Case of Generalized Tumor-Type Rosai–Dorfman Disease With Sarcoidosis-Like Histological Features and IgG4-Positive Plasma Cells

ABSTRACT Rosai-Dorfman disease (RDD, also known as sinus histiocytosis with massive lymphadenopathy) is a rare and benign non-Langerhans cell histiocytosis. Skin biopsy usually shows nodular or diffuse dermatitis. Rosai-Dorfman cells (RDD cells) and emperipolesis are the key to diagnosis. RDD cells express S-100 antigen, CD68, CD163, α1-antitrypsin, α1-antichymotrypsin, and ham-56, whereas Langerhans cell markers such as CD1a and langerin are negative. We presented a case of a 55-year-old man with varying sizes of many dark red nodules and lumps over the face, trunk, and limbs for approximately 1 year but without systemic involvement. The results of the laboratory evaluations were notable for an increased level of serum IL-6 and serum IgG4. Histopathological examination showed a diffused dense nodular infiltration of "nude" epithelioid histiocytes with infiltration of minimal lymphocytes and plasm cells around the epithelioid nodules. Immunohistochemistry identified nodular histiocytes being stained strongly positive for S-100 and CD68 but negative for CD1a. Plasma cells showed focally positive for IgG, IgG4, and CD38 and with a ratio of IgG4/IgG >40%. Considering these findings, we believe that our case meets the diagnostic description of "cutaneous Rosai-Dorfman disease" and is, therefore, a rare case with clinical features of multiple tumor-like nodules, sarcoidosis-like histological features, and immunohistochemistry of IgG4-positive plasma cells.