IgG4-positive plasma cells in cutaneous Rosai-Dorfman disease: an additional immunohistochemical feature and possible relationship to IgG4-related sclerosing disease

J Cutan Pathol. 2009 Oct;36(10):1069-73. doi: 10.1111/j.1600-0560.2008.01222.x. Epub 2009 Jan 27.


Background: Cutaneous Rosai-Dorfman disease (CRDD) shares the histopathological features of abundant plasma cells and stromal fibrosis with IgG4-related sclerosing disease. The possible role of IgG4+ plasma cells in CRDD was investigated.

Methods: Twelve cases of CRDD were reviewed, and their lesions were immunostained with anti-IgG4 and anti-IgG antibodies. The number of IgG4+ and IgG+ plasma cells and their ratios were estimated. Serum IgG4 and IgG concentrations were measured in two recent cases.

Results: Many IgG4+ and IgG+ plasma cells were found in all 12 cases. IgG4+ plasma cells ranged from 21 to 204 per high-power field (HPF) (mean 117/HPF), and IgG+ plasma cells ranged from 114 to 759/HPF (mean 349/HPF). All cases had more than 30 IgG4+ cells/HPF, except one case. The IgG4/IgG ratio ranged from 16% to 51% (mean 34%). Serum IgG4 concentration and serum IgG4/IgG ratio were increased in one recent case. Various degrees of stromal fibrosis were present in all cases.

Conclusions: The presence of many IgG4+ plasma cells and stromal fibrosis suggests that CRDD may be related to IgG4-related sclerosing disease. Many IgG4+ plasma cells is another feature of CRDD, and serum IgG4 may be elevated.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Histiocytosis, Sinus / immunology
  • Histiocytosis, Sinus / metabolism
  • Histiocytosis, Sinus / pathology*
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / immunology
  • Immunohistochemistry
  • Plasma Cells / immunology*
  • Plasma Cells / metabolism
  • Plasma Cells / pathology*
  • Skin Diseases / immunology*
  • Skin Diseases / metabolism
  • Skin Diseases / pathology*


  • Immunoglobulin G