Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients

I. Juhász, Gerald S. Lazarus, George F. Murphy, Ie Ming Shih, Meenhard Herlyn

Research output: Contribution to journalArticle

37 Citations (Scopus)

Abstract

Pemphigus vulgaris is an autoimmune blistering disease that is induced by binding of antibodies to a 130/85-kD protein complex on epidermal keratinocytes. An in vivo experimental model of this disease was developed by reconstituting severe combined immunodeficiency (SCID) mice with 1-10 × 107 PBL from patients with naturally occurring pemphigus vulgaris. Of 49 reconstituted mice, 34 (69%) produced human IgG levels of > 0.1 mg/ml. Circulating anti-pemphigus antibodies were found in 20 of the 34 successfully reconstituted mice; 44% of these animals had deposits of human IgG in their own skin after it was traumatized by either heat or cold. Spontaneous pemphigus vulgaris-like blisters associated with human IgG deposits were rarely found in mouse skin. By contrast, allogeneic human skin grafted to 10 of 12 mice before reconstitution with patients' PBL developed pemphigus vulgaris-like lesions containing human IgG deposits. These results demonstrate that SCID mice can serve as a model of an antibody-mediated human autoimmune skin disease.

Original languageEnglish
Pages (from-to)2401-2407
Number of pages7
JournalJournal of Clinical Investigation
Volume92
Issue number5
Publication statusPublished - 1993

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Severe Combined Immunodeficiency
Pemphigus
Lymphocytes
Immunoglobulin G
Skin
Autoimmune Diseases
Antibodies
Blister
Keratinocytes
Skin Diseases
Anti-Idiotypic Antibodies
Theoretical Models
Hot Temperature
Proteins

Keywords

  • Animal model
  • Antibodies
  • Pemphigus
  • Skin autoimmune disease

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients. / Juhász, I.; Lazarus, Gerald S.; Murphy, George F.; Shih, Ie Ming; Herlyn, Meenhard.

In: Journal of Clinical Investigation, Vol. 92, No. 5, 1993, p. 2401-2407.

Research output: Contribution to journalArticle

Juhász, I. ; Lazarus, Gerald S. ; Murphy, George F. ; Shih, Ie Ming ; Herlyn, Meenhard. / Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients. In: Journal of Clinical Investigation. 1993 ; Vol. 92, No. 5. pp. 2401-2407.
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