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. 2001 Dec;60(12):1097–1102. doi: 10.1136/ard.60.12.1097

Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis

R Brons 1, M C J M de Jong 1, N K de Boer 1, C Stegeman 1, C Kallenberg 1, J Tervaert 1
PMCID: PMC1753448  PMID: 11709450

Abstract

BACKGROUND—Wegener's granulomatosis (WG) is considered a pauci-immune systemic vasculitis based on the absence of immune deposits in renal biopsies of patients with active disease. In animal models of antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis, immune deposits along the glomerular capillary wall are present at early stages of lesion development. These deposits are degraded rapidly, resulting in "pauci-immune" lesions.
OBJECTIVE—To test the hypothesis that immune deposits can also be detected in early lesions of patients with WG, thereby initiating an inflammatory reaction that, in time, is augmented in the presence of ANCA, resulting in pauci-immune lesions later on.
METHODS—The presence of immune deposits in skin biopsies taken within 48 hours of lesion development was investigated. Direct immunofluorescence was used to examine 32 skin biopsies for the presence of immune deposits (IgG, IgA, IgM, C3c). When possible, a comparison was made between the immunofluorescence findings in renal and skin biopsies taken at the same time.
RESULTS—Four of 11 biopsies taken at initial presentation and four of 21 biopsies taken at the onset of a relapse of WG showed IgG and/or IgA containing immune deposits in the subepidermal blood vessels. All nine renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=5) or absence (n=4) of immune deposits in the skin biopsy.
CONCLUSION—A substantial number of skin biopsies showed immune deposits during active disease. These results could support the hypothesis that immune complexes may trigger vasculitic lesions in WG.



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Figure 1  .

Figure 1  

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(A) Leucocytoclastic vasculitis. There is infiltration of vessel walls with neutrophils and fibrinoid necrosis (black arrows) with leucocytoclasia and extravasation of red blood cells (white arrows) (haematoxylin and eosin, objective lens ×50). (B) Cutaneous granuloma annulare with large area of "necrobiosis" surrounded by a palisade of histiocytes (black arrow) (haematoxylin and eosin, objective lens ×25).

Figure 2  .

Figure 2  

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Immunofluorescence images of frozen skin sections from patients with Wegener's granulomatosis, counterstained with blue fluorescent bisbenzimide to visualise cell nuclei. Asterisks indicate the lumen of small blood vessels, the closed arrows point towards epidermal cell nuclei, and open arrows point towards endothelial cell nuclei. (A) Overview of the epidermis and superficial dermis in patient No 5 showing extensive granular staining for IgG in and around blood capillary walls in dermal papillae (objective lens ×40). (B) Higher magnification of the inset on figure A (objective lens ×20). (C) Blood capillary wall of dermal papilla of patient No 13 showing fine granular staining for IgA (objective lens ×40). (D) Blood capillary in dermal papilla of perilesional skin from patient No 4 showing negative staining for IgA (objective lens ×40).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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