgang S (1940) Lupus erythematosus profundus: report of an example with clinical resemblance to Darier-Roussy sarcoid. Arch Dermatol Syphilol 42:97–108

    Article  Google Scholar 

  • Kaposi MH (1872) Neue Beiträge zur Kenntnis des Lupus erythematosus. Arch Dermatol Syphilol 4:36–78

    Article  Google Scholar 

  • Kaposi M (1883) Pathologie und Therapie der Hautkrankheiten in Vorlesungen für praktische Ärzte und Studierende, 2nd edn. Urban & Schwarzenberg, Wien, p 642

    Google Scholar 

  • McCustion CH, Schoch EP Jr (1954) Possible discoid lupus erythematosus in newborn infant; report of a case with subsequent development of acute systemic lupus erythematosus in mother. AMA Arch Derm Syphilol 70:782–785

    Article  Google Scholar 

  • O’Leary PA (1934) Disseminated lupus erythematosus. Minn Med 17:637–644

    Google Scholar 

  • Pedro SD, Dahl MV (1973) Direct immunofluorescence of bullous systemic lupus erythematosus. Arch Dermatol 107:118–120

    Article  CAS  PubMed  Google Scholar 

  • Rowell NR, Beck S, Anderson JR (1963) Lupus erythematosus and erythema multiforme-like lesions. Arch Dermatol 88:176–180

    Article  CAS  PubMed  Google Scholar 

  • Scholtz M (1922) Lupus erythematosus acutus disseminatus hemorrhagicus. Arch Dermatol Syph 6:466475

    Google Scholar 

  • Sontheimer RD, Thomas JR, Gilliam JN (1979) Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol 115:1409–1415

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. University Dermatological Clinic, University Hospital Erlangen, Erlangen, Germany

    Michael Sticherling

  2. University of Muenster, Muenster, Germany

    Annegret Kuhn

Authors
  1. Michael Sticherling
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  2. Annegret Kuhn
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Correspondence to Michael Sticherling .

Editor information

Editors and Affiliations

  1. Department of Dermatology, University of Munich, Munich, Germany

    Gerd Plewig

  2. Department of Dermatology, University of Munich, Munich, Germany

    Lars French

  3. Department of Dermatology, University of Munich, Munich, Germany

    Thomas Ruzicka

  4. Department of Dermatology, Venerology and Allergology, Frankfurt University Hospital, Frankfurt, Germany

    Roland Kaufmann

  5. Clinic for Dermatology and Allergology, University of Marburg, Marburg, Germany

    Michael Hertl

Section Editor information

  1. Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany

    Michael Hertl

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Sticherling, M., Kuhn, A. (2020). Lupus Erythematosus. In: Plewig, G., French, L., Ruzicka, T., Kaufmann, R., Hertl, M. (eds) Braun-Falco´s Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-58713-3_54-1

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  • DOI: https://doi.org/10.1007/978-3-662-58713-3_54-1

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  • Print ISBN: 978-3-662-58713-3

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