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Leukozytoklastische Vaskulitis

Leukocytoclastic vasculitis

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Zusammenfassung

Die leukozytoklastische Vaskulitis (LcV) stellt die häufigste Form der Vaskulitis an der Haut dar. Sie ist meist durch die Ablagerung von Immunkomplexen an der Gefäßwand bedingt. Wenn primär IgA im Immunkomplex gebunden ist, liegt oft eine systemische Beteiligung im Sinne einer Purpura Schönlein-Henoch vor. Wenn sie im Erwachsenenalter auftritt, sind schwere Komplikationen häufiger. Vaskulitiden, bei denen eher IgG oder IgM als IgA involviert sind, zeigen klinisch häufiger nur einen kutanen Befall oder auch eine milde Systembeteiligung. Bei anderen Formen der LcV sind außer den immunkomplexbedingten Entzündungsreaktionen weitere Faktoren pathophysiologisch wirksam. Die LcV kann außerdem ein initiales oder begleitendes Symptom der schweren systemischen, ANCA-assoziierten Vaskulitiden sein. In manchen Fällen ist sie Ausdruck einer Bakteriämie. Die Diagnostik dient dazu, die genaue Form der LcV und damit das Ausmaß einer systemischen Beteiligung abzuklären sowie mögliche Ursachen aufzudecken. Wenn kein Auslöser beseitigt werden kann, sollte man unkomplizierte Formen der LcV symptomatisch behandeln. Kortikosteroide sind indiziert wenn sich Nekrosen und Ulzerationen ankündigen. Bei chronisch rezidivierender LcV bietet sich der Einsatz von Dapson und Colchizin an. Bei schwerer systemischer Vaskulitis werden immunsuppressive Strategien verfolgt.

Abstract

Leukocytoclastic vasculitis (LcV) is the most common form of cutaneous vasculitis. Often LcV results from deposition of immune complexes in the vascular wall. When IgA is the dominant immunoglobulin in these complexes, systemic involvement is likely (Henoch-Schönlein purpura), being more severe in adults. LcV in which immune complexes are composed of IgG or IgM are more often limited to the skin and may additionally show minor systemic involvement. In other forms of LcV additional pathophysiological factors play a role. LcV can also be a presenting or accompanying symptom of severe systemic ANCA-associated vasculitis. In some cases, LcV is a sign of bacteriemia. The aim of diagnostic procedures is to determine the specific type of vasculitis and degree of systemic involvement as well as possible causes. If no trigger or cause can be found, uncomplicated cases of LcV should be treated symptomatically. Corticosteroids are indicated at initial signs of necrosis or ulceration. Chronic recurrent LcV may respond to dapsone or colchicine. Severe systemic vasculitis requires immunosuppressive therapy.

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Danksagung

Die Abbildungen wurden freundlicherweise überlassen vom Fotolabor der Klinik und Poliklinik für Dermatologie des Universitätsklinikums Münster (wir danken Frau J. Bückmann, Herrn P. Wissel und Herrn Prof. Dr. T. Luger). Für die Hilfe bei der grafischen Gestaltung danken wir außerdem dem Fotolabor der Klinik für Dermatologie und Allergologie des Universitätsklinikums Ulm (Frau M. Mössner-Strohm, Herrn S. Reihle und Frau Prof. Dr. K. Scharffetter-Kochanek).

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Authors and Affiliations

  1. Universitätsklinik und Poliklinik für Dermatologie und Allergologie, Universitätsklinikum Ulm, Ulm

    C. Sunderkötter

  2. Institut für Experimentelle Dermatologie und Universitätskinderklinik, Universitätsklinikum Münster, Münster

    J. Roth

  3. Klinik und Poliklinik für Hautkrankheiten, Universitätsklinikum Münster, Münster

    G. Bonsmann

  4. Universitätsklinik und Poliklinik für Dermatologie und Allergologie, Maienweg 12, 89081 , Ulm

    C. Sunderkötter

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  1. C. Sunderkötter
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Correspondence to C. Sunderkötter.

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Sunderkötter, C., Roth, J. & Bonsmann, G. Leukozytoklastische Vaskulitis. Hautarzt 55, 759–785 (2004). https://doi.org/10.1007/s00105-004-0760-1

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