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Figure 4 | Orphanet Journal of Rare Diseases

Figure 4

From: Passive transfer of collagen XVII-specific antibodies induces sustained blistering disease in adult mice

Figure 4

Diseased mice show immuno- and histo-pathological features of the pemphigoid disease. At the end of the observation period, mice were sacrificed and perilesional biopsies were analyzed for immunoreactants deposition. By IF microscopy, the perilesional skin of a diseased mouse injected with (a) rabbit or (b) sheep pathogenic antibodies showed linear deposition of murine IgG at the DEJ. Additionally, tissue bound (d) rabbit and (e) sheep antibodies were shown to activate murine C3 in vivo as revealed by direct IF microscopy. In contrast, no (c) IgG or (f) complement deposits were found in mice injected with control sheep antibodies. The lesional skin of a mouse receiving (g) rabbit or (h) sheep BP180/ CXVII-specific IgG shows a dermal-epidermal separation accompanied by an inflammatory infiltrate. In contrast, the skin of a mouse receiving (i) control sheep antibodies showed no histopathological signs of disease (magnifications, 200x).

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172