Orbital Rosai–Dorfman disease: a case report and literature review

X, Su; Zhang, Liqing

doi:10.1177/0300060519878086

Cited by 6 publications

(1 citation statement)

References 15 publications

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“…More than 40% of RDD have at least one extranodal organ involvement, of which the skin, soft tissue, and the central nervous system are the most common, and digestive tract is less involved. 13,14 CRDD was mainly seen in women aged around 45 years, and in Asians, the mobility of CRDD is even higher than typical RDD. 15,16 The lesions in CRDD patients are usually seen on the face and neck, followed by the trunk, with various manifestations ranging from ,1 cm or .30 cm.…”

Section: Discusscionmentioning

confidence: 92%

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

Tan

Zhou²,

Zhan³

et al. 2020

The American Journal of Dermatopathology

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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, a1-antitrypsin, a1-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.

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Section: Discusscionmentioning

confidence: 92%