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10-04-2016 | Systemic lupus erythematosus | Book Chapter | Article

7. The Spectrum of Cutaneous Manifestations in Systemic Lupus Erythematosus and Novel Classification

Authors: Simone Ribero, Dan Lipsker, Luca Borradori

Publisher: Springer International Publishing

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Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune syndrome showing a broad and protean spectrum of clinical and immunological features. Cutaneous involvement is frequently found in SLE. Affected patients may show a variety of different “specific” and “nonspecific” cutaneous manifestations as either an initial leading sign of the disease or as complication in its course. Hence, knowledge of the typical and more unusual cutaneous features associated with SLE is important for the proper diagnosis and management of affected patient.
After articular involvement, the skin represents the second most frequently affected organ in SLE. In fact, approximately 80 % of patients will display skin manifestations during the course of the disease. This chapter will focus on cutaneous involvement in SLE, with special emphasis on classification, diagnostic, and treatment aspects.
Literature
1.
Obermoser G, Sontheimer RD, Zelger B (2010) Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates. Lupus 19(9):1050–1070CrossRefPubMed
2.
Rothfield N, Sontheimer RD, Bernstein M (2006) Lupus erythematosus: systemic and cutaneous manifestations. Clin Dermatol 24(5):348–362CrossRefPubMed
3.
Werth VP (2005) Clinical manifestations of cutaneous lupus erythematosus. Autoimmun Rev 4(5):296–302CrossRefPubMed
4.
Kuhn A, Ruland V, Bonsmann G (2011) Cutaneous lupus erythematosus: update of therapeutic options part I. J Am Acad Dermatol 65(6):179–193CrossRef
5.
Durosaro O, Davis MD, Reed KB, Rohlinger AL (2009) Incidence of cutaneous lupus erythematosus, 1965–2005: a population-based study. Arch Dermatol 145(3):249–253CrossRefPubMedPubMedCentral
6.
Gronhagen CM, Fored CM, Granath F, Nyberg F (2011) Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden. Br J Dermatol 164(6):1335–1341CrossRefPubMed
7.
Petri M, Orbai A-M, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG Jr, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G Jr, Magder LS et al (2012) Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 64(8):2677–2686CrossRefPubMedPubMedCentral
8.
Grönhagen CM, Gunnarsson I, Svenungsson E, Nyberg F (2010) Cutaneous manifestations and serological findings in 260 patients with systemic lupus erythematosus. Lupus 19(10):1187–1194CrossRefPubMed
9.
Fernández M, Alarcón GS, Calvo-Alén J, Andrade R, McGwin G Jr, Vilá LM, Reveille JD, LUMINA Study Group (2007) A multiethnic, multicenter cohort of patients with systemic lupus erythematosus (SLE) as a model for the study of ethnic disparities in SLE. Arthritis Rheum 57(4):576–584CrossRefPubMed
10.
Chiu Y-M, Lai C-H (2010) Nationwide population-based epidemiologic study of systemic lupus erythematosus in Taiwan. Lupus 19(10):1250–1255CrossRefPubMed
11.
Lee HJ, Sinha AA (2006) Cutaneous lupus erythematosus: understanding of clinical features, genetic basis, and pathobiology of disease guides therapeutic strategies. Autoimmunity 39(6):433–444CrossRefPubMed
12.
Freire EAM, Souto LM, Ciconelli RM (2011) Medidas de avaliação em lúpus eritematoso sistêmico (Assessment measures in systemic lupus erythematosus). Rev Bras Reumatol 51:75–80CrossRef
13.
Liu Z, Davidson A (2012) Taming lupus-a new understanding of pathogenesis is leading to clinical advances. Nat Med 18(6):871–882CrossRefPubMedPubMedCentral
14.
Deng y, Tsao BP (2010) Genetic susceptibility to systemic lupus erythematosus in the genomic era. Nat Rev Rheumatol 6(12):683–692CrossRefPubMedPubMedCentral
15.
Niewold TB, Hua J, Lehman TJ, Harley JB, Crow MK (2007) High serum IFN-alpha activity is a heritable risk factor for systemic lupus erythematosus. Genes Immun 8:492–502CrossRefPubMedPubMedCentral
16.
Jacob N, Stohl W (2011) Cytokine disturbances in systemic lupus erythematosus. Arthritis Res Ther 13:228CrossRefPubMedPubMedCentral
17.
Tüngler V, Silver RM, Walkenhorst H, Günther C, Lee-Kirsch MA (2012) Inherited or de novo mutation affecting aspartate 18 of TREX1 results in either familial chilblain lupus or Aicardi-Goutières syndrome. Br J Dermatol 167:212–214CrossRefPubMed
18.
Miyazaki T, Kim YS, Yoon J, Wang H, Suzuki T, Morse H (2014) The 3′-5′ DNA exonuclease TREX1 directly interacts with poly(ADP-ribose) polymerase-1 (PARP1) during the DNA damage response. J Biol Chem 289(47):32548–32558CrossRefPubMedPubMedCentral
19.
Kuhn A, Wozniacka A, Szepietowski J, Gläser R, Lehmann P, Haust M, Sysa-Jedrzejowska A, Reich A, Oke V, Hügel R, Calderon C, de Vries DE, Nyberg F et al (2011) Photoprovocation in cutaneous lupus erythematosus: a multicenter study evaluating a standardized protocol. J Invest Dermatol 131:1622–1630CrossRefPubMed
20.
Kind P, Lehmann P, Plewig G (1993) Phototesting in lupus erythematosus. J Invest Dermatol 100:53S–57SCrossRefPubMed
21.
Freutel S, Gaffal E, Zahn S, Bieber T, Tüting T, Wenzel J (2011) Enhanced CCR5+/CCR3+ T helper cell ratio in patients with active cutaneous lupus erythematosus. Lupus 20(12):1300–1304CrossRefPubMed
22.
Gilliam JN, Sontheimer RD (1981) Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol 4(4):471–475CrossRefPubMed
23.
Gilliam JN, Sontheimer RD (1982) Skin manifestations of SLE. Clin Rheum Dis 8(1):207–218PubMed
24.
Sontheimer RD (2009) Lichenoid tissue reaction/interface dermatitis: clinical and histological perspectives. J Invest Dermatol 129(5):1088–1099CrossRefPubMed
25.
Ackerman AB, Boer A, Benin B, Gottlieb GJ (2005) Histopathological diagnosis of inflammatory skin diseases, 3rd edn. Ardor Scribendi, New York
26.
Lipsker D (2010) The need to revisit the nosology of cutaneous lupus erythematosus: the current terminology and morphologic classification of cutaneous LE: difficult, incomplete and not always applicable. Lupus 19(9):1047–1049CrossRefPubMed
27.
Kuhn A, Lehmann P, Ruzicka T (2004) Clinical manifestations of cutaneous lupus erythematosus. In: Kuhn A, Lehmann P, Ruzicka T (eds) Cutaneous lupus erythematosus. Springer, Heidelberg, pp 59–92
28.
Boeckler P, Milea M, Meyer A, Uring-Lambert B, Heid E, Hauptmann G, Cribier B, Lipsker D (2005) The combination of complement deficiency and cigarette smoking as risk factor for cutaneous lupus erythematosus in men; a focus on combined C2/C4 deficiency. Br J Dermatol 152(2):265–270CrossRefPubMed
29.
Tebbe B, Mansmann U, Wollina U, Auer-Grumbach P, Licht-Mbalyohere A, Arsenmeier M, Orfanos CE (1997) Markers in cutaneous lupus erythematosus indicating systemic involvement. A multicenter study on 296 patients. Acta Derm Venereol 77:305–308PubMed
30.
Rémy-Leroux V, Léonard F, Lambert D, Wechsler J, Cribier B, Thomas P, Adamski H, Marguery MC, Aubin F, Leroy D, Bernard P (2008) Comparison of histopathologic-clinical characteristics of Jessner’s lymphocytic infiltration of the skin and lupus erythematosus tumidus: multicenter study of 46 cases. J Am Acad Dermatol 58(2):217–223CrossRefPubMed
31.
Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P (2000) Lupus erythematosus tumidus e a neglected subset of cutaneous lupus erythematosus: report of 40 cases. Arch Dermatol 136:1033–1041CrossRefPubMed
32.
Rongioletti F, Rebora A (1991) The new cutaneous mucinoses. A review with an up-to-date classification of cutaneous mucinoses. J Am Acad Dermatol 24:265–270CrossRefPubMed
33.
Ortize VG, Krishnan RS, Chen LL, Hsu S (2004) Papulonodular mucinosis in systemic lupus erythematosus. Dermatol Online J 10:16
34.
Gold SC (1954) An unusual papular eruption associated with lupus erythematosus. Br J Dermatol 66:429–433CrossRefPubMed
35.
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, Mejía JC, Aydintug AO, Chwalinska-Sadowska H, de Ramón E, Fernández-Nebro A, Galeazzi M, Valen M, Mathieu A, Houssiau F, Caro N, Alba P, Ramos-Casals M, Ingelmo M, Hughes GR, European Working Party on Systemic Lupus Erythematosus (2003) Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore) 82(5):299–308CrossRef
36.
Francès C, Piette JC (2000) The mystery of Sneddon syndrome: relationship with antiphospholipid syndrome and systemic lupus erythematosus. J Autoimmun 15(2):139–143CrossRefPubMed
37.
Englert HJ, Loizou S, Derue GG, Walport MJ, Hughes GR (1989) Clinical and immunologic features of livedo reticularis in lupus: a case-control study. Am J Med 87:408–410CrossRefPubMed
38.
Francès C, Niang S, Laffitte E, Pelletier F, Costedoat N, Piette JC (2005) Dermatologic manifestations of the antiphospholipid syndrome: two hundred consecutive cases. Arthritis Rheum 52(6):1785–1793CrossRefPubMed
39.
Ball E, Newburger A, Ackerman AB (2003) Degos’ disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol 25(4):308–320CrossRefPubMed
40.
Theodoridis A, Makrantonaki E, Zouboulis CC (2013) Malignant atrophic papulosis (Köhlmeier-Degos disease) – a review. Orphanet J Rare Dis 8:10CrossRefPubMedPubMedCentral
41.
Lipsker D (2012) In: Goldsmith L, Katz S, Gilchrest B, Paller A, Leffell D, Wolff K (eds) Fitzpatrick’s dermatology in general medicine. McGraw-Hill, New York, pp 2072–2076, Malignant atrophic papulosis (Degos disease)
42.
Wahl DG, Guillemin F, de Maistre E, Perret-Guillaume C, Lecompte T, Thibaut G (1998) Meta-analysis of the risk of venous thrombosis in individuals with antiphospholipid antibodies without underlying autoimmune disease or previous thrombosis. Lupus 7(1):15–22CrossRefPubMed
43.
Venencie PY, Winkelmann RK (1985) Monoclonal antibody studies in the skin lesions of patients with anetoderma. Arch Dermatol 121:747–749CrossRefPubMed
44.
Gusdorf L, Bessis D, Lipsker D (2014) Lupus erythematosus and neutrophilic urticarial dermatosis: a retrospective study of 7 patients. Medicine (Baltimore) 93(29):e351CrossRef
45.
Mitsias DI, Kapsogeorgou EK, Moutsopoulos HM (2006) Sjögren’s syndrome: why autoimmune epithelitis? Oral Dis 12:523–532CrossRefPubMed
46.
Lipsker D, Saurat JH (2008) Neutrophilic cutaneous lupus erythematosus. At the edge between innate and acquired immunity? Dermatology 216(4):283–286CrossRefPubMed
47.
Marzano AV, Ramoni S, Caputo R (2008) Amicrobial pustulosis of the folds. Report of 6 cases and a literature review. Dermatology 216(4):305–311CrossRefPubMed
48.
Lee HY, Pelivani N, Beltraminelli H, Hegyi I, Yawalkar N, Borradori L (2011) Amicrobial pustulosis-like rash in a patient with Crohn’s disease under anti-TNF-alpha blocker. Dermatology 222:304–310CrossRefPubMed
49.
Fujimoto W, Hamada T, Yamada J, Matsuura H, Iwatsuki K (2005) Bullous systemic lupus erythematosus as an initial manifestation of SLE. J Dermatol 32:1021–1027CrossRefPubMed
50.
Camisa C, Sharma HM (1983) Vesiculobullous systemic lupus erythematosus. J Am Acad Dermatol 9:924–933CrossRefPubMed
51.
Hall RP, Lawley TJ, Smith HR, Katz SI (1982) Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann Intern Med 197:165–170CrossRef
52.
Kieffer C, Cribier B, Lipsker D (2009) Neutrophilic urticarial dermatosis; a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore) 88:23–31CrossRef
53.
Vedove CD, Del Giglio M, Schena D, Girolomoni G (2009) Drug-induced lupus erythematosus. Arch Dermatol Res 301:99–105CrossRefPubMed
54.
Xiao X, Chang C (2014) Diagnosis and classification of drug-induced autoimmunity (DIA). J Autoimmun 48–49:66–72CrossRefPubMed
55.
Katz U, Zandman-Goddard G (2010) Drug-induced lupus: an update. Autoimmun Rev 10:46–50CrossRefPubMed
56.
Fritzler MJ (1994) Drugs recently associated with lupus syndromes. Lupus 3(6):455–459CrossRefPubMed
57.
Chlebus E, Wolska H, Blaszczyk M, Jablonska S (1998) Subacute cutaneous lupus erythematosus versus systemic lupus erythematosus: diagnostic criteria and therapeutic implications. J Am Acad Dermatol 38(3):405–412CrossRefPubMed
58.
Marzano AV, Lazzari R, Polloni I, Crosti C, Fabbri P, Cugno M (2011) Drug-induced subacute cutaneous lupus erythematosus: evidence for differences from its idiopathic counterpart. Br J Dermatol 165(2):335–341CrossRefPubMed
59.
Sontheimer RD, Maddison PJ, Reichlin M, Jordon RE, Stastny P, Gilliam JN (1982) Serologic and HLA associations in subacute cutaneous lupus erythematosus, a clinical subset of lupus erythematosus. Ann Intern Med 97:664–667CrossRefPubMed
60.
Marzano AV, Vezzoli P, Crosti C (2009) Drug-induced lupus: an update on its dermatologic aspects. Lupus 18:935–940CrossRefPubMed
61.
Rubin RL (2015) Drug-induced lupus. Expert Opin Drug Saf 2:1–18
62.
Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725CrossRefPubMed
63.
Kuhn A, Wenzel J, Weyd H (2014) Photosensitivity, apoptosis, and cytokines in the pathogenesis of lupus erythematosus: a critical review. Clin Rev Allergy Immunol 47(2):148–162CrossRefPubMed
64.
Nyberg F, Hasan T, Puska P, Stephansson E, Häkkinen M, Ranki A, Ros AM (1997) Occurrence of polymorphous light eruption in lupus erythematosus. Br J Dermatol 136(2):217–221CrossRefPubMed
65.
Kim A, Chong BF (2013) Photosensitivity in cutaneous lupus erythematosus. Photodermatol Photoimmunol Photomed 29(1):4–11CrossRefPubMedPubMedCentral
66.
Schmidt E, Tony H-P, Bröcker E-B, Kneitz C (2007) Sun-induced life-threatening lupus nephritis. Ann N Y Acad Sci 1108:35–40CrossRefPubMed
67.
Kuhn A, Ruland V, Bonsmann G (2010) Photosensitivity, phototesting, and photoprotection in cutaneous lupus erythematosus. Lupus 19(9):1036–1046CrossRefPubMed
68.
Klein R, Moghadam-Kia S, LoMonico J, Okawa J, Coley C, Taylor L, Troxel AB, Werth VP (2011) Development of the CLASI as a tool to measure disease severity and responsiveness to therapy in cutaneous lupus erythematosus. Arch Dermatol 147(2):203–208CrossRefPubMedPubMedCentral
69.
Bonilla-Martinez ZL, Albrecht J, Troxel AB, Taylor L, Okawa J, Dulay S, Werth VP (2008) The cutaneous lupus erythematosus disease area and severity index: a responsive instrument to measure activity and damage in patients with cutaneous lupus erythematosus. Arch Dermatol 144(2):173–180CrossRefPubMedPubMedCentral
70.
Okon LG, Werth VP (2013) Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol 27(3):391–404CrossRefPubMedPubMedCentral
71.
Kuhn A, Ruland V, Bonsmann G (2011) Cutaneous lupus erythematosus: update of therapeutic options part II. J Am Acad Dermatol 65(6):e195–e213CrossRefPubMed
72.
Kuhn A, Sigges J, Biazar C, Ruland V, Landmann A, Amler S, Bonsmann G, WUSCLE coauthors (2014) Influence of smoking on disease severity and antimalarian therapy in cutaneous lupus erythematosus: analysis of 1002 patients from the EUSCLE database. Br J Dermatol 171:571–579CrossRefPubMed
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