Der Hautarzt

, Volume 57, Issue 1, pp 47–50 | Cite as

Therapierefraktäre kutane nekrotisierende Vaskulitis

Erfolgreiche Sulfontherapie
Kasuistik

Zusammenfassung

Das Erscheinungsbild von Vaskulitiden variiert von selbst limitierenden oligosymptomatischen bis hin zu lebensbedrohlichen Multiorganerkrankungen. Im Folgenden beschreiben wir die Krankengeschichte einer Patientin, die seit über 10 Jahren an einer schwer verlaufenden kutanen, nekrotisierenden Vaskulitis erkrankt war. Verschiedene immunsuppressive Therapien inklusive Cyclophosphamid blieben trotz adäquater Dosierung und ausreichendem Applikationszeitraum erfolglos. Erst eine Therapie mit dem Sulfon DADPS (Diaminodiphenylsulfon, Dapson) führte zu einer vollständigen Abheilung der jahrelang bestehenden vaskulitischen Ulzera. Neben der Besprechung der Einsatzmöglichkeiten von DADPS zur Therapie entzündlicher Hauterkrankungen gibt der Fallbericht eine Übersicht über das Spektrum kutaner Vaskulitiden.

Schlüsselwörter

Nekrotisierende Vaskulitis Sulfone DADPS Immunmodulatoren 

Refractory cutaneous necrotizing vasculitis

Successful sulfone therapy

Abstract

The clinical picture in vasculitis varies from few self-limiting symptoms to life-threatening illnesses affecting multiple organs. We describe the course of a woman patient who had had a severe cutaneous necrotizing vasculitis for more than 10 years. Various immunsuppressants, including cyclophosphamide, had failed to improve the clinical course, although adequate doses were given for a sufficient period. Treatment with DADPS (diaminodiphenylsulfone, dapsone), however, finally led to complete remission of the severe and chronic vasculitic ulcerations. Indications for DADPS treatment of inflammatory cutaneous diseases are discussed, and an overview of cutaneous vasculitis is provided.

Keywords

Necrotizing vasculitis Sulfones DADPS Immunomodulators 

Literatur

  1. 1.
    Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98: 76–85PubMedGoogle Scholar
  2. 2.
    Fiorentino DF (2003) Cutaneous vasculitis. J Am Acad Dermatol 48: 311–340CrossRefPubMedGoogle Scholar
  3. 3.
    Fredenberg MF, Malkinson FD (1987) Sulfone therapy in the treatment of leukocytoclastic vasculitis. J Am Acad Dermatol 16: 772–778Google Scholar
  4. 4.
    Fukuhara K, Urano Y, Kimura S, Hori K, Arase S (2000) Pyoderma gangrenosum with rheumatoid arthritis and pulmonary aseptic abscess responding to treatment with dapsone. Br J Dermatol 139: 556–558Google Scholar
  5. 5.
    Grabbe J, Haas N, Moller A, Henz BM (2000) Erythema elevatum et diutinum—evidence for disease-dependent leucocyte alterations and response to dapsone. Br J Dermatol 143: 415–420Google Scholar
  6. 6.
    Hunder GG, Arend WP, Bloch DA et al. (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum 33: 1065–1067PubMedGoogle Scholar
  7. 7.
    Jennette JC, Falk RJ, Andrassy K et al. (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37: 187–192PubMedGoogle Scholar
  8. 8.
    Nürnberg W, Grabbe J, Czarnetzki BM (1995) Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline. Acta Derm Venereol 75: 54–56Google Scholar
  9. 9.
    Sharquie KE, Najim RA, Abu-Raghif AR (2002) Dapsone in Behcet’s disease: a double-blind, placebo-controlled, cross-over study. J Dermatol 29: 267–279Google Scholar
  10. 10.
    Thomas-Golbanov C, Sridharan S (2001) Novel therapies in vasculitis. Expert Opin Invest Drugs 10: 1279–1289Google Scholar
  11. 11.
    Thuong-Nguyen V, Kadunce DP, Hendrix JD, Gammon WR, Zone JJ (1993) Inhibition of neutrophil adherence to antibody by dapsone: a possible therapeutic mechanism of dapsone in the treatment of IgA dermatoses. J Invest Dermatol 100: 349–355Google Scholar
  12. 12.
    Van de Kerkhof PC (1994) On the efficacy of dapsone in granuloma faciale. Acta Derm Venereol 74: 61–62Google Scholar
  13. 13.
    Wozel G (1996) Dapson—Pharmakologie, Wirkmechanismus und klinischer Einsatz. Thieme, Stuttgart New York, S 24–48Google Scholar
  14. 14.
    Wozel G, Blasum C, Winter C, Gerlach B (1997) Dapsone hydroxylamine inhibits the LTB-4-induced chemotaxis of polymorphonuclear leukocytes into human skin: results of a pilot study. Inflamm Res. 46: 420–422Google Scholar

Copyright information

© Springer Medizin Verlag 2005

Authors and Affiliations

  1. 1.Dermatologische Klinik mit PoliklinikUniversitätsklinikumErlangen
  2. 2.Dermatologische Klinik mit PoliklinikUniversitätsklinikum ErlangenErlangen

Personalised recommendations