Induction of Pemphigus in Neonatal Mice by Passive Transfer of IgG from Patients with the Disease

Grant J. Anhalt, Ramzy S. Labib, John J. Voorhees, Theodore F. Beals, Luis A. Diaz

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We examined the role of circulating autoantibodies in the pathogenesis of pemphigus vulgaris by passively transferring IgG fractions from five patients with pemphigus vulgaris into neonatal Balb/c mice, in doses of 1.5 to 16 mg per gram of body weight per day. Cutaneous blisters and erosions with the histologic, ultrastructural, and immunofluorescence features of pemphigus occurred in 39 of 55 mice given intraperitoneal injections of IgG from patients with pemphigus and in none of 58 control mice given normal human IgG. IgG fractions with high titers of pemphigus antibodies were most effective in inducing disease, and this effect was dose dependent. Titers of circulating IgG in mouse serum closely correlated with the extent of disease induced (P<0.002). This study strongly supports the proposed role of pemphigus autoantibodies in the pathogenesis of pemphigus vulgaris in human beings and demonstrates that pemphigus can be passively transferred to laboratory animals. (N Engl J Med. 1982; 306:1189–96.) PEMPHIGUS vulgaris is a severe and often fatal blistering disease, affecting the skin and mucous membranes, in which intraepithelial vesicles form as a result of a distinctive process of cell detachment termed acantholysis.1 The immunologic abnormalities discovered two decades ago by Beutner and Jordon2 are consistently used as markers of the disease. These investigators showed by immunofluorescence techniques that the majority of patients with pemphigus have IgG-class autoantibodies directed against squamous-epithelial-cell surfaces. These autoantibodies are detected both bound to the diseased epithelium and circulating in the serum, but their relation to the pathologic process has been unclear. There is substantial.

Original languageEnglish (US)
Pages (from-to)1189-1196
Number of pages8
JournalNew England Journal of Medicine
Issue number20
StatePublished - May 20 1982
Externally publishedYes

ASJC Scopus subject areas

  • Medicine(all)


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