Rheumatology Advance Access originally published online on November 4, 2006
Rheumatology 2007 46(3):398-402; doi:10.1093/rheumatology/kel297
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Immunophenotyping of chimeric cells in localized scleroderma
1Mayo Clinic College of Medicine, Rochester, MN, 2Department of Medicine, Division of Rheumatology, 3Department of Dermatology, 4Texas Dermatology Associates, PA, Dallas, TX, and 5Department of Health Sciences Research, Division of Biostatistics, Mayo Clinic College of Medicine, Rochester, MN, USA.
Correspondence to: Ann M. Reed, MD, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA. E-mail: reed.ann18{at}mayo.edu
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Abstract |
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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.
KEY WORDS: Morphoea, Localized scleroderma, Chimerism, cGVHD
Submitted 20 March 2006;
revised version accepted 10 July 2006.
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