Skip to main content

Advertisement

SpringerLink
Log in

Lupus erythematodes

Symptomenvielfalt durch klinische Variabilitäten, Koexistenzen und Imitatoren

Lupus erythematosus

Wide range of symptoms through clinical variation, associated diseases and imitators

  • Übersichten
  • Published:
Der Hautarzt Aims and scope Submit manuscript

Zusammenfassung

Der Lupus erythematodes (LE) bietet an der Haut die vielfältigen und charakteristischen klinischen Zeichen des akuten, subakuten und chronischen Typs, des intermediären Lupus (Lupus tumidus), des Chilblain Lupus sowie des Lupus profundus. Des Weiteren treten das Schmetterlingserythem sowie auch ulzeröse Schleimhautveränderungen auf. Neben den typischen Lupusherden können auch unspezifische Veränderungen vorkommen wie eine dermale Muzinose, akneiforme Hautveränderungen, verschiedene Livedoarten, eine nekrotisierende Vaskulitis mit Ulzera, Purpura, Urtikariavaskulitis, neutrophile Dermatose, Hyperpigmentierungen, Haar- und Nagelveränderungen sowie Overlap-Syndrome mit Erythema exsudativum multiforme, Pemphigus, bullösem Pemphigoid, Sklerodermie, Sjögren-Syndrom, Raynaud-Phänomen, Lichen planus und Psoriasis. Lupusimitatoren und differenzialdiagnostisch gegen Lupus profundus schwierig abzugrenzen sind atypische Mykobakterieninfektionen und das subkutane T-Zell-Lymphom. Unspezifische Hautläsionen bei LE sind durch eine Maximalvariante der bei LE typischen pathologischen Veränderungen oder bestimmte Befundkonstellationen bedingt wie das Vorliegen von Antiphospholipidantikörpern oder durch Komplementmangel. Wenn letztere beide Laborbefunde pathologisch sind, ist mit schwererem klinischem LE-Verlauf, auch mit Komplikationen zu rechnen.

Abstract

The typical clinical forms of cutaneous lupus erythematosus (LE) are the butterfly rash, acute, subacute and chronic cutaneous lupus, intermediate lupus (lupus tumidus), chilblain- and bullous lupus, lupus profundus, and ulcerating lesions on the mucous membrane. Besides the typical lupus forms, nonspecific skin lesions are also observed such as dermal mucinosis, acneiform skin lesions, different variants of livedo, necrotizing vasculitis with ulcers, purpura, urticaria vasculitis, neutrophilic dermatosis, hyperpigmentation, hair and nail changes as well as overlap syndromes with erythema multiforme, scleroderma, Sjögren syndrome, Raynaud phenomenon, lichen planus, bullous pemphigoid und psoriasis. There are lupus imitators which create differential diagnostic challenges, such as infections with atypical mycobacteria or subcutaneous T-cell lymphoma both of which are similar to lupus profundus. All these skin lesions can present as maximal pathological findings seen in lupus or be caused by a variety of pathological laboratory findings such as the anti-phospholipid antibodies or a deficiency of complement factors. In the latter situation severe lupus often with complications can be expected.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6

Literatur

  1. Kuhn A, Ruzicka T (2004) Classification of cutaneous lupus erythematosus. In: Kuhn A, Lehmann P, Ruzicka T (Hrsg) Cutaneous lupus erythematosus. Springer, Berlin Heidelberg New York Tokyo, S 53–58

  2. Cardinali C, Caproni M, Bernacchi E et al (2000) The spectrum of cutaneous manifestations in lupus erythematosus – the Italian experience. Lupus 9:417–423

    Article  CAS  PubMed  Google Scholar 

  3. Kuhn A, Schuppe HC, Ruzicka T, Lehmann P (2000) Rare cutaneous manifestaions of lupus erythematosus. A clinical overview. Hautarzt 5:818–825

    Article  Google Scholar 

  4. Gammon WR, Briggaman RA (1993) Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol 100:28S–34S

    Article  CAS  PubMed  Google Scholar 

  5. Chan LS, Lapiere JC, Chen M et al (1999) Bullous systemic lupus erythematosus with autantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen. Arch Dermatol 135:569–573

    Article  CAS  PubMed  Google Scholar 

  6. Kacalak-Rzepka A, Zaluga E, Maleszka R et al (2004) Bullous systemic lupus erythematosus with antiphospholipid syndrome. J Eur Acad Dermatol Venereol 18:490–494

    Article  CAS  PubMed  Google Scholar 

  7. Sonntag M, Lehmann P, Megahed M et al (2003) Papulonodular mucinosis associated with subacute cutaneous lupus erythematosus. Dermatology 206:326–329

    Article  PubMed  Google Scholar 

  8. Del Pozo J, Pena C, Almagro M et al (2000) Systemic lupus erythematosus presenting with a reticular erythematous mucinosis-like condition. Lupus 9:144–146

    Article  Google Scholar 

  9. Deruelle-Khazaal R, Segard M, Cottencin-Charriere AC et al (2002) Chronic lupus erythematosus presenting as acneiform lesions. Ann Dermatol Venereol 129:883–885

    CAS  PubMed  Google Scholar 

  10. Motel PJ, Bernstein EF, Fazio M et al (1995) Systemic lupus erythematosus in patients diagnosed with treatment-resistant acne. Int J Dermatol 34:338–340

    Article  CAS  PubMed  Google Scholar 

  11. Bouaziz JD, Barete S, Le Pelletier F et al (2007) Cutaneous lesions of the digits in systemic lupus erythematosus: 50 cases. Lupus 16:163–167

    CAS  PubMed  Google Scholar 

  12. Viguier M, Pinquier L, Cavelier-Balloy B et al (2001) Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus. Medicine (Baltimore) 80:180–188

    Google Scholar 

  13. Alegre VA, Gastineau DA, Winkelmann RK (1989) Skin lesions associated with circulating lupus anticoagulant. Br J Dermatol 120:419–429

    Article  CAS  PubMed  Google Scholar 

  14. Richert B, Andre J, Bourguignon R, Brassine M de la (2004) Hyperkeratotic nail discoid lupus erythematosus evolving towards systemic lupus erythematosus: therapeutic difficulties. JEADV 18:728–730

    CAS  PubMed  Google Scholar 

  15. Riccieri V, Spadaro A, Ceccarelli F et al (2005) Nailfold capillaroscopy changes in systemic lupus erythematosus: correlations with disease activity and autoantibody profile. Lupus 14:521–525

    Article  CAS  PubMed  Google Scholar 

  16. Urowitz MB, Gladman DD, Chalmers A, Ogryzlo MA (1978) Nail lesions in systemic lupus erythematosus. J Rheumatol 5:441–447

    CAS  PubMed  Google Scholar 

  17. Vaughn RY, Bailey JP Jr, Field RS et al (1990) Diffuse nail dyschromia in black patients with systemic lupus erythematosus. J Rheumatol 17:640–643

    CAS  PubMed  Google Scholar 

  18. Miyakis S, Lockshin MD, Atsumi T et al (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4:295–306

    Article  CAS  PubMed  Google Scholar 

  19. Merrill JT (2004) Antibodies and clinical features of the antiphospholipid syndrome as criteria for systemic lupus erythematosus. Lupus 13:869–876

    Article  CAS  PubMed  Google Scholar 

  20. Weinstein C, Miller MH, Axtens R et al (1987) Livedo reticularis associated with increased titers of anticardiolipin antibodies in systemic lupus erythematosus. Arch Dermatol 123:596–600

    Article  CAS  PubMed  Google Scholar 

  21. Gibson GE, Su WP, Pittelkow MR (1997) Antiphospholipid syndrome and the skin. J Am Acad Dermatol 36:970–982

    Article  CAS  PubMed  Google Scholar 

  22. Diogenes MJ, Diogenes PC, Morais Carneiro RM de et al (2004) Cutaneous manifestations associated with antiphospholipid antibodies. Int J Dermatol 43:632–637

    Article  CAS  PubMed  Google Scholar 

  23. Moser KM, El Shabrawi-Caelen L, Koller S, Aberer E (2004) Systemic lupus erythematosus with atrophic blanche-like palmar lesions. J Dtsch Dermatol Ges 2:1009–1012

    PubMed  Google Scholar 

  24. Paira S, Cassano G, Korol V et al (2005) Erythromelalgia with subsequent digital necrosis, glomerulonephritis, and antiphospholipid antibodies. J Clin Rheumatol 11:209–212

    Article  PubMed  Google Scholar 

  25. McClain MT, Arbuckle MR, Heinlen LD et al (2004) The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum 50:1226–1232

    Article  PubMed  Google Scholar 

  26. Gomez-Puerta JA, Martin H, Amigo MC et al (2005) Long-term follow-up in 128 patients with primary antiphospholipid syndrome: Do they develop lupus? Medicine (Baltimore) 84:225–230

    Google Scholar 

  27. Cervera R, Garcia-Carrasco M, Font J et al (1997) Antiphospholipid antibodies in primary Sjögren’s syndrome: prevalence and clinical significance in a series of 80 patients. Clin Exp Rheumatol 15:361–365

    CAS  PubMed  Google Scholar 

  28. Sangle S, D’Cruz DP, Hughes GR (2005) Livedo reticularis and pregnancy morbidity in patients negative for antiphospholipid antibodies. Ann Rheum Dis 64:147–148

    Article  CAS  PubMed  Google Scholar 

  29. Davis MD, Daoud MS, Kirby B et al (1998) Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol 38:899–905

    Article  CAS  PubMed  Google Scholar 

  30. Zeitouni NC, Funaro D, Cloutier RA et al (2000) Redefining Rowell’s syndrome. Br J Dermatol 142:343–346

    Article  CAS  PubMed  Google Scholar 

  31. Davis MD, Brewer JD (2004) Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome. Immunol Allergy Clin North Am 24:183–213

    Article  PubMed  Google Scholar 

  32. Wisnieski JJ (2000) Urticarial vasculitis. Curr Opin Rheumatol 12:24–31

    Article  CAS  PubMed  Google Scholar 

  33. Provost TT, Zone JJ, Synkowski D et al (1980) Unusual cutaneous manifestations of systemic lupus erythematosus: I. Urticaria-like lesions. Correlation with clinical and serological abnormalities. J Invest Dermatol 75:495–499

    Article  CAS  PubMed  Google Scholar 

  34. Ramos-Casals M, Campoamor MT, Chamorro A et al (2004) Hypocomplementemia in systemic lupus erythematosus and primary antiphospholipid syndrome: prevalence and clinical significance in 667 patients. Lupus 13:777–783

    Article  CAS  PubMed  Google Scholar 

  35. D’Cruz DP, Wisnieski JJ, Asherson RA et al (1995) Autoantibodies in systemic lupus erythematosus and urticarial vasculitis. J Rheumatol 22:1669–1673

    Google Scholar 

  36. Sontheimer RD, Racila E, Racila DM (2005) C1q: its functions within the innate and adaptive immune responses and its role in lupus autoimmunity. J Invest Dermatol 125:14–23

    Article  CAS  PubMed  Google Scholar 

  37. Korb LC, Ahearn JM (1997) C1q binds directly and specifically to surface blebs of apoptotic human keratinocytes: complement deficiency and systemic lupus erythematosus revisited. J Immunol 158:4525–4528

    CAS  PubMed  Google Scholar 

  38. Berkel AI, Petry F, Sanal O et al (1997) Development of systemic lupus erythematosus in a patient with selective complete C1q deficiency. Eur J Pediatr 156:113–115

    Article  CAS  PubMed  Google Scholar 

  39. Lienesch DW, Sherman KE, Metzger A, Shen GQ (2006) Anti-Clq antibodies in patients with chronic hepatitis C infection. Clin Exp Rheumatol 24:183–185

    CAS  PubMed  Google Scholar 

  40. Saadoun D, Sadallah S, Trendelenburg M et al (2006) Anti-C1q antibodies in hepatitis C virus infection. Clin Exp Immunol 145:308–312

    Article  CAS  PubMed  Google Scholar 

  41. Koide M, Shirahama S, Tokura Y et al (2002) Lupus erythematosus associated with C1 inhibitor deficiency. J Dermatol 29:503–507

    PubMed  Google Scholar 

  42. Gammon WR, Wheeler CE Jr (1979) Urticarial vasculitis: report of a case and review of the literature. Arch Dermatol 115:76–80

    Article  CAS  PubMed  Google Scholar 

  43. Buckley D, Barnes L (1995) Childhood subacute cutaneous lupus erythematosus associated with homozygous complement 2 deficiency. Pediatr Dermatol 12:327–330

    Article  CAS  PubMed  Google Scholar 

  44. Lyon VB, Nocton JJ, Drolet BA, Esterly NB (2003) Necrotic facial papules in an adolescent: C2 deficiency with eventual development of lupus erythematosus. Pediatr Dermatol 20:318–322

    Article  PubMed  Google Scholar 

  45. Nousari HC, Kimyai-Asadi A, Provost TT (1999) Generalized lupus erythematosus profundus in a patient with genetic partial deficiency of C4. J Am Acad Dermatol 41:362–364

    Article  CAS  PubMed  Google Scholar 

  46. Franke W, Kuhn A, Megahed M et al (1999) Periorbital edema as the initial symptom of lupus erythematosus profundus. Case report and discussion of the literature. Hautarzt 50:889–892

    Article  CAS  PubMed  Google Scholar 

  47. Jacyk WK, Bhana KN (2006) Lupus erythematosus profundus in black South Africans. Int J Dermatol 45:717–721

    Article  CAS  PubMed  Google Scholar 

  48. Gleason BC, Zembowicz A, Granter SR (2006) Non-bullous neutrophilic dermatosis: an uncommon dermatologic manifestation in patients with lupus erythematosus. J Cutan Pathol 33:721–725

    Article  PubMed  Google Scholar 

  49. Hou TY, Chang DM, Gao HW et al (2005) Sweet’s syndrome as an initial presentation in systemic lupus erythematosus: a case report and review of the literature. Lupus 14:399–402

    Article  CAS  PubMed  Google Scholar 

  50. Levenstein MM, Fisher BK, Fisher LL, Pruzanski W (1991) Simultaneous occurrence of subacute cutaneous lupus erythematosus and Sweet syndrome. A marker of Sjogren syndrome? Int J Dermatol 130:640–643

    Article  Google Scholar 

  51. 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

    CAS  PubMed  Google Scholar 

  52. Reynaert S, Setterfield J, Black MM (2006) Hydroxychloroquine-induced pigmentation in two patients with systemic lupus erythematosus. J Eur Acad Dermatol Venereol 20:487–488

    Article  CAS  PubMed  Google Scholar 

  53. Baird JS, Johnson JL, Elliott-Mills D, Opas LM (1997) Systemic lupus erythematosus with acanthosis nigricans, hyperpigmentation, and insulin receptor antibody. Lupus 6:275–278

    Article  CAS  PubMed  Google Scholar 

  54. Chaudhuri S, Basu K, Dhar MC et al (2002) Alopecia universalis in a case of systemic lupus erythematosus. J Assoc Physicians India 50:1073–1074

    CAS  PubMed  Google Scholar 

  55. Seyahi E, Seyahi N, Fresko I et al (2006) Hair diameter in systemic lupus erythematosus. Lupus 15:282–284

    Article  CAS  PubMed  Google Scholar 

  56. Werth VP, White WL, Sanchez MR, Franks AG (1992) Incidence of alopecia areata in lupus erythematosus. Arch Dermatol 128:368–371

    Article  CAS  PubMed  Google Scholar 

  57. Tristano A, Villarroel J, Rodrigez M, Millan A (2006) Calcinosus cutis universalis in a patient with systemic lupus erythematosus. Clin Rheumatol 25:70–74

    Article  PubMed  Google Scholar 

  58. Zalla MJ, Muller SA (1996) The coexistence of psoriasis with lupus erythematosus and other photosensitive disorders. Acta Derm Venereol Suppl (Stockh) 195:1–15

    Google Scholar 

  59. Berthelot C, Nash J, Duvic M (2007) Coexistent psoriasis and lupus erythematosus treated with alefacept. Am J Clin Dermatol 8:47–50

    Article  PubMed  Google Scholar 

  60. Provost TT, Watson R, Simmons-O’Brien E (1997) Anti-Ro(SS-A) antibody positive Sjögren’s/lupus erythematosus overlap syndrome. Lupus 6:105–111

    Article  CAS  PubMed  Google Scholar 

  61. Kaufhold A, Marsch WC (1993) Sjögren-LE-syndrome with anti-Ro- and anti-La-antibodies. Aktuelle Derm 19:19–23

    Google Scholar 

  62. Meiss F, Fischer M, Hädecke J et al (2004) Graves disease. An important differential diagnostic consideration for systemic lupus erythematosus. Hautarzt 55:465–479

    Article  Google Scholar 

  63. Ramos-Casals M, Brito-Zeron P, Font J (2007) The overlap of Sjögren’s syndrome with other systemic autoimmune diseases. Semin Arthritis Rheum 36:246–255

    Article  PubMed  Google Scholar 

  64. Szabo E, Husz S, Kovacs L (1981) Coexistent atypical bullous pemphigoid and systemic lupus erythematosus. Br J Dermatol 104:71–75

    Article  CAS  PubMed  Google Scholar 

  65. Asherson RA, Angus H, Mathews JA et al (1991) The progressive systemic sclerosis/systemic lupus overlap: an unusual clinical progression. Ann Rheum Dis 50:323–327

    Article  CAS  PubMed  Google Scholar 

  66. Stevens HP, Ostlere LS, Rustin MH (1994) Systemic lupus erythematosus in association with ulcerative colitis: related autoimmune diseases. Br J Dermatol 130:385–389

    Article  CAS  PubMed  Google Scholar 

  67. Müller FB, Groth W, Mahrle G (2004) Multiple autoimmune syndrome. Reynolds-syndrome (acral scleroderma, primary biliary cirrhosis, Sjögren syndrome) associated with the lupus erythematosus/lichen planus overlap syndrome. Hautarzt 55:465–470

    Article  PubMed  Google Scholar 

  68. Sharp GC, Irvin WS, May CM et al (1976) Association of antibodies to ribonucleoprotein and Sm antigens with mixed connective-tissue disease, systematic lupus erythematosus and other rheumatic diseases. N Engl J Med 295:1149–1154

    Article  CAS  PubMed  Google Scholar 

  69. Grader-Beck T, Wigley FM (2005) Raynaud’s phenomenon in mixed connective tissue disease. Rheum Dis Clin North Am 31:465–481

    Article  PubMed  Google Scholar 

  70. Burdt MA, Hoffman RW, Deutscher SL et al (1999) Long-term outcome in mixed connective tissue disease: longitudinal clinical and serologic findings. Arthritis Rheum 42:899–909

    Article  CAS  PubMed  Google Scholar 

  71. Hsu PY, Yang YH, Hsiao CH et al (2002) Mycobacterium kansasii infection presenting as cellulitis in a patient with systemic lupus erythematosus. J Formos Med Assoc 101:581–584

    PubMed  Google Scholar 

  72. Czelusta A, Moore AY (1999) Cutaneous mycobacterium kansasii infection in a patient with systemic lupus erythematosus: case report and review. J Am Acad Dermatol 40:359–363

    Article  CAS  PubMed  Google Scholar 

  73. Elkayam O, Gat A, Lidgi M et al (2003) Atypical cutaneous findings in a patient with systemic lupus erythematosus. Lupus 12:413–417

    Article  CAS  PubMed  Google Scholar 

  74. Mok MY, Wong SS, Chan TM et al (2007) Non-tuberculous mycobacterial infection in patients with systemic lupus erythematosus. Rheumatology (Oxford) 46:280–284

    Google Scholar 

  75. Cassis TB, Fearneyhough PK, Callen JP (2004) Subcutaneous panniculitis-like T-cell lymphoma with vacuolar interface dermatitis resembling lupus erythematosus panniculitis. J Am Acad Dermatol 50:465–469

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  77. Ma L, Bandarchi B, Glusac EJ (2005) Fatal subcutaneous panniculitis-like T-cell lymphoma with interface change and dermal mucin, a dead ringer for lupus erythematosus. J Cutan Pathol 32:360–365

    Article  PubMed  Google Scholar 

  78. Gonzalez EG, Selvi E, Lorenzini S et al (2007) Subcutaneous panniculitis-like T-cell lymphoma misdiagnosed as lupus erythematosus panniculitis. Clin Rheumatol 26:244–246

    Article  PubMed  Google Scholar 

  79. Magro CM, Crowson AN, Kovatich AJ, Burns F (2001) Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia. J Cutan Pathol 28:235–247

    Article  CAS  PubMed  Google Scholar 

  80. Malik M, Ahmed AR (2007) Concurrence of systemic lupus erythematosus and pemphigus: coincidence or correlation? Dermatology 214:231–239

    Article  PubMed  Google Scholar 

  81. Friss AB, Cohen PR, Bruce S, Duvic M (1995) Chronic cutaneous lupus erythematosus mimicking mycosis fungoides. J Am Acad Dermatol 33:891–895

    Article  CAS  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Univ.-Klinik für Dermatologie, Medizinische Universität Graz, Auenbrugger Platz 8, 8036, Graz, Österreich

    E. Aberer

Authors
  1. E. Aberer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to E. Aberer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Aberer, E. Lupus erythematodes. Hautarzt 61, 676–682 (2010). https://doi.org/10.1007/s00105-010-1939-2

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00105-010-1939-2

Schlüsselwörter

Keywords

Search

Navigation