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Clinical Note |

Rosai-Dorfman Disease Presenting in the Parotid Gland With Features of IgG4-Related Sclerosing Disease

Tai-Di Chen, MD; Li-yu Lee, MD
Arch Otolaryngol Head Neck Surg. 2011;137(7):705-708. doi:10.1001/archoto.2011.52.
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Rosai-Dorfman disease (RDD) is an infrequently encountered disorder characterized by histiocytes exhibiting emperipolesis and varying degrees of chronic inflammatory cell infiltrates. It is generally considered an important differential diagnosis of IgG4-related sclerosing disease because of their similar histopathologic features. We report herein a case of RDD in the parotid gland with an increase in the absolute number of IgG4+ plasma cells as well as an increase in the ratio of IgG4+ to IgG+ plasma cells. We also discuss both disease entities and this overlapping phenomenon.

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Figure 1. Histopathologic images of the lesion. A, In low-power magnification, note the variegated dark-and-light-stained appearance (hematoxylin-eosin, original magnification ×40). B, Stromal fibrosis highlighted by a trichrome stain (Masson trichrome stain, original magnification ×100).

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Figure 2. Histopathologic images of the lesion. A, Engulfment of intact lymphocytes and plasma cells by the large histiocytes, a phenomenon known as emperipolesis (arrows) (hematoxylin-eosin, original magnification ×400). B, Large histiocytes are stained by S-100 protein; note the unstained lymphocytes and plasma cells (arrows) (S-100 protein immunohistochemical stain counterstained by hematoxylin-eosin, original magnification ×400).

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Figure 3. Histopathologic images of the lesion. A, Immunohistochemical staining for IgG labels most of the plasma cells in this high-power field (IgG immunohistochemical stain counterstained by hematoxylin-eosin, original magnification ×400). B, A large proportion of plasma cells also stained with IgG4 (IgG4 immunohistochemical stain counterstained by hematoxylin-eosin, original magnification ×400).

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