Skip to main content

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

Publication ,  Journal Article
Juhasz, I; Lazarus, GS; Murphy, GF; Shih, IM; Herlyn, M
Published in: J Clin Invest
November 1993

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 x 10(7) 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 to 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.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

J Clin Invest

DOI

ISSN

0021-9738

Publication Date

November 1993

Volume

92

Issue

5

Start / End Page

2401 / 2407

Location

United States

Related Subject Headings

  • Species Specificity
  • Skin Transplantation
  • Pemphigus
  • Mice, SCID
  • Mice
  • Lymphocyte Transfusion
  • Infant, Newborn
  • Immunology
  • Immunoglobulins
  • Humans
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Juhasz, I., Lazarus, G. S., Murphy, G. F., Shih, I. M., & Herlyn, M. (1993). Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients. J Clin Invest, 92(5), 2401–2407. https://doi.org/10.1172/JCI116846
Juhasz, I., G. S. Lazarus, G. F. Murphy, I. M. Shih, and M. Herlyn. “Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients.J Clin Invest 92, no. 5 (November 1993): 2401–7. https://doi.org/10.1172/JCI116846.
Juhasz, I., et al. “Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients.J Clin Invest, vol. 92, no. 5, Nov. 1993, pp. 2401–07. Pubmed, doi:10.1172/JCI116846.
Juhasz I, Lazarus GS, Murphy GF, Shih IM, Herlyn M. Development of pemphigus vulgaris-like lesions in severe combined immunodeficiency disease mice reconstituted with lymphocytes from patients. J Clin Invest. 1993 Nov;92(5):2401–2407.

Published In

J Clin Invest

DOI

ISSN

0021-9738

Publication Date

November 1993

Volume

92

Issue

5

Start / End Page

2401 / 2407

Location

United States

Related Subject Headings

  • Species Specificity
  • Skin Transplantation
  • Pemphigus
  • Mice, SCID
  • Mice
  • Lymphocyte Transfusion
  • Infant, Newborn
  • Immunology
  • Immunoglobulins
  • Humans