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Rheumatology Advance Access published online on November 4, 2006
Rheumatology, doi:10.1093/rheumatology/kel297
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Received March 20, 2006
Accepted July 10, 2006

Original Papers

Immunophenotyping of chimeric cells in localized scleroderma

K. T. McNallan 1, C. Aponte 2, R. el-Azhary 3, T. Mason 1, A. M. Nelson 3, J. J. Paat 1, C. S. Crowson 4, and A. M. Reed 1 *

1 Mayo Clinic College of Medicine, Rochester, MN, USA; Department of Medicine, Division of Rheumatology, PA, Dallas, TX, USA
2 Texas Dermatology Associates, PA, Dallas, TX, USA
3 Mayo Clinic College of Medicine, Rochester, MN, USA; Department of Dermatology, PA, Dallas, TX, USA
4 Mayo Clinic College of Medicine, Rochester, MN, USA; Department of Health Sciences Research, Division of Biostatistics, Mayo Clinic College of Medicine, Rochester, MN, USA

* To whom correspondence should be addressed.
A. M. Reed, E-mail: reed.ann18{at}mayo.edu


  Abstract

Objective. Localized scleroderma causes thickening of the skin due to excessive collagen deposition. This condition has clinical and histopathological similarities to chronic graft-vs-host disease. We wanted to identify whether chimeric cells are present in the affected tissue in localized scleroderma and to further investigate the role of chimerism by immunophenotyping the chimeric cells. We hypothesize that the presence of chimerism and immunotypic chimeric cells will lend to an understanding of the pathogenesis of localized scleroderma and possible mechanisms by which chimeric cells participate in autoimmunity.

Methods. We studied skin biopsies from 18 localized scleroderma patients and compared them with concurrent biopsies from unaffected skin in a subset of patients. Skin biopsies from morphoea and linear scleroderma patients were analysed for the presence of chimeric cells using male-female (X, Y) differences. Cell surface markers (CD4, CD8, CD19/20, CD68, S100, CD14 and CD56) were determined for cell phenotyping of chimeric cells.

Results. Overall, the affected tissue contained a greater number of lymphocytic inflammatory cells. In the affected tissue, 38% of the total chimeric cells were CD68+ (dendritic cell, monocyte and macrophage marker), 29% Langerin/S100+ (dendritic cell marker), 26% CD8+ (cytotoxic T-lymphocyte marker), 20% CD19/20+ (B-lymphocyte marker), 14% CD4+ (T-helper lymphocyte) and 0% CD56+ (natural killer cell marker).

Conclusions. We report that not only are chimeric cells present in affected localized scleroderma lesions but they also are more likely to be dendritic cells and B lymphocytes suggesting a role in the pathogenesis of localized scleroderma.

Keywords: Morphoea; Localized scleroderma; Chimerism; cGVHD.
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