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Kutane Vaskulitiden

Wege zur Diagnose

Cutaneous vasculitides

A diagnostic approach

Der Hautarzt volume 59, Article number: 374 (2008) Cite this article

Zusammenfassung

Gemeinsames Merkmal der Vaskulitiden ist die Gefäßentzündung mit Schädigung der abhängigen Gewebe. Zu den Vaskulitiden mit charakteristischen Hautveränderungen gehören die leukozytoklastische Vaskulitis (LcV), die Hennoch-Schönlein-Purpura (HSP), die kutane Polyarteriitis nodosa (kPAN), das Erythema elevatum et diutinum (EED) und die Urtikariavaskulitis (UV). Bei anderen Vaskulitisformen stehen systemische Manifestationen meist im Vordergrund, die fakultativ auftretenden Hautveränderungen sind hier vielfältig: Dazu gehören M. Kawasaski (MK), kryoglobulinämische Vaskulitis (KV), Wegener-Granulomatose (WG), Churg-Strauss-Syndrom (CSS) und mikroskopische Polyangiitis (MPA). Die Ursachen der Vaskulitiden sind heterogen, Auslöser können Infektionen, Medikamente, Kollagenosen, Autoimmunerkrankungen oder lymphoproliferative Erkrankungen sein. Idiopathische Vaskulitiden treten meist nur einmalig auf und sind in ihrem Verlauf selbstlimitiert. Die Diagnostik der Vaskulitiden orientiert sich am klinischen Bild und beinhaltet neben der Histologie die Beurteilung von Entzündungszeichen, den Nachweis von zirkulierenden Immunkomplexen, unterschiedlichen Typen von Kryoglobulinen und antineutrophilen zytoplasmatischen Antikörpern, eine kollagenosespezifische Immunserologie und weiterführende hämatologische Untersuchungen. Vaskulitiden können sich an zahlreichen Organen manifestieren und bedürfen daher besonders bei der WG, der MPA, dem CSS und der PAN einer weitreichenden bildgebenden Diagnostik.

Abstract

Vasculitis is characterized by an inflammatory reaction of vessel walls with damage to the dependent tissues. Forms of vasculitis which frequently have skin changes include leukocytoclastic angiitis (LcV), Henoch-Schönlein purpura (HSP), cutaneous polyarteriitis nodosum (cPAN), erythema elevatum et diutinum (EED) and urticarial vasculitis (UV). In other forms of vasculitis, systemic manifestations predominate but there are a variety of skin changes. Kawasaki disease (MK), cryoglobulinemic vasculitis (kV), Wegener granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangitis (MPA) belong to this group. The causes of vasculitis are heterogeneous. Triggers include infections, drugs, collagen vascular diseases, autoimmune diseases and lymphoproliferative disorders. Idiopathic vasculitis, particularly LcV and EED, occur only once and have a self-limited course. The diagnostic work up depends on the clinical picture and includes inflammatory markers, circulating immune complexes, different types of cryoglobulins and anti-neutrophilic cytoplasmic antibodies, collagen vascular disease specific autoantibodies and additional hematological studies. Vasculitis can manifest in many organs and requires a thorough work up specifically in cases where WG, MPA, CSS and PAN are under consideration.

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Literatur

  1. Braun GS, Horster S, Wagner KS et al. (2007) Cryoglobulinaemic vasculitis: classification and clinical and therapeutic aspects. Postgrad Med J 83: 87–94

    PubMed  Article  Google Scholar 

  2. Brouet JC, Clauvel P, Danon F et al. (1974) Biologic and clinical significance of cryoglobulins. Am J Med 57: 775–788

    PubMed  Article  CAS  Google Scholar 

  3. Carlson JA, Cavaliere LF, Grant-Kels JM (2006) Cutaneous vasculitis: diagnosis and management. Clin Dermatol 24: 414–429

    PubMed  Article  Google Scholar 

  4. Carlson JA, Chen K-R (2006) Cutaneous vasculitis update: small vessel neutrophilic vasculitis syndrome. Am J Dermatopathol 28: 486–506

    PubMed  Article  Google Scholar 

  5. Chung L, Kea B, Fiorentino DF (2008) Cutanneous vasculitis. In: Bolognia JL, Jorizzo JL, Rapini RP (eds) Dermatology. Mosby Elsevier, London, pp 347–367

  6. Creighton WD, Lambert PH, Miescher PA (1973) Detection of antibodies and soluble antigen-antibody complexes by precipitation with polyethylene glycol. J Immunol 111: 1219–1227

    PubMed  CAS  Google Scholar 

  7. Cuellar Ml (2002) Drug-induced vasculitits. Curr Rheumatol Rep 4: 55–59

    PubMed  Article  Google Scholar 

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

    PubMed  Article  CAS  Google Scholar 

  9. De Koning HD, Bodar EJ, van der Meer JW, Simon A (2007) Schnitzler syndrome: beyond the case reports: review and follow-up op 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 37: 137–148

    Article  Google Scholar 

  10. Fiorentino DF (2003) Cutaneous vasculitis. J Am Acad Dermatol 48: 311–340

    PubMed  Article  Google Scholar 

  11. Fritsch PO (1991) Nekrotisierende Vaskulitis. Hautarzt 42: 661–670

    PubMed  CAS  Google Scholar 

  12. Gause A, Lamprecht P (2003) Die kryoglobulinämische Vaskulitis. Internist 44: 165–174

    PubMed  CAS  Google Scholar 

  13. Günther C, Hertl M, Meurer M (2007) Autoantikörperdiagnostik. Hautarzt 58: 1063–1075

    PubMed  Article  Google Scholar 

  14. Hartung K, Seelig H-P (2007) Labordiagnostik der systemischen Autoimmunerkrankungen. Teil II: Rheumatoide Arthritis und Vaskulopathien. Z Rheumatol 66: 225–238

    PubMed  Article  CAS  Google Scholar 

  15. Hellmich B, Voswinkel J, Aries PM, Gross WL (2004) Primäre systemische Vaskulitiden: Wege zur Diagnose. Dtsch Med Wochenschr 129: 1322–1327

    PubMed  Article  CAS  Google Scholar 

  16. Hellmich B, Lamprecht P, Aries PM, Gross WL (2005) Frühdiagnose von Vaskulitiden. Z Rheumatol 64: 538–546

    PubMed  Article  CAS  Google Scholar 

  17. Hunder G, Arend WP, Bloch DA et al.T (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis. Arthritis Rheum 33: 1065–1067

    PubMed  CAS  Google Scholar 

  18. Jennette JC (1994) Vasculitis affecting the skin. Arch Dermatol 130: 899–906

    PubMed  Article  CAS  Google Scholar 

  19. Jennette JC, Falk RJ, Andrassy K et al. (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37: 187–192

    PubMed  Article  CAS  Google Scholar 

  20. Lamprecht P, Gause A, Gross W (1999) Cryoglobulinemic vasculitis. Arthritis Rheum 42: 2507–2516

    PubMed  Article  CAS  Google Scholar 

  21. Landthaler M (1980) Kryoproteinämien. Hautarzt 31: 633–638

    PubMed  CAS  Google Scholar 

  22. Marini A, Fenk R, Plettenberg H et al. (2006) Seltene Vaskulitiden als Hinweis auf Plasmozytom. Hautarzt 57: 137–143

    PubMed  Article  CAS  Google Scholar 

  23. Michel BA, Hunder GG, Boch DA, Calabrese LH (1992) Hypersensitivity vascultitis and Henoch-Schonlein purpura: a comparison between the 2 disorders. J Rheumatol 19: 721–728

    PubMed  CAS  Google Scholar 

  24. Nydegger UE (1974) Circulating immune complexes in the serum in systemic lupus erythematosus and in carriers of hepatitis B antigen. Quantitiation by binding to radiolabeled Clq. J Clin Invest 54: 297–309

    PubMed  Article  CAS  Google Scholar 

  25. Nydegger UE (2007) Immune complex pathophysiology. Ann N Y Acad Sci 1109: 66–83

    PubMed  Article  CAS  Google Scholar 

  26. Ozen S, Bakkaloglu A, Dusunsel R et al. (2007) Childhood vasculitides in Turkey: a nationwide survey. Clin Rheumatol 26: 196–200

    PubMed  Google Scholar 

  27. Poyrazoglu HM, Per H, Gündüz Z et al. (2003) Acute hemorrhagic edema of infancy. Pediatr Int 45: 697–700

    PubMed  Article  Google Scholar 

  28. Petzelbauer P, Peng LS, Poler JS (2008) The skin in inflammatory and other vascular disorders. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ (eds) Fitzpatrick’s dermatology in general medicine. McGraw-Hill, New York, pp 1585–1686

  29. Saadoun D, Landau DA, Calabrese LH, Cacoub PP (2007) Hepatitis C-associated mixed cryoglobulinaemia: a crossroad between autoimmunity and lymphoproliferation. Rheumatology 46: 1234–1242

    PubMed  Article  CAS  Google Scholar 

  30. Savige J, Gillis D, Benson E et al. (1999) International Consensus Statement on testing and reporting of antineutrophil cytoplasmic antibodies (ANCA). Am J Clin Pathol 111: 507–513

    PubMed  CAS  Google Scholar 

  31. Schäkel K, Meurer M (2004) Vasculitis allergica. In: Schultze-Werninghaus G, Fuchs T, Bachert C, Wahn U (eds) Manuale allergologicum. Dustri-Verlag, München Orlando, pp 1115–1125

  32. Schneider SW, Gaubitz M, Luger TA (2007) Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J Am Acad Dermatol 56: 120–122

    Article  Google Scholar 

  33. Stringa SG, Bianchi C, Casala AM, Bianchi O (1967) Allergic vasculitis Gougerot-Ruiter syndrome. Arch Derm 95: 23–27

    PubMed  Article  CAS  Google Scholar 

  34. Sepp N (2005) Vaskulitis. In: Braun-Falco O, Plewig G, Wolff HH et al. (Hrsg) Dermatologie und Venerologie. Springer, Berlin Heidelberg New York Tokyo, S 779–803

  35. Sunderkötter C, Roth J, Bonsmann G (2004) Leukozytoklastische Vaskulitis. Hautarzt 55: 759–785

    PubMed  Article  Google Scholar 

  36. Sunderkötter C, Sindrilaru A (2006) Clinical classification of vasculitis. Eur J Dermatol 16: 114–124

    PubMed  Google Scholar 

  37. Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD (1997) Schönlein-Henoch purpura in adult patients: predictive factors for IgA glomerulonephritis in a retospective study of 57 cases. Arch Dermatol 133: 438–442

    PubMed  Article  CAS  Google Scholar 

  38. Trendelenburg M, Courvoisier S, Spath PJ et al. (1999) Hypocomplementemic urticarial vasculitis or systemic lupus erythematosus? Am J Kidney Dis 34: 745–751

    PubMed  Article  CAS  Google Scholar 

  39. Ueki H, Yaoita H ((1989) A color atlas of dermato-immunohistocytology. Wolfe Med Publ, Tokyo

  40. Van der Woude FJ, Lobatto S, Permin H et al. (1985) Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet 1(8426): 425–429

    Google Scholar 

  41. Wiik A (2001) Clinical use of serological tests for antineutrophil cytoplasmic antibodies. Rheum Dis Clin N Am 27: 799–813

    Article  CAS  Google Scholar 

  42. Wiik A (2008) Drug-induced vasculitis. Curr Opin Rheumatol 20: 35–39

    PubMed  Article  Google Scholar 

  43. Wisnieski J, Baer AN, Christensen J, Cupps TR (1995) Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine 74: 24–41

    PubMed  Article  CAS  Google Scholar 

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  1. Klinik und Poliklinik für Dermatologie am Universitätsklinikum Carl Gustav Carus derTechnischen Universität Dresden, Fetscherstraße 74, 01307, Dresden, Deutschland

    K. Schäkel &  M. Meurer

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  1. K. Schäkel
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  2. M. Meurer
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Correspondence to M. Meurer.

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Schäkel, K., Meurer, M. Kutane Vaskulitiden. Hautarzt 59, 374 (2008). https://doi.org/10.1007/s00105-008-1546-7

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Schlüsselwörter

  • Kutane Vaskulitiden
  • Gefäßentzündungen
  • Diagnose
  • Immunserologie
  • Hämatologische Untersuchungen

Keywords

  • Cutaneous vasculitis
  • Vascular inflammation
  • Diagnosis
  • Immune serology
  • Hematological evaluation