Dermal dendritic cell population and blood vessels are diminished in the skin of systemic sclerosis patients: relationship with fibrosis degree and disease duration

Am J Dermatopathol. 2013 Jun;35(4):438-44. doi: 10.1097/DAD.0b013e3182712d1a.


Recent studies have suggested that the number of dermal dendritic cells is altered in the skin of patients with scleroderma and that these cells may have an important role in the pathogenesis of this disease. There is also a belief that insufficient blood flow to the affected organs may also be responsible for the disease. Our aim was to quantify CD34+ cells, factor XIIIa cells, and blood vessels in the skin of patients with systemic sclerosis and to correlate these data with fibrosis degree and duration of disease. Paraffin-embedded skin sections from patients with systemic sclerosis and from healthy subjects were immunolabelled with antibodies against CD34+ and factor XIIIa. Cells and blood vessels were quantified in the papillary and reticular dermis. Both, the number of CD34+ cells and factor XIIIa cells in the skin of patients with systemic sclerosis were reduced. The reduction of these cell types preceded the appearance of intense fibrosis, suggesting that fibrosis is not responsible of this phenomenon. Blood vessel volume and surface density were also reduced in the skin of systemic sclerosis patients. This reduction was also noted early in the evolution of the disease. Our results suggest that CD34+ cells and factor XIIIa cells may contribute to normal regulation of extracellular matrix assembly. We confirmed the observation that capillary density is diminished in scleroderma skin.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Antigens, CD34 / analysis
  • Biomarkers / analysis
  • Biopsy
  • Brazil
  • Capillaries / chemistry
  • Capillaries / immunology
  • Capillaries / pathology*
  • Case-Control Studies
  • Factor XIIIa / analysis
  • Female
  • Fibrosis
  • Humans
  • Immunohistochemistry
  • Langerhans Cells / immunology
  • Langerhans Cells / pathology*
  • Male
  • Middle Aged
  • Paraffin Embedding
  • Scleroderma, Systemic / immunology
  • Scleroderma, Systemic / metabolism
  • Scleroderma, Systemic / pathology*
  • Severity of Illness Index
  • Skin / blood supply*
  • Skin / immunology
  • Skin / pathology*
  • Young Adult


  • Antigens, CD34
  • Biomarkers
  • Factor XIIIa