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Arthritis und Rheuma 2010; 30(04): 205-210
DOI: 10.1055/s-0037-1618024
Vaskulitiden
Schattauer GmbH

Die leukozytoklastische Vaskulitis

Leukocytoclastic vasculitis
S. Unger
1   Sektion Rheumatologie, Department für Innere Medizin, Neurologie und Dermatologie, Universitätsklinikum Leipzig
,
C. Baerwald
1   Sektion Rheumatologie, Department für Innere Medizin, Neurologie und Dermatologie, Universitätsklinikum Leipzig
› Author Affiliations
Further Information

Publication History

Publication Date:
26 December 2017 (online)

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Zusammenfassung

Die leukozytoklastische Vaskulitis stellt als häufigste Vaskulitis der Haut eine differenzialdiagnostische Herausforderung für Dermatologen und Rheumatologen dar. Als Immunkomplexvaskulitis entsteht die leukozytoklastische Vaskulitis durch Ablagerung von Immunkomplexen an der Gefäßwand. In der Hautbiopsie mit direkter Immunfluoreszenz kann eine IgA- von einer IgM- beziehungsweise IgG-Ablagerung unterschieden werden. Die Purpura Schönlein-Henoch ist die häufigste Vaskulitis des Kindesalters und besitzt eine sehr gute Prognose. Bei Vorhandensein von IgM/IgG-Immunkomplexen sollten differenzialdiagnostisch Infektionen, maligne Grunderkrankungen, Medikamentennebenwirkungen und entzündlich-rheumatische Erkrankungen ausgeschlossen werden. Unkomplizierte Verlaufsformen der leukozytoklastischen Vaskulitis können symptomatisch behandelt werden. Kortikosteroide sind bei ausgedehnten Nekrosen und Ulzerationen der Haut indiziert. Systemische Beteiligungen müssen frühzeitig detektiert und gegebenenfalls immunsuppressiv behandelt werden. Ingesamt besitzt die leukozytoklastische Vaskulitis jedoch eine sehr gute Prognose und zeigt meist einen selbstlimitierenden Verlauf.

Summary

Leukocytoclastic vasculitis is the most common cutaneous vasculitis and represents a challenge in differential diagnosis for dermatologists and rheumatologists. Disease manifestation results from deposition of immune complexes in the vascular wall. IgA-complexes can be distinguished from IgM/IgG-complexes by means of direct immunofluorescence from cutaneous biopsy. Henoch-Schönlein purpura is the most common vasculitis in children with an excellent prognosis. If IgM/IgG-complexes are detected underlying infections, neoplastic diseases, drug reactions and rheumatic diseases should be considered. Uncomplicated cases of leukocytoclastic vasculitis can be treated symptomatically. Steroids are indicated if cutaneous necrosis or ulcerations occur. Systemic involvement has to be detected early and should be treated with immunosuppressants. However, in most cases leukocytoclastic vasculitis is self-limiting with an excellent prognosis.

Schlüsselwörter

Leukozytoklastische Vaskulitis - kutane Vaskulitis - Vaskulitis - Purpura Schönlein-Henoch

Keywords

Leukocytoclastic vasculitis - cutaneous vasculitis - vasculitis - Henoch-Schönlein purpura
 

  • Literatur

  • 1 Black AK. Urticarial vasculitis. Clin Dermatol 1999; 17: 565-569.
    CrossrefPubMedGoogle Scholar
  • 2 Callen JP, Ekenstam E. Cutaneous leukocytoclastic vasculitis: clinical experience in 44 patients. South Med J 1987; 80: 848-851.
    CrossrefPubMedGoogle Scholar
  • 3 Callen JP. et al. Azathioprine. An effective, corticosteroid-sparing therapy for patients with recalcitrant cutaneous lupus erythematosus or with recalcitrant cutaneous leukocytoclastic vasculitis. Arch Dermatol 1991; 127: 515-522.
    CrossrefPubMedGoogle Scholar
  • 4 Callen JP. A clinical approach to the vasculitis patient in the dermatologic office. Clin Dermatol 1999; 17: 549-553.
    CrossrefPubMedGoogle Scholar
  • 5 Chandesris MO. et al. Infliximab in the treatment of refractory vasculitis secondary to hepatitis C-associated mixed cryoglobulinaemia. Rheumatology (Oxford) 2004; 43: 532-533.
    CrossrefPubMedGoogle Scholar
  • 6 Cupps TR. at el. Chronic, recurrent small-vessel cutaneous vasculitis. J Am Med Assoc 1982; 247: 1994-1998.
    CrossrefGoogle Scholar
  • 7 Davis MD. et al. Clinicopathologic correlation of hypocomplementemic urticarial vasculitis. J Am Acad Dermatol 1998; 38: 899-905.
    PubMedGoogle Scholar
  • 8 Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol 2003; 48: 311-340.
    CrossrefPubMedGoogle Scholar
  • 9 Gibson LE. Cutaneous vasculitis update. Dermatol Clin 2001; 19: 603-615.
    CrossrefPubMedGoogle Scholar
  • 10 Jarrett SJ. et al. Anti-tumor necrosis factor-alpha therapy-induced vasculitis: case series. J Rheumatol 2003; 30: 2287-2291.
    Google Scholar
  • 11 Jennette JC. et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37: 187-192.
    CrossrefPubMedGoogle Scholar
  • 12 Jessop SJ. Cutaneous leucocytoclastic vasculitis: a clinical and aetiological study. Br J Rheumatol 1995; 34: 942-945.
    CrossrefPubMedGoogle Scholar
  • 13 Martinez-Taboada VM. et al. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med 1997; 102: 186-191.
    CrossrefPubMedGoogle Scholar
  • 14 Mehregan DR. et al. Urticarial vasculitis: a histopathologic and clinical review of 72 cases. J Am Acad Dermatol 1992; 26: 441-448.
    CrossrefPubMedGoogle Scholar
  • 15 Mohan et al. Leukocytoclastic vasculitis associated with tumor necrosis factor-alpha blocking agents. J Rheumatol 2004; 31: 1955-1958.
    Google Scholar
  • 16 Ong CS, Benson EM. Successful treatment of chronic leukocytoclastic vasculitis and persistent ulceration with intravenous immunoglobulin. Br J Dermatol 2000; 143: 447-449.
    CrossrefPubMedGoogle Scholar
  • 17 Padeh S, Passwell JH. Successful treatment of chronic Henoch-Schonlein purpura with colchicine and aspirin. Isr Med Assoc J 2000; 2: 482-483.
    PubMedGoogle Scholar
  • 18 Pertuiset E. et al. Adult Henoch-Schonlein Purpura associated with malignancy. Semin Arthritis Rheum 2000; 29: 360-367.
    CrossrefPubMedGoogle Scholar
  • 19 Pillebout E. et al. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 2002; 13: 1271-1278.
    CrossrefPubMedGoogle Scholar
  • 20 Pyne D. et al. Colchicine for the treatment of recurrent Henoch-Schonlein Purpura in an adult. Rheumatology (Oxford) 2001; 40: 1430-1431.
    CrossrefPubMedGoogle Scholar
  • 21 Ramelli GP, Bianchetti MG. Dapsone in cutaneous Henoch-Schonlein syndrome-worth a trial. Acta Paediatr 1997; 86: 337.
    PubMedGoogle Scholar
  • 22 Ruiz Villaverde R. et al. Annular leukocytoclastic vasculitis: response to dapsone. J Eur Acad Dermatol Venereol 2002; 16: 544-546.
    CrossrefPubMedGoogle Scholar
  • 23 Russell JP. et al. Primary cutaneous small vessel vasculitis: approach to diagnosis and treatment. Int J Dermatol 2006; 45 (01) 3-13.
    CrossrefPubMedGoogle Scholar
  • 24 Sais G. et al. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol 1995; 131: 1399-1402.
    CrossrefPubMedGoogle Scholar
  • 25 Sais G. et al. Prognostic factors in leukocytoclastic vasculitis. A clinicopathologic study of 160 patients. Arch Dermatol 1998; 134: 309-315.
    CrossrefPubMedGoogle Scholar
  • 26 Sarma PS. et al. Dapsone in Henoch-Schonlein Purpura. Postgrad Med J 1994; 70 (842): 464-465.
    CrossrefPubMedGoogle Scholar
  • 27 Saulsbury FT. Henoch-Schonlein Purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 1999; 78: 395-409.
    CrossrefPubMedGoogle Scholar
  • 28 Somer T, Finegold SM. Vasculitides associated with infections, immunization, and antimicrobial drugs. Clin Infect Dis 1995; 20: 1010-1036.
    CrossrefPubMedGoogle Scholar
  • 29 Sunderkötter C. et al. Leukocytoclastic vasculitis. Hautarzt 2004; 55 (08) 759-783.
    CrossrefPubMedGoogle Scholar
  • 30 Sunderkötter C. et al. Management of leukocytoclastic vasculitis. J Dermatolog Treat 2005; 16 (04) 193-206.
    CrossrefPubMedGoogle Scholar
  • 31 ten Holder SM. et al. Cutaneous and systemic manifestations of drug-induced vasculitis. Ann Pharmacother 2002; 36: 130-147.
    CrossrefPubMedGoogle Scholar
  • 32 Tosca AD. et al. Cyclosporin A in the treatment of cutaneous vasculitis. Clinical and cellular effects. J Eur Acad Dermatol Venereol 1996; 6: 135-141.
    CrossrefGoogle Scholar
  • 33 Uthman IW. et al. Response of deep cutaneous vasculitis to infliximab. J Am Acad Dermatol 2005; 53: 353-354.
    CrossrefPubMedGoogle Scholar
  • 34 Weiler-Bisig D. et al. Henoch-Schonlein purpura associated with esophagus carcinoma and adenocarcinoma of the lung. Clin Nephrol 2005; 63: 302-304.
    CrossrefPubMedGoogle Scholar
  • 35 Wisnieski JJ. Urticarial vasculitis. Curr Opin Rheumatol 2000; 12: 24-31.
    CrossrefPubMedGoogle Scholar
  • 36 Zhu YI, Stiller MJ. Dapsone and sulfones in dermatology: overview and update. J Am Acad Dermatol 2001; 45: 420-434.
    CrossrefPubMedGoogle Scholar